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PART, , VI Congenital Heart Disease, in the Adult, 29, , Congenital Heart Disease in the Adult, , Definition 1063, Section I: Overview    1063, Prevalence, 1063, Survival versus Cure 1063, Categories of Adult Congenital Heart, Disease, 1064, , Results 1078, Indications for Operation 1079, Section III: Patent Foramen Ovale    1079, Definition 1079, Morphology 1079, Clinical Features and Diagnostic Criteria, , PRIMARY CONGENITAL HEART DISEASE    1064, , Newly Diagnosed Anomalies    1064, Previously Diagnosed Anomalies with Benign, Pathophysiology    1064, Previously Diagnosed Anomalies Thought to Be, Inoperable    1064, , SECONDARY CONGENITAL HEART DISEASE    1064, , Management and Organization of Health, Care, 1064, Special Circumstances 1066, Overview    1066, Maternal Cardiac Surgery during, Pregnancy    1067, , PULMONARY ARTERIAL HYPERTENSION AND, EISENMENGER PHYSIOLOGY    1067, HEART FAILURE AND TRANSPLANTATION    1069, , Heart Failure    1069, Transplantation    1069, , ENDOCARDITIS    1070, ARRHYTHMIAS    1071, OTHER ORGAN SYSTEMS    1073, SYNDROMES ASSOCIATED WITH CONGENITAL HEART, DISEASE    1073, REPEAT STERNOTOMY    1074, , Overview    1074, Technical Considerations    1074, Outcomes    1074, , SPECIFIC ANOMALIES    1075, , PRESENTATION    1078, DIAGNOSIS    1078, , Technique of Operation, , 1078, , Natural History, 1080, Technique of Operation 1080, Results 1080, Indications for Operation 1080, Section IV: Ventricular Septal Defect    1080, Definition 1080, Morphology 1081, Clinical Features and Diagnostic Criteria, , 1081, , PRESENTATION    1081, DIAGNOSIS    1081, , PREGNANCY AND CONTRACEPTION    1066, , Section II: Atrial Septal Defect    1077, Definition 1077, Morphology, 1078, Clinical Features and Diagnostic Criteria, , 1079, , PRESENTATION    1079, , 1078, , Natural History, 1081, Technique of Operation 1081, Results 1082, Indications for Operation 1082, Section V: Atrioventricular Septal Defect    1082, Definition 1082, Morphology 1082, Clinical Features and Diagnostic Criteria 1082, PRESENTATION    1082, DIAGNOSIS    1083, , Natural History, 1083, Technique of Operation, Results 1083, , 1083, , DEATH    1083, LEFT ATRIOVENTRICULAR VALVE AND LEFT, VENTRICULAR OUTFLOW TRACT LESIONS    1084, , Indications for Operation 1084, Section VI: Patent Ductus Arteriosus    1084, Definition 1084, Morphology 1084, Clinical Features and Diagnostic Criteria, PRESENTATION    1085, DIAGNOSIS    1085, , Technique of Operation, Results 1086, , 1085, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1085

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1062, , PART VI, , Congenital Heart Disease in the Adult, , Indications for Operation, 1086, Section VII: Bicuspid Aortic Valve    1087, Definition 1087, Morphology, 1087, Clinical Features and Diagnostic Criteria, , 1088, , PRESENTATION    1088, DIAGNOSIS    1088, , Natural History, 1088, Technique of Operation 1088, Results, 1089, Indications for Operation, 1090, Section VIII: Subaortic Stenosis    1091, Definition 1091, Morphology, 1091, Clinical Features and Diagnostic Criteria, , Natural History    1107, Technique of Operation    1107, Results    1107, Indications for Operation    1107, DOUBLE OUTLET RIGHT VENTRICLE    1107, , Definition    1107, Morphology    1107, Natural History    1107, Technique of Operation    1108, , TRANSPOSITION OF THE GREAT ARTERIES    1109, , Definition    1109, Natural History    1109, Technique of Operation    1109, , 1091, , CONGENITALLY CORRECTED TRANSPOSITION OF THE, GREAT ARTERIES    1109, , Natural History, 1092, Technique of Operation 1092, Results, 1092, Indications for Operation, 1092, Section IX: Supravalvar Aortic Stenosis    1093, Definition 1093, Morphology, 1093, Clinical Features and Diagnostic Criteria 1093, , Section XII: Transposition of the Great Arteries    1110, Definition 1110, Morphology 1110, Clinical Features and Diagnostic Criteria 1110, , PRESENTATION    1091, DIAGNOSIS    1091, , PRESENTATION    1093, DIAGNOSIS    1093, , Natural History, 1093, Technique of Operation 1093, Results, 1094, Indications for Operation, 1095, Section X: Aortic Arch Obstructive Problems    1095, Definition 1095, Morphology, 1095, Clinical Features and Diagnostic Criteria 1095, PRESENTATION    1095, DIAGNOSIS    1095, , Natural History, 1097, Technique of Operation 1097, Results, 1097, Indications for Operation, 1100, Section XI: Tetralogy of Fallot    1101, Definition 1101, Morphology, 1101, Clinical Features and Diagnostic Criteria, , 1102, , Natural History, 1103, Technique of Operation 1103, Results, 1103, Indications for Operation, 1105, Special Situations and Controversies, , PRESENTATION    1110, DIAGNOSIS    1111, , Natural History, 1111, Technique of Operation, , 1111, , AFTER ATRIAL SWITCH REPAIR    1111, AFTER ARTERIAL SWITCH REPAIR    1115, , Results 1115, Indications for Operation, , 1115, , AFTER ATRIAL SWITCH REPAIR    1116, AFTER ARTERIAL SWITCH REPAIR    1117, , Section XIII: Congenitally Corrected Transposition of, the Great Arteries    1117, Definition 1117, Morphology 1117, Clinical Features and Diagnostic Criteria 1117, Natural History, 1117, Technique of Operation 1118, Results 1118, Indications for Operation 1118, Section XIV: Ebstein Anomaly    1119, Definition 1119, Morphology 1119, Clinical Features and Diagnostic Criteria, PRESENTATION    1119, DIAGNOSIS    1120, , 1106, , PULMONARY ATRESIA WITH VENTRICULAR SEPTAL, DEFECT    1106, , Definition    1106, Morphology    1106, Clinical Features and Diagnostic Criteria    1106, Technique of Operation    1106, , TRUNCUS ARTERIOSUS    1107, , DOUBLE OUTLET LEFT VENTRICLE    1110, , PRESENTATION    1117, DIAGNOSIS    1117, , PRESENTATION    1102, DIAGNOSIS    1103, , CATHETER-BASED INTERVENTIONS    1105, SURGICAL INTERVENTIONS    1106, , Definition    1109, Morphology    1109, Natural History    1109, Technique of Operation    1109, Results    1109, , Definition    1107, Clinical Features and Diagnostic Criteria    1107, , 1119, , Natural History, 1120, Technique of Operation 1120, Results 1121, Indications for Operation 1122, Section XV: Coronary Artery Problems Related to, Congenital Heart Disease    1123, Definition 1123, Morphology 1123, Clinical Features and Diagnostic Criteria 1123, PRESENTATION    1123, DIAGNOSIS    1123, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, , Technique of Operation 1126, Results, 1126, Indications for Operation, 1126, Section XVI: Single Ventricle    1127, Definition 1127, Morphology, 1127, PRESENTATION    1127, DIAGNOSIS    1128, , Natural History, 1128, Technique of Operation 1129, Results, 1129, Indications for Operation, 1130, Section XVII: Other Anomalies Rarely Requiring Surgery, in the Adult    1131, Isolated Pulmonary Stenosis 1131, DEFINITION    1131, MORPHOLOGY    1131, TECHNIQUE OF OPERATION    1131, RESULTS    1131, INDICATIONS FOR OPERATION    1132, , Total Anomalous Pulmonary Venous, Connection 1132, DEFINITION    1132, MORPHOLOGY    1132, CLINICAL FEATURES AND DIAGNOSTIC, CRITERIA    1132, NATURAL HISTORY    1132, TECHNIQUE OF OPERATION    1133, RESULTS    1133, , Vascular Ring and Associated Arch, Anomalies, 1133, , Congenital Heart Disease in the Adult, , CLINICAL FEATURES AND DIAGNOSTIC, CRITERIA    1133, NATURAL HISTORY    1133, TECHNIQUE OF OPERATION    1133, RESULTS    1133, INDICATIONS FOR OPERATION    1133, , Cor Triatriatum, , 1133, , DEFINITION    1133, MORPHOLOGY    1133, CLINICAL FEATURES AND DIAGNOSTIC, CRITERIA    1133, TECHNIQUE OF OPERATION    1134, RESULTS    1134, INDICATIONS FOR OPERATION    1134, , Sinus of Valsalva Aneurysm, , 1134, , DEFINITION    1134, MORPHOLOGY    1134, CLINICAL FEATURES AND DIAGNOSTIC, CRITERIA    1134, NATURAL HISTORY    1134, TECHNIQUE OF OPERATION    1134, INDICATIONS FOR OPERATION    1134, , Double-Chambered Right Ventricle, , 1134, , DEFINITION    1134, MORPHOLOGY    1134, CLINICAL FEATURES AND DIAGNOSTIC, CRITERIA    1134, TECHNIQUE OF OPERATION    1134, RESULTS    1135, INDICATIONS FOR OPERATION    1135, , DEFINITION    1133, MORPHOLOGY    1133, , DEFINITION, Congenital heart disease in the adult is presence of unrepaired, or repaired congenital heart disease in patients aged 21 years, or older. A more practical working definition is not straightforward. Because physical and emotional maturity is variable,, the distinction between an adult and non-adult is unclear., The designation “adult” implies provision of specific methods, of caregiving best delivered in an adult care environment. The, patient age at which this approach is advisable varies, ranging, from mid-teens to mid-20s, depending on the individual., It has been recommended that the process of transitioning, young patients successfully to an adult healthcare environment should begin by age 12 years.F15 Several models of care, fit the definition of “adult healthcare environment,” including adult congenital heart disease programs based in pediatric, hospitals and clinics, those based in adult hospitals and clinics,, and hybrid arrangements. None has proven superior., , Section I, , Overview, , PREVALENCE, Survival of patients with congenital heart disease (CHD) has, steadily improved over the past 4 decades since reparative, , surgery has become commonplace. Since the 1970s, more, than 80% of patients have survived into adult life. The 32nd, Bethesda Conference report (Bethesda Report) in 2000 contains an estimate that approximately 800,000 adults in the, United States have CHD. With current surgical mortality less, than 10%, it is expected that in the next decade almost 1 in, 150 young adults will have some form of CHD.M9,W12, The level of development of health care in a particular, environment will strongly influence the prevalence and profile, of adult congenital heart patients. In countries with underdeveloped healthcare systems, fewer congenital heart disease, patients will survive to adulthood, and a preponderance of, these will have unrepaired anomalies. Consequently, many of, these patients will have advanced sequelae consistent with the, natural history of the particular anomaly. In this chapter, we, emphasize adult congenital heart disease as it presents in, environments with state-of-the-art pediatric congenital heart, disease management., , SURVIVAL VERSUS CURE, Survival does not necessarily, and usually does not, mean, cure.D8,W11 Cure is best defined as a state that results when, survival and quality of life are indistinguishable from normal., Fig. 29-1 illustrates this concept, listing a selection of congenital cardiac anomalies and where each stands on the spectrum of survival and cure following intervention. Clearly,, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1063

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PART VI, , Congenital Heart Disease in the Adult, Mortality by Age, , Long-Term Survival versus Cure, Survival, equals, cure, , 12, , 8, , Figure 29-1 Cure following repair of congenital heart disease. A, selection of anomalies is shown, with each positioned along a spectrum conceptually measuring how close survival following surgical, repair comes to cure. Key: ASD, Atrial septal defect; AVSD, atrioventricular septal defect; COARCT, aortic coarctation; CONDUIT, right, ventricular-to–pulmonary artery conduit; PDA, patent ductus arteriosus; SINGLE V, single-ventricle physiology; TF, tetralogy of Fallot;, TGA, transposition of the great arteries; VSD, ventricular septal, defect., , many patients surviving surgery or intervention for CHD are, not cured, and these patients’ residual or recurrent lesions, frequently will require repeat surgery or intervention later in, life. Even patients who are hemodynamically cured with no, residual lesions may have reduced quality of life compared, with the population without CHD.L5, , CATEGORIES OF ADULT CONGENITAL, HEART DISEASE, Primary Congenital Heart Disease, Primary CHD in the adult refers to previously untreated, anomalies. These anomalies tend to cause relatively benign, pathophysiologic perturbations, allowing survival into adulthood without treatment. Primary CHD is less common than, secondary CHD., Newly Diagnosed Anomalies, Newly diagnosed anomalies fall into two categories. The first, consists of patients with anomalies that not only allow survival, to adulthood, but are sufficiently benign to escape detection, even in environments with well-organized healthcare systems., Typical anomalies include those causing left-to-right shunt, (atrial septal defect [ASD], partial atrioventricular septal, defect [AVSD], restrictive ventricular septal defect [VSD],, and restrictive patent ductus arteriosus [PDA]) and those, causing minor valvar obstruction or regurgitation (bicuspid, aortic valve). The second consists of patients with anomalies, that allow survival to adulthood, but are sufficiently malignant to cause serious pathophysiologic changes; typically,, these patients spend their childhood in environments without, the capability of diagnosing or treating the anomaly, and only, as adults enter an environment capable of detecting it. Typical, anomalies include all of those listed in the first category, but, they are attended by more serious pathophysiologic perturbations (larger, less restrictive VSDs), as well as selected cases, of many forms of cyanotic congenital heart disease (tetralogy, of Fallot, pulmonary stenosis, and even some forms of single, ventricle)., Previously Diagnosed Anomalies with, Benign Pathophysiology, Typical anomalies include those resulting in small or restrictive left-to-right shunts and minor valvar lesions. They are, detected in infancy or childhood, but because of lack of, , %, , V, ND, UI, SIN T, GL, E, , TF, , CO, , AV, SD, AR, CT, CO, , TG, A, , VS, D, , AS, D, , 10, , PD, A, , 1064, , 6, 4, 2, 0, Neonate, , Infant, , Child, , Over 18 y, , Age, , Figure 29-2 Mortality after surgical repair of congenital heart, disease by age ranges., , important symptoms and pathophysiologic changes, are left, untreated. These anomalies may progress in adulthood,, causing symptoms (bicuspid aortic valve) or complications, that result in symptoms (restrictive VSD with endocarditis)., Previously Diagnosed Anomalies Thought, to Be Inoperable, Occasionally, adult patients are encountered who were diagnosed with complex congenital heart disease in infancy;, however, because their pathophysiology was not life threatening and their structural heart disease was so complex as to be, thought inoperable, they have been managed without surgical correction into adulthood. New surgical approaches may, by then have become available. An example is the occasional, patient with pulmonary atresia, VSD, or aortopulmonary collaterals, with mild cyanosis. This patient may be a candidate, for unifocalization and intracardiac repair as an adult., Secondary Congenital Heart Disease, Secondary congenital heart disease refers to patients with previously treated CHD, which is more common in the adult than, primary CHD. It covers the entire spectrum of congenital, anomalies. As illustrated in Fig. 29-1, some but by no means, all patients who have undergone surgery for CHD as infants, and children are cured and are not considered to have secondary CHD as adults., , MANAGEMENT AND ORGANIZATION, OF HEALTH CARE, Currently, delivery of appropriate health care to adults with, CHD is not fully met, even in the developed world.M35,S40, This is partly due to inadequately trained healthcare providers, managing these patients after they transition from pediatric, care, partly to poor organization, and partly to loss of health, insurance when these patients become adults—up to 20% of, adults with CHD may be uninsured.W13 Lapse of care for, adults with CHD is associated with adverse outcome.Y6, According to the Society of Thoracic Surgeons (STS) database, early mortality following cardiac surgery for CHD is, higher in adults than in children, although neonates and, infants have the highest mortality (Fig. 29-2). This higher, mortality in adults may be caused partially by lack of, healthcare organization and experience., , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, Table 29-1 Personnel and Services Recommended for Regional, Adult Congenital Heart Disease Centers, Type of Service, , Personnel/Resources, , Cardiologist specializing in ACHD, , One or several 24/7, , Congenital cardiac surgeon, , Two or several 24/7, , Nurse/physician assistant/nurse, practitioner, , One or several, , Cardiac anesthesiologist, , Several 24/7, , Echocardiographya (includes TEE,, intraoperative TEE), , Two or several 24/7, , Diagnostic catheterizationa, , Yes, 24/7, , Noncoronary interventional, catheterization, , Yes, 24/7, , Electrophysiology/pacing/AICD, implantationa, , One or several, , Exercise testing, , Echocardiography, , Congenital Heart Disease in the Adult, , Box 29-1 Diagnoses in Adult Patients with Congenital Heart, Disease of Low Complexitya, , Native Disease, , Isolated congenital aortic valve disease, Isolated congenital mitral valve disease (e.g., except, parachute valve, cleft leaflet), Small atrial septal defect, Isolated small ventricular septal defect (no associated lesions), Mild pulmonary stenosis, Small patent ductus arteriosus, , Repaired Conditions, , Previously ligated or occluded ductus arteriosus, Repaired secundum or sinus venosus atrial septal defect, without residua, Repaired ventricular septal defect without residua, Modified from Warnes and colleagues.W13, These patients can usually be cared for in the general medical community., , a, , Radionuclide, Cardiopulmonary, Metabolic, Cardiac imaging/radiologya, , Cardiac MRI, CT scanning, Nuclear medicine, , Multidisciplinary teams, , High-risk obstetrics, Pulmonary hypertension, Heart failure/transplant, Genetics, Neurology, Nephrology, Cardiac pathology, Rehabilitation services, Social services, Vocational services, Financial counselors, , Information technology, , Data collection, Database support, Quality assessment review/, protocols, , Modified from Warnes and colleagues.W13, a, These modalities must be supervised/performed and interpreted by, physicians with expertise and training in congenital heart disease., Key: 24/7, Available 24 hours a day, 7 days a week; ACHD, adult congenital, heart disease; AICD, automatic implantable cardioverter defibrillator;, CT, computed tomography; MRI, magnetic resonance imaging;, TEE, transesophageal echocardiography., , The Bethesda Report recommends organizing care of, adults with CHD within a regionalized system of specialized, adult CHD units, with each unit providing education, care,, and research for its designated region.W12 Table 29-1 summarizes the resources required for such a unit.W13 The, Bethesda Report describes three levels of training for adult, cardiovascular specialists managing adults with CHD.W16, These training levels emphasize cardiology training, but do, not focus on specifics of training for surgeons who care for, these patients. It is recommended that cardiothoracic surgeons caring for adults with CHD have formal fellowship, , Box 29-2 Diagnoses in Adult Patients with Congenital Heart, Disease of Moderate Complexitya, Aorto–left ventricular fistulas, Anomalous pulmonary venous drainage, partial or total, Atrioventricular septal defects (partial or complex), Coarctation of the aorta, Ebstein anomaly, Infundibular right ventricular outflow obstruction of, significance, Ostium primum atrial septal defect, Patent ductus arteriosus (not closed), Pulmonary valve regurgitation (moderate to severe), Pulmonary valve stenosis (moderate to severe), Sinus of Valsalva fistula/aneurysm, Sinus venosus atrial septal defect, Subvalvular AS of SupraAS (except HOCM), Tetralogy of Fallot, Ventricular septal defect with:, Absent valve or valves, Aortic regurgitation, Coarctation of the aorta, Mitral disease, Right ventricular outflow tract obstruction, Straddling tricuspid/mitral valve, Subaortic stenosis, Modified from Warnes and colleagues.W13, a, These patients should be seen periodically at regional adult congenital heart, disease centers., Key: AS, Aortic stenosis; HOCM, hypertrophic obstructive cardiomyopathy;, SupraAS, supravalvular aortic stenosis., , training in pediatric heart surgery. There is evidence obtained, from an analysis of national practice patterns involving more, than 40,000 patients that mortality following CHD surgery, in adults is lower if the surgeon performing the operation is, an experienced pediatric heart surgeon.K5 The cardiothoracic, surgeon managing adults with CHD should be fully integrated into the adult CHD unit and may take a leadership, role in the functioning of the unit., The combined American College of Cardiology (ACC)/, American Heart Association (AHA) Guidelines Committee, for the Management of Adults with CHD recognizes, three levels of complexity of congenital heart disease in adults, (Boxes 29-1 to 29-3) and makes specific recommendations, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1065

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1066, , PART VI, , Congenital Heart Disease in the Adult, , Box 29-3 Diagnoses in Adult Patients with Congenital Heart, Disease of Great Complexitya, Conduits, valved or nonvalved, Cyanotic congenital heart (all forms), Double-outlet ventricle, Eisenmenger syndrome, Fontan procedure, Mitral atresia, Single ventricle (also called double inlet or outlet, common,, or primitive), Pulmonary atresia (all forms), Pulmonary vascular obstructive disease, Transposition of the great arteries, Tricuspid atresia, Truncus arteriosus/hemitruncus, Other abnormalities of atrioventricular or ventriculoarterial, connection not included above (e.g., crisscross heart,, isomerism, heterotaxy syndromes, ventricular inversion), Modified from Warnes and colleagues.W13, a, These patients should be seen regularly at adult congenital heart disease, centers., , for the management protocols based on these levels (Box, 29-4).W13, , SPECIAL CIRCUMSTANCES, Pregnancy and Contraception, Overview, The most common cardiac cause of morbidity and mortality, in pregnant women in North America is congenital malfor­, mations.S37 Ideally, women with CHD should receive counseling by an adult CHD expert before becoming pregnant., Both fetal and maternal risks should be discussed. If pregnancy occurs, fetal echocardiography should be obtained and, the consequences of pregnancy discussed.S37, If functional class and systemic ventricular function are, good, the outcome of pregnancy is favorable in most women, with CHD. Even in women with well-compensated cardiac, status, however, specific risks are present. In those with intracardiac shunts, air entry into intravenous lines may cause, paradoxical embolism. Any degree of immobilization of the, pregnant woman should be attended by prophylaxis for, deep vein thrombosis, particularly if there is the potential for, right-to-left intracardiac shunting.W13, Pulmonary hypertension, especially when above 70% systemic, presents a serious risk during pregnancy. Pulmonary, hypertensive events may occur after delivery.S36 If Eisenmenger physiology is present, maternal mortality is up to 50%, fetal loss at a similar level. Even after a successful pregnancy,, maternal mortality may increase in the first several days after, delivery. Anticoagulation during pregnancy, even to a level, required for mechanical valves, is not a strict contraindication, to pregnancy; however, it poses an increased risk to both, mother and fetus.S35, In a small group of women with complex CHD or with, decompensated cardiac status, pregnancy is either dangerous, or contraindicated. These women should be managed and, delivered in specialized centers with expertise in adult CHD,, obstetrics, anesthesiology, and neonatology. Vaginal delivery, is preferable for most women with CHD; cesarean section, , Box 29-4 American College of Cardiology/American Heart, Association Recommendations for Access to Care for Adults, with Congenital Heart Disease, 1. An individual primary caregiver or cardiologist without, specific training and expertise in ACHD should manage, the care of adults with complex and moderate CHD only, in collaboration with level 2 or level 3 ACHD specialists., 2. For ACHD patients in the lowest-risk group, cardiac, follow-up at a regional ACHD center is recommended at, least once to formulate future needs for follow-up., 3. Frequent follow-up (generally every 12 to 24 months), at a regional ACHD center is recommended for the larger, group of adults with complex and moderate CHD. A, smaller group of adults with very complex CHD will, require follow-up at a regional ACHD center at a minimum, of every 6 to 12 months., 4. Stabilized adult patients with CHD who require admission, for urgent or acute care should be transferred to a, regional ACHD center, except in some circumstances after, consultation with the patient’s primary level 2 or level 3, ACHD specialist.W13, 5. Diagnostic and interventional procedures, including, imaging (i.e., echocardiography, MRI, CT), advanced, cardiac catheterization, and electrophysiology procedures, for adults with complex and moderate CHD should be, performed in a regional ACHD center with appropriate, experience in CHD and in a laboratory with appropriate, personnel and equipment. Personnel performing such, procedures should work as part of a team with expertise, in the surgical and transcatheter management of patients, with CHD., 6. Surgical procedures that require general anesthesia or, conscious sedation in adults with moderate or complex, CHD should be performed in a regional ACHD center with, an anesthesiologist familiar with ACHD patients., 7. ACHD patients should be transferred to an ACHD center, for urgent or acute care of cardiac problems., 8. Adult patients with complex or high-risk CHD should be, transferred to an ACHD center for urgent or acute, noncardiac problems., 9. An ACHD specialist should be notified or consulted when, a patient with simple or low-risk CHD is admitted to a, non-ACHD center., Modified from Warnes and colleagues.W13, Key: ACHD, Adults with congenital heart disease; CHD, congenital heart, disease; CT, computed tomography; MRI, magnetic resonance imaging., , and delivery is recommended for obstetric reasons and for, women fully anticoagulated with warfarin at the time of delivery, because of the risk of fetal intracranial hemorrhage., Although pregnancy is not contraindicated in women with, repaired congenital anomalies, increased complications may, occur. An excess of miscarriages, preterm delivery, and maternal hypertension is found after successful coarctation repair, and repair of congenital aortic stenosis.B10,V18,Y2, Certain medications are contraindicated during pre­, gnancy.W13 Angiotensin-converting enzyme (ACE) inhibitors, and angiotensin receptor blockers (ARB) cause congenital, and renal disorders in the fetus.S13 Warfarin should be used, only after full discussion with the patient about its risks, during pregnancy.S9 Endocarditis is a recognized risk for, maternal morbidity; however, endocarditis prophylaxis at the, time of delivery is not universally recommended. Some, believe that risk of bacteremia is low; others routinely, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, administer antibiotics.P20,W13 Intravenous amoxicillin and gentamicin should be considered for women with high-risk, anatomy or previous history of endocarditis., Estrogen-containing oral contraceptives are generally, contraindicated in women at risk of thromboembolism., Those containing progesterone are contraindicated in women, with heart failure because of their tendency to cause fluid, retention. The risk of endocarditis with intrauterine devices, is controversial, and recommendations should be individualized on the basis of discussions between the adult CHD, specialist and gynecologist.W13, Maternal Cardiac Surgery during Pregnancy, Cardiac surgery during pregnancy is rarely necessary. About, 1% to 4% of pregnancies are complicated by cardiac disease.B6, Occasionally, owing to progression of cardiac disease during, pregnancy or to cardiovascular changes induced by pregnancy, cardiac surgical intervention is indicated. Although, about 20% of adverse cardiac events during pregnancy will, require surgery or an invasive interventional procedure, the, majority of these can be managed medically. Maximal interdisciplinary efforts and proper assessment of maternal and, fetal risks are mandatory in managing these patients., The maternal-fetal conflict of interest, nonelective presentation for surgery, and vulnerability during the postpartum, period contribute to a higher risk of cardiovascular operations, during pregnancy and postpartum than in the nonpregnant, population.W19, Mortality risk of cardiac surgery is high, 2% to 9% for the, mother and 20% to 30% for the fetus. Thus, the risk of maternal death during pregnancy increases 500- to 3000-fold if, cardiac surgery is required.W19 On the other hand, the 2% to, 9% maternal mortality risk is probably onefold to twofold, higher than the risk of the same cardiac operation in a nonpregnant woman of the same age. Several recent reports, suggest that maternal mortality is not increased relative to the, risk in nonpregnant women.P4,P7 Maternal risk will vary, depending on the cardiac lesion. From a literature review of, 161 cardiac operations during pregnancy, the greatest maternal risk was found to be associated with cardiac operations, for pulmonary embolism (22%) and aortic dissection (22%),, followed by operations for either native (9%) or prosthetic, valve disease (9%).W19 The underlying etiology of the embolism, dissection, and valve disease was not given; however,, from the age range of the pregnant women, it is reasonable, to assume, at least for the native and prosthetic valve categories, that congenital anomalies represent the underlying cause, for a substantial number of the cases. In this same review, a, separate category of “congenital anomalies” accounted for 11, (7%) of the 161 cases. Of these 11, 6 required cardiopulmonary bypass (CPB) to accomplish the repair. None of these, 11 patients died. The other common underlying etiology of, heart disease in pregnant women requiring cardiac surgery is, likely rheumatic disease.A24, Other risk factors for death in pregnant women undergoing cardiac surgery include moderate or severe obstruction of, the aortic or mitral valve, left ventricular ejection fraction, below 40%, higher preoperative New York Heart Association, (NYHA) functional class, and a preoperative history of stroke, from arrhythmias.A19,C14 Risk factors for fetal death are shown, in Table 29-2.A19, To minimize risk to the fetus, if surgery is being considered during the third trimester, controlled delivery before the, , Congenital Heart Disease in the Adult, , Table 29-2 Incremental Risk Factors Associated with Fetal, Death after Cardiac Surgery Requiring Cardiopulmonary Bypass, in Pregnant Women, Death, Risk Factor, , Yes, , No, , >35, , 27.3%, , 72.7%, , <35, , 70.0%, , 30.0%, , Yes, , 66.7%, , 33.3%, , No, , 26.2%, , 73.8%, , Emergency, , 70.6%, , 29.4%, , Planned, , 18.9%, , 81.1%, , P Value, , Maternal Age, .023, , Reoperation, .016, , Surgery, <.001, , Preoperative NYHA Class, IV, , 66.7%, , 33.3%, , III, , 20.0%, , 80.0%, , II, , 16.7%, , 83.3%, , Cardioplegic, , 66.7%, , 33.3%, , Anoxic, , 28.9%, , 71.1%, , .003, , Myocardial Protection, .053, , From Arnoni and colleagues.A19, Key: NYHA, New York Heart Association., , mother’s cardiac operation should be considered. If cardiac, surgery is required at an earlier stage of gestation, alterations, in managing the operation are necessary. Fetal bradycardia is, a common complication; thus, fetal heart rate monitoring,, and ideally fetal echocardiographic monitoring, should be, performed. CPB adjustments are important to maximize, uterine circulation and maintain fetal heart rate.M3,P4,P7 These, adjustments include increasing perfusion flow rates, main­, taining high perfusion pressure (60 mmHg), avoiding, hypothermia, maintaining high hematocrit, avoiding vasoconstrictive agents, and using pulsatile perfusion. The latter, can be achieved using an intraaortic balloon pump during, CPB, and this has been shown to improve uterine and fetal, perfusion.W21 Uterine contractions occur in response to CPB,, possibly as a response to the dilution of progesterone, which, stabilizes the uterus; thus, tocolytic pharmacologic therapy, may be beneficial during CPB.P4, Pulmonary Arterial Hypertension, and Eisenmenger Physiology, Irreversible pulmonary arterial hypertension (PAH) associated with CHD usually results from anomalies that allow, long-standing left-to-right shunts. All such shunts cause PAH, from birth onward; however, initially the PAH is flow related,, , is increased and pulmeaning pulmonary blood flow (Qp), monary vascular resistance (Rp) is low. Over time, PAH may, evolve from flow related to resistance related, meaning that,  decreases. Flow-related PAH is, Rp becomes elevated and Qp, reversible after eliminating the shunt with surgery or other, intervention. Resistance-related PAH is generally irreversible., , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1067

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1068, , PART VI, , Congenital Heart Disease in the Adult, , The shunt’s type, size, and duration influence the likelihood, that, and rapidity with which, irreversible PAH will develop., Thus, these factors will be important in determining the age, at which patients with irreversible PAH present. Type and, size of the shunt determine the magnitude of shunt flow,, which in turn determines the amount of shear stress on the, endothelial surface of resistance-level pulmonary arteries., Shear stress induces vasoactive changes and ultimately permanent obstructive structural changes in these arteries. Pulmonary vascular histology in shunt-induced irreversible PAH, resembles that described for idiopathic PAH, with medial, thickening and plexiform lesions in severe cases.T24, Individuals with atrial-level left-to-right shunts are least, likely to develop irreversible PAH, those with ventricularlevel shunts are more vulnerable, and those with arterial-level, shunts are at greatest risk. Whether the variation in risk, among these different levels is solely related to shunt flow or, to an underlying genetic predisposition is unknown. A, number of specific congenital heart anomalies can lead to, irreversible PAH. Unrepaired large ASD, VSD, AVSD, and, PDA account for most cases, simply because these defects are, common.W13 However, many less common complex lesions,, such as partial or total anomalous pulmonary venous return,, unrepaired or palliated conoventricular defects, including, truncus arteriosus or transposition of the great arteries (TGA),, and single-ventricle variants, can also result in development, of irreversible PAH. Other congenital causes of PAH unrelated to shunting include pulmonary vein stenosis and pulmonary veno-occlusive disease.W13, Over time, as severe vascular obstructive changes develop,, Rp approaches and exceeds systemic vascular resistance (Rs),, causing a bidirectional or predominantly right-to-left shunt, accompanied by oxygen-unresponsive hypoxemia, identified, as Eisenmenger physiology.W27 In patients with large ventricular- and arterial-level left-to-right shunts or unrepaired, complex congenital heart defects, irreversible PAH can, develop as early as the first year of life and Eisenmenger, physiology within the first decade of life (see “Pulmonary, Vascular Disease” under Natural History in Section I of, Chapter 35); however, in patients with medium or larger, ASDs, Eisenmenger physiology may not appear at all, but, when it does, it typically appears in the second, third, or, fourth decade. Pregnancy may unmask pending Eisenmenger, physiology., PAH and Eisenmenger physiology may develop late after, surgical repair of left-to-right shunts. The most common, explanation is that the repair was performed too late or was, incomplete. However, additional factors such as left ven­, tricular hypertrophy and diastolic dysfunction, valve abnormalities, pulmonary venous hypertension or obstruction,, restrictive or hypoventilatory lung disease, chronic liver, disease, and toxin use must be considered and, if present,, addressed to the degree possible.W13, Dyspnea on exertion is the most common presenting, symptom of patients with severe PAH and Eisenmenger, physiology, followed by palpitations, peripheral edema,, volume retention, hemoptysis, syncope, and progressive, cyanosis.W27 Morbidity is progressive and becomes substantial, typically by the third decade of life. Hypoxemia-related, secondary erythrocytosis leads to increased blood viscosity, and intravascular sludging. Organ damage may result in the, brain from cerebrovascular changes brought about by sludging, with resultant stroke, and in the kidneys, with altered, , renal function. Right heart pressure and volume overload, cause elevated systemic venous pressure leading to hepatic, dysfunction. Hyperuricemia may result in gout. Hemoptysis, is potentially life threatening. Chest pain due to right ventricular ischemia, coronary artery compression by a dilated, pulmonary artery, or arteriosclerosis may occur with exertion, or at rest. Ultimately, right heart failure is inevitable. Poor, functional status is an important predictor of mortality, as are, serologic evidence of low systemic organ perfusion, worsening hypoxemia, and left ventricular systemic dysfunction.C2, Premature death is the rule. The immediate modes of death, include right ventricular failure, severe hemoptysis from, bronchial artery rupture or pulmonary infarction, com­, plications during pregnancy, and cerebral vascular events,, including occlusive strokes, systemic paradoxical embolization, and brain abscesses.S2,V17 Death during noncardiac, surgery also occurs., Changes that occur with left-to-right shunt-related PAH, can be reversible after eliminating the shunt, provided that, the surgery is performed during the vasoactive stage of PAH, development, before irreversible obstructive pulmonary vascular changes occur. Catheterization-based calculations of, , individualized measurements of resistance in isolated, Qp,, lung segments, and direct measurement of pulmonary venous, pressure are typically used to assess PAH reversibility and, likelihood of surgical success. One hundred percent inspired, oxygen, inhaled nitric oxide, and intravenously administered, prostacyclin are frequently used in such investigations to, determine the degree of pulmonary vascular reactivity and, the potential to subsequently lower pulmonary artery pressure with surgical correction of the shunt. Increasingly, acute, and chronic pharmacologic pulmonary vasodilatory and vascular remodeling therapy accompanies surgery in these cases., Specific data are not available that firmly establish the pressures, flows, and resistances that determine if operation to, remove the shunt is indicated. Typically, Rp less than 10 to, ,  less than or equal to 2/3 are, 14 Wood units and a Qp/Qs, associated with better surgical outcomes.C32,S48 Even less clear, is the predictive value of degree of vasodilatation achieved in, the catheterization laboratory in response to vasodilatory, agents. An additional confounding factor is that calculated,  based on flow; Rp calculated under, Rp itself can vary with Qp,  may actually be lower than, shunt conditions with high Qp, that calculated after the high-flow condition is eliminated, by surgical repair. Pulmonary vessels that were recruited,  may be lost after flow is reduced to, because of high Qp, normal following surgery, resulting in higher postoperative, Rp and pulmonary pressure than was anticipated using the, catheterization data., If evaluation determines that surgical closure of the shunt, is indicated, a multidisciplinary team approach is mandatory,, including an anesthesia team and intensive care team experienced in managing both PAH and the adult with CHD. The, surgical procedure itself will often be technically simple;, however, pre- and postoperative management will not. The, optimal type and mode of anesthetic administration should, be individualized (see Chapter 4). Risk of right-to-left embolization warrants avoiding bubbles following intravenous, catheter placement. Use of inhaled nitric oxide both pre- and, postoperatively should be considered., Diagnosis of Eisenmenger physiology requires a detailed, history, documenting all previous cardiac surgical and interventional procedures and medical treatments. Thorough, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, documentation of the current cardiac morphology and cardiopulmonary physiology is mandatory using chest radiography,, electrocardiography, echocardiography, cardiac catheterization, computed tomography (CT), pulmonary function, studies, and assessment of all end-organ function. Once the, diagnosis is made, the option of surgical treatment by repair, of the anomaly causing the shunt is no longer an option, because this approach will result in physiologic decompensation from severe PAH, right heart failure, and mortality., Proven treatment options are strictly medical, with the exception of lung or heart-lung transplantation (see Chapter 21)., Transplantation offers a limited survival benefit, given the, unpredictability of transplant-free survival and significantly, higher perioperative mortality in this cohort of patients,, although individual outcomes may warrant individual, considerations.T22 Medical treatment options are complex and, must be tailored to the individual patient, as discussed in detail, in the ACC/AHA 2008 guidelines.W13 The evolving concept, of treat and repair, meaning initially using advanced pharmacologic regimens to treat PAH followed by surgical repair of, the structural anomaly, has the potential to change some, patients from “inoperable” to “operable,” although longterm benefit is currently unknown.D18, Heart Failure and Transplantation, Heart Failure, Myocardial dysfunction resulting in depressed cardiac, function (heart failure) is present more frequently in adults, being considered for surgery to correct a structural heart, anomaly than in neonates, infants, and children. Distinguishing between heart failure and existing structural heart disease, as the cause of cardiopulmonary decompensation is critical to, successful decision making and managing of adults with, CHD. Recognizing heart failure may be difficult because, the associated congenital cardiac condition may mimic symptoms of heart failure. For example, dyspnea on exertion, may be due to cyanosis and not heart failure.S25 As a result,, NYHA functional status may be inadequate in this patient, population.W13, The blurring between heart failure and structural heart, disease is further complicated because the underlying disease, itself may lead to heart failure in the adult, and thus both may, be present in the same patient. The ACC/AHA guidelines, identify the most common underlying cardiac structural, substrates leading to late heart failureW13:, ■, , ■, , ■, ■, ■, ■, ■, , ■, , ■, ■, , Severe aortic stenosis or regurgitation secondary to, bicuspid aortic valve and variants, Subvalvar or supravalvar left ventricular outflow tract, pathology, Coarctation of the aorta, Severe congenital mitral stenosis or regurgitation, Unoperated ASD or partial AVSD, Congenitally corrected TGA, TGA after Mustard or Senning atrial switch procedures, in which the morphologic right ventricle is the systemic, ventricle, Tetralogy of Fallot with early-era surgery, long-standing, shunt, or severe pulmonary regurgitation or stenosis, after repair, Single-ventricle mixed circulation and chronic cyanosis, Single ventricle after a Fontan procedure, , Congenital Heart Disease in the Adult, , Heart failure can be accelerated further in this patient, population by unrelated conditions and chronic degenerative, processes common in all adultsW13:, ■, ■, ■, ■, ■, ■, ■, ■, ■, ■, ■, ■, , Acquired valvar heart disease, Coronary artery disease, Systemic hypertension, Diabetes mellitus, Pregnancy, Endocarditis, Chronic pulmonary disease, Cardiotoxic chemotherapy and mediastinal irradiation, Illicit drug use, Acquired renal or liver disease, Obstructive sleep apnea, Hyperthyroidism or hypothyroidism., , Arrhythmia and heart block therapy may play a role in, heart failure. Rhythm disturbances are common sequelae of, cardiac surgery for CHD, and progressively deteriorating, hemodynamics and rhythm disturbances often coincide, without a clear etiology. Nevertheless, the functional effect, of combined heart failure and rhythm disturbance in the adult, with CHD is additive. A surgically placed epicardial or transvenously placed endocardial right heart sequential atrioventricular pacing system may benefit the patient with heart, block or sinus node disease and related bradycardia.P10, Patients with heart failure induced by abnormal activation, sequences from right ventricular pacing, and selected patients, with structurally abnormal hearts, heart failure, and normal, sinus rhythm, may benefit from resynchronization therapy.J1,T4, Resynchronization therapy is not of proven benefit in singleventricle patients with heart failure., Pharmacologic therapy is first-line therapy for many, arrhythmias; however, transvenous or surgical ablation techniques may benefit selected patients with both atrial and, ventricular tachyarrhythmias. The maze procedure may, have therapeutic value in patients with atrial fibrillation or, flutter, particularly when combined with reconstructive surgical procedures such as Fontan revision, repair of Ebstein, anomaly, and right ventricular outflow tract surgery in tetralogy of Fallot., Other factors, such as a history of early-era surgery with, poor myocardial protection or prior surgery with prolonged, CPB and myocardial ischemia, inadequate surgical reconstructive techniques, or other surgical sequelae can also contribute to heart failure. When heart failure and structural, heart disease coexist with these chronic pressure or volume, overload and cyanotic conditions, case-by-case judgment, must be made with respect to specific management. Options, include surgical correction of the structural defect and simultaneous medical management of the heart failure, medical, management of both the heart failure and structural disease,, and heart or heart-lung transplantation., Transplantation, When irreversible heart failure is judged to be the predominant factor limiting survival, and it is due to an uncorrected, structural anomaly or residual structural heart disease after, surgery, reconstructive heart surgery should be considered., Otherwise, if reconstructive surgery is not possible, heart, transplantation should be considered. If pulmonary vascular, obstructive disease is present and limits survival, lung or, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1069

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1070, , PART VI, , Congenital Heart Disease in the Adult, , heart-lung transplantation should be considered. The decision between lung and heart-lung transplantation is made, based on the complexity of the structural heart anomaly. If a, PDA, ASD, or simple VSD is present, lung transplantation, along with reconstructive surgery for the heart may be, possible.S42 If the CHD is more complex, or if heart failure, accompanies one of these simple defects, heart-lung transplantation is most appropriate., Pre-transplantation evaluation is multidisciplinary, similar, to that for any other heart failure patient. Rp may be elevated, in any patient with heart failure; however, it is more commonly encountered when there is a history of long-standing, CHD in addition to heart failure, particularly if the CHD is, associated with left-to-right shunting. If there is no evidence, of intracardiac or arterial-level shunting, Rp-related contraindications to heart transplantation in the adult with CHD are, similar to those for any patient and include Rp greater than, or equal to 6 Wood units or a transpulmonary gradient above, 15 mmHg that is unresponsive to pulmonary vasodilator, therapy in the catheterization laboratory.A3,K14 Transpulmonary gradient alone is less helpful in the presence of either,  which is commonly encountered, increased or decreased Qp,, in many forms of uncorrected CHD. In addition, magnetic, resonance imaging (MRI) or CT of the chest may be helpful, in defining extracardiac morphology, such as systemic venous, anomalies, arch anomalies, and the relationship of the aorta,, pulmonary trunk, conduit (if present), or ventricular mass to, the posterior table of the sternum., Noncardiac contraindications to transplantation in CHD, are similar to those for transplantation in acquired cardiac, disease:, , Chapter 15).C24,D7,D19,H23,M48 More than 10% of patients, with endocarditis have a history of CHD, and endocarditis is, the cause for 4% of hospital admissions for adults with, CHD.M27,V4 Some anomalies carry a higher risk of endo­, carditis than others, including bicuspid aortic valve, unrepaired VSD, PDA, tetralogy of Fallot, TGA, single-ventricle, anomalies with systemic to pulmonary artery shunts,, and those whose repair includes a conduit or prosthetic, valve.B29,C1,C24,D19,D20,F7,G12,J9,K4,L4,L7,M22,M40,M48,S4 Surgical closure, of a VSD reduces the risk of endocarditis, and when endocarditis develops at the site of a surgically repaired defect, a, residual patch leak is frequently observed. In a series of adults, with CHD admitted for a diagnosis of endocarditis, certain, anomalies were underrepresented, including ASD, completely, closed VSD, unrepaired Ebstein anomaly, and Mustard and, Senning atrial switch repairs.M27, Definitive diagnosis of infectious endocarditis requires, positive blood cultures with appropriate organisms and physical evidence of endocardial involvement (typically identified, by echocardiography). Surface echocardiography may be, adequate, but transesophageal echocardiography may be particularly helpful, especially when complex structural anomalies are present.D25,K28 Once the diagnosis is made or suspected,, further management should occur at a center with an established adult CHD program. Consultation with a cardiac, surgeon who has a focus on adult CHD should be undertaken early in the patient’s course, because rapid deterioration, requiring surgery is common.D7 Relative indications for, surgery areB22,C28,D24:, ■, ■, , ■, ■, , ■, ■, ■, ■, ■, , Uncontrolled infection, Positive serology for human immunodeficiency virus, (HIV) or hepatitis C infection, Uncontrolled metabolic disease, Additional severe congenital anomalies, Multisystem organ failure, Uncontrolled malignancy, Psychosocial disability affecting compliance, , Additionally, previous thoracotomy, especially if multiple and, associated with chronic cyanosis, is a relative contraindication,, particularly for lung or heart-lung transplantation, because of, the likelihood of fatal bleeding from collaterals., Mortality risk is doubled in the first year after transplantation if the indication has been adult CHD.T4 Outcomes after, lung and heart-lung transplantation for PAH in adults with, CHD are comparable with those reported for children, with, actuarial survival at 10 years of 20%. There is increased, risk of early death compared with transplantation for obstructive pulmonary disease or cystic fibrosis because of perioperative complications related to increased complexity of the, operation. Outcomes for combined lung transplantation, and cardiac repair are similar to those for heart-lung, transplantation.W6, Endocarditis, As stated earlier in this chapter, most forms of CHD are not, cured by surgery or other intervention. Residual defects or, surgical and interventional remnants present in most adults, with CHD often predispose to infective endocarditis (see, , ■, , ■, , ■, ■, ■, , Development of hemodynamic decompensation, Evidence of embolic complications, Intractable infection despite appropriate antibiotic, therapy, Infection of prosthetic valves, conduits, or other, material, Abscess development, Contained rupture, Development of heart block, , The indication to operate may be clear, or it may be ambiguous. Consultation among the cardiologist, infectious disease, specialist, and surgeon should take place in all cases under, consideration for surgery., Recommendations for infectious endocarditis prophylaxis, have changed in recent years. The 2007 AHA guidelines, recommend selective use of preventive antibiotic therapy, but, also emphasize behavioral elements.W23 The latter include, maintaining daily oral hygiene and skin hygiene, particularly, with respect to acne, and avoiding nail biting.W13 Prophylactic, antibiotic therapy is confined to dental procedures (no longer, gastrointestinal or genitourinary procedures) in patients with, prior endocarditis, prosthetic heart valves, conduits, shunts,, unrepaired cyanotic CHD, CHD repaired with prosthetic, patches or other material within 6 months of surgery, residual, defects after reparative surgery for CHD if the residual defect, is in the proximity of prosthetic material, and valve lesions in, transplant patients., Niwa and colleagues reported 69 cases of endocarditis, in adults with CHD.N7 Prior cardiac surgery and a history, of cyanosis were common. Involvement of the left and, right sides of the heart was equally common. Dental procedures, cardiac surgery, and pneumonia commonly preceded, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, endocarditis. Streptococcus and Staphylococcus accounted for, 87% of cases, with Streptococcus the most common organism., Surgical intervention was needed in 26% of cases, and the, indication for surgery was large vegetations in 45% and heart, failure in 29%. Endocarditis-related mortality was 8% in, patients treated medically and 11% in those treated surgically., Di Filippo and colleagues note that adults increasingly, account for cases of endocarditis in patients with CHD,, and that complex cyanotic heart disease is also increasingly, common.D16 Streptococcus and Staphylococcus remain the most, prevalent organisms. Again, dental procedures and cardiac, surgery frequently preceded endocarditis, but these authors, note an increasing frequency of cutaneous infections in recent, years. A precipitating cause for endocarditis, however, is often, not identified. Awadallah and colleagues identified a predisposing event in 56% of cases, but Gersony and colleagues in, only 32%.A25,G12, Arrhythmias, Both atrial and ventricular arrhythmias are a more important, source of morbidity in adults with CHD than in infants, and children. Ventricular arrhythmias in particular, uncommon in young patients, are frequent in adults. Arrhythmias, can be observed in all adult clinical groups: surgically repaired, anomalies,W5 surgically palliated and single-ventricle anoma­, lies,F10 and unrepaired anomalies (Table 29-3). Cause of conduction system pathophysiology is multifactorial, including, cyanosis, volume and pressure overload, surgical incisions,, suture lines, and patches with subsequent scarring; ischemic, insults of any etiology, including coronary embolism in, patients with left-to-right shunting; and inadequate myocardial protection during previous surgery. In general, the longer, the inciting cause is present, such as cyanosis or volume, overload, the more likely arrhythmias will occur. It is this, cumulative effect that results in the higher prevalence of, arrhythmias in the adult population., Rhythm disturbances may come to the attention of the, surgeon in several ways. Most commonly, a patient being, evaluated for reconstructive surgery, such as repair of a large, ASD or replacement of a right ventricle to pulmonary trunk, conduit, will have an associated rhythm disturbance that may, influence intraoperative and postoperative care. Antiarrhythmic therapy may be required, and existence of an arrhythmogenic substrate may influence the choice or concentration of, inotropic support used., The arrhythmia may require surgical therapy concomitant, with the reconstructive surgical procedure, such as an atrial, maze procedure or placement of ventricular cryoablation, lesions (see Chapter 16). In other circumstances the sole, or, primary, indication for surgery may be the rhythm disturbance. The atrial maze procedure, an epicardial pacemaker, system for heart block or bradycardia, a biventricular pacemaker system for resynchronization therapy,T8 or placement, of an implantable cardioverter-defibrillator (ICD) are examples of surgery that may be needed., Intraatrial reentrant tachycardia (IART), or atrial flutter,, is the most common rhythm disturbance in adults with CHD., It usually develops late postoperatively, most often in patients, who have had a right atrial incision or some other right atrial, suture line. The amount of right atrial surgery tends to correlate with prevalence of IART, the greatest being in patients, who have had Mustard atrial switch intracardiac type Fontan, , Congenital Heart Disease in the Adult, , Table 29-3 Rhythm Disturbances in Adults with Congenital, Heart Disease, Rhythm Disturbance, , Associated Lesions, , Tachycardias, Wolff-Parkinson-White, syndrome, , Ebstein anomaly, Congenitally corrected transposition, , Intraatrial reentrant, tachycardia (atrial flutter), , Postoperative Mustard, Postoperative Senning, Postoperative Fontan, Tetralogy of Fallot, Other, , Atrial fibrillation, , Mitral valve disease, Aortic stenosis, Tetralogy of Fallot, Palliated single ventricle, , Ventricular tachycardia, , Tetralogy of Fallot, Aortic stenosis, Other, , Bradycardias, Sinus node dysfunction, , Postoperative Mustard, Postoperative Senning, Postoperative Fontan, Sinus venosus ASD, Heterotaxy syndrome, , Spontaneous AV block, , AV septal defects, Congenitally corrected transposition, , Surgically induced AV block, , VSD closure, Subaortic stenosis relief, AV valve replacement, , Modified from Warnes and colleagues.W13, Key: ASD, Atrial septal defect; AV, atrioventricular; VSD, ventricular septal, defect., , procedures. It can, however, occur after ASD closure. Pharmacologic therapy and catheter ablation are the first- and, second-line therapeutic choices. Pacemaker placement to, increase baseline heart rate may suppress fibrillation episodes, if sinus bradycardia coexists. If a pacemaker is indicated, a, transvenous approach is preferred; however, numerous contraindications exist, including presence of any intracardiac, shunt (even if trivial, such as a small patent foramen ovale),, previous bidirectional Glenn or extracardiac-type Fontan procedure, single-ventricle physiology of any kind, and upper, body central venous thrombosis. In these situations, surgical, pacemaker placement is indicated. Surgical therapy with a, concomitant right atrial maze procedure is indicated if, reconstructive intracardiac surgery is planned. Isolated right, atrial maze may be considered if IART is poorly controlled, by other means. The right atrial maze procedure and its, modifications have been shown to be effective in eliminating, IART in Fontan patients undergoing concomitant conversion, of an intracardiac-type Fontan to an extracardiac type (see, Chapter 41).M17, Atrial fibrillation occurs most often in adults with congenital aortic stenosis, congenital mitral valve disease, or, single ventricle.K15 Medical therapy includes anticoagulation,, pharmacologic ventricular rate control, and electrical cardioversion. There is no role for catheter ablation. Indications for, pacemaker therapy if sinus bradycardia coexists are similar to, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1071

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1072, , PART VI, , Congenital Heart Disease in the Adult, , those for IART. A concomitant combined left and right atrial, maze procedure may be beneficial and should be considered, if reconstructive surgery is planned., Wolff-Parkinson-White accessory pathway is particularly, common in Ebstein anomaly (see Chapter 42).R9 Symptoms, related to tachycardia increase in frequency and become more, problematic in adulthood. Chronic tricuspid regurgitation, induces atrial dilatation, leading to atrial flutter or fibrillation, and accelerated conduction across the accessory pathway., Catheter ablation in the electrophysiology laboratory is firstline therapy; however, it is less successful and is more likely, to recur when structural heart defects are present.C19,K12,R9, Intraoperative ablation of the accessory pathway should be, performed in patients not responding to catheter-based, therapy and in those undergoing surgery on an Ebstein, tricuspid valve. Additionally, an atrial maze procedure is, indicated if atrial fibrillation or flutter is present., Ventricular arrhythmias may develop in the adult with, CHD. Macroreentrant ventricular tachycardia can develop, late after ventricular surgery, related to ventriculotomy or, VSD patching. In repaired tetralogy of Fallot, reentry circuits, typically form through narrow conduction pathways created, by right ventricular outflow tract scarring. Prevalence of late, ventricular tachycardia or sudden death for repaired tetralogy, is 0.5% to 6.0%.N10,W5 Older age at repair, right ventricular, dilatation, and QRS duration longer than 180 ms have been, identified as risk factors for development of ventricular tachycardia and sudden death in tetralogy of Fallot patients.C12,G4,G7, Palpitations, dizziness, or syncope warrant electrophysiologic, testing in the adult with repaired tetralogy of Fallot. These, symptoms may be elicited at the time of evaluation for surgical therapy for recurrent or residual right ventricular outflow, tract disease. Electrophysiologic testing should be performed, prior to surgery., Ventricular tachycardia can develop in any form of CHD, in the adult, even if there has never been a ventricular incision, or suture line. The onset may coincide with deteriorating, ventricular function.C12, Complete hemodynamic and electrophysiologic evaluation is required before therapy for ventricular tachycardia is, undertaken. If sustained ventricular tachycardia is documented or the patient has a history of cardiac arrest, the, next step is to determine whether there is a physiologically, significant residual or recurrent structural anomaly. If there, is no structural anomaly, the treatment option of choice is, implanting an ICD.A7 A surgically placed ICD is indicated, for patients with single-ventricle physiology, obstructed, upper body systemic veins, bidirectional cavopulmonary, anastomosis, a Fontan procedure, residual intracardiac, shunts, or other unusual or distorted intracardiac morphology; otherwise, transvenous systems are available. In selected, cases, catheter-based ablation can also be performed to, reduce the like­lihood or frequency of ventricular tachycardia, episodes.B35,G17 Catheter-based ablation is unreliable as sole, therapy, with recurrence that may exceed 20%.G18,M42 Pharmacologic therapy alone is currently considered inadequate, if sustained ventricular tachycardia or a history of cardiac, arrest exists, but may be indicated if less serious ventricular, arrhythmias are present., If structural cardiac anomalies with important hemodynamic impairment are present in an adult with ventricular, tachycardia, surgical repair of the anomaly combined with, either concomitant surgical ablation or concomitant surgical, , ICD placement may be indicated. In such cases, it is necessary, to map the ventricular tachycardia, either by preoperative, electrophysiologic testing or intraoperative mapping.T10 If a, discrete focus of ventricular tachycardia is inducible and there, is no clinical history of cardiac arrest, surgical ablation may, be the best option. A typical situation appropriate for this, form of therapy is the patient with tetralogy of Fallot originally repaired with a transanular patch who presents late with, severe pulmonary regurgitation, a dilated right ventricle, and, inducible ventricular tachycardia mapped to the right ventricular outflow tract. Surgical therapy includes placing a, pulmonary valve prosthesis and creating cryoablation lesions, from the outflow patch to the pulmonary trunk and from the, outflow patch to the tricuspid anulus. Follow-up electrophysiologic evaluation is indicated in all such cases to determine if ventricular tachycardia is controlled. Placement of an, ICD is indicated if ventricular tachycardia is inducible at, follow-up. In the patient presenting with a history of cardiac, arrest whose evaluation reveals structural disease as well as, ventricular tachycardia, or the patient with structural disease, and poorly localized ventricular tachycardia, the best choice, of therapy is probably structural repair and concomitant, surgical ICD placement., Sinoatrial (SA) node dysfunction in adults with CHD, typically is acquired, occurring as a result of localized trauma, or ischemia following previous cardiac surgery. The most, common procedures that result in SA node dysfunction, are the Mustard, Senning, Glenn, and Fontan oper­, ations.F10,F13,M6,W26 Less frequently, SA node dysfunction is, congenital, associated with some forms of heterotaxy, syndrome. Patients with SA node dysfunction may be symptomatic as a result of chronotropic incompetence or of development of atrial fibrillation or flutter, which are more likely, to occur when SA node dysfunction is present. Ventricular, tachycardia can also develop as a result of prolonged sinus, pauses. Placing a pacemaker system is indicated in several, circumstances. Implantation of an atrial, or atrioventricular, sequential, pacing system with activity responsiveness is indicated for symptoms related to chronotropic incompetence,, tachy-bradycardia syndrome, recurrent atrial tachycardias,, and pause-dependent ventricular tachycardia.E4 It is also indicated for the asymptomatic adult patient with a resting heart, rate of less than 40 beats per minute or atrial pauses greater, than 3 seconds. Typically, atrioventricular conduction is, normal when SA node dysfunction is present; therefore, atrial, pacing alone is effective therapy. Nevertheless, atrioventricular sequential pacing systems are recommended in all cases,, with appropriate programming of the system such that atrial, pacing occurs along with natural atrioventricular conduction.E4, Pacemaker systems can be placed transvenously or surgically., Surgical placement is indicated in the presence of singleventricle physiology, bidirectional cavopulmonary anastomosis, Fontan surgery, distorted or thrombosed upper body, central veins, and any intracardiac shunt; otherwise, transvenous placement is preferred., Atrioventricular (AV) block in the adult with CHD may, be acquired or congenital. Acquired block is more common, and results from surgical trauma to the AV node or surrounding tissues during intracardiac repair. Block usually develops, during surgery. Typical operations that may result in block, include closure of perimembranous or inlet VSDs, resection, of left ventricular outflow tract obstruction, and surgery, to repair or replace the inlet valves. Transient heart block, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, with full recovery of AV conduction is common after these, operations, occurring in over half of all patients who suffer, block at surgery. Recovery typically occurs within 10 days.W17, Transvenous or surgical placement (see indications for each, in the preceding text) of an AV sequential pacemaker system, is indicated if postoperative second- or third-degree block, has not recovered after 10 days of observation. A relative, indication for pacemaker placement is presence of persistent, bifascicular block., The AV node and bundle of His may also be congenitally, abnormal, associated with specific anomalies such as con­, genitally corrected TGA and AVSD.A13,T13,V5 These patients, are more likely to develop block with any form of intracardiac, manipulation and to develop spontaneous block either, before or after surgery. Spontaneous development of secondor third-degree heart block is an indication for either transvenous or surgical placement of an AV sequential pacemaker, system., Other Organ Systems, Other organ systems may be abnormal in the adult with, CHD. These abnormalities may result from altered hemodynamics, chronic cyanosis, or associated syndromes (see “Syndromes Associated with Congenital Heart Disease” in text, that follows), and may contribute important morbidity and, mortality risks when cardiac surgery is performed in the adult, with CHD.D17, Altered hemodynamics can lead to pulmonary vascular, abnormalities. This subject is discussed under “Pulmonary, Arterial Hypertension and Eisenmenger Physiology” earlier, in this section. An altered hemodynamic state is well documented in patients with coarctation of the aorta, both repaired, and unrepaired. Systemic hypertension and decreased systemic vascular compliance contribute to, and may even play, a causal role in, development of and morbidity related to, intracranial aneurysms. All adults with a history of coarctation, should undergo evaluation of the cerebral vasculature to rule, out vascular aneurysms, particularly if repeat aortic surgery is, being contemplated. Long-standing abnormal right-sided, hemodynamics, particularly in patients with tetralogy of, Fallot, single-ventricle morphology with Fontan surgery,, and Ebstein anomaly, may result in chronic hepatic venous, hypertension and hepatic congestion, leading to hepatic dysfunction and cirrhosis, gastroesophageal varices, and even, hepatocellular carcinoma. Particularly in Fontan patients,, additional problems may include protein-losing enteropathy,T12, plastic bronchitis,Z1 and renal compromise.D17, Chronic cyanosis leads to erythrocytosis, iron deficiency,, and clotting disorders.P9 Blood viscosity is increased. Combined erythrocytosis and iron deficiency leading to microcytosis increases risk of thrombosis and stroke, which may be, particularly relevant perioperatively. Additionally, cyanosisrelated platelet dysfunction and deficiency of plasma and, clotting factors due to erythrocytosis combine to increase the, risk of hemorrhagic complications, again of particular relevance perioperatively. An additional complication of erythrocytosis is an increased rate of red cell turnover, leading to, abnormal bilirubin metabolism and development of gall, stones. The risk of cholecystitis and pancreatitis perioperatively is increased. Chronic cyanosis also leads to renal, glomerulosclerosis.F12 Glomerular filtration rate is decreased,, resulting in creatinine elevation., , Congenital Heart Disease in the Adult, , Renal dysfunction is found in adult patients with a widespectrum CHD.D17, Scoliosis is common in patients with chronic cyanosis. This, may lead to deformity of the thorax, causing ventilatory compromise. Pulmonary function tests are required in all adult, patients with CHD and scoliosis who are under consideration, for cardiac surgery., There is a risk of stroke after all cardiac procedures in, patients of all ages. It was found to be 0.8% in 124 adults, undergoing surgery for CHD. Interestingly, this is lower than, the risk of stroke in adults undergoing several different types, of surgery for various acquired heart problems.A15, Syndromes Associated with Congenital Heart Disease, A number of syndromes are associated with CHD, many, associated with neurologic, developmental, and cognitive, deficits (Table 29-4). Many of these syndromes include, other coexisting disease processes in other organ systems, that represent specific risks during anesthesia and surgery, (Table 29-5)., The deficits may be mild enough in many cases to allow, these individuals to live somewhat independently. When the, cardiac surgeon is asked to consult on the adult patient with, CHD, he or she must keep in mind that the patient may, have one of these syndromes. Additionally, many adults with, CHD who do not have specific syndromes may be overprotected by caring family members and may not have the, emotional or intellectual maturity expected for their age., Accordingly, these patients may have limited ability to fully, appreciate the complexities, risks, complications, and alternatives to a proposed surgical procedure. Family members, and primary care providers should be included in such, consultations., , Table 29-4 Syndromes Associated with Congenital, Heart Disease, Syndrome, , Typical Congenital Heart Disease, , Down, , AVSD, TF, , DiGeorge, , TF, IAA, TA, , Williams, , Supravalvar AS, PS, , Noonan, , PS, , Turner, , CoA, AS, , Key: AS, Aortic stenosis; AVSD, atrioventricular septal defect; CoA, aortic, coarctation; IAA, interrupted aortic arch; PS, pulmonary stenosis; TA, truncus, arteriosus; TF, tetralogy of Fallot., , Table 29-5, , Syndromes and Associated Diseases, , Syndrome, , Associated Diseases, , Down, , Hypothyroidism, obstructive airway disease, , DiGeorge, , Immune deficiency, endocrinopathies, , Williams, , Hypercalcemia, diabetes mellitus, , Noonan, , Clotting disorders, hydrocephalus, , Turner, , Hypothyroidism, osteoporosis, diabetes, mellitus, renal abnormalities, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1073

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1074, , PART VI, , Congenital Heart Disease in the Adult, , Repeat Sternotomy, Overview, Many adults undergoing surgery for CHD have had at least, one, and often several, previous cardiac operations via median, sternotomy. Risk of life-threatening hemorrhage is present, with any repeat sternotomy. Other risks include entry of air, into the circulation and arrhythmias.R22 Thus, special preparation is required when repeat sternotomy is planned. First, all, previous operative notes should be obtained and reviewed., Along with the details of previous cardiac procedures, they, may provide important information regarding whether a, prosthetic barrier was placed between the sternum and cardiac, structures, whether the native pericardium was reapproximated, and whether difficulty was encountered during the, previous sternotomy. Also, comments warning about such, things as conduit placement in proximity to the posterior, sternal border may be given. Second, CT or MRI of the chest, can be helpful in defining the position of the anterior border, of the heart, ascending aorta, pulmonary trunk, brachiocephalic vein, and conduits relative to the posterior sternal table, (Fig. 29-3)., Technical Considerations, Several options are available for patients at high risk of injury, during repeat sternotomy. An attempt to open the sternum, slowly under direct vision, beginning inferiorly at the xiphoid, and progressing superiorly, dissecting along the posterior, table of the sternum, is the best initial approach in most cases., Using this approach, a segment of posterior sternal table is, , Figure 29-3 Three-dimensional computed tomography reconstruction, lateral view, of adult with L-transposition of the great, arteries, double inlet single left ventricle, and previous median sternotomy. Note proximity of ventricular mass to posterior aspect of, sternum. Superiorly, the transposed aorta is separated from the, sternum by interposed lung tissue. Both observations are important, for planning sternal reentry., , dissected, and only then is the oscillating saw used to split, the freed portion of the sternum. This process proceeds in, steps until the sternum is completely split. If, however, at any, point the posterior sternal table dissection can no longer be, performed under direct vision, or if even minimal bleeding is, encountered, dissection is stopped. Peripheral cannulation for, CPB is then performed by several means. The femoral artery, and vein may be exposed and cannulated and CPB established. If the patient has a completely separated two-ventricle, circulation and risk of injury is to any right-sided structures,, including right ventricle to pulmonary trunk conduits, then, sternotomy can be performed as soon as CPB is established., If the patient has single-ventricle physiology, Fontan physiology, any potential for intracardiac shunting, or two-ventricle, physiology but the aorta is at risk of injury, then he or she, must be cooled to deep hypothermic temperatures prior to, further attempts to open the sternum., Femoral vascular abnormalities may be present secondary, to previous cardiac catheterization or operative procedures., Knowledge of the status of femoral vessels is critically important in all patients undergoing repeat sternotomy. Alternative methods of cannulation for CPB exist (see Chapter 2),, and these may be preferable in some patients, including, those with femoral vessel abnormalities. If sternotomy, cannot be performed safely, the sternal skin incision can, be extended superiorly, and the brachiocephalic artery superior to the brachiocephalic vein is exposed and cannulated., The inferior vena cava is also exposed and cannulated within, the pericardial space by dissecting inferior to the xiphoid, along the diaphragm surface. CPB can then be initiated,, and the operation proceeds as described for femoral, cannulation., Outcomes, There are no large studies examining repeat sternotomy specifically in adults with CHD. Three studies examine repeat, sternotomy in patients with CHD; however, the average age, was 2.1, 3.6, and 4.7 years,K16,M37,R22 although all involved, some adults. Overall, the risk of life-threatening hemorrhage, during repeat sternotomy in these three series was low—, 0.3%, 0.7%, and 5.2%—with no specific analysis of adults., Risk factors included presence of right ventricular to pulmonary trunk conduits and increasing number of previous, sternotomies.K16 In another series of 2555 adult patients with, acquired heart disease undergoing repeat sternotomy, 3%, suffered major injury at sternal opening. Mortality, if injury, occurred, was 25%.P2, There are specific congenital anomalies and situations for, which risk may be increased. Presence of PAH (regardless of, the specific cardiac morphology) may be associated with an, enlarged right ventricle and right atrium, both of which have, elevated pressure and may be in close proximity to the, sternum.P2 Mustard and Senning patients will have markedly, hypertrophied and often dilated right ventricles positioned, directly behind the sternum. Additionally, the morphologic, right atrium, which serves as the physiologic left atrium, may, be markedly dilated, and its free wall or appendage may be, positioned anteriorly behind the sternum. Injury to this structure during sternotomy, with blood loss and hypovolemia,, may result in massive air embolism to the systemic circulation, as attempts to control the hemorrhage are undertaken. All, conotruncal anomalies, including tetralogy of Fallot, truncus, arteriosus, TGA, and double outlet right ventricle, have an, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, , Congenital Heart Disease in the Adult, , Table 29-6 Original Primary Anatomic Diagnoses, Classified According to European Association for Cardiothoracic Surgery, Congenital Database, Number (%), (N = 963), , Anatomic Diagnosis, , Number (%), (N = 963), , Anatomic Diagnosis, , Septal Defects, , 332 (34.5%), , Thoracic Arteries and Veins, , 98 (10.2%), , Atrial septal defect, secundum, , 196 (20.4%), , Aortic coarctation, , 43 (4.5%), , Ventricular septal defect, , 57 (5.9%), , Aortic coarctation + aortic valve stenosis, , 34 (3.5%), , Atrioventricular septal defect, partial, , 56 (5.8%), , Aortic aneurysm, , 3 (0.3%), , Atrial septal defect, sinus venosus, , 12 (1.2%), , Anomalous origin of left coronary artery from, pulmonary artery, , 3 (0.3%), , Coronary fistula, , 3 (0.3%), , Coronary artery anomaly, origin, , 1 (0.1%), , Patent ductus arteriosus, , 6 (0.6%), , Ventricular septal defect + aortic coarctation, , 3 (0.3%), , Atrioventricular septal defect, complete, , 3 (0.3%), , Truncus arteriosus, , 5 (0.5%), , Left Heart Lesions, , 242 (25.12%), , Aortic stenosis, valvar, , 162 (16.8%), , Aortic stenosis, subvalvar, , 28 (2.9%), , Aortic insufficiency, , 18 (1.9%), , Aortic insufficiency + stenosis, , 11 (1.1%), , Aortic stenosis, subvalvar + valvar, , 7 (0.7%), , Mitral valve stenosis, , 7 (0.7%), , Sinus of Valsalva aneurysm, , 5 (0.1%), , Mitral insufficiency, , 2 (0.2%), , Cardiomyopathy, , 2 (0.2%), , Right Heart Lesions, , 194 (20.1%), , Tetralogy of Fallot, , 123 (12.8%), , Ebstein disease, , 31 (3.2%), , Pulmonary stenosis, valvar, , 18 (1.9%, , Pulmonary stenosis, valvar + subvalvar, , 8 (0.8%), , Pulmonary atresia, VSD including TOF/PA, , 8 (0.8%), , Pulmonary atresia, IVS, , 3 (0.3%), , Pulmonary stenosis, subvalvar, , 2 (0.2%), , Double-chambered right ventricle (DCRV), , 1 (0.1%), , Vascular ring, , 5 (0.5%), , Transposition of the Great Arteries (TGA), , 37 (3.8%), , TGA, IVS, including LVOTO, , 17 (1.8%), , TGA, VSD, including LVOTO, , 12 (1.4%), , Congenitally corrected TGA (ccTGA), , 4 (0.4%), , ccTGA, VSD, including LVOTO, , 4 (0.4%), , Single Ventricle, , 35 (3.6%), , Tricuspid atresia, , 18 (1.9%), , Double inlet left ventricle (DILV), , 8 (0.8%), , Mitral atresia, , 4 (0.4%), , Double outlet right ventricle (DORV), , 4 (0.4%), , DILV and DORV, , 1 (0.1%), , Electrophysiologic, , 16 (1.7%), , Arrhythmia, heart block, congenital, , 16 (1.7%), , Pulmonary Venous Anomalies, , 7 (0.7%), , Partial anomalous pulmonary venous connection, , 5 (0.5%), , Cor triatriatum, , 2 (0.2%), , Miscellaneous, , 2 (0.2%), , Aneurysm, atrial, , 2 (0.2%), , From Putman and colleagues.P23, , aorta positioned more anteriorly than usual within the superior mediastinum. Injury to the aorta during sternotomy, inevitably will be attended by massive hemorrhage and systemic air embolism. Right ventricle to pulmonary trunk, conduits, particularly those positioned in the midline as in, patients with TGA, VSD, and pulmonary stenosis or atresia,, or in any patient with dextrocardia, are also particularly, vulnerable.K16, Specific Anomalies, Definition, surgical history, morphology, and natural history,, as well as general aspects of pathophysiology, clinical presentation, diagnosis, and treatment of specific anomalies discussed in the remainder of this chapter, are described, elsewhere in this book in the specific chapters named for each, anomaly. The following sections focus on preoperative, operative, and postoperative care issues that are specifically related, to adults with these anomalies., , Several studies provide an overview of the practice of adult, congenital cardiac surgery. Putman and colleagues report a, single-institution experience with 963 adult congenital, cardiac surgical procedures in 830 patients (mean age 39, years, 50% male) between 1990 and 2007.P23 Underlying, diagnoses are shown in Table 29-6; 51% were primary operations and 49% reoperations. Underlying anomalies tended to, be more complex in the reoperation group and simpler in the, primary operation group. The most common operations were, those involving aortic valve replacement (26%); ASD closure, (18%); pulmonary valve replacement (13%); mitral valve operation (7.1%); pacemaker placement (6.4%); VSD closure, (5.5%); and coarctation repair (4.6%) (Table 29-7). Concomitant coronary artery bypass grafting was performed in, 3.4%. CPB was used in 90%. Overall early mortality was 1.5%,, and actuarial survival at 17 years was 71% (Fig. 29-4). Risk, factors for mortality are shown in Table 29-8. Functional, status, estimated by NYHA functional classification, was, improved in most survivors., , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1075

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1076, , PART VI, Table 29-7, , Congenital Heart Disease in the Adult, Actual Procedures Performed According to European Association for Cardiothoracic Surgery Congenital Databasea, , Main Procedures, , Number (%), (N = 963), , Left Heart Lesions, , 366 (38%), , Aortic valve replacement, mechanical: 10×, LVOT, 3× VSD, 4× MVP, 3× MVR, 4× CABG,, 3× ASD, , 77 (8.0%), , Aortic valve replacement, homograft: 5× LVOT,, 1× CABG, 1× PAA, , 67 (7.0%), , Ross procedure: 2× PAA, 2× aortoplasty, 1×, CABG, 2× LVOT, , 51 (5.5%), , Aortic root replacement, mechanical (Bentall):, 1× ASD, 2× LVOT, 1× MVR, 2× pulmonary, mechanical root, 3× PHG, 3× MVP, 1× CABG,, 2× prosthetic aortic arch, , 46 (4.8%), , Mitral valvuloplasty: 15× ASD, 1× AHG, 5×, CABG, 1× PDA, 1× MAZE, , 41 (4.0%), , Mitral valve replacement: 7× ASD, 6× TVP, 1×, AVSD, 2× aortic valve, 1× MAZE, 2× CABG,, 1× PDA, , 31 (3.1%), , Subvalvular aortic repair: 2× AVRM, 1× PHG,, 1× VSD, 1× RVOT, 1× shunt takedown, 1×, aortoplasty, 1× CABG, , 22 (2.3%), , Aortic valve replacement, bioprosthetic: 2×, MVP, 3× ASD, 1× CABG, , 17 (1.7%), , Main Procedures, , Number (%), (N = 963), , RVOT procedure: 2× ASD, 1× VSD, 1× LVOT,, 1× DCRV, , 6 (0.6%), , Pulmonary artery plasty, , 3 (0.3%), , Double-chambered right ventricle (DCRV), repair, , 1 (0.1%), , Occlusion MAPCA(s), , 1 (0.1%), , Electrophysiologic, , 62 (6.4%), , Pacemaker procedure, , 62 (6.4%), , Thoracic Arteries and Veins, , 58 (6.0%), , Coarctation repair, end-to-end, , 25 (2.6%), , Coarctation repair, interposition graft, , 17 (1.8%), , Coronary artery fistula ligation, , 3 (0.3%), , ALCAPA: 1× coronary fistulaa, , 3 (0.3%), , PDA closure: 1× TVP, , 2 (0.2%), , Vascular ring repair, , 3 (0.3%), , Aortic aneurysm repair, , 3 (0.3%), , Coronary artery bypass for anomalous coronary artery, , 1 (0.1%), , Single Ventricle, , 14 (1.4%), 10 (1.0%), , Aortic valvuloplasty: 1× PAA, 2× LVOT, 1× MVP, , 6 (0.6%), , Fontan, revision or conversion (re-do Fontan)b, , Sinus of Valsalva, aneurysm repair, , 3 (0.3%), , Fontan, TCPC, lateral tunnel: 1× MAZE, 1× PM, , 3 (0.3%), , Transplant, heart, , 3 (0.3%), , Palliative Procedures, , 8 (0.8%), , Aortic root replacement, valve sparing: 1× MVP, , 3 (0.3%), , Glenn procedure: 1× PDA, 2× Blalock, takedown, 1× PM, 1× PAP, , 3 (0.3%), , Shunt, modified Blalock-Taussig, , 3 (0.3%), , Shunt, central, , 1 (0.1%), , Shunt, ligation and takedown, , 1 (0.1%), , Transposition of the Great Arteries, , 8 (0.8%), , Atrial baffle procedure, Mustard revision: 2×, RVOT, 2× PM, 1× ASD, , 8 (0.8%), , Pulmonary Venous Anomalies, , 6 (0.6%), , Partial anomalous pulmonary venous, connection repair, , 4 (0.4%), , Cor triatriatum repair, , 2 (0.2%), 6 (0.6%), , Septal Defects, , 255 (26.5%), , Atrial septal defect repair: 7× MAZE, 15× CABG,, 3× PAR, 13× MVP, 10× TVP, 1× PVP, , 176 (18.3%), , Ventricular septal defect repair: 7× ASD, 7×, PHG, 7× RVOT, 1× LVOT, 2× DCRV, 2× AVRM,, 1× CABG, , 53 (5.5%), , Partial atrioventricular septal defect repair: 1×, LVOT, 4× TVP, 4× ASD, 1× AHG, , 26 (2.7%), , Right Heart Lesions, , 180 (18.8%), , Pulmonary valve replacement: 12× VSD, 30×, RVOT, 7× PAP, 10× ASD, 2× AVRM, 2× TVR,, 10× TVP, 4× Pm, , 121 (12.6%), , Ebstein repair: 2× Glenn, 1× ASD, 1× PM, , 17 (1.8%), , Miscellaneous Procedures, , Tricuspid valve replacement, , 12 (1.2%), , Aneurysm, atrial, repair, , 2 (0.2%), , 11 (1.1%), , Cardiac tumor resection: 2× ASD, , 2 (0.2%), , 8 (0.9%), , Pulmonary embolectomy, acute, , 1 (0.1%), , Pulmonary embolectomy, chronic: 1× ASD, , 1 (0.1%), , Tetralogy of Fallot primary repair, Valvuloplasty tricuspid valve: 4× ASD, 1× MAZE,, 2× MVR, 1× MVP, P23, , Modified from Putman and colleagues., a, Data following colon after each procedure shows the concomitant procedures and their frequency (“×” is shorthand for a multiplier)., b, Includes two re-do procedures for sclerosed homografts and eight conversions (two extracardiac and six lateral tunnel)., Key: AHG, Aortic homograft procedure; ALCAPA, anomalous origin of the left coronary artery from the pulmonary artery; ASDL, atrial septal defect closure;, AVRM, mechanical aortic valve replacement; AVSD, atrioventricular septal defect repair; LVOT, left ventricular outflow tract procedure; MVP, mitral valvuloplasty;, MVR, mitral valve replacement; PAA, prosthetic ascending aorta; PAP, plasty of the pulmonary artery(ies); PDA, patent ductus arteriosus closure; PHG, pulmonary, homograft procedure; PM, pacemaker procedure; PVP, pulmonary valvuloplasty; RVOT, right ventricular outflow tract procedure; TVP, tricuspid valvuloplasty;, TVR, tricuspid valve replacement; VSD, ventricular septal defect closure., , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, Cohort Survival, , Survival probability, , 1.0, 0.8, 0.6, 0.4, 0.2, 0.0, , 830, , 629, , 535, , 448, , 349, , 2, , 4, , 6, , 8, , 0, , A, , 165, , 10, , 12, , 106, , 14, , 40, , 16, , 18, , 20, , Years, Subgroup Survival, , 1.0, Survival probability, , 259, , A&V, Right heart, , 0.8, , Septal, , 0.6, , Congenital Heart Disease in the Adult, , In a multi-institutional (37 Child Health Corporation of, America [CHCA] centers) study of 719 adult congenital, cardiac operations performed between 2005 and 2007, Mahle, and colleagues report that the most frequent principal procedures were pacemaker placement (29%), pulmonary valve, replacement (17%), aortic valve replacement (8.3%), and, Fontan revision (5.2%).M2 Early mortality was 1.9%. Among, the 37 freestanding children’s hospitals that make up this, consortium, 0% to 11% of all cardiac operations were performed in adults., Data from the CONCOR (CONgenital CORvitia) Dutch, national registry of adults with CHD show several notable, gender-specific outcomes in 7414 patients.V14 Women had a, 33% higher risk of pulmonary hypertension, a 33% lower risk, of aortic events, a 47% lower risk of endocarditis, and a 55%, lower risk of arrhythmias and ICD placement. There were no, gender-related mortality differences., , Left heart, , 0.4, 0.2, 0.0, , B, , 259, 179, 357, 59, , 195, 127, 265, 49, , 172, 105, 206, 43, , 145, 94, 171, 36, , 114, 81, 124, 27, , 2, , 4, , 6, , 8, , 0, , 77, 64, 92, 20, , 53, 45, 51, 13, , 10, , 40, 19, 28, 12, , 12, , 14, , 16, 7, 10, 6, , 16, , Septal, Right heart, Left heart, A&V, , 18, , 20, , Years, , Figure 29-4 Survival after surgery for adult congenital heart, disease. A, Survival of entire cohort (blue line with 95% confidence, limits bars) and of an age-matched population (red line). B, Survival, of four largest subgroups of procedures. Key: A & V, Thoracic arteries, and veins. (From Putman and colleagues.P23), , Table 29-8, , Section II, , Atrial Septal Defect, , DEFINITION, Definition, morphology, and basic physiology of atrial septal, defect (ASD) are described in Chapter 30. ASD is one of the, most common anomalies found in adults. It typically presents, as newly diagnosed primary disease, or previously diagnosed, primary disease with benign physiology. It constitutes 25% to, , Incremental Risk Factors for Early and Late Mortality Following 963 Adult Congenital Heart Operations, 30-Day, , 1-Year, , Long-Term, , Univariate OR, (95% CI), , Multivariate OR, (95% CI), , Univariate OR, (95% CI), , Multivariate OR, (95% CI), , Univariate HR, (95% CI), , Multivariate HR, (95% CI), , Pulmonary HT, , 7.82 (2.08-29.38), , 7.72 (1.99-29.86), , 7.20 (2.54-20.38), , 7.59 (2.55-22.59), , NS, , —, , Arrhythmia, , 2.02 (1.01-4.03), , 1.92 (0.90-4.07), , 1.79 (1.04-3.07), , NS, , 2.02 (1.44-2.82), , 1.97 (1.34-2.91), , Age at surgery, , 1.03 (1.00-1.06), , 1.03 (0.99-1.06), , 1.03 (1.01-1.06), , 1.03 (1.00-1.05), , 1.05 (1.03-1.06), , 1.04 (1.02-1.06), , Impaired VEF, , NS, , —, , 3.71 (1.69-8.15), , 3.61 (1.60-8.15), , 4.02 (2.52-6.42), , 3.71 (2.27-6.07), , Active endocarditis, , NS, , —, , 6.30 (1.74-22.80), , NS, , NS, , —, , Preoperative, ventilation, , NS, , —, , 8.04 (2.21-29.23), , 6.67 (1.66-26.86), , NS, , —, , Additive EuroSCOREa, , NS, , —, , 1.32 (1.14-1.54), , NS, , 1.29 (1.17-1.42), , NS, , Logistic EuroSCOREa, , NS, , —, , 1.10 (1.04-1.16), , NS, , 1.08 (1.04-1.14), , NS, , NYHA III or IV, , NS, , —, , 3.28 (1.49-7.21), , NS, , 3.01 (1.89-4.82), , —, , Cyanosis, , NS, , —, , NS, , —, , 1.90 (1.07-3.37), , 2.37 (1.30-4.33), , Diabetes, , NS, , —, , NS, , —, , NS, , —, , COPD, , NS, , —, , NS, , —, , 4.82 (2.39-9.74), , 2.57 (1.19-5.52), , Creatinine, , NS, , —, , NS, , —, , NS, , —, , Smoking, , NS, , —, , NS, , —, , 2.31 (1.18-4.55), , 3.17 (1.58-6.36), , Extracardiac artery, , NS, , —, , NS, , —, , NS, , —, , Male sex, , NS, , —, , NS, , —, , NS, , —, , P23, , Modified from Putman and colleagues., NOTE: Thirty-day and one-year mortality were analyzed using logistic regression, long-term mortality was analyzed using Cox regression., a, See reference P24 for description of EuroSCORE., Key: CI, Confidence interval; COPD, chronic obstructive pulmonary disease; HR, hazard ratio; HT, hypertension; NS, nonsignificant; —, not tested multivariate;, NYHA, New York Heart Association; OR, odds ratio; VEF, ventricular ejection fraction., , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1077

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1078, , PART VI, , Congenital Heart Disease in the Adult, , 30% of newly diagnosed congenital heart disease (CHD), cases in adults.R20, , MORPHOLOGY, Each of the four morphologic forms of ASD is found in, adults, with associated cardiac defects in up to a third (see, Chapter 30). Most commonly, these are classic associations, found with sinus venosus and ostium primum defects. Mitral, valve prolapse and valvar pulmonic stenosis may be seen, with ostium secundum defects.B3,R13,S15 Patent foramen ovale, (PFO) is of particular interest., , TECHNIQUE OF OPERATION, Options for ASD closure include surgical and percutaneous, device approaches. Surgical techniques used are the same as, those used in children (see Chapter 30). Because most defects, that come to surgery in the current era are large, patch closure, should always be used in adults, even for secundum ASDs;, primary closure should be avoided. Concomitant procedures, such as tricuspid valve repair and the maze procedure, uncommonly used in children, may be required., Percutaneous device closure of secundum ASDs can be, performed in adults regardless of age., , RESULTS, CLINICAL FEATURES AND DIAGNOSTIC CRITERIA, Presentation, The chronic right-sided volume and pressure overload found, with large ASDs leads to reduced aerobic capacity, atrial, arrhythmias, and respiratory infections. Dyspnea and palpitations are the most common presenting symptoms, typically, in the third and fourth decades of life.C37,K22 Atrial fibrillation, or flutter and paradoxical embolism may also lead to presentation. Pulmonary arterial hypertension (PAH) is usually mild, and primarily flow related; however, severely elevated pulmonary vascular resistance (Rp) and obstructive pulmonary, vascular disease leading to Eisenmenger physiology occur in, a minority of patients., Smaller ASDs—those less than 5 mm in diameter—and, PFOs do not cause these changes, but can be the source (as, can larger ASDs) of paradoxical emboli.L11,S29,W9 Defects, smaller than 1 cm may not cause symptoms for many decades,, but the left-to-right atrial shunt may increase later in life as, left ventricular compliance decreases because of acquired, cardiac diseases such as coronary artery disease and hypertension, causing symptoms to develop late.C37, Diagnosis, The electrocardiographic and chest radiographic findings are, the same in the adult as in the child (see Chapter 30). The, mainstay of diagnosis is echocardiography. In adults, transthoracic studies may produce inadequate images of the atrial, septum. Transesophageal studies often produce more accurate atrial septal images and detail the dimensions and position of the defect.K29,M26,M27,P5 PAH is estimated by measuring, velocity of tricuspid regurgitation flow, if present. Contrast, echocardiography can be used to confirm atrial-level shunting if direct imaging and color Doppler evaluation are not, definitive. Both magnetic resonance imaging (MRI) and, computed tomography (CT) angiography may be helpful, if echocardiography is not definitive, and are particularly, helpful in defining the pulmonary venous anatomy in, sinus venosus ASD. MRI is preferred to CT, which requires, substantial radiation exposure.B27,H18,H26,T3 Cardiac catheterization is reserved for three situations: to assess pulmonary, vascular hemodynamics if PAH is suspected or confirmed;, to assess presence of coronary artery disease, typically, in patients over age 35 (male) and 40 (female); and as, a therapeutic procedure if percutaneous device closure, is planned., , Early mortality following surgery is less than 1%, and longterm survival approaches that of the general population for, straightforward cases without associated anomalies or PAH., Closure effectively relieves shunt-related symptoms. Newonset late atrial arrhythmias can develop in patients after, surgery. The maze procedure is effective in reducing, but not, always eliminating, atrial fibrillation and flutter., Percutaneous device closure of secundum ASDs can be, performed in adults with less than 1% mortality and low, morbidity, with demonstration of reduction in right ventricular size and pulmonary artery pressure in all age groups.H25, A recent study of 100 adults with secundum ASD revealed, similar, but not identical, outcomes using closure by surgery, or percutaneous device.S53 The 52 surgical patients underwent treatment between 2001 and 2003, and the 48 device, closure patients between 2003 and 2005. The procedure was, successful in all surgical patients (100%; CL 96%-100%) and, in 45 of the 48 (94%; CL 88%-97%) percutaneous device, patients. There was no mortality in either group (CL 0%-3.6%, for surgical closure and 0%-3.9% for device closure). The, number of complications was similar, but the surgical group, had more serious ones. Postprocedure length of stay was, shorter in the percutaneous device group. At 1-year follow-up,, there was no mortality and equal reduction in right ventricular size and pulmonary artery pressure., In a series of 25 adults with surgical repair of sinus venosus, ASD and partial anomalous pulmonary venous return, there, was no early mortality (CL 0%-7.3%) and one late death due, to heart failure.J5 One patient (4%; CL 0.6%-13%) had superior vena caval obstruction. In a series of 75 adult patients, with either secundum or sinus venosus ASD, there was one, early death (1.3%; CL 0.2%-4.4%) in a patient with secundum, ASD, recurrent pulmonary emboli, and severe PAH.S1, An ostium primum ASD repair was performed in 51, patients (mean age 27 years) with an early mortality of 2.0%, (CL 0.3%-6.5%).A4 Preoperative left atrioventricular (AV), valve regurgitation was moderate in 35% and severe in 4%., With respect to the left AV valve, cleft closure was performed, in all patients, but anuloplasty in only two. At 36-month, follow-up, 21% had moderate regurgitation and 8% severe, regurgitation; one had mitral valve replacement. Postoperative regurgitation was progressive (Fig. 29-5). Risk factors for, postoperative moderate or severe mitral regurgitation were, female gender and preoperative PAH. Interestingly, moderate or severe preoperative left AV valve regurgitation was not, a risk factor., In another series, there was no early (CL 0%-12%) or late, mortality in 15 patients (mean age 31 years).J4 The left AV, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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100, , Percentage, , 80, 60, 40, % Free from severe MR, % Free from moderate MR, , 20, 0, , 3, , 12, , 24, , 36, , 60, , 100, , Time (months), , Figure 29-5 Freedom from moderate (red line) or severe (blue line), mitral regurgitation (MR) after surgical repair of ostium primum, atrial septal defect and cleft closure in 51 adult patients. (From, , Systolic pulmonary arterial pressure (mmHg), , Chapter 29, , Congenital Heart Disease in the Adult, , 120, 100, 80, 60, 40, 20, 0, 20, , 40, 60, Age (years), , 80, , Agarwal and colleagues.A4), , Figure 29-6 Correlation between systolic pulmonary artery pressure and age in 236 adults with secundum atrial septal defect before, closure (r = .65, P < .0001). (From Agarwal and colleagues.A4), , valve cleft was closed in 12 and a DeVega anuloplasty performed in 8. In a small series reported in 1976, seven patients, (mean age 41 years) underwent surgery with no early mortality (CL 0%-24%) and one late death at 18 months.M11, There are only case reports of surgical repair of coronary, sinus ASD in the adult.J6,O11, , adults with secundum ASD, 84% met criteria for device, closure and 16% underwent surgical closure.H25, Older age is generally not a contraindication to, surgical ASD closure, although there is some contro­, versy.G6,K22,S21,W8,W14 Two older studies demonstrate symptom, improvement and survival benefit in patients over age 60, undergoing surgical closure.J10,N1 A more recent randomized, trial of surgical ASD closure in patients over age 40 showed, symptom improvement but no survival benefit.A22 If percutaneous device closure is not contraindicated, this approach, may provide a better risk/benefit ratio than surgery in older, patients.R20, , INDICATIONS FOR OPERATION, Patients with unrepaired ASDs of 5 mm or less in diameter, without symptoms or associated lesions can be followed., Continued follow-up is required because symptoms may, develop as left ventricular compliance decreases with age and, the left-to-right shunt increases. Paradoxical embolism is an, indication for closure regardless of ASD size, as may be onset, of atrial arrhythmias. Larger ASDs usually are associated with, right ventricular enlargement and should be closed even if, symptoms are absent at time of diagnosis because the natural, history predicts eventual morbidity. Closure of moderate and, large defects provides demonstrated benefit.E3, PAH may be a contraindication to closure if Eisenmenger physiology is present. PAH increases with age, providing evidence that ASDs should be closed when diagnosis, is made in order to minimize this complication (Fig. 29-6)., Lung biopsy reveals pathologic evidence of pulmonary vascular disease in 59% of patients with secundum and sinus, venosus ASDs.S1, Presence of paroxysmal or persistent atrial fibrillation or, flutter is an indication for performing a maze procedure., Indications for surgical closure include all sinus venosus,, ostium primum, and coronary sinoseptal ASDs. Ostium, secundum ASDs may be closed surgically or with a percutaneous device. Percutaneous closure is contraindicated if the, defect is very large, has inadequate rims,D14,D26,F9 or is associated with other intracardiac anomalies requiring surgery, such, as important tricuspid valve regurgitation or atrial arrhythmia, requiring a maze procedure. Stretched ASD size greater than, 36 mm and native size greater than 25 mm are contraindications for device closure.H25 Relative contraindications include, multiple ASDs, fenestrated septum primum, and redundant, or aneurysmal septum primum. In one large study of 236, , Section III, , Patent Foramen Ovale, , DEFINITION, Patent foramen ovale (PFO) is incomplete closure of the, septum primum resulting in a valve-like flap closure. It allows, intermittent interatrial shunting, which may occur in the leftto-right or right-to-left direction., , MORPHOLOGY, PFO appears as a small slit at the upper margin of the fossa, ovale (see Chapter 1). It represents incomplete obliteration, of the fetal foramen ovale., , CLINICAL FEATURES AND DIAGNOSTIC CRITERIA, Presentation, Of the 280,000 individuals who suffer a cryptogenic stroke, in the United States every year, a PFO is found twice as often, as in the normal population, suggesting an association, between PFO and stroke.W1 Nevertheless, three prospective, class 1 studies addressing cryptogenic stroke all suggest that, when patients who suffer an initial cryptogenic stroke are, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1079

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1080, , PART VI, , Congenital Heart Disease in the Adult, , followed, recurrent cryptogenic stroke has no relationship to, the size or presence of a PFO.S19 Yet several longitudinal, observational studies examining the likelihood of recurrent, stroke in patients with PFO suggest that recurrent strokes, occur less often when the PFO is treated with a device or, with surgical closure rather than with antiplatelet or anticoagulation therapy.C7,S16 Larger PFO size has also been associated with increased risk of recurrent cryptogenic stroke, following an initial event.S16 Thus, cryptogenic stroke has, multiple causes, only one of which is paradoxical embolism, through a PFO; however, just because a cryptogenic stroke, occurs in the presence of a PFO does not mean that the, PFO is causative. These studies do not provide definitive, enough data to formulate a management plan for preventing, recurrent cryptogenic stroke in PFO patients., Atrial septal aneurysm with or without a PFO has, also been found with increased prevalence in patients with, cryptogenic stroke, suggesting a possible association between, it and cryptogenic stroke.A5, , NATURAL HISTORY, PFO is a common condition, estimated to occur in, about 25% of the adult population.G11 It causes minimal, hemodynamic consequences; however, it allows intermittent, interatrial shunting, which may occur in the left-to-right, or right-to-left direction. When flow occurs in the rightto-left direction, the potential for paradoxical embolism, exists. There is probably an association between PFO, and cerebrovascular events; however, the nature of the relationship is unclear and controversial, as described in the, preceding text., , TECHNIQUE OF OPERATION, Percutaneous device closure is currently favored over surgical, closure; however, surgical PFO closure is an option if a, patient refuses device closure, there is another reason for, open heart surgery, or there is a contraindication. In patients, with atrial septal aneurysm without PFO, surgical resection, with reconstruction of the atrial septum can be considered, when anticoagulation therapy has failed., Unfortunately, a surgical closure arm is not included in, any of the ongoing randomized trials.O3 This oversight may, be pertinent because the preliminary report of the CLOSURE, 1 trial noted that in some cases thrombus has been identified, on the closure device at follow-up., , question are currently in progress, but no evidence base can, determine whether PFO closure is superior to antiplatelet and, anticoagulation therapy for preventing recurrent stroke.O3 A, preliminary report from one of these randomized trials, the, CLOSURE 1 trial, was presented at the American Heart, Association 2010 Scientific Sessions. No difference in recurrent stroke was noted between anticoagulation therapy and, the STARFlex PFO closure device. Certain design flaws in, this study have been noted., The association of PFO and migraine headache is even, more controversial than the association between PFO and, cryptogenic stroke. It appears, based on an extensive metaanalysis, that percutaneous device closure of PFO may cure, or improve migraine headache symptoms in a subset of, migraine patients who have a PFO and suffer a cryptogenic, stroke.B36 In general, the cause of migraines is multifactorial,, with not all migraines due to paradoxical embolism or paradoxical streaming of causative humoral factors.W1 This may, explain the findings in the Migraine Intervention with, STARFlex Technology (MIST) trial, a randomized, doubleblinded, sham procedure controlled study of PFO closure in, patients with PFO and migraine. In this study population,, there was no other indication (i.e., no cryptogenic stroke) for, PFO closure other than migraine headache. The study showed, no benefit to PFO closure in this broader group of migraine, patients.D23, , INDICATIONS FOR OPERATION, The joint advisory recommends antiplatelet therapy as the, first-line treatment for patients with PFO and cryptogenic, stroke, vitamin K antagonist anticoagulation if there is associated hypercoagulable state or atrial fibrillation, and PFO, device closure if recurrent stroke occurs on anticoagulation.O3, PFO closure is also indicated in patients exposed to alterations in atmospheric pressure, increasing the risk of paradoxical air embolism., At the present time, PFO closure is not considered standard medical practice for treating migraine headache.G11, There is substantial evidence, however, that patients with, PFO who suffer a cryptogenic stroke and also suffer from, migraines will have their migraines cured or improved about, 80% of the time after PFO device closure performed to, prevent recurrent paradoxical embolism.D2,W1, , Section IV, , RESULTS, Antiplatelet therapy, anticoagulation therapy, percutaneous, device closure, and surgical closure are all used to prevent, recurrent stroke in patients with PFO. No consensus has been, reached about whether one form of therapy is better than the, others.C3,O3,S19 A number of studies examining the effectiveness of these different treatment options in preventing recurrent stroke point to an advantage of closure over antiplatelet, or anticoagulation therapy; however, the criticism of all these, studies is that none of them randomized the treatment, options.C7,P1,S16,W24 A science advisory published jointly by the, American Heart Association, American Stroke Association,, and American College of Cardiology in 2009 notes that, five different prospective randomized trials addressing this, , Ventricular Septal, Defect, , DEFINITION, Definition, morphology, and basic physiology of ventricular, septal defect (VSD) are described in Chapter 35. In the, adult, VSD presenting for surgical closure is rare. When, it does occur, it is unusual for it to present as newly, diagnosed primary disease. More commonly, VSD presents, as previously diagnosed primary disease with benign physiology, such as a restrictive defect, with new development of a, specific VSD-related complication requiring intervention., It may also present as a secondary disease, such as late after, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, , Congenital Heart Disease in the Adult, , surgical VSD closure, in association with a new VSD-related, complication., , MORPHOLOGY, Each of the morphologic forms of VSD is found in the adult, (see Chapter 35)., , CLINICAL FEATURES AND DIAGNOSTIC CRITERIA, Presentation, Adults with a history of VSD closure as an infant or child may, present with infectious endocarditis in the presence of a small, residual defect; distortion of the tricuspid valve septal leaflet, due to previous VSD closure, resulting in clinically important, tricuspid regurgitation or progressive aortic regurgitation due, to surgical injury; distortion of the valve during VSD closure;, or prolapse of the valve into the VSD prior to VSD closure, that progresses after closure., Diagnosis, Transthoracic echocardiography is usually diagnostic for, patients with unrepaired or previously repaired VSD unless, the surface windows do not provide adequate images. In that, case, transesophageal echocardiography is usually diagnostic., It is important not only to focus on size and position of, the native or residual VSD, but also to rule out aortic valve, prolapse and regurgitation, tricuspid regurgitation, doublechamber right ventricle, subaortic membrane, membranous, septal aneurysm, primary pulmonary arterial hypertension, (PAH), and ventricular dysfunction., If PAH is suggested by echocardiography, diagnostic, cardiac catheterization is indicated to assess status of the, pulmonary vascular bed (see “Pulmonary Arterial Hypertension and Eisenmenger Physiology” in Section I). Cardiac, catheterization may also be indicated in the patient with a, small to moderate VSD, either unrepaired or repaired with a, residual defect, for whom the indications for surgical closure, are equivocal. Specific data obtained at catheterization may, assist in the decision to close the VSD, including magnitude, of the shunt, left ventricular end-diastolic pressure, pulmonary artery pressure, and pulmonary vascular resistance (Rp)., Catheterization and angiography may also be indicated to, assess the coronary arteries if arteriosclerotic disease is suspected, if the patient is older than age 35 (male) or 40, (female), or to further characterize unusual structural problems, such as membranous septal aneurysms (Fig. 29-7)., Magnetic resonance imaging (MRI) and computed, tomography (CT) may play a role in defining anatomic details, if echocardiography is not definitive. These imaging modalities may help define multiple VSDs, unusually positioned, muscular VSDs, and suspected associated pulmonary artery, or vein anomalies., , NATURAL HISTORY, Unrepaired large (unrestrictive, > 50% aortic diameter) VSD, first presenting in the adult is rare. When it occurs, there is, likely to be PAH or Eisenmenger physiology. Occasionally,, evaluation reveals a reactive pulmonary vascular bed. Unrepaired moderate (restrictive, 25%-50% aortic diameter) VSD, , Figure 29-7 Left ventricular angiogram in left anterior oblique, projection demonstrating aneurysm of membranous septum in a, 29-year-old man. A perimembranous ventricular septal defect forms, the base of the aneurysm, and contrast enters right ventricle,   = 1.2)., through a trivial defect at apex of the aneurysm (Qp/Qs, Aneurysm protrudes into right ventricular outflow tract, resulting in, pressure gradient of 32 mmHg across outflow tract. (From Yilmaz, and colleagues.Y8), , presenting in the adult is also rare. When it does, there is, pulmonary overcirculation and symptoms of high-output, heart failure. Unrepaired small (highly restrictive, <25%, aortic diameter) VSD may be newly diagnosed or, more, likely, previously diagnosed. These patients are hemodynamically asymptomatic., Secondary complications related to a small VSD may, develop in the adult. These include aortic, mitral, and tricuspid regurgitation, double-chamber right ventricle, subaortic, membrane, and endocarditis.S41 Aneurysms of the membranous septum may develop and progress in long-standing, unrepaired perimembranous VSD. In one large series of 254, adults with perimembranous VSD, aneurysms developed in, 51 cases (20%).Y8 When aneurysms form, flow restriction, occurs through the VSD, resulting in pulmonary to systemic, ,  of less than 2 : 1. Aneurysms may enlarge, flow ratio (Qp/Qs), over time, causing important secondary hemodynamic, changes, including right ventricular outflow tract obstruction, tricuspid regurgitation, and rupture of the aneurysm,, ,  (see Fig. 29-7).Y8, resulting in an acute increase in Qp/Qs, Prevalence of endocarditis and aortic valve prolapse and, regurgitation may increase when a membranous septal aneurysm is present.Y8, , TECHNIQUE OF OPERATION, Surgical techniques used to close VSDs in adults are the same, as those used in infants and children (see Chapter 35). Percutaneous device VSD closure may be used for selected muscular VSDs remote from ventricular inlet and outlet., If aneurysm of the membranous septum is present, it, should be completely excised via exposure through the right, atrium, with standard patch closure of the anatomic borders, of the perimembranous VSD. Closure of the shunt by, approximating aneurysmal tissue should be avoided because, recurrence of the aneurysm and residual leaks have been, described.Y8 Associated tricuspid regurgitation or aortic, regurgitation should be addressed concomitantly., , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1081

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1082, , PART VI, , Congenital Heart Disease in the Adult, , RESULTS, Early mortality for uncomplicated VSD closure in the adult, is less than 1%. If complex associated problems or pulmonary, vascular disease coexist, early mortality is 5% to 10%.M14,O9 In, a series of 51 adults (mean age 22 years, age range 15-59, years) with perimembranous VSDs complicated by aneurysm, of the membranous septum, there was no early mortality (CL, 0%-3.7%).Y8 In another experience, there was no early mortality (CL 0%-4.0%) in 46 adult patients (mean age 34 years), with perimembranous and subarterial VSDs.M32, Late mortality in patients without associated comorbidity, is low: 5% at a mean follow-up of 10 years, 5% at a mean, follow-up of 15 years, and 0% at a mean follow-up of 5.6, years in three separate series.M32,O9,Y8, Surgical complications associated with VSD closure in, adults are similar to those seen in younger patients, including, residual VSD requiring reoperation, complete heart block,, and injury to aortic and tricuspid valves.M32, In a series of 220 patients with small perimembranous, VSDs followed into adulthood, 7% required surgical closure, over a 6-year observation period.S41 In the remaining 93%, 4%, had spontaneous closure, 1% died of a cardiac cause, and 4%, developed endocarditis. Prevalence of PAH increased from 3%, ,  was 1.2. These data, to 9%. In this study the average Qp/Qs, emphasize that a small VSD is not always benign. In another, analysis of 125 adolescent and adult patients (mean age 23, years, age range 10-51 years) with unrepaired VSD, 41 were, treated surgically, 70 were considered to have no indication, for surgery (small VSD and no associated problems), and 14, were inoperable due to PAH.O9 At 15-year follow-up, even, though the group with no indication for surgery had less, complex defects, mortality was twice that of the operated, group, and there was a higher occurrence of endocarditis and, new valvar lesions. New York Heart Association (NYHA), functional class deteriorated in the unoperated group and, improved in the operated group. Pulmonary artery pressure, rose in the unoperated group and fell in the operated group., In the small group of 14 patients in which surgery was contraindicated because of PAH, mortality at 15 years was 71%., , INDICATIONS FOR OPERATION, Indications for operation are the same whether the VSD is, unrepaired or a residual defect following attempted surgical, or device closure., Large VSDs should be closed if cardiac catheterization, demonstrates reversible PAH. They should not be closed if, fixed-resistance severe PAH or Eisenmenger physiology is, present. Levels of Rp and PAH that contraindicate surgical, closure are described under “Pulmonary Arterial Hypertension and Eisenmenger Physiology” in Section I. Moderate, VSDs should be closed. They almost always cause pulmonary, ,  of 1.5 or greater. They are someovercirculation and Qp/Qs, what restrictive and do not cause Eisenmenger physiology., There is evidence that surgical closure provides long-term, benefit for these patients.O9 Traditionally, surgical closure has, not been recommended for small VSDs. Most will have a, ,  of less than 1.5. However, considering the low morQp/Qs, bidity and mortality of surgical closure of VSD in the current, era and the morbidity and mortality in adults with small, unrepaired VSDs,O9,S41 the traditional recommendation to, not close a small VSD in the adult should be questioned., , Other indications for surgical intervention include development of important associated problems, usually in the, setting of a small restrictive VSD: aortic regurgitation, tricuspid regurgitation, subaortic membrane, double-chamber, right ventricle, large aneurysm of the membranous septum,, and infectious endocarditis. Surgery may require addressing, the VSD and the associated problem concomitantly, or, the associated problem alone if the VSD has been previously, closed. In one series of 20 patients (mean age 43 years), an associated problem was the indication for surgery in, 35%; in another series of 42 patients (mean age 27 years), an associated problem was the indication for surgery, in 52%.M14,S54, , Section V, , Atrioventricular Septal, Defect, , DEFINITION, The definition, morphology, and basic physiology of atrioventricular septal defect (AVSD) are described in Chapter 34., Most adults presenting with AVSD fall into the category, of secondary congenital heart disease, having undergone, surgical repair in infancy or childhood., , MORPHOLOGY, AVSD may be partial or complete. Adults rarely present with, complete unrepaired AVSD, and when they do, it is usually, inoperable because of pulmonary arterial hypertension (PAH)., This is because of the combination of unrestrictive ventricular- and atrial-level shunting and the high likelihood of Down, syndrome. Partial AVSD may present unrepaired in the adult, and is usually operable. Down syndrome is uncommon in, partial AVSD., The most common indications for surgery in the adult, with repaired AVSD are left-sided atrioventricular (AV) valve, stenosis or regurgitation, followed by left ventricular outflow, tract obstruction., , CLINICAL FEATURES AND DIAGNOSTIC CRITERIA, Presentation, The clinical presentation of repaired AVSD depends on the, nature of residual or recurrent lesions after repair, and on, development of new lesions. The most common residual or, recurrent lesion is left-sided AV valve regurgitation, which, presents with left ventricular volume overload and failure,, left atrial dilatation, and atrial fibrillation. The next most, common lesion is subaortic left ventricular outflow tract, obstruction, which may be residual, recurrent, or new onset., Signs and symptoms are the same as for any patient with left, ventricular outflow obstruction. Other presentations include, signs and symptoms related to left or right AV valve stenosis,, right AV valve regurgitation, residual ventricular septal defect, (VSD), endocarditis related to any of these residual structural, lesions, or PAH, particularly in patients with repaired complete AVSD., , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. 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Chapter 29, Diagnosis, The electrocardiogram shows typical superior left-axis deviation, and this finding alone in a previously undiagnosed, adult is highly suggestive of AVSD. In adults with residual, or recurrent lesions, electrical findings of left atrial enlargement, left ventricular hypertrophy, and right ventricular, hypertrophy may be present. Atrial fibrillation or flutter may, also be present., The chest radiograph will show a prominent pulmonary, artery bulb and distal pulmonary artery pruning if PAH is, present, cardiomegaly if valve regurgitation or left-to-right, shunting exists, and pulmonary venous congestion if leftsided AV valve regurgitation is present. Echocardiography is, diagnostic, just as it is in infants and children. In previously, repaired patients, this study should focus on determining, presence of residual atrial or ventricular shunts, right and left, AV valve function, left ventricular outflow tract patency, and, signs of PAH., Cardiac catheterization is performed in all unrepaired, adults under consideration for surgical repair to assess the, pulmonary vasculature. Coronary angiography is indicated if, the patient is over age 35 (male) or 40 (female) or if coronary, insufficiency is suspected. Catheterization may also be indicated to assess PAH in repaired patients and general hemodynamics in patients with equivocal indications for surgical, intervention. Magnetic resonance imaging can be helpful in, assessing regurgitant fraction when AV valve regurgitation is, present in patients with equivocal indications for surgical, intervention., , NATURAL HISTORY, Presentation of partial AVSD in the adult has some similarities to that of a large atrial septal defect (ASD), with chronic, right heart volume overload leading to right ventricular, failure. Mild PAH is present, but Eisenmenger physiology is, uncommon. Unlike a large ASD, however, regurgitation of, the right-sided, left-sided, or both AV valves is common,, causing earlier onset of ventricular failure and atrial fibrillation, and flutter., Presentation of the adult with unrepaired complete, AVSD will be similar to that of a large or unrestrictive VSD,, with PAH and likely Eisenmenger physiology. Additionally,, important AV valve regurgitation may be present, increasing, the likelihood of ventricular failure and atrial fibrillation, or flutter., , TECHNIQUE OF OPERATION, The surgical approach to unrepaired AVSD, whether partial, or complete, is the same in the adult as in infants and children, (see Chapter 34)., Postrepair residual or recurrent left AV valve regurgitation, requires repeat surgery in 5% to 10% of patients. It may be, due to an open cleft or breakdown of a previous cleft closure., Surgical closure of the cleft is performed. Reduction anuloplasty is almost always indicated. If the etiology of regurgitation is more complex, then standard techniques used for, mitral valve repair are used (see Chapter 11). Rigid valve, anuloplasty rings may be contraindicated because the shape, of the anulus in repaired AVSD is different from that of, the normal mitral valve. Mixed regurgitation and stenosis is, , Congenital Heart Disease in the Adult, , particularly difficult to repair, and valve replacement may be, required. The inferiorly displaced position of the AV node, and bundle of His must be kept in mind to avoid causing, heart block., Left ventricular outflow tract obstruction is rarely due to, a simple subaortic membrane. Typically, there is an elongated, narrow, muscular tunnel with or without the addition, of AV valve chordal tissue. The chordal tissue is rarely functional. Most commonly these chords were previously normal, components of the superior bridging leaflet of a Rastelli type, A defect. They become nonfunctional as part of standard, original AVSD repair. Surgical correction of late left ventricular outflow obstruction is best performed through the aortic, valve, with extensive circumferential myectomy and resection, of the obstructive AV valve and chordal tissue. Damage to, the mitral valve can still occur during this procedure, as it can, for any left ventricular outflow tract resection; however,, injury to the conduction system is not of concern because the, AV node is displaced inferiorly. Occasionally, myectomy will, not be effective, and a Konno operation will be required (see, Chapter 12)., , RESULTS, Death, Early mortality for primary repair of partial AVSD in the, adult can be less than 1%; however, some older series report, early mortality as high as 6%. There was no (CL 0%-4.8%), early mortality in a series of 39 patients (mean age 36 years), in whom the indication for surgery was left-to-right shunt.G5, One patient required concomitant left AV valve replacement, and 37 underwent cleft closure, five of whom also underwent, reduction anuloplasty. At a median follow-up of 7 years, there, were six late deaths, five of which were cardiac in origin. In, a series of 132 patients with partial AVSD, 10% of whom were, older than age 20 years, early mortality was 4.5% (CL 2.7%7.2%) and late mortality 3.2%.C26 By univariable analysis,, older age (more than 10 years at initial repair) was among, the risk factors. By multivariable analysis, however, only preoperative PAH and a grossly deformed left AV valve were risk, factors, suggesting that the association of death with older, age is due to development of PAH and valve deformity over, time. In a series of 31 patients with partial AVSD, all of whom, were over age 40, early mortality was 6.4% (CL 2.2%-15%);, however, some of the operations were performed as long ago, as 1958, and the two deaths occurred in 1967 and 1981.B18, There were nine additional deaths at late follow-up (Fig., 29-8). In a separate report from the same authors, an analysis, of all patients with partial AVSD showed that age older than, 20 years at repair was a risk factor for death.E2 In a series of, 29 adolescents and adults (mean age 28 years) undergoing, repair of partial AVSD, early mortality was 3.4% (CL 0.6%11%), and actuarial survival after 25 years was 79%.L15 There, was important left AV valve regurgitation in 68% of the longterm survivors and important arrhythmias in 20%. In a larger, series of reoperations in 96 adults (median age 26 years) with, prior repair of partial AVSD, early mortality was 5.2% (CL, 2.9%-8.7%); however, three of the deaths occurred prior to, 1983. Since 1983, 2 of 76 patients experienced early death, (2.6%; CL 0.9%-6.1%) (Fig. 29-9).S50, Primary repair of complete AVSD in the adult is rare;, however, there are isolated case reports of such repairs.K24, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1083

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PART VI, , Congenital Heart Disease in the Adult, , (31), , 100, , (26), , 90, , (19), , (17), , 80, , (12), , Survival %, , 70, 60, 50, 40, 30, 20, 10, 0, , 0, , 5, , 10, , 15, , Years, , Figure 29-8 Survival of 31 patients aged 40 years or older, after, repair of partial atrioventricular septal defect. Numbers in parentheses represent number of traced patients at that time point. (From, , Bergin and colleagues.B18), , 100, 80, Survival (%), , 1084, , > 1983, , INDICATIONS FOR OPERATION, , 60, 1983, , 40, 20, 0, , other in 6%.S50 About half of the patients requiring reoperation for left AV valve regurgitation underwent valve repair,, and the other half underwent valve replacement., Reoperations following prior repair of complete AVSD, have been reported in a series of 50 patients.S51, As expected, the primary repair was performed early in, life (median age 1 year), and the median interval between, primary repair and reoperation was 15 months. Thus, most, reoperations were performed in young children, although, some were performed in adults as old as 38 years. Left AV, valve regurgitation was the indication for reoperation in 41, patients. There were two early deaths, both in young, patients; thus, there were no deaths in adults, although the, specific number of adults treated is not designated. In two, other series examining long-term outcomes after AVSD, repair in infancy and childhood, freedom from reoperation, was 80% at 25 years in one and 76% at 20 years in the, other.B23,H20 In both studies the majority of first reoperations, were for left AV valve problems, and these reoperations, occurred relatively early. In one of these studies, mean age, at first reoperation was 2 years.H20 Thus, few patients present, for their first left AV valve reoperation in adulthood. Second, reoperations on the left AV valve are common, with freedom, from reoperation after first reoperation of only 42% at, 15 years.H20, , P  .03, 0, , 5, , 10, Follow-up (years), , 76, 18, , 43, 13, , 20, 11, , 15, 11, 9, , Figure 29-9 Survival after reoperation for various indications in 94, patients (median age 26 years) with prior partial atrioventricular, septal defect repair, according to era of surgery. Difference is greater, than expected by chance (P = .03) when reoperation was performed, after 1983. (From Stulak and colleagues.S50), , Surgery is indicated for all unrepaired patients with partial, AVSD unless important PAH is present. One analysis suggests that outcome after surgery is better than expected, with medical management.H29 Surgery is indicated only, rarely for the adult with complete AVSD because of the, high likelihood of advanced pulmonary vascular obstructive, disease. Repaired patients with residual lesions should, undergo surgery if these cause important symptoms. If, residual lesions cause no or minimal symptoms, then standard physiologic criteria are used for residual shunts, AV, valve regurgitation or stenosis, and left ventricular outflow, tract obstruction. A maze procedure may be indicated concomitant with the structural repair if atrial fibrillation or, flutter is present. Coronary artery bypass grafting is indicated, as a concomitant procedure if standard criteria are met (see, Chapter 7)., , Left Atrioventricular Valve and Left Ventricular, Outflow Tract Lesions, Early mortality after repair of residual or recurrent left AV, valve lesions or left ventricular outflow tract lesions is similar, to that after mitral valve or left ventricular outflow tract, procedures performed in adults without AVSD. One report, describes 11 patients with prior repair of partial AVSD in, whom surgery as an adult was required.G5 Indications were, left AV valve regurgitation in six (two of whom required, valve replacement), subaortic stenosis in three, left AV, valve stenosis in one, and atrial shunt in one. There were, no early deaths (CL 0%-16%), and at median follow-up of, 7 years, there were two late deaths, one of which was cardiac, in origin., In a series of 96 reoperations in adults after partial AVSD, repair, indications for reoperation were left AV valve regurgitation in 67%, subaortic stenosis in 25%, right AV valve, regurgitation in 22%, residual atrial septal defect in 11%, and, , Patent Ductus, Arteriosus, , Section VI, , DEFINITION, Patent ductus arteriosus (PDA) is rare in the adult. It almost, always presents as newly diagnosed primary disease, but may, present as previously diagnosed primary disease with benign, physiology., , MORPHOLOGY, PDA in the adult may be complicated by calcification or, aneurysm., , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, , Congenital Heart Disease in the Adult, , CLINICAL FEATURES AND DIAGNOSTIC CRITERIA, Presentation, Small PDA is asymptomatic, causing clinically unimportant, left-to-right shunt. A continuous murmur may or may not be, detectable, depending on size of the PDA. The patient may, present with endocarditis or endarteritis., Moderate PDA results in restrictive left-to-right shunting, of variable magnitude, depending on its size. The larger the, PDA, the more likely it will cause shortness of breath, fatigue,, a wide pulse pressure, left atrial and ventricular enlargement,, and some elevation of pulmonary artery pressure. In some, cases, initial presentation is an incidental finding of ductal, calcification or aneurysm on chest radiography or other, imaging., Large PDA is nonrestrictive and produces a large left-toright shunt, pulmonary arterial hypertension (PAH), and, almost always Eisenmenger physiology. Lower body cyanosis, develops with advanced Eisenmenger physiology. Left and, right ventricular failure may be present., , A, , Diagnosis, The electrocardiogram is abnormal with large PDA, showing, left atrial enlargement and left (volume-loaded) and right, (pressure-loaded) ventricular hypertrophy. Chest radiography, varies from normal to abnormal depending on shunt size., With larger shunts, cardiomegaly from left atrial, left ventricular, and right ventricular enlargement is seen. The pulmonary trunk is prominent. Calcification of the ductus may, be detected., Echocardiography confirms the diagnosis by using color, Doppler to identify flow across the ductus. If PAH is present,, pressure gradient and flow across the ductus are small, and, echocardiography may fail to identify the PDA. Cardiac catheterization is performed in most cases of adult PDA, either, as a diagnostic tool to assess the state of the pulmonary vasculature in large PDAs, or as a therapeutic tool to close small, and some moderate PDAs. Magnetic resonance imaging or, computed tomography may be useful if the PDA is complicated by aneurysm. Using these, the specific size and position, of the aneurysm and its adjacency to other structures can be, determined. Most reported aneurysms are patent at only one, end, either aortic or pulmonary, but cases of true patency, have been reported (Fig. 29-10)., , B, , TECHNIQUE OF OPERATION, Surgical closure can be performed either via median sternotomy or left thoracotomy. This is partially surgeon preference; however, other factors may influence the choice. A prior, left thoracotomy or other left pleural space problem make a, thoracotomy approach less advisable. Additional cardiac, disease requiring surgery, such as associated ventricular septal, defect or coronary artery occlusive disease, demands a median, sternotomy approach. If cardiopulmonary bypass (CPB) is, required or likely to be required to close the PDA, median, sternotomy is preferred., If the PDA is not complicated by calcification, aneurysm,, or very short length, closure is performed using techniques, similar to those described for children. When calcification, is present, these techniques are contraindicated because, simple ligation and division carries substantial risk of rupture., , C, Figure 29-10 Aneurysm of ductus arteriosus. A, Contrastenhanced computed tomography image showing 50-mm aneurysm (arrows) of ductus arteriosus with mural thrombus and, calcification. B, Magnetic resonance image showing aneurysm, (arrows) arising from distal aortic arch. C, Angiography at catheterization also showing aneurysm (arrows) and its communication with, both aorta and pulmonary artery. (From Tofukuji and colleagues.T18), , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1085

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1086, , PART VI, , Congenital Heart Disease in the Adult, The same approach may be used for large PDA with little, or no length. Another option for this anatomy, especially, if there is no calcification of the ductus or aorta, does not, use CPB and can be performed by either median sternotomy, or left thoracotomy. The pulmonary artery and aorta at, the ductal site are clamped, the ductus is divided, and the, pulmonary artery and aorta are either sutured primarily, or patched., Aneurysm resection and repair is performed using median, sternotomy and CPB, and the technique is similar to that, used for arch aneurysm repair (see Chapter 26). Patching the, aorta or pulmonary artery may be required.T18, , Pulmonary artery, end of patent ductus, arteriosus, , A, , B, Figure 29-11 Technique of closing patent ductus arteriosus (PDA), when calcium is present. A, After cardiopulmonary bypass is initiated, pulmonary trunk is incised to the left and right pulmonary, arteries. B, An 8F Foley catheter is inserted into PDA through pulmonary trunk. Inset, Two or more pledgeted 4-0 polypropylene, mattress sutures are placed around pulmonary artery orifice of PDA., Catheter is removed before sutures are tied. Pulmonary arteriotomy, incision is closed. (From Kataoka and colleaguesK6 and Tekin and, T6, , colleagues. ), , CPB via median sternotomy, with internal patch or primary, closure of the ductal orifice through the pulmonary trunk,, is the preferred approach (see Chapter 37).F11,J7,N3 It may, be helpful to use a catheter device with a balloon, such as, a Foley catheter, to temporarily occlude the ductus after it, is exposed via the pulmonary arteriotomy and prior to definitive surgical closure. Cardioplegic arrest is not necessary, (Fig. 29-11).K6,T6,T17, , RESULTS, Early mortality for surgical PDA closure in adults is low, but, probably slightly higher than that in infants and children,, which approaches zero. This is due to the increased technical, demands of the procedure in adults. In a series of 53 adults, (mean age 24 years) reported in 1971, there was no early, mortality (CL 0%-3.5%).B21 Currently, with alternative therapeutic options, series of this size no longer exist; however, it, is reasonable to assume that mortality has decreased. In a, series of nine adults (mean age 55 years) reported in 2000,, there were no early deaths (CL 0%-19%).T17 CPB with temporary balloon occlusion was used in this series, along with, direct suture and patch closures from within the pulmonary, artery. Pulmonary artery pressure decreased from 55 mmHg, systolic prior to surgery to 35 mmHg at 6-month follow-up., In a series of 25 complex patients, many of whom had heavy, calcification, aneurysm, heart failure, or PAH, early mortality, was 4% (CL 0.7%-13%).C11 In a series of 29 patients age 50, or older at surgery, early mortality was 3.4% (CL 0.6%11%).N3 In another series of 71 adults (mean age 24 years), with relatively uncomplicated PDA, there was no early or late, mortality (CL 0%-2.6%).Y9 Many of the patients in this series, (35%) were asymptomatic; 91.5% were treated with simple, surgical ligation and 8.5% with surgical division., Premature late death after PDA closure in adults is related, to chronic changes in left ventricular function and in the, pulmonary vascular bed resulting from long-standing left-toright shunt., Outcomes after repair of ductal aneurysm are not well, documented because the lesion is so rare. There are case, reports of successful surgical management.O5,T18, , INDICATIONS FOR OPERATION, Surgery is rarely indicated for PDA in adults. Most, small and small to moderate PDAs are closed percutaneously, at cardiac catheterization with coils or other occlusive, devices.A8,B24,H7,H16,H19,I2,R6,S28,T19,Y1 Most patients with large, PDAs have Eisenmenger physiology and are not candidates, for closure., An emerging technology that can be applied to selected, cases of PDA is endovascular stent-grafting. Stents are placed, into the aorta and deployed to occlude the aortic opening of, the ductus (Fig. 29-12).M46,R19 Hybrid approaches, with, access to the aorta via surgical incision and deployment of an, endovascular device, have been described and may be useful, in selected cases.L3, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, Surgery is indicated for any PDA that causes shunt-related, symptoms, shunt-related cardiac enlargement, or PAH, or for, a PDA that cannot be closed percutaneously because of endarteritis. Typical cases include those with a large lumen and, short length, those complicated by aneurysm, and those with, other unusual anatomic features., In contrast to infants and children, adults requiring surgery, for intracardiac problems who have a coexisting PDA should, have the PDA closed percutaneously prior to the cardiac, operation., , Section VII, , Congenital Heart Disease in the Adult, , Bicuspid Aortic Valve, , DEFINITION, The definition, morphology, and basic physiology of bicuspid, aortic valve (BAV) are described in Chapter 12 and Chapter, 47. Most commonly, BAV presents in the adult as primary, congenital heart disease, either newly diagnosed or previously, diagnosed with benign physiology. It may present as secondary congenital heart disease, because some patients may have, undergone previous surgical or interventional procedures on, the aortic valve., , MORPHOLOGY, Primary disease presenting in the adult is usually an isolated, lesion, with the exception of associated aortic disease. Frequency of ascending aortic dilatation varies widely. It has, been reported to be as low as 10% to 12%B9 and as high as, 83%.D12 These variations are largely due to differences in, patient population, length of follow-up, and definition of, dilatation. Dilated aortas are at increased risk of developing, complications (Fig. 29-13).C31, BAV presenting in infants and children may be associated, with other left-sided obstructive lesions, including coarctation (which is particularly commonS38), subvalvar aortic stenosis, parachute mitral valve, and supramitral ring. When, multiple lesions occur together, the term Shone complex is, applied. Rarely, adults present with newly diagnosed Shone, complex; however, secondary presentation occurs in adulthood in essentially all survivors, because most of the cardiac, lesions are palliated and not cured during childhood intervention. BAV may be a component of William and Turner, syndromes.S38, Bicuspid aortic valve is a gross morphologic oversimplification. Two large and equally sized cusps are unusual, in, , 0.00, 0.07, 0.14, 0.20, 0.25, 0.30, 0.34, 0.37, 0.41, 0.44, 0.46, 0.49, 0.51, 0.53, , – 0.72, – 0.50, , – 0.32, – 0.19, – 0.07, , 8, 7, 6, 5, 4, 3, 2, 1, 0, , R, , A, , (MD-PD)/MD, , B, Figure 29-12 Endovascular stent-grafting for selected cases of, patent ductus arteriosus (PDA). A, Arteriography of thoracic aorta, via left brachial artery showing large PDA with aneurysmal pulmonary artery. Placing an occlusion device via this access was considered contraindicated. B, Access to thoracic aorta via right femoral, artery was obtained and a stent-graft placed distal to left subclavian, artery with PDA closure. (From Munoz and colleagues.M46), , Figure 29-13 Risk of aortic complications based on diameter of, ascending aorta. Graph shows that all patients with abnormally, increased diameters have increased risk of an aortic complication., For a given diameter, risk is higher for patients with either Marfan, syndrome or bicuspid aortic valve relative to patients who do not, manifest either of these. Blue dotted line represents patients without, bicuspid aortic valve or Marfan syndrome; red line, patients with, bicuspid aortic valve; and green line, patients with Marfan syndrome. Key: MD, Measured diameter of ascending aorta; PD, predicted normal diameter of ascending aorta; R, relative risk of aortic, complication. (From Codecasa and colleagues.C31), , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1087

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PART VI, , Congenital Heart Disease in the Adult, , contrast to the bicuspid pulmonary valve seen in tetralogy of, Fallot. Typically, BAV morphology has one large well-formed, cusp, usually making up 40% to 50% of the anular circumference, and a second cusp consisting of a fusion of two cusps, with a thick raphe representing the point of fusion. This, abnormal cusp may prolapse, causing regurgitation, or it may, calcify, particularly at the immobile raphe, leading to late, stenosis. In cases of early stenosis, there is usually associated, anular hypoplasia or variable degrees of fusion of the two, other relatively normally formed commissures., , CLINICAL FEATURES AND DIAGNOSTIC CRITERIA, Presentation, Patients with primary or secondary disease often present, with gradual stenosis and/or regurgitation that eventually, leads to symptoms or physiologic criteria for surgical, intervention.B15,R17 Less often, the primary presentation is, ascending aorta dilatation and, rarely, aortic dissection, aneurysm, or rupture. Occurrence of dissection may be tenfold, higher than in the normal population. Associated coarctation, appears to increase risk of dissection.B12,L8,N6,R14,R23,S12 Occasionally, BAV presents with signs and symptoms of infective, endocarditis., Adults with secondary disease may present with a failed, aortic valve repair, failed bioprosthetic aortic valve, failed or, outgrown mechanical aortic valve, or failed pulmonary autograft (Ross procedure) in the aortic position. Bioprosthetic, valves tend to require earlier replacement in young adults, compared with older adults. This is likely due to a combination of patient growth after original placement and more, rapid calcific degeneration. Need for replacement of mechanical valves is mostly related to patient growth, but gradual, encroachment of pannus may also play a role. After the Ross, procedure, neoaortic regurgitation necessitates reoperation, in up to 10% of patients within a decade.S13,S26,S31 Neoaortic, root dilatation occurs in about half of cases by 7 years and, may or may not be associated with neoaortic regurgitation., Risk of dissection or aneurysm formation in the dilated, neoaortic root is not clear. Coronary obstruction may also, present late after the Ross procedure because of scarring and, kinking of the translocated coronary arteries and from compression by the calcified right ventricle to pulmonary trunk, conduit. One of the most common late developments after, the Ross operation is right ventricular outflow tract conduit, failure. Freedom from conduit reoperation is better following, the Ross operation than for conduits placed for other congenital heart diseases, such as tetralogy of Fallot. Brown and, colleagues demonstrated freedom from conduit reoperation, of 96% at 10 years.B30 Raanani and colleagues report one, reoperation for conduit failure in 109 patients at a mean, follow-up of 39 months, although moderate to severe stenosis was noted in 3.8% and moderate to severe regurgitation, in 9.5%.R1, Diagnosis, Electrocardiography and chest radiography show typical findings associated with aortic valve disease. Echocardiography is, the mainstay of diagnosis. It is able to assess the degree of, stenosis or regurgitation once the diagnosis is made and can, identify when physiologic criteria are met for intervention., , Cardiac catheterization is performed when coronary assessment is indicated, primarily in patients over age 40, or if there, is concern that primary coronary insufficiency is present or, that coronary scarring or compression has developed following a Ross procedure or other aortic root replacement procedure. Magnetic resonance imaging (MRI) and computed, tomography are indicated to assess ascending aorta size and, to rule out dissection and aneurysm. Additionally, MRI can, be used to quantify aortic regurgitation if symptoms and, echocardiographic findings disagree., , NATURAL HISTORY, BAV is the most common congenital heart defect, occurring, in up to 2% of the population.S38 In many cases it is associated, with normal physiology for years or even decades. Aortic, stenosis or regurgitation may develop at any time. Dilatation, of the ascending aorta occurs, caused by connective tissue, aortopathy with a genetic basis.B8,D13,N8,S38 Many cases of neonatal and infant aortic stenosis requiring intervention have, underlying BAV. If aortic valve physiology is normal in, infancy and childhood, the typical age for surgical intervention is 60 years.F5,H15 Among adults requiring surgery for, aortic stenosis, a congenital abnormality of the valve is considered the cause in 54%.R15, In a natural history study of 642 adults with BAV (mean, age 35 years at baseline) followed for 9 years (mean), one or, more primary cardiac events, including death, surgical intervention, aortic dissection, and heart failure, occurred in 25%, of patients at a mean age of 44 years.T29 Nevertheless, fatal, events were rare, with actuarial survival comparable with that, of the general population. In another large series of adults, (mean age 32 years at baseline) with mean follow-up of 15, years, cardiac events occurred in 40% at a mean age of 52, years.M30 Again, however, actuarial survival was indistinguishable from that of the general population. The frequency of, adverse cardiovascular events in adults with BAV is stratified, based on risk profile, with risk factors including older age,, moderate or severe aortic stenosis, and moderate or severe, aortic regurgitation (Fig. 29-14).T29, , TECHNIQUE OF OPERATION, Many surgical techniques used for adults with BAV and its, associated lesions are the same as those used in children with, Primary cardiac events (%), , 1088, , 70, , >1 risk factor 65 ± 5%, , 60, 50, 40, 30, , All subjects factor 25 ± 2%, , 20, , 1 risk factor 18 ± 3%, , 10, , No risk factor 6 ± 2%, , 0, , n, , 0, 642, , 2, , 4, 6, 8, Follow-up duration (years), , 639, , 296, , 413, , 309, , 10, 198, , Figure 29-14 Frequency of adverse cardiovascular events in adults, with bicuspid aortic valves, stratified by risk profile. Risk factors, include age greater than 30 years, moderate or severe aortic stenosis, and moderate or severe aortic regurgitation. (From Siu and, colleagues.S38), , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, congenital heart disease and adults with acquired aortic valve, disease. The various techniques used for simple aortic valve, replacement, aortic root enlargement, aortic root replacement, and pulmonary autograft aortic root replacement are, described in Chapters 12 and 47., When surgical intervention is indicated for valvar disease,, options include surgical valvuloplasty (for stenosis or regurgitation, but usually not for mixed disease); simple aortic, valve replacement with a bioprosthetic valve, mechanical, valve, or pulmonary autograft (Ross procedure); Konno procedure or other forms of aortic root enlargement; aortic root, and ascending aorta replacement; and valve-sparing aortic, root replacement (see following text). No single prosthetic, type has been shown to be superior to others.S56 A Ross, procedure is controversial because pathologic abnormalities, found in the medial wall of the aorta are also found in the, wall of the pulmonary trunk, raising concern that dilatation, of the neoaortic root will be inevitable following this, procedure.S38 This controversy is not resolved.D4,O12,S7 Many, patients with Shone complex survive to adulthood and may, , A, , D, , B, , Congenital Heart Disease in the Adult, , require either primary or repeat surgery for aortic arch, obstruction and mitral valve disease in addition to left ventricular outflow tract surgery., Reduction aortoplasty, with or without external aortic, support, for ascending aorta dilatation is described and, depicted in Figs. 29-15 and 29-16, respectively. Other variations on external aortic support are described by Robicsek, and colleagues and by Cohen and colleagues.C33,R16 Some, controversy remains about whether a supportive wrap is a, beneficial accompaniment to reduction aortoplasty in patients, with BAV. Some even question the utility of reduction, aortoplasty.S27, , RESULTS, Outcomes for adults undergoing aortic valve replacement for, congenital aortic valve disease are similar to those for adults, with acquired aortic valve disease (see Chapter 12). Early and, midterm outcomes after reduction aortoplasty for dilated, ascending aorta associated with BAV, either alone or in, , C, , Figure 29-15 Repair of ascending aorta dilatation using reduction aortoplasty, without external support in patient with bicuspid aortic valve. Aortic valve typically is repaired or replaced at the same operation. Valve is addressed after, aorta is opened. After valve procedure is completed, reduction aortoplasty is, performed. Valve component of procedure is not shown in these depictions., A, Typical appearance of ascending aortic dilatation. Outer curvature of, ascending aorta is involved, and typically sinuses of Valsalva and aortic arch, are spared. Dotted line shows longitudinal incision in aorta directly over area, of dilatation. Operation is performed on cardiopulmonary bypass (CPB) with, arterial cannulation above area of dilatation or in aortic arch and single venous, cannulation of right atrium. Mild or moderate hypothermia and cardioplegic, arrest are used. B, Aortic incision is then made, and aortic tissue on each side, of incision is resected, reducing aortic diameter to normal. Shape of resected, aortic tissue approximates an oval (dotted lines). C-D, After aortic valve procedure is completed, aortic wall is closed in two layers, a continuous mattress, suture line followed by an over-and-over suture line, to achieve a normal aortic, diameter. Myocardial reperfusion, rewarming, and separation from CPB are, standard. (From Bauer and colleagues.B9), , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1089

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1090, , PART VI, , A, , E, , Congenital Heart Disease in the Adult, , B, , C, , D, , F, , association with aortic valve repair or replacement, are excellent. In a series reported by Bauer and colleagues,, 115 patients (mean age 56 years) underwent reduction, aortoplasty.B9 There were no early deaths (CL 0%-1.6%). At, a mean follow-up of 40 months, there was one cardiac death, due to myocardial infarction. In 106 of the 115 patients, no, external support of the aorta was used at the time of reduction aortoplasty; among these, 97 showed no postoperative, dilatation during follow-up. In the nine patients who showed, progressive postoperative dilatation, further review indicated, that the original reduction was inadequate, not achieving a, diameter of 35 mm or less. The authors argue that external, support is not required if the reduction is adequate. There, were no cases of postoperative dilatation and no complications in the nine patients who underwent reduction aortoplasty with external polyester graft support. There were no, reoperations in the entire series., Others have raised concerns about outcomes when reduction aortoplasty is performed without external support,, arguing that the results reported by Bauer and colleagues are, unreliable because follow-up was not long enough and citing, the two causes of aortic dilatation: the hemodynamic principle of the Law of Laplace and the intrinsic aortopathy found, in these patients.K30,P17,T26 These authors recommend external, support for all cases of reduction aortoplasty. Cohen and, colleagues report 102 adult patients (mean age 54 years) with, ascending aorta dilatation, 80% of whom also had aortic valve, disease.C33 All underwent a procedure involving external polyester mesh support of the ascending aorta, with or without, concomitant reduction aortoplasty, aortic valve surgery, or, , Figure 29-16 Repair of ascending aorta dilatation using reduction aortoplasty with external support in patient with bicuspid, aortic valve. Steps outlined in Fig. 29-15 are performed. Either, before or after separation from cardiopulmonary bypass, an, external support sleeve is fashioned from a polyester tube. Tube, graft is cut longitudinally (A and B), and two pieces are excised, from the ends of the graft (C), forming a “butterfly” shape (D)., E, Graft is then wrapped around aorta as shown so that it fits, the aortic contour. F, Graft is sutured longitudinally along anterior aspect using 4-0 polypropylene suture, making sure it fits, tightly around aorta and does not impinge on coronary ostia., Additional tacking sutures are placed circumferentially at both, proximal and distal edges of graft to prevent migration. (From, Bauer and colleagues.B9), , coronary artery surgery. There was no early mortality (CL, 0%-1.8%) and no late mortality related to aortic disease. At a, mean follow-up of 5.7 years, mean increase in aortic diameter, was 2.6 mm., Aortic root replacement with concomitant aortic valve, replacement can be performed with low early mortality., Nazer and colleagues report early mortality of 2.1% (CL, 0.9%-4.2%).N2 Diminished late survival was related to older, age at operation. Valve-sparing aortic root replacement has, been reported. In one series of 190 patients, 60 (mean age, 53 years) had BAV.A6 There was no early mortality (CL, 0%-3.1%) and no late mortality at 5-year follow-up. Function, of the spared BAVs was similar to a comparison group of, 130 patients undergoing valve-sparing root replacement, with tricuspid aortic valves. In another series of 153 patients, (mean age 51 years) with BAV, early mortality was 0.6% (CL, 0.1%-2.2%).S10 Survival was 99% at 5 years and 91% at, 10 years. At 10 years, freedom from valve replacement was, excellent (Fig. 29-17)., The Ross procedure, with concomitant ascending aorta, reduction or polyester graft replacement, was reported by, Conaglen and colleagues in 154 patients (mean age 32 years)., There was no early (CL 0%-1.2%) or late mortality and no, cardiac reoperations at a mean follow-up of 9 years.C34, , INDICATIONS FOR OPERATION, Indications for intervention in adult patients with BAV, include the standard symptoms and hemodynamic and physiologic thresholds associated with any form of aortic stenosis, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, 100, , 97%, , Congenital Heart Disease in the Adult, , 97%, , Section VIII, , 90, , Subaortic Stenosis, , 80, 70, , DEFINITION, , %, , 60, 50, 40, 30, 20, , 122 104 87 76 68 60 47 39 28 19 11 5, , 1, , 10, 0, , 0 12 24 36 48 60 72 84 96 108 120 132 144 156, Follow-up (months), , Figure 29-17 Freedom from subsequent aortic valve replacement, in 153 patients with bicuspid aortic valve after initial valve-sparing, aortic root replacement. (From Schafers and colleagues.S10), , or regurgitation (see Chapter 12). If aortic stenosis exists, without regurgitation and without a dilated ascending, aorta, percutaneous balloon valvotomy is indicated. If isolated aortic regurgitation, combined aortic stenosis and, regurgitation, or associated dilatation of the ascending aorta, exists, then surgical intervention is indicated. If balloon, valvotomy fails to relieve the gradient or causes important, regurgitation, surgical intervention is indicated., An ascending aorta diameter of 5 cm or more or a change, in aortic diameter of 0.5 cm · y−1 are absolute indications for, intervention.B25 Indications for surgery on ascending aortas, with lesser degrees of aortic dilatation are not as clear. Most, agree that an ascending aorta diameter of 3.5 to 4.9 cm, should be surgically addressed if surgery is otherwise indicated to treat aortic valve disease. Many, but not all, recommend surgery for an ascending aortic diameter of 3.5 to, 4.9 cm even if there is no indication for aortic valve disease;, some recommend observation in this situation. Valve-sparing, prosthetic aortic root replacement, composite prosthetic valve, and root replacement, Ross procedure, reduction aortoplasty, with or without external aortic support, and isolated external, support of the ascending aorta have been recommended for, surgical management of a dilated ascending aorta.B9,C27,C33, Clinical judgment comes into play when strict criteria for, intervention are not met. For example, in the young adult,, new-onset trace or mild regurgitation and an enlarging, ascending aorta that has not yet reached 5 cm in diameter, may be considered for surgical intervention. Aortic root, replacement at this point in the disease process may allow a, valve-sparing procedure. Another example is the patient with, severe aortic stenosis and an ascending aorta that is dilated,, but does not meet criteria for replacement. The best advice, is to surgically address the aorta and replace the valve., In women of childbearing age, especially those planning, pregnancy or likely to become pregnant, timing of intervention may be altered. Intervention may be considered when, milder physiologic alterations are present, anticipating the, cardiovascular demands during the third trimester of pregnancy. Additionally, choice of intervention may be altered., Mechanical valves, with the attendant requirement for, anticoagulation therapy, are poor choices for the pregnant, woman (see “Pregnancy and Contraception” under Special, Circumstances in Section I)., , The definition, morphology, and basic physiology of sub­, aortic stenosis are described in Chapter 47. In the adult it, may present as a primary disease, either newly diagnosed or, previously diagnosed with benign physiology, or as a secondary disease. In a report from 1978, 36 of 138 patients (26%), undergoing surgery for primary subaortic obstruction presented in adulthood.S55 It is likely that in the current era,, improved diagnostic imaging has resulted in a smaller percentage of patients presenting for surgery in adulthood., , MORPHOLOGY, As with subaortic stenosis in infants and children, morphology may range from a discrete fibrous membrane, to mixed, fibromuscular obstruction, to tunnel-like muscular obstruction. Accessory atrioventricular (AV) valve tissue may play a, role in the obstruction. As in children, the angle formed by, the ventricular septum and aorta (aortoseptal angle) is more, acute in adults with isolated discrete subaortic obstruction.Y3, In contrast to the case in children, there is evidence that this, angle, as well as other left ventricular outflow tract geometric, abnormalities found in patients with isolated subaortic stenosis, does not remodel postoperatively in adults.B5, , CLINICAL FEATURES AND DIAGNOSTIC CRITERIA, Presentation, Subaortic stenosis presents in a variety of ways in adults. It, may be a primary and isolated lesion with the typical signs, and symptoms of aortic stenosis, sometimes with associated, aortic valve regurgitation. In one study, aortic regurgitation, was present in 80% of patients, but was hemodynamically, important in only 20%.O8 Subaortic stenosis may also be a, primary lesion associated with ventricular septal defect (VSD),, atrioventricular septal defect (AVSD), or a conotruncal, anomaly with subaortic conus. About half of all primary cases, are isolated, and half are associated with other cardiac, anomalies.O8, Subaortic stenosis may also be a secondary lesion that, develops after surgical repair of a spectrum of anomalies,, including left ventricular outflow tract obstruction, perimembranous VSD, posterior malalignment VSD with arch obstruction, AVSD, and conotruncal anomalies such as double outlet, right ventricle or certain types of transposition of the great, arteries.C29 Signs and symptoms at presentation are similar to, those of valvar aortic stenosis., Diagnosis, Electrocardiography and chest radiography show typical, findings associated with aortic stenosis (and aortic regurgitation if present). Echocardiography will demonstrate the morphologic characteristics of the subaortic region and proximity, of the subaortic lesion to the aortic valve, assess aortic regurgitation, estimate the pressure gradient, and demonstrate, left ventricular function. As with most complex intracardiac, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1091

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PART VI, , Congenital Heart Disease in the Adult, , lesions in adults, transesophageal echocardiography may add, important details to the surface echocardiogram. Cardiac, catheterization plays a limited role in subaortic stenosis, primarily to assess the coronary arteries if necessary. There is no, role for therapeutic catheterization. In complex cases, magnetic resonance imaging may provide a more detailed estimation of the left ventricular outflow tract., , NATURAL HISTORY, Subaortic stenosis is relatively uncommon among adults with, congenital heart disease, accounting for 6.5% (134 of 2057, patients) of presenting cases in one series.O8 In this series,, 22% (29/134) presented with severe obstruction and no, prior surgical history, 48% (64/134) had no indication for, surgery and no prior surgical history, and 30% (41/134) had, surgery for subaortic obstruction during childhood., Subaortic stenosis is a progressive lesion that creates, greater degrees of obstruction over time and causes progressive aortic valve damage, which leads to aortic regurgitation., In adults, progression of obstruction and aortic regurgitation, is slower than in children.S47 It is not unusual for an adult to, be followed with known uncomplicated mild subaortic stenosis for a period of time, only to develop more progressive, stenosis, aortic regurgitation, or infective endocarditis. Aortic, regurgitation is more likely the higher the pressure gradient, in the subaortic region.G10,M24 Severe obstruction carries the, same risks as severe valvar aortic stenosis., , Survival, , Freedom (%), , 1092, , 100, 90, 80, 70, 60, 50, 40, 30, 20, 10, 0, , 0, , 5, , 10, , 15, , 20, , 25, , Years after surgery, , Figure 29-18 Survival in 52 adult patients following resection of, subaortic obstruction. (From Stassano and colleagues.S47), , Table 29-9, , Associated Lesions and Correction Procedures, , Anomaly, , No., , Procedures, , Ostium secundum, , 5, , ASD closure, , Ostium primum, , 1, , ASD closure, , 14, , VSD closure, , ASD:, , VSD, Bicuspid aortic valve, , 9, , Commissurotomy or AVR, , Valvular AS with normal annulus, , 4, , Commissurotomy, , Supravalvular AS, , 2, , Repair with patch, , TECHNIQUE OF OPERATION, , Coarctation, , 7, , Patchplasty, , The appropriate operation for subaortic obstruction depends, on the morphology of the obstruction—membranous, fibromuscular, or tunnel-like—and presence and severity of, aortic regurgitation. Procedures include membrane resection,, myectomy, aortic valve repair, aortic valve replacement, and, Konno or modified Konno procedures.E5 Operations and, principles determining their application are described in, Chapter 47, those for aortic valve replacement in Chapter, 12, and those for aortic valve regurgitation in Chapter 35, (see Section II, Ventricular Septal Defect and Aortic, Regurgitation)., , PDA, , 4, , Division or ligation, , Infundibular stenosis, , 1, , Resection of RVOT, , Valvular PS, , 2, , Commissurotomy, , RESULTS, Early mortality in the current era is 3% to 4%, with late survival substantially lower than that of the general population, and a relatively high rate of reoperation. In a 1976 report of, 36 adults undergoing surgery for subaortic stenosis, only 53%, had an isolated anomaly and 25% presented with infectious, endocarditis, reflecting the complex nature of the patients in, this study.S55 Early mortality was 8.3% (CL 3.7%-16%)., In a more contemporary series of 52 patients (mean age, 25 years) reported in 2005, early mortality was 3.8% (CL, 1.3%-8.8%).S47 Concomitant aortic valve replacement was, required in 29% because of chronic aortic regurgitation, graded as 3+ or 4+. The mean age of patients requiring valve, replacement was older than those not requiring replacement, (37 vs. 21 years). Late mortality was 16% (Fig. 29-18)., Recurrent obstruction requiring another operation occurred, in five patients over a mean follow-up period of 17 years., In another series of 88 patients (mean age 20 years), reported in 2005, 66% (58/88) had discrete obstruction and, , RVOT stenosis:, , From Erentug and colleagues.E5, AS, Aortic stenosis; ASD, atrial septal defect; AVR, aortic valve replacement;, PDA, patent ductus arteriosus; PS, pulmonary stenosis; RVOT, right ventricular, outflow tract; VSD, ventricular septal defect., , 34% (30/88) had diffuse tunnel-like obstruction.E5 Moderate, or worse aortic regurgitation requiring a concomitant aortic, valve procedure was present in 15%. Aortic valve hypoplasia, requiring a Konno procedure was present in 17%. Associated, cardiac anomalies requiring concomitant surgery were present, in 45% (Table 29-9). Early mortality was 3.4% (CL 1.5%6.7%) and late mortality 1.1% at a mean follow-up of 6.1, years. Diffuse tunnel-like obstruction was a risk factor for, early mortality by multivariable analysis. Reoperation for progressive aortic regurgitation or recurrent left ventricular, outflow tract obstruction was required in 16.5% of patients, over the follow-up period (Fig. 29-19)., , INDICATIONS FOR OPERATION, Surgical resection of subaortic stenosis is indicated in, several circumstances. The first relates to classic obstructive, physiology: peak echocardiographic gradient of 50 mmHg or, greater or a lesser gradient associated with left ventricular, hypertrophy and strain or documented symptoms. The, second relates to status of the aortic valve: presence of subaortic stenosis, regardless of gradient, is an indication for, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. 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Chapter 29, , artery hypoplasia with multiple peripheral stenoses. The, intima, media, and adventitia of the coronary arteries may all, be involved with fibrotic and dysplastic changes.M12,V8,Y7, , Actuarial freedom from reoperation (%), , 1.0, , .9, , CLINICAL FEATURES AND DIAGNOSTIC CRITERIA, Presentation, , .8, , .7, , .6, , 0, , 2, , 4, , 6, , 8, , 10, , 12, , 14, , 16, , Years, , Figure 29-19 Freedom from reoperation following initial operation for subaortic obstruction in 88 adult patients. Indication for, reoperation was either recurrent obstruction or progressive aortic, regurgitation. (From Erentug and colleagues.E5), , surgery if new-onset aortic regurgitation develops or more, than mild regurgitation is present. Mild subaortic stenosis in, the absence of aortic regurgitation, particularly if the membrane is in contact with or in close proximity to the aortic, valve, is considered an indication for resection by some., Aortic valve repair is indicated at the time of resection if aortic, regurgitation is more than mild. Aortic valve replacement, is indicated for moderate or severe regurgitation if repair is, not possible., , Section IX, , Supravalvar Aortic, Stenosis, , DEFINITION, Definition, morphology, and basic physiology of supravalvar, aortic stenosis are described in Chapter 47. The adult with, supravalvar aortic stenosis most commonly presents with secondary congenital heart disease, having undergone surgery, for the disease in childhood. If mild disease is present, the, adult may present with newly diagnosed disease or previously, diagnosed disease with benign physiology., , MORPHOLOGY, Supravalvar aortic stenosis comes in three forms:, ■, ■, ■, , Congenital Heart Disease in the Adult, , Associated with William syndrome, Familial type, Sporadic type, , All forms have potential for diffuse pathology throughout the, systemic and pulmonary vascular tree, including the signature, discrete narrowing and thickening of the aorta just above the, sinutubular junction, variable hypoplasia of the ascending, aorta and even descending aorta, renal artery hypoplasia, coronary artery obstruction and dysplasia, and branch pulmonary, , Because the pathologic processes in the arterial wall are, diffuse and progressive, disease that was asymptomatic in, childhood may lead to systemic hypertension (renal arteries, and diffuse aortic hypoplasia), late-onset discrete systemic, outflow obstruction (progression of supravalvar aortic stenosis), pulmonary hypertension (peripheral branch pulmonary, artery stenosis and hypoplasia), or cardiac ischemia (coronary, artery ostial obstruction, sinus of Valsalva inflow obstruction, from progression of the supravalvar obstructive process, or, coronary artery aneurysm or dissection).D22,M12,V8,Y7 These, modes of presentation can occur in the adult whether or not, the patient underwent surgery in childhood to address supravalvar aortic stenosis., Diagnosis, The electrocardiogram will show varying degrees of left ventricular hypertrophy, reflecting the degree of left-sided, outflow obstruction. If there is coronary artery involvement,, there may be signs of ischemia. The chest radiograph may also, show signs of left ventricular hypertrophy. Surface echocardiography reliably demonstrates the abnormal supravalvar, aortic morphology, but cannot completely define the coronary artery, pulmonary artery, or diffuse changes in the, remainder of the aorta and its branches. Transesophageal echocardiography may further define the intracardiac and proximal, aortic morphology, but has the same limitations as surface, echocardiography with respect to more peripheral artery and, coronary artery problems. Both magnetic resonance imaging, and computed tomography (CT) are useful in defining the, ascending and descending aorta and its major branches and, the peripheral branch pulmonary arteries. Although CT can, define some coronary artery abnormalities, cardiac catheterization and angiography are indicated for precise definition., Obstruction to coronary arterial flow may be caused by progression of the supravalvar stenosis leading to inhibition of, blood entering the sinus of Valsalva, or from intrinsic coronary artery ostial obstruction from intimal thickening. In the, most severe cases the sinus of Valsalva can be totally occluded, as the free edge of the valve cusp fuses to the overhanging, fibrous ridge of the discrete supravalvar ring., , NATURAL HISTORY, Supravalvar aortic stenosis is typically diagnosed and treated, in childhood. In the William syndrome form, it is rare for the, individual to reach adulthood undiagnosed, even if the cardiovascular manifestations are mild, because the distinctive, associated facial and neurodevelopmental abnormalities make, the diagnosis obvious., , TECHNIQUE OF OPERATION, Standard techniques used for aortic reconstruction in children are also used in unrepaired adults, including various, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1093

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1094, , PART VI, , Congenital Heart Disease in the Adult, , Type I, , Type II, , Ostial narrowing, , Type III, , Cusp-ridge fusion, , Fusiform narrowing, , Figure 29-20 Classification of left main coronary artery obstruction in patients with supravalvar aortic stenosis. Type I primarily involves, intimal thickening at the ostium, as thickened supravalvar ridge pathology extends to include the coronary sinus. Type II is caused by fusion, of left coronary cusp edge with supravalvar ridge, preventing blood from entering the coronary sinus from aortic lumen. Type III involves, diffuse narrowing of left main coronary artery, which may extend into left anterior descending or circumflex coronary branches. (From Thistle-, , thwaite and colleagues.T14), , Ostial, stenosis, , Figure 29-21 Repair of supravalvar aortic, stenosis in the adult. A, Supravalvar aortic stenosis and left main coronary artery ostial stenosis. Ostium is drawn into supravalvar ridge, and becomes involved with intimal thickening, and cusp edge thickening. B, Incisions into, right coronary, noncoronary, and left coronary, sinuses. Left sinus incision is extended into, left main coronary artery. C, Ostial and left, sinus patchplasty and noncoronary and right, coronary sinus patchplasty are performed., Polyester may be used in the noncoronary and, right coronary sinuses; however, autologous, glutaraldehyde-treated pericardium is recommended for the left coronary sinus and coronary artery patch. (From Inan and colleagues.I1), , A, , B, Dacron, patchplasty, , Pericardial, patchplasty, , C, , forms of patch aortoplasty and sliding aortoplasty (see, Chapter 47). Coronary obstruction can be managed in, several ways, depending on the etiology (Fig. 29-20). Resection of the supravalvar ridge effectively relieves obstruction, to sinus inflow. If ostial stenosis is present, direct ostial reconstructionT14 or coronary artery bypass grafting (see Chapter, 7) can be performed. Direct ostial reconstruction is preferred, in children, especially very young ones; it also remains an, option in the adult (Fig. 29-21). If long-segment proximal, or distal coronary artery stenosis is present, bypass grafting is, the appropriate procedure.T14, Valvar and subvalvar abnormalities, including anular, hypoplasia, bicuspid aortic valve, and subvalvar obstruction,, can coexist with supravalvar aortic stenosis. These problems, are managed surgically as described in Sections VII and, VIII. Other vascular procedures may be indicated, including, reconstruction of stenotic or hypoplastic ascending aorta,, , arch, head and neck arteries, and branch pulmonary, arteries., , RESULTS, Evidence-based outcome estimates for outcomes of supravalvar aortic stenosis repair in adults are based on case, reports and the few adult patients included in larger pediatric series.C36,S45,V6 Early mortality after repair of supravalvar, aortic stenosis in adults is probably in the range of 2% to, 5%. Mortality should be no higher than for similar operations in children unless important complicating factors are, present.V6 At least one large series, mostly involving children, (median age 7 years), reports an early mortality of 9%;, however, most of the deaths occurred in the 1950s and, 1960s or involved patients brought to the operating room, in extremis.S45, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, It is probably true that adult patients with supravalvar, aortic stenosis will demonstrate sequelae not usually seen in, children, primarily related to the coronary arteries. Inan and, colleagues report a case of a 21-year-old man with associated, left main coronary artery stenosis caused by cusp fusion and, thickening and intimal thickening of the coronary ostium.I1, Surgical repair was uncomplicated. Yilmaz and colleagues, report two cases, age 20 and 21 years, of associated coronary, artery aneurysm. Both patients underwent repair of the, supravalvar aortic stenosis without surgically addressing the, aneurysms. Postoperatively, anticoagulation therapy was, instituted. Both patients were doing well at midterm, follow-up.Y7 Thistlethwaite and colleagues report on a, 32-year-old patient with long-segment left main coronary, artery narrowing successfully managed with supravalvar aortic, repair and concomitant saphenous vein coronary artery bypass, grafting.T14, As in children, risk factors for late survival and reoperation, are likely to be diffuse supravalvar aortic hypoplasia and concomitant aortic valve disease.S45,V6, , INDICATIONS FOR OPERATION, , Congenital Heart Disease in the Adult, , be present in patients with coarctation, leading to intracranial, bleeding and stroke., A spectrum of associated left-sided cardiac structural, anomalies can occur with coarctation, most commonly bicuspid aortic valve, but also other anomalies that, with coarctation, make up Shone complex. These are supramitral ring,, parachute mitral valve, and subaortic membrane. All may be, part of the presentation of either recurrent or residual coarctation, or well-repaired coarctation in the adult. With the, exception of bicuspid aortic valve, these associated anomalies, are much less likely to be part of the presentation of newly, diagnosed coarctation in the adult, because most patients, with multiple associated anomalies will present much earlier., These associated left-sided obstructive problems are not, discussed further in this section. Aortic valve and subvalvar, aortic obstruction are discussed in Sections VII and VIII of, this chapter., , CLINICAL FEATURES AND DIAGNOSTIC CRITERIA, Presentation, , The definitions, morphology, and basic physiology of coarctation of the aorta and interrupted aortic arch are described, in Chapter 48. Problems related to aortic coarctation are, relatively common among adults with congenital heart, disease. Often these problems relate to secondary disease,, with the patient having undergone a surgical or interventional, procedure as an infant or child. Patients can, however, present, with native disease in adulthood.B7,B20,B26,J3,W20, , The adult with unrepaired coarctation typically presents with, systemic hypertension. This may be accompanied by symptoms such as headache and lower extremity weakness, especially with exercise. On further evaluation a differential, between upper body and lower body blood pressure is, commonly noted; however, long-standing obstruction leads, to collateral development, which can blunt or even eliminate, the pressure differential. A lower body pulse delay remains in, all cases., Interrupted aortic arch rarely presents in adulthood as, primary disease; rather, the majority of patients present critically ill as neonates and either undergo surgical repair or die., Adults with secondary disease following prior repair of interrupted aortic arch will present with signs and symptoms, similar to those of patients with prior coarctation repair., There will be, however, a higher prevalence of late aortic valve, and subaortic problems., Patients with previously repaired coarctation or interrupted aortic arch most commonly present with residual or, recurrent coarctation or hypertension, but may present with, any of the signs and symptoms related to aortic, coronary, or, cerebral vascular disease, or associated cardiac structural, anomalies. Aortic aneurysm at the repair site is particularly, likely in patients with prior patch aortoplasty repair (Fig., 29-22).K19 Even if there is no recurrent obstruction, adults, with a history of coarctation repair in childhood have reduced, exercise capacity compared with normal individuals, particularly if systemic hypertension is present or repair was performed at an older age.T20, , MORPHOLOGY, , Diagnosis, , Many patients with a history of previous coarctation repair, have some form of residual disease as adults, and lifelong, follow-up is required after repair at any age. Residual disease, may take many forms. There may be obstruction at the coarctation repair site or, particularly if the repair technique, involves a prosthetic patch, aneurysm at the repair site. Diffuse, aortopathy is recognized as part of the coarctation disease, process, and this can contribute to chronic hypertension, dissection, aneurysm, and rupture. Intracranial aneurysm may, , In unrepaired coarctation, the electrocardiogram will show, left ventricular hypertrophy. Chest radiography may show cardiomegaly from left ventricular hypertrophy, aortic silhouette, irregularities such as the reverse-3 sign of unrepaired coarctation or dilatation and ectasia associated with prior repair, and, rib notching from intercostal arterial collaterals. Echocardiographic imaging of the thoracic aorta is difficult in the adult,, although color Doppler may show an obstructive pattern at, the coarctation site, flow in intercostal collaterals, and blunted, , Surgery is the only therapeutic option for supravalvar aortic, stenosis; there are no percutaneous techniques applicable to, this lesion. The hemodynamic indication for surgery is a, 50-mmHg mean echocardiographic gradient. If symptoms, are present, there is left ventricular hypertrophy or failure, or, increased cardiac demand is expected (e.g., an active lifestyle,, anticipation of pregnancy), surgery is indicated for less severe, resting obstruction. Evidence of myocardial ischemia with, obstruction to coronary flow is an indication for surgery, regardless of aortic gradient., , Section X, , Aortic Arch Obstructive, Problems, , DEFINITION, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1095

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PART VI, , Congenital Heart Disease in the Adult, 38.7, , 40, , Percentage (%), , 1096, , A, , 30, , 26.8, , 20, 15.1, , 10, 1.3, , 0, , 1, , 5, , 0.3, , 2.1, , 10, Years, , 4.2, , 15, , B, 20, , Figure 29-22 Cumulative incidence of descending thoracic aortic, aneurysms after native coarctation repair by (A) patch aortoplasty, (n = 494) or (B) other methods (n = 397). (From Knyshov and, , colleagues.K19), , A, , C, , pulsation distal to the coarctation. Echocardiography is essential for documenting associated intracardiac structural anomalies and myocardial function. Magnetic resonance imaging, (MRI) and computed tomography (CT) are the preferred, methods for precisely defining the morphologic details of, both primary coarctation and previously repaired coarctation, or interrupted aortic arch in the adult, particularly when, three-dimensional reconstruction is obtained (Figs. 29-23, and 29-24).H2,T25, Accurate measurement of reduction in luminal diameter, at the primary coarctation or repair site is an important factor, used in management decisions. MRI can be used to calculate, the amount of collateral flow present by subtracting flow in, the aorta just distal to the primary coarctation or repair site, from flow in the aorta at the diaphragm. It can also assess, functional elastic properties of the aortic wall.H1 These data, may be particularly helpful in cases of both primary and, , B, , D, , Figure 29-23 Computed tomography images of a 36-year-old woman with severe native coarctation of the aorta. Sagittal multiplanar, reformatted (A) and left lateral volume-rendered (B) images show severe aortic narrowing (white arrows) below left subclavian artery., Enlarged internal thoracic arteries and dilated posterior collateral intercostal arteries connecting to the postcoarctation descending thoracic, aorta are seen. C, Anterior volume-rendered image shows enlarged internal thoracic arteries (black arrows) and dilated superior thoracic, and thoracoacromial arteries (white arrows). D, Coronal maximum-intensity projection image shows dilated posterior collateral intercostal, arteries causing rib notching. (From Turkvatan and colleagues.T25), , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, ■, , ■, , ■, , Figure 29-24 Oblique sagittal plane image through aortic arch, obtained by magnetic resonance. Aorta of a 21-year-old patient, with coarctation of the aorta after undergoing polyester patch aortoplasty at age 21 years. Depicted are vessel wall abnormalities at, the isthmus level (arrow). (From Hager and colleagues.H2), , recurrent coarctation when there is a smaller gradient than, expected across the coarctation site and reduction in luminal, diameter is equivocal., MRI of the head is indicated in all adults to rule out, intracranial aneurysm. Diagnostic cardiac catheterization is, indicated primarily to assess the coronary arteries; however,, in cases with questionable criteria for intervention, a catheter, pullback peak-to-peak gradient across the coarctation may, provide definitive information. Therapeutic catheterization, may be indicated., , NATURAL HISTORY, If coarctation is unrecognized for many years, premature, coronary artery obstructive disease, cerebrovascular disease,, and aortic disease may lead to myocardial infarction, heart, failure, stroke, intracranial hemorrhage, infective endarteritis,, or aortic dissection or rupture. Aortic complications are more, likely with advanced age and with presence of a bicuspid, aortic valve.O7 Life expectancy is approximately half of normal, in patients with unrepaired coarctation. Survival at 30-year, follow-up after surgical repair ranges from 72% to 82%.W15, , TECHNIQUE OF OPERATION, There are many surgical options for addressing both native, and recurrent coarctation in the adult. The actual techniques, are no different from those used for coarctation in infants and, children, which are described in detail in Chapter 48. Decision making used in choosing among these, however, is very, different from that used in infants and children. The difference is based on several factors:, ■, , Somatic growth does not have to be considered in the, adult., , Congenital Heart Disease in the Adult, , Adults have much less elasticity in their aortic tissue than, children., Adults are much more likely to have had one or more, prior operations for coarctation., Adults are much less likely to have intracardiac anomalies, requiring surgery concomitant with coarctation repair., , Thus, for native coarctation in the adult, it is more likely, that an interposition graft will be required rather than resection and primary anastomosis, especially if there is any length, to the coarctation or if proximal arch hypoplasia is present., For native coarctation, the surgical approach will usually be, by left thoracotomy in adults, because intracardiac anomalies, are relatively uncommon. Use of various forms of extended, resection and primary anastomosis are essentially never an, option in the adult because lack of elasticity in the adult aorta, precludes the degree of mobilization required. Resection and, primary anastomosis is reserved for discrete coarctation with, no proximal arch hypoplasia. If coronary artery or aortic valve, disease is present and requires surgical treatment, median, sternotomy is used.M41,T15, For recurrent coarctation, the surgeon must consider, whether previous repairs were performed by sternotomy or, left thoracotomy. If by sternotomy, and the recurrent, obstruction is in the distal arch, then left thoracotomy may, be the best approach. Resection and primary anastomosis is, rarely possible in this setting or in any recurrent setting in, the adult; most often, either patch repair or interposition, grafting will be performed. If the previous operation was by, left thoracotomy, a median sternotomy with cardiopulmonary bypass (CPB) may be the best approach, especially if, the recurrent obstruction is in the proximal arch. If the, obstruction is distal in the arch and relatively simple anatomically, and there has been only one previous thoracotomy, a repeat thoracotomy may be considered. CPB can, also be considered when a thoracotomy is chosen, either for, native or recurrent coarctation, if the repair appears to be, complex (anticipated long aortic clamp time) and there are, few or no collaterals (Fig. 29-25).B1 Deep hypothermic circulatory arrest has been described for complex coarctation, repair via thoracotomy.G22 As the number of previous thoracotomies and anatomic complexity of the recurrent, obstruction increase, the more attractive becomes the option, of performing the repair using one of the many extraanatomic graft reconstruction techniques.A10,A16,C8,D1,M13,S14,W7, Two of these techniques, one using median sternotomy and, one left thoracotomy, are shown in Figs. 29-26 and 29-27., Right thoracotomy can also be used., Therapeutic catheter-based intervention for recurrent, obstruction is preferred in many institutions. Endovascular, approaches, including balloon aortoplasty and covered stenting, may be used.C18,W3 In selected complex patients with, previous surgery or with important comorbidities, hybrid, approaches may be applicable (Fig. 29-28).C5,C6, , RESULTS, Early mortality after surgical repair of native coarctation, in the adult is less than 1%, and hypertension is reliably, improved and even normalized in many cases, with reduced, requirement for antihypertensive medication.V16 Jatene and, colleagues report on 50 adults (mean age 25 years) who, underwent surgical repair of native coarctation via left, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1097

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1098, , PART VI, , Congenital Heart Disease in the Adult, , Venous, cannula, , Kirklin fence, , LA appendage, , Phrenic, nerve, Pericardium, , Lung, Left vagus, nerve, Recurrent, laryngeal, nerve, , Lung, , PDA, Coarctation, , Aortic cannula, , A, , B, To pump, , Ligamentum, divided, , C, , From, pump, , Figure 29-25 Repair of coarctation of the aorta in adults., A, Through a posterolateral thoracotomy, lung has been retracted, anteriorly with a “Kirklin fence.” Aortic cannula is shown in position in the descending thoracic aorta distal to site for distal aortic, vascular clamp. Coarctation site and aortic arch have been dissected. “Patent ductus arteriosus” (PDA) represents either ductus, or ligamentum arteriosum, which may or may not be present., B, Lung has now been retracted posteriorly (temporarily) and, pericardium opened posterior to phrenic nerve. Venous cannula, has been inserted into left atrial (LA) appendage. C, Patient is, placed on partial cardiopulmonary bypass. Venous drainage must, be carefully controlled by perfusionist such that left atrium is not, drained completely, allowing enough left ventricular filling so that, upper body perfusion is maintained by left ventricular ejection., Proximal and distal vascular clamps have been applied. Ligamentum, if present, is ligated and divided. Dotted lines indicate extent, of coarctation resection. In this case, two intercostal collateral, vessels have been ligated and divided., , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, , Congenital Heart Disease in the Adult, , To pump, , Recurrent, laryngeal, nerve, , D, , From, pump, , Hemashield, interposition graft, , Left vagus, nerve, , E, , Figure 29-25, cont’d D, Coarctation specimen has been excised. A preclotted polyester interposition graft is sutured in place. E, Completed, interposition graft. Cannulation sutures have been tied. Pleura is closed over interposition graft if it is the initial operation. (From Backer and, colleagues.B1), , thoracotomy.J3 Procedures used included resection with, anastomosis in 40%, patch augmentation in 44%, and interposition graft in 16%. There was no early mortality (CL, 0%-3.7%). At a mean follow-up of 46 months, there was one, late death due to endocarditis. Blood pressure was normal in, more than 90%, with 75% free of antihypertensive medication, (Fig. 29-29). The gradient was reduced from a mean of, 61 mmHg preoperatively to a mean of 19 mmHg and was, independent of the surgical technique used. Similarly,, Bouchart and colleagues report on 35 adults (mean age 28, years) with native coarctation.B26 Resection with anastomosis, and interposition graft were the dominant techniques used., Mean follow-up was 165 months. There was no mortality, (CL 0%-5.3%) and no reoperation for recoarctation over the, course of follow-up. At follow-up, 66% (23/35) were normotensive without medication; however, 35% (8/23) had, a hypertensive response to exercise testing at 6-month, follow-up. Six patients underwent subsequent surgery for, aortic valve disease. Wells and colleagues report on 26 adults, (mean age 32 years) undergoing surgical repair of native, coarctation, with a mean follow-up of 2.3 years.W20 There was, no mortality (CL 0%-7.0%). At rest, 88% were normotensive,, but most remained on medication. Hashemzadeh and colleagues report on 38 adults (mean age 26 years) undergoing, surgical repair using the techniques of resection with anastomosis, patch repair, and graft interposition, with a mean, follow-up of 37 months.H9 Results were similar to the previously cited studies, with no mortality (CL 0%-4.9%) and relief, of resting hypertension in the majority of patients. Bhat and, colleagues report on 84 adults (mean age 29 years) undergoing surgical repair of native coarctation, with a mean follow-up, of 5.2 years.B20 There was 1 early death (1.2%; CL 0.2%-4.0%), and no deaths at follow-up. All survivors experienced significant regression of hypertension, with 42% off medication, and, , persistent hypertension was observed in 31%. Bauer and colleagues report 15 patients over the age of 50 years undergoing surgical repair of native aortic coarctation, with a mean, follow-up of 4 years.B7 There was no mortality (CL 0%-21%)., Two minor strokes occurred perioperatively with full resolution, and there was one case of endocarditis. Hypertension, was relieved in 80% (12/15), but 73% (8/11) of evaluated, patients who were normotensive at rest had a hypertensive, response to exercise., Complications after repair of native coarctation in adults, are similar to coarctation repair in children, including blood, loss, phrenic nerve injury, recurrent nerve injury, hypertension, residual coarctation, pseudoaneurysm, and paraplegia., Interventional procedures for native coarctation in the, adult, either balloon angioplasty or stenting, provide similar, gradient relief and hypertension control as surgery.M1 Earlier, reports of aneurysm formation and intimal tears following, balloon angioplasty in up to 13% of patients may be reduced, as experience increases and technology advances to include, stents and covered stents.C18,W3 Reports as recent as 2008,, however, show aneurysm formation in 7.5%.F2 Several deaths, have also occurred using interventional techniques.M44 Longterm sequelae of endoluminal prostheses remain an important, unanswered question, and this concern is the major objection, to their use in native coarctation. It is also the major argument for surgical management of native coarctation, especially when end-to-end anastomosis can be achieved, thereby, avoiding prosthetic material., Early mortality for surgical repair of recurrent coarctation, varies from 1% to 14%, depending on complexity of, the surgery and presence of comorbidities.K19 There are, reports of no surgical mortality.B16,S14 Early mortality, using extra-anatomic bypass techniques varies from 0% to, 4.6%.A10,A16,C8,D1,M13,S14,W7 Late outcome, ranging up to 22, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1099

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1100, , PART VI, , Inferior, vena cava, , Congenital Heart Disease in the Adult, , Diaphragm, , Figure 29-26 Extra-anatomic graft placed via median sternotomy, using cardiopulmonary bypass (CPB). Pericardium is opened. CPB, is established using aortic and right atrial cannulation. After cooling, to moderate hypothermia, ascending aorta is clamped and antegrade cardioplegia is infused. Heart is then retracted cephalad and, to patient’s right. Posterior pericardium is incised longitudinally, directly over descending thoracic aorta. Aorta is dissected to allow, placing a partially occluding vascular clamp. Unnecessary dissection, in the region of the esophagus is avoided, and an end-to-side graft, to aorta anastomosis is constructed using continuous 4-0 polypropylene suture. Graft is allowed to fill retrograde, and a clamp is, placed on its proximal portion. Ascending aortic clamp is removed,, reestablishing perfusion to the heart. Graft is routed anterior to, inferior vena cava and led around right atrium and anastomosed to, right lateral aspect of ascending aorta using a side-biting clamp., Patient is separated from CPB in standard fashion. This procedure, can also be performed without CPB, using essentially the same, exposure.S14 This approach may be applicable if no additional intracardiac procedures are required. Cardiac stability must be maintained during the posterior pericardial dissection and distal, anastomosis. (From Wang and colleagues.W7), , years in these studies, is excellent, with no reported graftrelated complications or reoperations., Endovascular approaches, including balloon aortoplasty,, stenting, and covered stenting, are as effective as surgery in, relieving the gradient in adults with recurrent coarctation., These approaches avoid much of the morbidity associated, with reoperative surgery and complex reconstruction;, however, they are themselves complex procedures. Morbidity, includes vascular injury at the site of percutaneous puncture,, residual and recurrent coarctation, hypertension, aneurysm, formation at the coarctation site, aortic leaks, and vascular, compromise. In a series of 11 cases (mean age 47 years), of recurrent coarctation treated by stents and endografts,, there was no early mortality and no paraplegia (CL 0%-16%)., , Figure 29-27 Extra-anatomic repair of recurrent coarctation via, left thoracotomy without cardiopulmonary bypass. A left posterolateral thoracotomy through the fourth intercostal space is performed. Adhesions between chest wall and lung are dissected to, mobilize the lung and expose the descending thoracic aorta. Pericardium is incised ventral to phrenic nerve, with great care taken, to avoid damaging it. Ascending aorta is dissected from pulmonary, artery. Systemic anticoagulation (1 mg heparin · kg−1) is established., A partial exclusion clamp is placed on ascending aorta under continuous blood pressure control of right radial and femoral artery. A, preclotted polyester graft is anastomosed end to side using a, running 4-0 polypropylene suture. Aortic arch and tissue in recoarctation area are left untouched. Graft is placed anatomically parallel, to native aortic arch. A partial exclusion clamp is placed on descending aorta, maintaining a mean femoral artery pressure of at least, 60 mmHg. This anastomosis is performed end to side again with a, running 4-0 polypropylene suture. After clamp is released, heparin, is reversed with protamine. One pleural tube and one intrapericardial tube are inserted for drainage and the chest closed. (From, , Daebritz and colleagues.D1), , Surgical reconstruction of the left subclavian artery was necessary in two patients because of coverage of the origin of the, artery and arm ischemia. At a mean follow-up of 12 months,, three cases of leaks around the endograft were documented,, one as late as 2 years after implantation.K25 Long-term clinical, effectiveness has not been demonstrated., , INDICATIONS FOR OPERATION, Intervention may be surgery or percutaneous catheterbased therapy. Indications for intervention include a gradient, of 20 mmHg or more (documented by catheterization, in equivocal cases) across the coarctation and a luminal narrowing of 50% or more (by MRI, CT, or catheter-based, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, , Congenital Heart Disease in the Adult, , aortography) at the coarctation site. If the gradient is less, than 20 mmHg and luminal narrowing is less than 50%, MRI, documentation of important collateral flow is an indication, for intervention., Some institutions consider percutaneous catheter-based, balloon dilatation and stenting as an option for adult native, coarctations that meet specific morphologic criteria, but, others consider surgery to be the procedure of choice for all, native coarctations, reserving percutaneous therapy for recurrent coarctation. Most institutions with experienced interventional cardiology teams consider percutaneous catheter-based, therapy as the procedure of choice for recurrent coarctations, that meet these specific morphologic criteria:, ■, ■, ■, , ■, , ■, , Figure 29-28 Hybrid arch reconstruction for complex aortic arch, disease. This example shows an aneurysm of arch. Complex arch, obstruction with involvement of head and neck arteries can also be, managed with this technique. Surgical reconstruction of head and, neck vessels is performed first via median sternotomy; cardiopulmonary bypass is not used. Either bifurcated or simple polyester grafts, are used to reconstruct head and neck vessels as shown. Head and, neck vessels are transected and oversewn at their aortic origin with, a running polypropylene suture and anastomosed to corresponding, limb in an end-to-end fashion. If perfusion pressure distal to occlusion is greater than 60 mmHg in the brachiocephalic artery, no, additional cerebral protection is used. If it is less than 60 mmHg, a, temporary shunt is inserted. The stent-graft can be introduced, retrograde through the femoral artery or antegrade through the, graft attached to the proximal aorta that will eventually be used for, left subclavian artery reconstruction. (From Carrel and colleagues.C6), , Systolic pressure, , 200, , mmHg, , 175, 150, 125, 100, 75, 12, , Surgery is indicated for recurrent coarctation if any one of, these criteria is not met or if percutaneous therapy is not, successful. For those institutions that prefer percutaneous, techniques for selected native coarctation, the criteria that, must be met are the same as for recurrent coarctation., , Section XI, , Tetralogy of Fallot, , DEFINITION, Definition, morphology, and basic physiology of tetralogy of, Fallot are described in Chapter 38. Tetralogy of Fallot is, common in adults presenting with congenital heart disease., Almost exclusively, adult patients have secondary disease,, having undergone repair in infancy or childhood. Rarely, the, adult with tetralogy will present with primary disease., , MORPHOLOGY, , 225, , 0, , Coarctation must be discrete., There must be no associated proximal arch hypoplasia., Coarctation must be remote from the origin of head and, neck arteries., There must be no aneurysm, dissection, or important, ectasia associated with the coarctation., No intracardiac anomalies requiring surgical repair are, present., , 24, , 36, , Months of follow-up, , Figure 29-29 Preoperative and postoperative systolic blood pressure in 45 adult patients undergoing surgical repair of native aortic, coarctation. (From Jatene and colleagues.J3), , Most often, previously repaired patients present as adults with, residual right ventricular (RV) outflow tract (RVOT) disease,, either pulmonary stenosis, regurgitation, or both. This may, develop in the native RVOT, in a prosthetic valve placed in, the native RVOT, or in a RV-to–pulmonary trunk conduit., When stenosis is present, it may be subvalvar, valvar, or, supravalvar. Residual RVOT disease is common because this, problem is built into the standard initial repair. Pulmonary, valve anular hypoplasia and abnormal cusp development, occur in the majority of tetralogy patients. Initial repair, involves a transanular patch in more than 50% of patients,, causing obligatory important pulmonary regurgitation. Surgical pulmonary valvotomy is performed in many of the, remaining patients, leaving them vulnerable to both regurgitation and stenosis., Other less common reasons for adult presentation include, residual ventricular septal defect (VSD), residual atrial septal, defect, tricuspid regurgitation, aortic regurgitation, dilated, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1101

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PART VI, , Congenital Heart Disease in the Adult, 40, , 60, IART/AF, AF, IART, VT/VF, , 50, , 30, 20, , 25, 20, 15, 10, , 10, , Number of, patients, , VT/VF, , 30, , 40, , 0, , IART/AF, , 35, , Prevalence (%), , Prevalence (%), , 5, <25, , 92, , 25-35, , 180, , 35-45, Age (years), 152, , 45-55, , ≥55, , 0, 1, , 76, , 56, , Figure 29-30 Prevalence of tachyarrhythmias in 556 surgically, repaired tetralogy of Fallot patients by age category. Key: AF, Atrial, fibrillation; IART, intraatrial reentrant tachycardia; VF, ventricular, fibrillation; VT, ventricular tachycardia. (From Khairy and colleagues.K11), , Number of, patients, , 2, 3, 4, Number of cardiac surgeries, , 152, , 187, , 102, , 66, , ≥5, , 49, , Figure 29-31 Prevalence of atrial and ventricular arrhythmias, according to number of cardiac operations in 556 repaired tetralogy, of Fallot patients. Key: AF, Atrial fibrillation; IART, intraatrial reentrant, tachycardia; VF, ventricular fibrillation; VT, ventricular tachycardia., (From Khairy and colleagues.K11), , aortic root, branch or peripheral pulmonary artery stenosis,, stroke,C25 and various arrhythmias., , 15, 14, , P<.001, , 13, , CLINICAL FEATURES AND DIAGNOSTIC CRITERIA, , 12, , Presentation, , 11, , The typical adult with previously repaired tetralogy of Fallot, presents with signs and symptoms related to pulmonary, regurgitation, including exercise intolerance and palpitations., If intervention is not undertaken at that time, RV dilatation, progresses and right heart failure or atrial or ventricular tachycardia may be the mode of presentation. The effect of RVOT, dilatation, “aneurysm,” and akinesis, independent of the pulmonary regurgitation itself, on the RV is currently the focus, of attention.D6,U2 The role of these factors is not clear at this, time. One study shows that the best predictors of reduced, quality of life are reduced RV ejection fraction and later age, at repair.L13, Many patients in developed countries are followed carefully into adulthood after infant or childhood tetralogy repair,, and the presentation may be of an asymptomatic patient who, at follow-up evaluation eventually meets a number of imaging,, hemodynamic, and electrophysiologic criteria for intervention. These criteria continue to evolve and are designed to, trigger intervention before irreversible myocardial or electrical damage occurs in the right atrium and ventricle., The arrhythmia burden is substantial in repaired tetralogy, patients, and it increases dramatically with age (Figs. 29-30, to 29-32 and Table 29-10)., Presentation in the adult may be altered by any of the, residual or secondary lesions mentioned in Section I of this, chapter., Although about 15% of adult tetralogy patients have a, dilated ascending aorta (associated with a history of long, shunt-to-repair interval and with pulmonary atresia), it rarely, , LV E/e′, , 1102, , 10, 9, 8, 7, 6, 5, 4, 3, No VT/VF, , VT/VF, , Figure 29-32 Relationship between left ventricular (LV) diastolic, dysfunction and ventricular arrhythmias in 321 adult tetralogy, patients. Tissue Doppler studies for diastolic dysfunction calculated, using the ratio (LV E/e′) of early transmitral flow velocity (LV E) and, early diastolic mitral anular velocity (LV e′). Box-and-whiskers plots, show patients with and without ventricular tachycardia (VT) or, ventricular fibrillation (VF). Lower and upper edges of box indicate, 25th and 75th percentile of values (quartiles); horizontal line in box, represents median value. Lower and upper bars (whiskers) indicate, 10th and 90th percentiles. (From Khairy and colleagues.K11), , leads to presentation.N9 Of 671 acute aortic dissections, reported in one large series, only one was in a patient with, tetralogy of Fallot.S52 A few additional isolated single case, reports of aortic dissection and aneurysm in tetralogy patients, also exist; however, given the number of adults with tetralogy, these events are rare.K13,R7,R21 Dilated ascending aorta,, however, is associated with important aortic regurgitation.N9, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, Table 29-10 Arrhythmia Burden in 556 Adults (Mean Age, 37 Years) with Surgically Repaired Tetralogy of Fallot, Characteristic, , Prevalence %, , 95% CI, , Sustained tachyarrhythmia:, , 29.9, , 26.2-33.7, , Atrial tachyarrhythmia:, , 20.1, , 17.0-23.6, , 11.5, , 9.0-14.3, ,    AF, , 7.4, , 5.4-9.7, ,    Other, , 6.7, , 4.8-8.9, , 14.6, , 11.8-17.7, ,    VT, , 14.2, , 11.5-17.3, ,    VF, , 0.5, , 0.1-1.4, , 21.4, , 18.1-24.9, , 7.2, , 5.2-9.5, , 18.3, , 15.3-21.7, ,    IART, , Ventricular tachyarrhythmia:, , At least one arrhythmia, intervention:, Transcatheter ablation, Implanted cardiac arrhythmia, device:,    Pacemaker, , 7.9, , 6.0-10.5, ,    ICD, , 10.4, , 8.1-13.1, , Sustained tachyarrhythmia, and/or intervention, , 43.3, , 39.3-47.5, , From Kairy and colleagues.K11, Key: AF, Atrial fibrillation; CI, confidence interval; IART, intraatrial reentrant, tachycardia; ICD, implantable cardioverter-defibrillator; VF, ventricular, fibrillation; VT, ventricular tachycardia., , Diagnosis, The diagnosis is already known in the great majority of adults, with tetralogy of Fallot. Further diagnostic workup is aimed, at identifying residual defects (primarily pulmonary regurgitation) and determining when intervention is indicated. The, electrocardiogram (ECG) is critical to evaluation, identifying, the predominant rhythm (sinus, junctional, or atrial fibrillation or flutter), presence of ventricular ectopy, and QRS, complex duration. Holter monitoring and formal electrophysiologic evaluation should be undertaken to fully characterize any rhythm disturbance (see “Arrhythmias” in Section, I of this chapter)., Echocardiography estimates severity of pulmonary regurgitation, RVOT obstruction, RV function and size, tricuspid, regurgitation, residual ventricular and atrial septal defects, as, well as left-sided valvar and ventricular function., Magnetic resonance imaging (MRI) is particularly helpful, in quantifying pulmonary regurgitant fraction, tricuspid, regurgitant fraction, and RV end-diastolic volume.D6,V9 It, may also provide important information about the peripheral, branch pulmonary arteries. If MRI is contraindicated, computed tomography (CT) can be used to determine RV volume, and to image the branch pulmonary arteries.B28,K20,R3, Cardiac catheterization is indicated to assess the coronary, arteries and to determine pulmonary vascular resistance,, which may be abnormal, particularly in patients formerly, palliated with shunts. Coronary arteries are imaged to rule, out arteriosclerosis and to characterize the coronary artery, anatomy if it is not already known. Specifically, the left anterior descending coronary artery arises from the right sinus of, Valsalva and crosses the RVOT in about 10% of tetralogy, patients and thus is vulnerable to injury during RVOT intervention. Catheterization may also be indicated to obtain, , Congenital Heart Disease in the Adult, , complete hemodynamic evaluation if alterations in right or, left ventricular function require further clarification., , NATURAL HISTORY, Tetralogy is a common form of congenital heart disease, (5%-7% of all congenital defects), and most patients survive, to adulthood after repair. Thirty-year survival after repair is, 80% to 85%. Surgical cure is rare, however.H14, When an adult presents with unrepaired tetralogy, the, physiology is usually that of mild or mild to moderate RVOT, obstruction., , TECHNIQUE OF OPERATION, The surgical procedures used in adults with tetralogy of, Fallot will be for residual or recurrent disease in most cases., Rarely, the adult will present with unrepaired tetralogy, either, with a history of previous palliative shunt or with no previous, surgery. The procedures used in these patients are the same, as in children and are detailed in Chapter 38. In adults with, previously repaired tetralogy who have pulmonary regurgitation, porcine, xenograft, bovine pericardial, and allograft, valved conduits may be placed into the RVOT. The technique of operation is described in Chapter 38, as is surgical, reconstruction of the RVOT., Catheter-based placement of a pulmonary valve is possible, as a “valve-in-valve” procedure requiring a previously placed, bioprosthetic conduit 18 to 20 mm in diameter. Peripheral, or segmental pulmonary artery stenoses may be addressed by, balloon dilatation, either proceeding the procedure or as a, hybrid procedure. Catheter-based methods may be applicable, for residual atrial or ventricular septal defects, branch pulmonary artery stenoses, aortopulmonary collateral arteries, and, closure of previously placed surgical shunts., , RESULTS, Surgical outcomes after operations on the RVOT in adults, with previously repaired tetralogy are excellent, with early, mortality of 0% to 2%.C20,D21,T11 Lesser performed operations,, such as closure of residual atrial or ventricular septal defect, or repair of branch pulmonary artery stenosis, probably have, similar mortality. Late survival is also excellent, with mortality, of 0.5% per patient-year.C20 Sudden death from arrhythmias, is the main mode of premature death. Ventricular tachycardia, is the most common cause of this; however, acute atrial, tachycardia and heart block may also contribute. Its risk, factors include a long interval before repair (this risk is present, whether the patient has received no operation or a palliative, shunt); residual RVOT disease (regurgitation or stenosis);, known ventricular ectopy; poor left ventricular function; and, prolonged QRS duration. Prolonged QRS duration and ventricular ectopy both correlate with poorly functioning and, dilated RVs and with cardiac events. Prolonged QRS also, correlates with left ventricular dysfunction, and left ventricular dysfunction is a risk factor for sudden death.G14,T28, Normalization of RV hemodynamics (placing a competent, pulmonary valve and relieving RVOT obstruction) can reduce, ventricular arrhythmias.B2,B19,C12,C39,D2,D5,G7,G14,H5,H17,T10,W5 The, operation can also reduce QRS duration in some cases, but, not in others.H6,M28 The study by Scherptong and colleagues, showed that QRS duration improved in about two thirds of, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1103

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PART VI, Table 29-11, , Congenital Heart Disease in the Adult, Observed Annualized Cardiac Event Rate According to QRS Duration, No., , Patient-Years, , Events, , Events/100 Patient-Years, , 90, , 483.8, , 13, , 2.7, , ≤180 ms + PO QRS reduction, , 44, , 241.8, , 0, , 0, , ≤180 ms − PO QRS reduction, , 27, , 157.0, , 8, , 5.1, , 2.2-10.0, , >180 ms + PO QRS reduction, , 14, , 75.3, , 3, , 4.0, , 0.8-11.6, , >180 ms − PO QRS reduction, , 5, , 9.7, , 2, , 20.6, , 2.5-74.4a, , ≤180 ms + PO QRS reduction, , 52, , 294.8, , 2, , 0.7, , 0.1-2.4, , ≤180 ms − PO QRS reduction, , 21, , 123.7, , 6, , 4.9, , 1.8-10.6, , >180 ms + PO QRS reduction, , 6, , 22.4, , 1, , 4.5, , 0.1-24.9, , >180 ms − PO QRS reduction, , 11, , 43.0, , 4, , 9.3, , 2.5-23.8b, , Overall, , 95% CI, 1.4-4.6, , Preoperative QRS Duration, NA, , Postoperative QRS Duration, , Modified from Scherptong and colleagues.S11, a, P = .007 vs. the overall group., b, P = .030 vs. the overall group., Key: CI, Confidence interval; NA, not applicable; PO, postoperative., , patients after operation, and this occurred whether the preoperative QRS was below or above 180 ms.S11 Postoperative, cardiac events were most likely to occur in the subset of, patients with preoperative QRS of greater than 180 ms and, no postoperative reduction in duration (Table 29-11). In, another study, placing a competent pulmonary valve did not, reduce QRS duration in any patient, and late follow-up, showed no reduction in cardiac events.H6 Why some patients, show reduction in QRS duration after placement of a competent valve and others do not is unclear. Nevertheless, these, studies imply that preserving RV function is probably the best, way to minimize late fatal arrhythmias. This is best achieved, by early primary repair with attention to myocardial preservation, and aggressive intervention in adults with residual or, recurrent hemodynamic abnormalities that threaten to compromise RV function., Improvement in both systolic and diastolic function, and in RV dimensions has also been documented after, placement of a competent pulmonary valve. Some studies,, however, show continued RV dilatation and reduced function, despite improvement in functional health status of the, patient. These discrepancies may be due largely to timing, of inter­vention, again underscoring the benefit of early inter­, vention.C20,D5,E8,G9,H12,T11,V10,V15, Performance of prosthetic pulmonary valves placed into, the RVOT in adults with previously repaired tetralogy and, pulmonary regurgitation has been studied by Fiore and, colleagues.F8 In comparing porcine (Medtronic Mosaic valve),, bovine pericardial (Carpentier-Edwards), and pulmonary, allograft valved conduit (Cryolife) prostheses, they found that, allograft valved conduits initially had the lowest gradients;, however, they also developed regurgitation and required, reintervention sooner (Fig. 29-33). Outcomes for adult, tetralogy patients undergoing right heart procedures and, concomitant aortic valve or ascending aortic surgery have, been reported by Stulak and colleagues.S52 In their study of, 81 patients, early mortality was 7.4% (CL 4.4%-12%);, however, there was no mortality after 1991 in the last 56, patients (CL 0%-6.4%)., Adults presenting with unrepaired tetralogy of Fallot are, becoming a vanishing subset. Most reports of large series are, , 100, PE, 80, , % Freedom, , 1104, , PO, , 60, 40, , AL, , 20, 0, 0, , 30, , 60, , 90, , 120, , 150, , 0, 2, 1, , 0, 1, 0, , 0, 0, 0, , Months, Patients at risk, PE, 18, PO, 49, AL, 15, , 12, 19, 6, , 4, 3, 5, , Figure 29-33 Freedom from valve explantation in patients who, underwent initial pulmonary valve replacement for chronic pulmonary insufficiency. Key: AL, Allograft; PE, pericardial; PO, porcine; Pts,, patients. (From Fiore and colleagues.F8), , either from the 1960s and 1970s or, if more recent, are from, countries with emerging healthcare technology.G15 Attenhofer and colleagues recently reported on 52 patients (mean, age 50 years) operated on between 1970 and 2007, half of, whom had no previous palliation and half of whom did.A21, Early mortality was 5.8% (CL 2.6%-11%). At 15-year, follow-up, 29 of 48 early survivors had died at a mean age of, 65 years. Previously palliated patients who died did so at a, mean age of 59 years, and patients with no previous palliation, who died did so at a mean age of 70 years. Survival in the, entire group was lower than in the general population 10, years after repair, consistent with other evidence that earlier, repair provides the most benefit. Surviving patients were, functionally improved. Alizadeh and colleagues reported on, 51 patients (mean age 22 years) operated on between 1995, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, and 2005.A9 A previous systemic-to–pulmonary artery shunt, had been performed in 16%. Early mortality was 2.0%. (CL, 0.05%-10%). During a mean follow-up of 42 months, one, additional death occurred. Functional improvement was documented in survivors. Atik and colleagues reported on 39, patients (mean age 27 years) operated on between 1982 and, 2001.A20 A previous shunt had been performed in 10%. Early, mortality was 5.1% (CL 0.6%-17%), and actuarial survival at, 15 years was 68%. Again, functional improvement was seen, in survivors. Lu and colleagues report on 57 patients (mean, age 25 years) operated on between 1990 and 2004.L14 Early, mortality was 7.0% (CL 1.9%-17%), and survival was 73% at, 14 years. Functional improvement was documented, with, 76% of patients in New York Heart Association (NYHA), functional class I. Horer and colleagues report on 52 patients, (mean age 29 years) operated on between 1974 and 2003.H21, Early mortality was 15% (CL 6.9%-28%). There were two late, cardiac deaths at a mean follow-up of 12 years; 88% of survivors were in NYHA functional class I. Rammohan and, colleagues report on 100 patients (mean age 20 years) operated on between 1991 and 1996.R4 A previous shunt was, present in 22%. Early mortality was 4% (CL 1.1%-9.9%), and, there was one late death at a mean follow-up of 3.4 years. Of, survivors, 94% were in NYHA functional class I., These studies indicate that primary repair can be performed in the adult with varying, but generally low, early, mortality risk, and with substantial improvement in functional status and quality of life. Late survival, however,, is substantially lower than in the general population, and, lower than for tetralogy patients who undergo operation, earlier in life., , INDICATIONS FOR OPERATION, Decisions relating to intervention in the adult with previously, repaired tetralogy of Fallot occur commonly and are com­, plex.C21,T8,T9 There are multiple potential defects, many of, which may occur simultaneously and with varying degrees, of severity. Appropriate intervention may be by surgery,, percutaneous catheter-based intervention, or both. The, adult congenital cardiologist, pediatric cardiologist, adult, congenital cardiothoracic surgeon, and electrophysiologist, should all be involved in formulating the individualized management plan for each patient. In patients with equivocal, indications for intervention, cardiopulmonary exercise testing, may be helpful.G16, Careful judgment should be used for the adult who presents with long-standing profound cyanosis and no previous, surgery. This patient may struggle with severe right or even, biventricular failure following primary repair even if no residual lesions are present and excellent myocardial protection is, used. There are many reasons for this, including chronic, cyanosis and long-standing RV hypertension., There is another important variable: It must be appreciated that with markedly reduced pulmonary blood flow, this, patient will have a combined ventricular output far less than, normal, in the range of 1.3 to 1.5 times normal systemic, output, rather than the normal combined ventricular, output of 2 times normal systemic output. Primary repair, of tetralogy, even when there are no residual defects, represents an acute volume load as biventricular output is obligatorily increased to 2 times normal systemic output. In the, setting of a very noncompliant, small hypertrophied RV, the, , Congenital Heart Disease in the Adult, , circulation may be unsustainable after repair. In such cases a, preliminary systemic-to–pulmonary artery shunt procedure,, followed by shunt takedown and repair after 3 to 6 months,, should be considered. This approach will increase volume, load during the shunt phase, effectively training the two, ventricles to work at normal volumes prior to repair., Catheter-Based Interventions, Catheter-based intervention may be applicable in selected, cases of RVOT obstruction or regurgitation, residual atrial, and ventricular septal defect, and branch and peripheral pulmonary artery stenosis that meet the same indications as for, surgical intervention. Catheter-based intervention is preferred, for closing acquired aortopulmonary collaterals and often for, closing previously placed surgical shunts., Catheter-based therapy may be considered for RVOT, obstruction if the obstruction is at the valve level or beyond,, but not if it is in the infundibulum. Balloon dilatation of, valvar obstruction (either native valve or conduit), with or, without stenting, is commonly performed; however, it is difficult to demonstrate objectively the efficacy of this procedure. Gradients can diminish but are not eliminated; however,, regurgitation is made worse. The tradeoff in this physiologic, change is of questionable benefit., Catheter-based placement of a pulmonary valve in combination with balloon dilatation and stenting of obstruction is, a more rational approach that has gained favor in recent years., This approach can also be used in selected cases of isolated, regurgitation after repair. Catheter-based placement of a pulmonary valve, however, is only applicable in about 10%, of repaired tetralogy patients with RVOT problems. Criteria, for this procedure include requirement of a previously placed, RV-to–pulmonary trunk conduit with a diameter of at least, 16 mm, or previously placed bioprosthetic valve with a diameter of 18 to 20 mm.M20,Z2 Thus, the great majority of repaired, tetralogy patients—those with native RVOTs or transanular, patches—are not candidates., Most atrial septal defects are candidates for percutaneous, device closure, with the criteria the same as for closure of, isolated atrial septal defect. Selected muscular and patchrelated residual VSDs are candidates for percutaneous, device closure.K18 Size and position of the defect must be such, that impingement of the device on the tricuspid, mitral, or, aortic valve is not of concern. Most acquired collaterals can, be occluded with percutaneously placed coils or other occlusive devices., Open surgical shunts are exceedingly rare in adult tetralogy patients in the current era; however, the occasional, patient who reaches adulthood with palliated tetralogy may, be a candidate for device closure. Branch and peripheral, pulmonary artery stenosis can be considered for percutaneous, balloon dilatation, particularly if surgical intervention is not, in the immediate future of the patient.K27,L9,O6 Stenting of, branch pulmonary arteries should be avoided in most cases,, even though it is fully acknowledged that some institutions, recommend them.F6,M23 The central branch pulmonary arteries are easily accessible surgically, even when the surgeon does, not have particular expertise in pulmonary artery reconstruction. When placed in central pulmonary artery branches,, stents do not relieve obstruction with the same efficacy as, surgical reconstruction, and furthermore, stents can erode, into the tracheobronchial tree and aorta., , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1105

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1106, , PART VI, , Congenital Heart Disease in the Adult, , Surgical Interventions, If surgical reconstruction of the RVOT is indicated, then, balloon dilatation or stenting of central branch pulmonary, artery stenosis should not be performed. Rather, the branch, pulmonary artery obstruction should be repaired surgically as, part of the RVOT procedure. If no immediate surgical procedure is anticipated for a patient, balloon dilatation of isolated central branch pulmonary artery stenosis is a reasonable, approach, but stenting should be avoided., If surgery is planned for RVOT disease, concomitant more, peripheral stenoses in lobar or segmental pulmonary arteries, may be addressed by balloon dilatation prior to surgery or at, surgery. This decision should be made on an individual institutional basis depending on available surgical and interventional expertise. Stenting of peripheral lobar and segmental, pulmonary arteries that do not respond to balloon dilatation, should not be performed if surgical expertise is available for, direct repair., , SPECIAL SITUATIONS AND CONTROVERSIES, A number of cardiac anomalies have morphologic characteristics similar to those of tetralogy of Fallot. These are all, conotruncal anomalies. They include all types of pulmonary, atresia with VSD, all types of truncus arteriosus, and some, types of double outlet right ventricle, transposition of the, great arteries (TGA), congenitally corrected transposition of, the great arteries, and double outlet left ventricle. The similar, characteristics include normally developed atria, atrioventricular (AV) valves, and ventricles, with large conoventricular, type VSD, overriding or transposed aorta, and pulmonary, stenosis or atresia. It follows that surgical repair of these, anomalies will be similar to that of tetralogy of Fallot. It also, follows that many of the late complications and management, issues seen in adults with these anomalies will be similar to, those seen with tetralogy., The majority of these anomalies will be repaired in infancy,, and repair requires two main components: VSD closure, such that the left ventricle aligns with the aorta, and RV-to–, pulmonary trunk reconstruction, often involving a valved, conduit. Thus, adults with these anomalies almost always, present with secondary disease, having been repaired in, infancy or childhood. Issues related to the late follow-up, status of the RV and RVOT in all these anomalies are similar, to those seen in tetralogy; each, however, has unique additional morphologic characteristics that can lead to other late, management problems not typically seen in tetralogy. These, are discussed in the following text., Pulmonary Atresia with Ventricular Septal Defect, Definition, See Chapter 38., Morphology, There are two forms of this anomaly. The duct-dependent, form is similar to classic tetralogy of Fallot. Because of the, atretic pulmonary valve, initial reconstruction in infancy typically involves a valved conduit. By the time these patients, reach adulthood, essentially all will have a RV-to–pulmonary, trunk conduit. Late problems in adults are similar to those in, tetralogy patients who have received a conduit. Dilatation of, , the ascending aorta, and associated aortic regurgitation, is, more common in tetralogy with pulmonary atresia than in, classic tetralogy., The other form of pulmonary atresia with ventricular, septal defect does not have a ductus arteriosus, but instead, has large aortopulmonary collateral arteries. Branch and, peripheral pulmonary artery abnormalities, such as deficient, arborization, hypoplasia, or complete absence, are the hallmark of this anomaly., Clinical Features and Diagnostic Criteria, Because of the complex and variable pulmonary artery morphology of this anomaly, individual patients will be managed, differently during childhood and will therefore present differently in adulthood. The spectrum of presentation includes, primary disease that is newly diagnosed (rarely), primary, disease that was previously diagnosed but with benign physiology (rarely), primary disease that was previously diagnosed, but deemed inoperable, and secondary disease after previous, repair or palliation., In adults with newly diagnosed primary disease, mode of, presentation will likely be with signs and symptoms of cyanosis, but also may be with complications related to longstanding mixed circulation, such as paradoxical embolus,, endocarditis, arrhythmias, or heart failure. Formal evaluation, with echocardiography to assess ventricular function and, valve function, and cardiac catheterization to assess the pulmonary artery and collateral morphology and physiology, are, required., Technique of Operation, Surgical management decisions are the same as for children, diagnosed with this disease (see Chapter 38), although adults, have a greater degree of pulmonary vascular obstructive, disease and collateral loss as well as cardiac dysfunction., Unoperated adults with preserved cardiac function and a full, complement of patent but partially stenotic collaterals may, be operable and fully correctable. In the large series by Malhotra and Hanley, unifocalization and intracardiac repair was, possible in selected patients as old as 45 years.M4 If evaluation, reveals that intracardiac repair is contraindicated, palliative, shunt or conduit operations to improve oxygenation may be, warranted if the dominant symptoms are from cyanosis., Heart-lung transplantation may be considered in patients, with decompensated cardiac function (see Chapter 21)., In adults with previously diagnosed primary disease with, benign physiology, careful evaluation is similarly warranted., The original assessment that the patient had benign physiology is likely to have been incorrect if that designation was, based only on adequate systemic oxygen saturation and not, on detailed morphologic and physiologic assessment of the, individual collaterals. Appropriate evaluation of each patient, in this category, as in the former category, will determine, whether repair, palliation, or transplantation is indicated., In adults presenting with previously diagnosed primary, disease thought to be inoperable, full evaluation is again, indicated. Surgical management options are the same as for, the previous categories just discussed. Many such patients, previously thought to be uncorrectable will in fact be correctable using techniques developed over the last 10 to 15, years.M4, In adults with secondary disease, there are two subgroups:, those who reach adulthood palliated and those that reach, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, , A, , B, , Congenital Heart Disease in the Adult, , C, , Figure 29-34 Repair of regurgitant truncal valve. A, Quadricuspid truncal valve with deficient cusp. B, Repair is accomplished using cusp, union to create either a functional tricuspid (B) or bicuspid (C) valve. Additional commissuroplasty sutures are used as needed to provide, additional support at commissural posts. (From Kaza and colleagues.K9), , adulthood after complete repair. The palliated subgroup will, have received childhood palliative procedures such as shunts, to either native pulmonary arteries or to collaterals, partial, unifocalization, collateral ligation, or RV-to–pulmonary trunk, conduits of varying types. Progressive cyanosis is the usual, mode of presentation; however, many patients will present, with other signs and symptoms associated with long-standing, mixed circulation, such as paradoxical embolus, arrhythmias,, endocarditis, or heart failure. Full evaluation as described, earlier in this section is indicated. Some in this group will be, candidates for pulmonary artery reconstruction, unifocalization, and intracardiac repair; those who are not may benefit, from further palliation or heart-lung transplantation., The second subgroup consists of those who have undergone unifocalization and complete intracardiac repair in, childhood. Adult management issues in this group are similar, to those of repaired tetralogy; however, pulmonary hypertension and distal pulmonary artery stenoses are much more, common, and these abnormalities influence the requirement, for and timing of conduit changes and peripheral pulmonary, artery interventions. Cardiac catheterization with pulmonary, angiography and distal pressure measurement in each segmental pulmonary artery is a mandatory component of preoperative evaluation. The principle of preserving underlying, RV function is the basis for all surgical management decisions, just as in tetralogy., Truncus Arteriosus, Definition, See Chapter 43., Clinical Features and Diagnostic Criteria, RVOT sequelae in previously repaired truncus are similar, to those in tetralogy. The truncal valve, which functions, as the aortic valve after the repair, is structurally abnormal., Late “aortic” regurgitation is much more common than in, tetralogy., Natural History, Unrepaired truncus arteriosus is rare in the adult, and when, it is encountered it is almost always unrepairable because of, Eisenmenger physiology. There are no reported series or case, reports of primary surgical repair of truncus in the adult., There are several case reports of unrepaired truncus presenting in adulthood with complications such as pulmonary, hypertension with Eisenmenger physiology, endocarditis, and, fatal truncal root dissection.C13,C22,E1,G23,M38,V13, , Technique of Operation, Because the truncal valve commonly has more than three, cusps and the anulus is large, the regurgitant valve can often, be repaired using techniques that are not usually applicable, in true aortic valves (Figs. 29-34 to 29-36)., Results, Outcomes and techniques for truncal valve repair are reported,, but mostly for younger patients. In the series of 17 repairs, by Kaza and colleagues, there was a single older patient (age, 42 years).K9, Indications for Operation, Patients with truncal regurgitation usually present for repair, at the time of neonatal correction, or within the first 10, to 15 years after repairH13,K9,M15; therefore, presentation in, the adult is unusual. Indications for repair or replacement, are standard., Double Outlet Right Ventricle, Definition, The type of double outlet right ventricle (DORV) with, subaortic VSD and pulmonary stenosis is discussed in this, section (see Chapter 53). It is the most common form, of DORV., Morphology, DORV with subaortic VSD and pulmonary stenosis or atresia, is managed surgically like tetralogy of Fallot. Late problems, occur primarily in the reconstructed RVOT, as in tetralogy., Additionally, there are concerns about the left ventricular, outflow tract (LVOT) because of the subaortic conus and the, more complex patch (or baffle) that is required to close the, VSD and align the left ventricle with the aorta. The result is, a complex tunnel-like LVOT. A combination of hypertrophy, of the conus muscle and patch distortion lead to a greater, chance of late subaortic stenosis than in tetralogy of Fallot., The obstruction is often morphologically complex, and repair, may require replacing the VSD patch (baffle), with or without, resection of conus muscle., Natural History, Individuals with DORV rarely survive to adulthood without, surgical repair. Those without pulmonary stenosis who do, survive to adulthood have Eisenmenger physiology and are, inoperable.D15 Those with pulmonary stenosis who survive, and are cyanotic from low pulmonary blood flow may be, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1107

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1108, , PART VI, , Congenital Heart Disease in the Adult, , B, C, , A, , E, D, Figure 29-35 Valve repair of quadricuspid truncal valve by cusp excision and anular remodeling. A, Common arterial trunk is transected,, showing regurgitant quadricuspid truncal valve. If just one cusp is grossly abnormal, the approach described is particularly applicable. In, this patient, small prolapsed cusp does not involve sinus of Valsalva. B, Prolapsed leaflet is removed, with care being taken to leave neighboring cusps attached. C, Sinus of Valsalva truncal wall is resected in preparation for remodeling procedure. D, Pledgeted sutures are used, to tighten anulus, thereby bringing remaining cusps together. This remodels truncal valve into a smaller, competent, and nonstenotic, neoaortic valve. Newly formed commissure is carefully aligned in the process of anular remodeling. E, Lateral view of remodeled neoaortic, valve. (From Mavroudis and colleagues.M15), , C, , A, B, , Figure 29-36 Reimplantation procedure for abnormal cusps involving a sinus of Valsalva. Coronary artery is reimplanted into neighboring, sinus of Valsalva, resecting the abnormal cusp and remodeling the anulus. A, Coronary button has been removed from affected sinus of, Valsalva. Abnormal cusp is excised. B, Anulus is tightened and coronary artery button reimplanted, using much the same technique as for, arterial switch operation. C, Lateral projection of anular remodeling technique. (From Mavroudis and colleagues.M15), , candidates for surgical repair, similar to individuals with unrepaired tetralogy of Fallot.E6,H4,M21,M47, Technique of Operation, The initial surgical approach should be through an incision, in the aortic root, with examination of the LVOT, looking, , retrograde through the aortic valve. If hypertrophied conus, muscle is the primary cause of the obstruction, it can be, resected effectively using this approach, similar to resection, of typical isolated subaortic obstruction. If examination, through the aortic root reveals more complex obstruction, involving the previously placed patch (baffle), then a right, ventriculotomy is made in the hood of the RV-to–pulmonary, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, trunk conduit, and the patch (baffle) is either revised or, removed and replaced to relieve the obstruction., Transposition of the Great Arteries, Definition, Only transposition of the great arteries (TGA) with VSD and, pulmonary stenosis or atresia is discussed in this section (see, Chapter 52 for in-depth discussions)., Natural History, Individuals with TGA rarely survive to adulthood without, surgical repair. There are only three case reports of survival, to adulthood in individuals with various forms of TGA. Ironically, none of these patients had TGA with VSD and pulmonary stenosis, which is managed surgically somewhat like, tetralogy of Fallot and has the physiology most consistent, with long-term survival., Technique of Operation, Repair in infancy or childhood requires use of a RV-to–, pulmonary trunk conduit in most cases. Thus, the usual late, “tetralogy-like” RVOT problems are encountered. The intracardiac baffle created to close the VSD and align the left, ventricle with the aorta at initial repair is even more complex, than that in DORV. Thus, the same late LVOT concerns exist, for the repaired adult TGA patient. Additionally, it is more, likely that these transposition patients will be palliated with, a shunt procedure in the neonatal period, so iatrogenic, peripheral pulmonary artery stenosis is another concern., Because of the anterior position of the aorta, the RV-to–, pulmonary trunk conduit must often be placed directly in the, midline behind the sternum, rather than positioned to the, left side as is typical in tetralogy. The conduit may have been, placed to the left or to the right of the ascending aorta. This, conduit position causes two additional problems: (1) conduit, compression between the mass of the heart and the sternum,, leading to more frequent conduit reoperations, and (2) repeat, sternotomy, which carries a greater risk of hemorrhagic complications than in tetralogy, with both the conduit and the, anterior aorta vulnerable (see “Repeat Sternotomy” in Section, I of this chapter)., Congenitally Corrected Transposition of, the Great Arteries, Definition, Only congenitally corrected transposition of the great, arteries (CTGA) with VSD and pulmonary stenosis or atresia, is discussed in this section (see Chapter 55)., Morphology, CTGA with VSD and pulmonary stenosis or atresia shares, certain features with tetralogy of Fallot: surgical repair, requires VSD closure and placement of a conduit from the, heart to the pulmonary trunk. The definitive repair in infancy, or childhood is either the “classic” repair or the “double, switch” repair. There are important sequelae in the adult after, both operations. In the classic repair, VSD closure is straightforward, and the conduit is placed from the morphologic left, ventricle to the pulmonary trunk. The expected late conduit, problems are a concern with this anomaly, just as with the, other anomalies under discussion in this section. However,, , Congenital Heart Disease in the Adult, , obstruction in the conduit may be better tolerated because, the morphologic left ventricle and mitral valve are part of the, pulmonary circulation., An additional important late problem following “classic”, repair is function of the tricuspid valve and morphologic RV, that are positioned in the systemic circulation. Premature, failure of these structures is common at late follow-up after, “classic” repair, as it is under any other circumstance when, the RV is left in the systemic circulation. (This subject is, covered in more detail in Sections XII and XIII of this, chapter.) When this circumstance is encountered after, “classic” repair, the option of training the left ventricle and, subsequent reoperation to place the left ventricle in the systemic circulation, under any circumstance a controversial, choice, is almost always inadvisable, because the left ventricle, already has a large ventriculotomy in it (raising concerns, about its long-term function in the systemic circulation), and, the operation would require placing a prosthetic valved, conduit from the left ventricle to the aorta. The alternative, of cardiac transplantation is the better option., In the other type of repair in infancy or childhood, the, “double switch” repair, the left ventricle is aligned with, the aorta via the VSD using a patch or baffle, a conduit is, placed from the morphologic RV to the pulmonary trunk,, and an atrial switch is performed. Because of the position of, the heart and aorta in CTGA, the conduit must often be, positioned in the midline in this operation. The late concerns, of conduit compression from the sternum are similar to those, previously discussed for TGA. Additionally, in the double, switch repair, atrial baffle complications, including intra-atrial, shunting, systemic or pulmonary venous obstruction, and sick, sinus syndrome or junctional rhythm, can occur, similar to, complications found after the Mustard or Senning operation, for simple TGA., Natural History, CTGA with VSD and pulmonary stenosis may present as, primary disease in adulthood, but in the current era, most, patients with this form of CTGA undergo surgery in infancy, or childhood. This may involve initial palliation with a systemic-to–pulmonary artery shunt followed by later definitive, repair, or initial definitive repair. After any kind of repair of, CTGA, complete heart block is frequent, occurring either at, the time of initial repair or spontaneously at any time., Technique of Operation, In Connelly and colleagues’ series of 52 patients with all, forms of corrected transposition presenting in adulthood,, 64% underwent definitive surgical repair.C35 The most, common procedure was the “classic” repair for patients with, associated VSD and pulmonary stenosis, performed in 80%, (20/25) of the definitive repairs. In six of these patients, the, systemic tricuspid valve was replaced. Early mortality was 5%., Two patients who had no systemic tricuspid valve regurgitation preoperatively developed severe regurgitation after, repair, probably related to shift of the ventricular septum after, left ventricular pressure was reduced. Exercise testing in survivors was subnormal. There was no increase in systemic, ventricular ejection fraction with exercise., Results, In a series of 189 patients of all ages with all forms of, corrected transposition, 152 had associated VSD and either, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1109

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1110, , PART VI, , Congenital Heart Disease in the Adult, , pulmonary stenosis or atresia.S24 Although mean age at, surgery was 8 years, the upper end of the age range was 47, years, implying that at least some patients with VSD and, pulmonary stenosis presented for initial repair in adulthood., Double Outlet Left Ventricle, There are several forms of this extremely rare anomaly (see, Chapter 54). In the repaired adult, in addition to the typical, right-sided conduit problems, the LVOT must be monitored, for late obstruction, just as in the other anomalies with left, ventricular–to-aortic intracardiac patch or baffles. Commonly,, repair involves complex closure of a VSD (with varying, degrees of baffling required) and placement of a RV-to–, pulmonary trunk conduit., , Transposition of, the Great Arteries, , Section XII, , DEFINITION, The morphology, physiology, and natural history of, transposition of the great arteries (TGA) are described in, Chapter 52., , MORPHOLOGY, There are several common morphologic variants of TGA:, with intact ventricular septum (simple TGA); with ventricular, septal defect (VSD); with arch obstruction; and with VSD, and pulmonary stenosis or atresia. TGA with VSD and pulmonary stenosis or atresia is discussed in Section XI. The, other variants require neonatal or infant surgical correction, for the patient to survive., , CLINICAL FEATURES AND DIAGNOSTIC CRITERIA, Presentation, The adult presentation of these variants of TGA will almost, exclusively be that of secondary congenital heart disease following early corrective surgery. The predominant corrective, surgery for these variants between 1959 and about 1985 was, some form of the atrial switch operation, either Senning or, Mustard. The predominant corrective surgery between about, 1985 and the present has been the arterial switch. Thus, currently, adults older than about age 25 almost certainly will, present with a background of atrial switch surgery, whereas, those under age 25 are likely to present with a background, of arterial switch surgery.L10 These two populations present, distinctly differently in adulthood., A small but notable subset of the population with prior, atrial switch surgery is made up of patients with failing systemic right ventricles (RV) who have undergone pulmonary, trunk banding in order to train the morphologic left ventricle, (LV) in anticipation of atrial switch takedown and conversion, to an arterial switch. These patients may enter adulthood, at varying stages of LV training or after conversion to the, arterial switch., , Survivors may have a spectrum of conditions, including, atrial arrhythmias (most commonly junctional rhythm with, sick sinus syndrome [50% at 20 years] but also atrial fibrillation and flutter [35% at 20 years]), ventricular arrhythmias, (9%), heart block, systemic and pulmonary venous obstruction within the atrial baffle, atrial septal defect, systemic (morphologic right) ventricular failure, systemic (tricuspid valve), atrioventricular valve regurgitation, and dynamic LV outflow, tract obstruction.L12,S18, Systemic venous baffle obstruction within the superior, limb leads to superior vena caval syndrome: swelling of the, head and upper body, sometimes accompanied by headache, or chylothorax. Systemic venous baffle obstruction in the, inferior limb can lead to hepatic congestion, cirrhosis, ascites,, and dependent peripheral edema. Baffle leaks will result in, shunting, typically physiologically left to right, with pulmonary overcirculation; however, large defects may lead to, mixing and systemic cyanosis or paradoxical embolus. Functional status of the systemic RV also influences magnitude, and direction of the shunt., Pulmonary venous pathway baffle obstruction leads to, pulmonary venous congestion and pulmonary hypertension., All the sequelae mentioned in the preceding paragraphs may, already be well established in childhood, and commonly,, pacemaker placement and revision of the atrial baffle, due to, either leak or obstruction, may have already been performed, before adulthood is reached. These sequelae have a substantial effect on quality of life and survival.G8,M34,M36,P22,R18,S8,W22, These multiple sequelae notwithstanding, systemic right, heart function (RV dysfunction, tricuspid valve regurgitation,, or both) is the major issue in adults being followed with an, atrial switch operation. Some degree of systemic right heart, dysfunction is present in most patients by the time they reach, adulthood., Patients with prior arterial switch present in adulthood, with fewer sequelae than do those with prior atrial switch., Some studies indicate that patients are physiologically and, functionally on par with the age-matched normal popu­, lation.C38 One study of 65 patients shows, however, that, exercise capacity was 73% of normal in patients studied at a, mean age of 19 years.T16, Sequelae are not only less prevalent than after the atrial, switch, those that occur are also distinctly different, reflecting, the differences between the two operations. In a study of, 65 patients by Tobler and colleagues, 17% (12/65) of adults, had at least one important cardiac lesion.T16 Arrhythmias, accounted for 7%, with atrial flutter in three patients, sick, sinus syndrome in one, and ventricular tachycardia in one., Structural lesions accounted for the other 10%, with RV, dysfunction in three patients, severe pulmonary regurgitation, in two, and LV dysfunction, severe tricuspid regurgitation,, and LV outflow tract obstruction in one each. They also, noted aortic root dilatation (>36 mm diameter) in 31%, but, no intervention was required. Aortic regurgitation occurred, in 52%; however, it was mild or less in all. There were no, documented coronary artery lesions in this series; however,, in other series coronary lesions were noted in 7% of cases at, late follow-up.W25, Other arterial switch series emphasize RV outflow tract, obstruction, coronary insufficiency and its sequelae, and, aortic root dilatation and aortic regurgitation.F14,L10,P6,S17,V2, RV outflow tract obstruction occurs in 5% to 15% of patients, and tends to develop within several years of the arterial, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, switch; rarely will this become a new problem late post­, operatively. Most patients with serious stenosis will undergo, revision of the RV outflow tract during childhood, and some, will require multiple revisions. This may ultimately result in, important pulmonary regurgitation or need for a prosthetic, valve or valved conduit. As adults, these patients require, further surgical management of the RV outflow tract for, problems related to right-sided conduit failure., Coronary artery problems develop late in up to 8% of, patients after arterial switch.L10 Some of these patients may, be asymptomatic, but most will present with typical signs and, symptoms of coronary insufficiency., Aortic root dilatation is well documented late following, the arterial switch, but it occurs in only a small percentage, of cases.H27, LV dysfunction may develop in patients with a history of, initial atrial switch followed by late pulmonary trunk banding, for LV training and conversion to arterial switch. The dysfunction is currently poorly understood, but is probably, related to inadequacy of the LV training process in most, cases. Coronary artery obstruction is usually not implicated., All patients with a history of LV dysfunction and late conversion to the arterial switch should undergo frequent evaluation, to assess LV function., Diagnosis, Evaluation of the adult with prior atrial switch surgery may, include electrocardiogram (ECG), echocardiography, magnetic resonance imaging (MRI), computed tomography, (CT), and cardiac catheterization. ECG documents baseline, rhythm, status of sinoatrial and atrioventricular nodes, and, presence of ventricular ectopy. Holter monitoring is commonly helpful if any disturbance is present., Echocardiography is the most important tool in assessing, the status of both systemic venous limbs of the atrial baffle,, pulmonary venous pathway, baffle leakage, systemic RV function, and systemic tricuspid valve function. Transesophageal, echocardiography may enhance the findings of surface studies., Nevertheless, it is acknowledged that echocardiography has, limited ability to assess RV function.C23,T5 Coronary artery, morphology is not well imaged by echocardiography., MRI and CT provide superior imaging information about, the atrial baffle and its pathways and the great arteries and, extracardiac central veins, and can provide additional valuable, information about the systemic RV and tricuspid valve. In, particular, MRI can be used to calculate tricuspid valve mass, and volume, ejection fraction, and tricuspid regurgitant, fraction. Myocardial delayed enhancement can evaluate RV, myocardial viability.P18, Cardiac catheterization is critical in the evaluation. Angiography is the standard for imaging the coronary arteries. A, complete hemodynamic evaluation, and in particular measurement of systemic RV end-diastolic pressure, provides, crucial information for managing the systemic right heart., Electrical, morphologic, and hemodynamic data obtained, from these studies are used to determine management of, the TGA patient with a systemic RV. Management options, include medical (pharmacologic) management of rhythm or, ventricular function problems, or both; surgery to correct, rhythm and hemodynamic problems (atrial baffle leaks or, obstruction, VSD, severe RV-to–pulmonary trunk obstruction, maze procedure for atrial flutter or fibrillation, standard, , Congenital Heart Disease in the Adult, , atrioventricular sequential or biventricular pacemaker placement); initiation of a program to train the LV in preparation, for conversion to an arterial switch by placing a pulmonary, trunk band; completion of conversion to an arterial switch in, a previously banded patient; and heart transplantation., Routine evaluation in the adult with prior arterial switch, includes ECG and echocardiography. Cardiac catheterization, with coronary angiography should be performed at least, once in the adult, and more frequently if coronary insufficiency is suspected by symptoms or by other studies.W13 More, frequent coronary angiography should also be considered if, the original operative note from the arterial switch operation, indicates an unusual coronary artery pattern or difficulty with, coronary artery translocation. ECG is most helpful in showing, ischemic changes either at rest or with exercise; however, it, will also document atrial and ventricular arrhythmias., Echocardiography assesses function of all four valves (with, particular focus on the semilunar valves), ventricular function,, and size of the aortic root. The coronary arteries cannot be, fully assessed by echocardiography, but indirect evidence, of coronary insufficiency, such as ventricular wall motion, abnormalities and mitral regurgitation, can be., MRI and CT with angiography are also used selectively,, for example, when more precise imaging is needed than can, be provided by echocardiography (for serial assessment of, aortic root size), when qualitative functional valve assessment, is needed (for serial assessment of aortic regurgitation), or, when functional myocardial evaluation is needed (magnetic, resonance myocardial delayed enhancement)., In atrial switch adult patients who are considered candidates for LV training and eventual conversion to arterial, switch, baseline evaluation of the morphologic LV and mitral, valve is required. This includes ECG for rhythm and ectopy;, echocardiography to assess morphologic LV systolic function,, wall thickness, and mitral valve function; cardiac catheterization to measure LV end-diastolic pressure; and MRI to determine LV mass. All of these studies are then repeated after, placing a pulmonary trunk band according to a specific, protocol, to determine if and when the patient becomes a, candidate for conversion to arterial switch., Electrophysiologic evaluation is indicated in all adults with, TGA, regardless on the type of surgery, if clinical course,, ECG, or Holter monitoring suggests important atrial or, ventricular rhythm disturbance., , NATURAL HISTORY, Adults with prior atrial switch surgery survive to adulthood., Twenty-five-year survival is 65%, and is even better (80%) if, TGA is not complicated by VSD.O2 Late mortality risk after, the first 5 years following surgery appears to be linear at, 0.5% per year.L12 Studies show a concerning trend in measures, of systemic ventricular function over time, with both cardiac, index and stroke volume index inversely related to age, (Figs. 29-37 and 29-38).B33, , TECHNIQUE OF OPERATION, After Atrial Switch Repair, Several surgical options are available for superior and inferior, vena caval baffle limb obstruction. The preferred approach, involves direct revision of the systemic venous component of, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1111

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PART VI, , Congenital Heart Disease in the Adult, , Cl at 100 watt (L · min–1 · m–2), , 9.00, , 8.00, , 7.00, , 6.00, , 5.00, , 4.00, 20, , 24, , 28, , 32, , Age (years), , Figure 29-37 Relationship between age and cardiac index (CI) at, 100 watts in 22 adult patients (mean age 21 years) with prior, Senning or Mustard operation for simple transposition of the great, arteries. Blue line is regression line (P = .0001), and dots are individual patient data. (From Budts and colleagues.B33), , 50.00, SVI at 100 watt (mL · m–2), , 1112, , 40.00, , 30.00, , 20, , 24, , 28, , 32, , Age (years), , Figure 29-38 Relationship between age and stroke volume index, (SVI) at 100 watts in 22 adult patients (mean age 21 years) with, prior Senning or Mustard operation for simple transposition of the, great arteries. Blue line is regression line (P = .004), and dots are, individual patient data. (From Budts and colleagues.B33), , the baffle itself. This involves cardiopulmonary bypass (CPB), using bicaval venous cannulation and aortic clamping with, cardioplegia administration. The systemic venous component, of the baffle is exposed using an incision into the morphologic right atrial free wall, which is part of the pulmonary, venous pathway of the baffle. Patch augmentation of, the affected limb of the systemic venous baffle is then, performed., An alternative option for superior limb obstruction is to, perform a bidirectional superior cavopulmonary anastomosis., This option may be preferred if there is long-standing longsegment occlusion of the superior limb or very complex, , nonocclusive obstruction. This option is contraindicated if, pulmonary vascular resistance is elevated above about 3, Wood units., Another option for superior limb obstruction to consider, when obstruction is complex is placing an extracardiac, polytetrafluoroethylene (PTFE) conduit from the patent, superior vena cava to the inferior vena cava at the level of the, diaphragm, with the conduit positioned along the right, lateral border of the heart. This option should be considered, only if the inferior limb of the systemic baffle is fully evaluated, and deemed large enough to carry the entire systemic venous, output without obstruction. An option that may occasionally, be considered for complex inferior limb obstruction is placing, an extracardiac conduit in the reverse direction, from the, patent inferior vena cava at the diaphragm up to the superior, vena cava. The superior limb of the baffle must be large, enough to carry the entire systemic venous return without, obstruction., Pulmonary venous baffle obstruction almost always occurs, at the midpoint of the pathway as it crosses the plane of the, (normal) atrial septum and wraps around the systemic venous, component of the baffle. Surgical approach involves CPB as, described previously for systemic venous baffle obstruction., Patch augmentation of the external wall of the morphologic, right atrium (which is part of the pulmonary venous pathway),, with the patch extending down onto the right pulmonary, veins, usually relieves the obstruction., Operation for baffle leakage is performed using the same, exposure described previously for systemic venous baffle, obstruction. The leak may be at the suture line or through, multiple atrial muscle trabeculations remote from the patch, suture line. After opening the morphologic right atrial wall, and entering the chamber that accepts pulmonary venous, blood, suture line leaks are usually obvious and are best closed, with additional patch material. Muscular trabeculation leaks, are best assessed by performing the additional step of opening, the chamber that accepts systemic venous blood and examining the internal surface of the systemic venous pathway., Direct suture closure or patching of all communications from, this vantage point is then carried out., Subvalvar LV-to–pulmonary trunk obstruction is, approached using CPB with bicaval venous cannulation and, aortic clamping with administration of cardioplegia. Access, to the subvalvar obstruction is through an incision in the, pulmonary trunk, with retrograde exposure through the, pulmonary valve. Muscular and fibromuscular resection is, performed using the same techniques used for subaortic, obstruction (see Chapter 47)., Tricuspid valve surgery in atrial switch patients is usually, not indicated. When it is, it rarely involves valve repair. Very, occasionally, a tethered septal leaflet will be an isolated cause, of regurgitation in a patient with a history of atrial switch, with concomitant closure of a VSD. The tethered leaflet is, iatrogenic. If systolic RV function is normal, repair of the, tricuspid valve with partial closure of the septal anterior commissure at the site of tethering may be effective. More complex, etiologies of tricuspid regurgitation in the setting of normal, RV function are best managed using tricuspid valve replacement. Both bioprosthetic and mechanical valves are appropriate options. The decision is based on the typical advantages, and disadvantages of each type of prosthesis., Atrial switch takedown and conversion to arterial switch, technique is shown in Fig. 29-39., , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Aorta, Pulmonary, artery band, Pulmonary, artery, , Pulmonary, artery, , Tricuspid, valve, , Mitral, valve, , A, , B, , Pulmonary, artery, (neoaortic), , Pulmonary, artery, , Neoaortic root, Left coronary, artery, , Left coronary, artery, , Aorta, , C, , Right coronary artery, , D, , Single pericardial, Neopulmonary patch, root, , Figure 29-39 Takedown of Mustard operation and conversion to arterial switch. A, In the months after placing a pulmonary trunk band,, patient is followed with echocardiography, cardiac catheterization, and magnetic resonance imaging (see text for details) to assess biventricular function and acquisition of morphologic left ventricular (LV) mass, gradient across band, ejection fraction, LV end-diastolic pressure,, and valvar function. If the LV fails to acquire a substantial amount of mass within 6 months and gradient across band predicts LV pressures, below 70% of estimated right ventricular pressure, it is necessary to reoperate to tighten pulmonary trunk band. Optimal duration of, banding, rate of LV mass acquisition, and gradient across band are likely to vary from patient to patient and have yet to be definitively, established. However, compared with the rapid two-stage arterial switch for infants with transposition of the great arteries and intact ventricular septum, the process of LV mass acquisition is much more gradual, and the pulmonary trunk band is left in place substantially longer., The LV should be documented to be working at systemic-level pressure for about 1 year. B, After adequate LV mass acquisition, the Mustard, configuration is converted to an arterial switch. Arterial switch is performed before Mustard baffle is removed, primarily to ensure that the, switch is accomplished successfully before proceeding. Heart, aorta, pulmonary trunk, branch pulmonary arteries, and both cavae are, exposed and dissected thoroughly and carefully through a midline sternotomy. Aorta and branch pulmonary arteries are mobilized extensively. Purse-string sutures are placed high in aorta and in both cavae, after which cardiopulmonary bypass is initiated with aortic and bicaval, cannulation. Systemic (right) ventricle is vented through appendage of morphologically right atrium. Patient is cooled to 25°C. Aorta is, then clamped and cardioplegia administered into aortic root. Aorta and pulmonary trunk are transected, and semilunar valves are inspected., After band around pulmonary trunk is removed, narrowed tissue at band site is excised and proximal and distal edges are trimmed carefully., C, After identifying coronary ostia, coronary arteries are mobilized on generous buttons of sinus wall. Counter incisions are made high in, the facing sinuses of the proximal neoaorta (pulmonary trunk), and the coronary arteries are reimplanted with continuous 5-0 or 6-0 absorbable monofilament suture. In patients with abnormal coronary artery branching patterns, coronary reimplantation is modified as necessary., D, After coronary buttons are reimplanted into neoaortic root, the pulmonary trunk is brought anterior to ascending aorta (Lecompte, maneuver). It is important that branch pulmonary arteries have been mobilized to the pulmonary hila to prevent tension on pulmonary, trunk anastomosis, which may increase risk of postoperative supravalvar pulmonary stenosis. Distal ascending aorta is then anastomosed to, neoaortic root in end-to-end fashion using continuous 4-0 or 5-0 absorbable monofilament suture. Coronary button defects in neopulmonary, artery preferably are patched with autologous pericardium, and with allograft or synthetic patch material if pericardium is not obtainable., Continued, Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Superior vena cava, , Sinoatrial node, Left, pulmonary, veins, Right, pulmonary, veins, , Mustard, baffle, , Pulmonary, venous, atrium, , Inferior vena cava, , E, , F, , Mitral, valve, , Pericardial, patch, , Mitral, valve, , Tricuspid, valve, Left pulmonary, vein orifices, , Coronary sinus, , Tricuspid valve, , G, , H, , Figure 29-39, cont’d E, Although a standard oblique atriotomy is the most common approach to performing a Mustard operation, other, types of atrial incision may be used. In approaching a take-down of the Mustard baffle and reseptation of atria, original atriotomy should, be identified and used. F, On opening pulmonary venous atrium, Mustard baffle is exposed. When Mustard procedure was performed, the, coronary sinus septum may have been opened superiorly into the morphologic left atrium to more easily incorporate the coronary sinus, into the systemic venous atrium. If that is the case, it will not be visible until the patch is removed. Suture lines are typically easy to identify., After incising the superior baffle suture line, entire patch is removed with sharp dissection using either a knife or scissors. Care should be, taken when excising the patch around the opening of the superior vena cava because the sinoatrial node is located in close proximity to, the anterior aspect of the suture line. G, After removing Mustard baffle, mitral valve and caval orifices are exposed. If the coronary sinus, was incorporated into the systemic venous atrium, it will also be exposed. It is important to carefully remove as much scar tissue as possible, from the suture lines, especially from the orifices of coronary sinus and cavae, to prevent any obstruction. H, A pericardial or polytetrafluoroethylene (PTFE) synthetic patch is then fashioned to recreate the interatrial septum. Patch is sewn to remaining rim of native atrial septum, using continuous 4-0 or 5-0 polypropylene suture. In patients who had coronary sinus septum opened at time of Mustard procedure, the, sinus orifice must be reconstructed by bringing the patch down and incorporating it as the posterior wall of the orifice. Alternatively, the, patch can be carried along the suture line of the Mustard baffle, such that the coronary sinus drains to the left atrium. Before tightening, final sutures, left atrium is de-aired by sustained pulmonary hyperinflation. A left ventricular vent is placed across mitral valve, through either, the posterior suture line or a separate purse string. Clamp is removed from aorta, and right atrium is closed and pulmonary outflow tract, reconstructed without cardioplegic arrest during rewarming., Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, , I, , Congenital Heart Disease in the Adult, , J, , Figure 29-39, cont’d I, Right atriotomy is then closed with continuous 5-0 polypropylene as rewarming is commenced. J, Neopulmonary, trunk is then reconstructed by anastomosing distal pulmonary trunk to reconstructed neopulmonary root using continuous 5-0 or 6-0, absorbable monofilament suture. A cardiotomy suction catheter is placed in pulmonary trunk to prevent backflow from obscuring operative, field. (From Reddy and colleagues.R8), , After Arterial Switch Repair, Repair of RV outflow tract problems after the arterial switch, can usually be managed using normothermic beating heart, CPB with a single venous cannula in the right atrium. Because, of the Lecompte maneuver, access to the entire RV outflow, tract, including subvalvar, valvar, and supravalvar areas, and, branch pulmonary arteries, is excellent. Patch augmentation, of obstructive lesions at any level is effective. Valvar obstruction may be managed by valvuloplasty, transanular patch, or, valve replacement. Transanular patching will result in some, degree of pulmonary regurgitation. Valve options include, bioprosthetic valves and pulmonary and aortic allograft valved, conduits. Isolated subvalvar obstruction can be resected,, working retrogradely through the pulmonary valve, or by a, longitudinal infundibular incision with resection or infundibular patching., Coronary artery obstruction and aortic valve and root, problems are managed with techniques described in Chapters, 7 and 12. Surgical access to the aortic root, valve, and coronary arteries is more difficult than usual because of their, posterior position behind the pulmonary trunk following the, Lecompte maneuver. If the pulmonary branches and trunk, are mobile, they may be dissected from the underlying aorta, intact and retracted, allowing access to the aortic root. Often, the pulmonary trunk is not easily mobilized, so it may be, necessary to transect it just above the pulmonary valve or to, transect the right pulmonary artery at its origin from the, pulmonary trunk, mobilizing the pulmonary trunk to access, the aortic root. Coronary ostial lesions may be managed by, , standard techniques of coronary artery bypass grafting or by, ostial patching., The classically described maze procedure is performed in, patients with the arterial switch and atrial fibrillation, and an, appropriately modified maze procedure is used when there is, an atrial baffle in place (see Chapter 16)., Transplantation is performed with the appropriate arterial, and venous anastomotic adjustments (see Chapter 21)., , RESULTS, There are no large studies of adult TGA patients providing, outcomes for the various operations described in this section., Based on outcomes for operations with similar complexity in, non-TGA patients, early mortality for most of the procedures, in patients with a history of either atrial or arterial switch, should be in the range of 1% to 2%. The one exception is the, atrial switch patient undergoing conversion to arterial, switch.P14 In the series of one of the authors (FLH), early, mortality was 20% (1/5; CL 3.2%-53%) among adult patients,, with five additional patients not meeting criteria for conversion after LV training. Benzaquen and colleagues report one, successful adult conversion.B14, , INDICATIONS FOR OPERATION, In all adult patients with transposition, regardless of their, surgical history, arrhythmias may require intervention. Indications for pacemaker insertion include sick sinus syndrome,, symptomatic bradycardia, and selected patients with a stable, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1115

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1116, , PART VI, , Congenital Heart Disease in the Adult, , junctional rhythm that does not meet bradycardia criteria., Although the atrial morphology may be altered, particularly, in atrial switch patients, transvenous pacing systems can, usually be placed. Surgical placement of epicardial pacing, systems remains an option for those with unusual morphology, intracardiac shunts, or occluded central veins, and for, those requiring biventricular pacing. Sudden death is, described in adult atrial switch patients, mostly related to, ventricular tachycardia and fibrillation.K2 Placing an internal, cardioverter-defibrillator (ICD) is indicated if a sudden death, episode is documented or electrophysiologic evaluation demonstrates inducible ventricular tachycardia or fibrillation. The, maze procedure, or appropriate modifications if atrial structures are altered, may be indicated for any adult patient with, TGA if atrial fibrillation or flutter is present and other cardiac, surgery is planned., After Atrial Switch Repair, Both surgical and catheter-based interventional procedures, are available for residual and recurrent lesions in adults with, prior atrial switch surgery. Intervention for the structural, lesions described in the following text is indicated if the, lesions cause important symptoms or if hemodynamic alterations caused by these lesions meet standard criteria for, intervention., Catheter-based dilatation or stenting is the preferred, method for treating symptomatic superior caval, inferior, caval, and pulmonary venous pathway obstruction, and for, baffle leaks. It is also an option, but not necessarily the preferred one, for supravalvar pulmonary trunk and branch pulmonary artery stenosis. Surgical intervention is indicated for, these same lesions when catheter-based techniques are unsuccessful or contraindicated. As an example, catheter-based, therapy may be contraindicated for long-segment occlusion, of the superior baffle limb. Many consider surgical reconstruction the preferred method for treating supravalvar and, branch pulmonary artery stenosis, whether these lesions occur, alone or with valvar and subvalvar stenosis., Surgical intervention for LV outflow tract obstruction in, atrial switch patients is indicated only if LV pressure becomes, suprasystemic or symptoms are attributable to the obstruction. Moderate or even severe obstruction, as judged by, pressure gradient, may not affect LV function. Additionally,, the argument has been made that some elevation of LV pressure may be beneficial, keeping the ventricular septum from, bowing away from the RV, thereby stabilizing the subvalvar, mechanism of the tricuspid valve and reducing development, of systemic tricuspid valve regurgitation., Surgical intervention may be indicated in selected cases of, moderate or severe systemic tricuspid valve regurgitation, but, only when systemic RV function is preserved. Repair of the, tricuspid valve working under systemic loading conditions, remains a challenge. In most cases, tricuspid valve regurgitation is associated with a dilated and poorly functioning systemic RV. The progression may be ventricular dysfunction, leading to regurgitation; however, the causality may be the, reverse as well. Either way, tricuspid valve repair or replacement is contraindicated when there is associated moderate or, severe ventricular dysfunction., Either pulmonary trunk banding with eventual conversion, to an arterial switch or heart transplantation is indicated when, RV dysfunction accompanies tricuspid regurgitation. There, , are no clear guidelines for choosing between these; however,, important atrial or ventricular arrhythmias, morphologic LV, dysfunction, and mitral valve dysfunction all strongly favor, moving toward transplantation. Conversion to arterial switch, remains controversial.L12 Some institutions never recommend, this option. The operation, however, can be performed successfully in highly selected cases in institutions experienced, with it. Indications for proceeding with atrial switch takedown and conversion to arterial switch are complex. Several, stages of selection are required. In the initial selection process,, all patients with an atrial switch procedure can be evaluated., Generally, patients with normal or near-normal function of, the systemic RV and tricuspid valve are not considered for, conversion. Typically, moderate or severe systemic ventricular, dysfunction or tricuspid regurgitation will be present, and, these findings lead to further evaluation for conversion., However, other sequelae, such as baffle obstruction or leak,, may also lead to additional evaluation. Candidates are eliminated from further consideration if important atrial or ventricular arrhythmias are present or if there is evidence of, morphologic LV or mitral valve dysfunction., In the unusual case that the LV is already working under, conditions that will allow it to perform adequately in the, systemic circulation (chronic moderate to severe LV outflow, tract obstruction, or pulmonary hypertension from atrial, baffle obstruction in the pulmonary venous pathway), the, patient may proceed directly to atrial switch takedown and, conversion to arterial switch without undergoing pulmonary, trunk banding. In the more common case the LV will be, chronically working under normal afterload conditions of the, pulmonary circulation and will not be prepared for systemic, workload conditions. These patients must enter a process of, LV preparation by pulmonary trunk banding. The aim is to, gradually achieve afterload conditions that allow the LV to, remodel and hypertrophy such that it will be capable of, working efficiently under the conditions of the systemic circulation. The training process takes a minimum of 1 year and, may take longer. During this time it is likely that more than, one banding procedure will be needed in order to achieve, proper training.B14 At each stage in the banding process,, reevaluation with echocardiography, cardiac catheterization,, and MRI is performed to obtain the data required to evaluate, LV preparedness. The criteria used by one of the authors, (FLH) before the LV is considered prepared are:, ■, ■, ■, ■, ■, , Ventricular ejection fraction of 55% or greater, Less than mild mitral regurgitation, Ventricular systolic pressure 90% systemic or greater, Ventricular end-diastolic pressure 10 mmHg or less, Ventricular mass 60 g · m−2 or greater, , If these criteria are met, conversion is performed., In a series of 31 patients managed by one of the authors, (FLH), 42% who began training with the band achieved, criteria for conversion. Of those achieving conversion, five, were adults aged 22 to 29 years. There was 80% survival in, this group. An additional five adult patients who did not meet, the criteria were turned down for conversion after attempted, LV training. Age was not a deterrent to successful conversion, as long as these strict inclusion criteria were adhered to. In, the study by Poirier and colleagues of 39 patients undergoing, banding, 71% underwent conversion; however, early mortality was 17% (CL 11%-27%) and late cardiac death 13%. This, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, study identified older age as a risk factor, with no successful, conversions in patients beyond age 16 years.P15 The differences in these two series may be explained by the stricter, training selection criteria used in the series with successful, adult conversions., , There are several morphologic variants of CTGA:, ■, ■, ■, ■, , After Arterial Switch Repair, Indications for surgery in adults with prior arterial switch and, RV outflow tract lesions are the same as those for tetralogy, of Fallot (see Section XI of this chapter). Indications for, coronary artery surgery are similar to those in patients with, arteriosclerotic disease (see Chapter 7) and include signs and, symptoms of myocardial ischemia and coronary artery luminal, narrowing of 50% or greater. Because coronary artery sequelae, after the arterial switch are almost always limited to the, ostium or adjacent proximal segment, direct ostial augmentation may frequently be indicated, although coronary artery, bypass grafting remains an option as well., Risk of aortic dissection and rupture associated with aortic, dilatation after the arterial switch is not known. However,, based on physiologic principles, it is reasonable to assume, that it is the same as that in native aortic dilatation. Thus, it, is reasonable to follow the indications for root replacement, recommended for native aortic disease (see Chapters 25 and, 12). If aortic valve function is normal, a valve-sparing root, replacement should be considered. If root dilatation is accompanied by more than mild aortic regurgitation, root replacement with aortic valve replacement, or aortic valve repair, is, indicated., , Congenitally, Corrected Transposition of, the Great Arteries, Section XIII, , DEFINITION, Definition, morphology, and basic physiology of congenitally, corrected transposition of the great arteries (corrected transposition, CTGA) are described in Chapter 55. CTGA with, pulmonary stenosis or atresia and ventricular septal defect, (VSD), the single most common form, is discussed in detail, under Special Situations and Controversies in Section XI of, this chapter. All other forms are discussed in this section., , MORPHOLOGY, CTGA is a rare anomaly and thus is not often found in adults., It may present in adulthood as newly diagnosed disease,, previously diagnosed disease with benign physiology, or secondary disease with a history of previous surgery. Presentation will be variable because all but 1% to 2% of patients have, one or more major associated structural cardiac anomalies,, including pulmonary stenosis, VSD, and systemic atrioventricular (AV) valve abnormalities, and these largely determine, the nature of presentation. Even the rare patient without, associated structural anomalies will eventually develop problems with conduction or with systemic right ventricular, (RV) function., , Congenital Heart Disease in the Adult, , ■, , Without associated structural anomalies, With VSD, With pulmonary stenosis or atresia, With structurally abnormal (“Ebsteinoid”) tricuspid, valve, With various combinations of these structural anomalies, , CLINICAL FEATURES AND DIAGNOSTIC CRITERIA, Presentation, Initial presentation may be spontaneous heart block, which, is well documented to occur at a rate of about 2% per year, in CTGA.H24 Patients with prior history of VSD closure will, present in adulthood in the same way as unoperated patients, without associated structural anomalies, that is, with systemic, RV failure; however, the presentation may be more accelerated. Patients with prior placement of a prosthetic valve in, the tricuspid valve position may present with dysfunction or, outgrowth of the valve, whether it is a bioprosthetic or, mechanical valve. These patients may also present with heart, failure from progressive systemic RV dysfunction. Patients, with a history of isolated pulmonary stenosis repair may, present with recurrent stenosis, or, if one has been placed,, with left ventricle–to–pulmonary trunk conduit failure., Patients with a history of double switch (arterial switch–type, double switch) will present with a combination of all the, sequelae seen after both the arterial and atrial switch (see, Section XII, “Transposition of the Great Arteries,” earlier in, this chapter). Signs and symptoms of systemic RV failure,, systemic tricuspid valve regurgitation, and complete heart, block are the same as in any patient with left-sided heart, failure or heart block and therefore are nonspecific for CTGA., Evaluation will lead to the diagnosis., Diagnosis, The electrocardiogram will show definitive evidence of ventricular inversion. The chest radiogram is not definitive, but, may show abnormal contour of the ventricular shadow, a, narrowed superior mediastinal shadow due to the position, of the great arteries, and abnormal cardiac position such as, mesocardia or dextrocardia. The echocardiogram is diagnostic, identifying AV and ventriculoarterial discordant connections, as well as the major associated anomalies such as VSD,, pulmonary stenosis, and systemic tricuspid valve structural, abnormalities and function.S30 Apical displacement of the tricuspid valve is easily identified, and complexity of pulmonary, stenosis, including valvar and subvalvar components, can, usually be determined. Quantitative assessment of systemic, RV function is often difficult by echocardiography. Magnetic, resonance imaging (MRI) is used to assess systemic RV function and volume and to quantify systemic tricuspid valve, regurgitation. Cardiac catheterization is often helpful in, assessing systemic RV function, using both ventriculography, and hemodynamics (RV end-diastolic pressure)., , NATURAL HISTORY, The undiagnosed asymptomatic adult may present incidentally during evaluation for some other problem.B11,F17 The, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1117

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1118, , PART VI, , Congenital Heart Disease in the Adult, , asymptomatic adult with known unrepaired CTGA may be, referred to the adult congenital cardiac clinic simply because, there is a known diagnosis. Most commonly, the unrepaired, patient without associated structural anomalies, or with, mild associated structural anomalies, will present in the, fourth or fifth decade of life with signs and symptoms of, systemic RV failure, systemic tricuspid valve regurgitation, or, arrythmias.B11,P19 In one single-institution study, by age 50, years, 66% of patients were in heart failure, but none died, over a 10-year observation period thereafter.P19 In a multiinstitutional longitudinal study, 25% of patients with CTGA, and no other structural anomalies presented with signs and, symptoms of heart failure by age 45 years. If associated structural anomalies were present, heart failure by age 45 years, increased to 67%.G19, Patients presenting in adulthood without prior surgery, rarely have severe pulmonary stenosis, large VSDs, or severely, abnormal systemic tricuspid valves, because the natural history, of these associated anomalies rarely allows patients to reach, adulthood in a healthy state. Isolated moderate, or even, moderate to severe, pulmonary stenosis, however, may be, well tolerated because the morphologic left ventricle will be, working under afterload conditions that it tolerates well., , TECHNIQUE OF OPERATION, The surgical techniques recommended in this section—direct, repair of associated anomalies and the double switch, operation—are described in Chapter 55. Other techniques, used to repair sequelae related to atrial and arterial switches, are described in Chapters 7, 12, and 52., , RESULTS, Early surgical mortality in adults after repair of the associated, anomalies found with CTGA is not well documented but, is likely very low, similar to that associated with repair of, these anomalies in younger patients. In the series of 44, patients (mean age 44 years) presenting in adulthood for, surgery to correct various associated structural anomalies, (atrial septal defect, VSD, severe aortic regurgitation, severe, tricuspid regurgitation, severe mitral regurgitation, severe, pulmonary stenosis), there were no early deaths (CL, 0%-4.2%).B11 Four patients who underwent tricuspid valve, replacement for severe regurgitation required cardiac transplantation at a mean interval of 56 months after initial, surgery. In another series, early mortality for VSD closure, and placement of a left ventricular–to–pulmonary trunk, conduit in adults was 5.8% (3/52; CL 2.6%-11%).C35 Isolated tricuspid valve replacement can be performed with low, mortality. Mongeon and colleagues reported no early deaths, (CL 0%-4.0%) in 46 patients (mean age 34 years) and actuarial survival of 69% at 10 years.M33 Patients with initial right, ventricular ejection fraction greater than 45% had better, 10-year survival than those with lower ejection fraction, 94%, vs. 55%., Coronary artery bypass grafting for arteriosclerotic coronary artery disease has been reported in a 47-year-old diabetic, adult with CTGA.B4, The double switch in adult patients with naturally prepared left ventricles has an early mortality that is probably, similar to that in younger patients, about 2%. Extrapolating, from experience with atrial switch conversions, requiring in, , CTGA left ventricular training, 50% will meet criteria to, proceed to the double switch, for which early mortality is, about 20%., , INDICATIONS FOR OPERATION, Surgery may be indicated in adults with unrepaired CTGA if, additional structural anomalies are present and are causing, symptoms, or if there are no additional structural anomalies, but the systemic RV is failing. Options for surgery if structural, anomalies are present include isolated surgical repair of the, anomaly or anomalies causing the symptoms, double switch, with concomitant management of the associated anomaly or, anomalies, and cardiac transplantation., Criteria for isolated VSD closure, pulmonary stenosis, repair, or systemic tricuspid valve repair or replacement for, regurgitation are generally similar to the criteria used for, similar isolated anomalies in otherwise normal hearts;, however, there are some notable modifications. The status of, the systemic RV must be considered carefully when a small, VSD is present. The already afterload-strained ventricle may, not compensate for even a small volume load; thus, small, VSDs may require closure if the systemic RV is dilated or, dysfunctional. The same argument can be used when assessing systemic tricuspid valve regurgitation. Tricuspid valve, repair is difficult in the systemic tricuspid valve, especially if, there are structural valve abnormalities. Valve replacement is, usually the procedure of choice. Pulmonary stenosis, on the, other hand, may be better tolerated with a morphologic left, ventricle in the pulmonary circulation, and surgical intervention may not be necessary for moderate or even moderate to, severe obstruction., The double switch is an option that should be considered, in selected cases. It is an attractive option if the left ventricle, is already prepared for a systemic workload due to a combination of VSD and pulmonary or subpulmonary stenosis (see, Special Situations and Controversies in Section XI of this, chapter). The double switch is less attractive when the left, ventricle is unprepared for the systemic workload, as is the, case when there are no, or minor, associated structural anomalies. The double switch in this setting will require preparatory left ventricular training (see Section XII, Transposition, of the Great Arteries, earlier in this chapter). In unrepaired, adults without associated structural anomalies, there is no, indication for surgery if systemic RV function and tricuspid, valve function are normal or near normal. Occasionally, such, patients will present with a request for double switch evaluation based on their own research. This approach may be, pursued if the patient is fully informed of the left ventricular, training process, including its risks, time course, likelihood of, multiple banding procedures, and likelihood of success to, double switch., In unrepaired adults without associated structural anomalies who have untreatable systemic RV failure, the options are, left ventricular training and double switch or cardiac transplantation. The considerations are similar to those used to, decide between these two options in patients with failing, systemic RVs following the atrial switch for simple transposition (see Section XII, Transposition of the Great Arteries,, earlier in this chapter). Double switch is not a good option, when isolated pulmonary valve stenosis is present. Although, the left ventricle is prepared, absence of a VSD renders a, Rastelli impossible, and the pulmonary stenosis renders the, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, arterial switch impossible unless a prosthetic root replacement, is concomitantly performed. Occasionally, the pulmonary, valve itself is normal or near normal, and there is isolated, subvalvar left ventricular outflow tract obstruction. The, double switch is a good option in this case, because the left, ventricle is prepared, the arterial switch is possible, and the, left ventricular outflow tract resection is relatively simple., In selected unrepaired adults without associated structural, anomalies who have important tricuspid regurgitation but, preserved RV function (ejection fraction > 45%), tricuspid, valve replacement may be considered instead of the double, switch or transplantation.M33 There is a single case report of, a 64-year-old patient who presented with complete heart, block and severe tricuspid valve regurgitation who underwent, placement of a sequential AV pacemaker, resulting in markedly improved tricuspid valve function.K21, In adults with previous surgery for CTGA in whom the, systemic ventricle remains the morphologic RV, indications, for surgery and surgical options are generally the same as, for unrepaired patients. Patients with left ventricle–to–, pulmonary trunk conduits in place will eventually require, conduit replacement for conduit failure. Moderate and moderate to severe conduit obstruction may be well tolerated, when the left ventricle is in the pulmonary circulation, and, in fact some elevation of left ventricular pressure may be, beneficial, helping to stabilize the ventricular septum in midposition, thereby preventing systemic tricuspid valve regurgitation. When severe conduit obstruction is present and, surgery is necessary, the fact that the left ventricle is prepared, for the systemic circulation may make the double switch seem, attractive; however, presence of the left ventriculotomy (from, the conduit) and lack of a straightforward option for creating, left ventricle to aortic continuity make the double switch, inadvisable in most cases., In adults with previous double switch surgery for CTGA,, indications for surgery are a combination of the indications, for surgery in patients with simple transposition and prior, atrial switch and in patients with simple transposition and, prior arterial switch. (See Section XII, Transposition of the, Great Arteries, earlier in this chapter.), , Congenital Heart Disease in the Adult, , MORPHOLOGY, A hallmark of Ebstein anomaly is its wide spectrum of morphologic deformity of the tricuspid valve and the associated, wide spectrum of physiologic derangement. Severity of morphologic deformity generally correlates with severity of valve, regurgitation, but there are exceptions. Unique to adults, the, chronic abnormalities of the right heart in this disease may,, over time, result in left ventricular alterations due to, ventricular-ventricular interaction.B13, , CLINICAL FEATURES AND DIAGNOSTIC CRITERIA, Presentation, Newly diagnosed adults commonly present with palpitations,, dyspnea, or fatigue following new onset of atrial tachyarrhythmias, such as atrial flutter, atrial fibrillation, or reentrant, tachycardia through an accessory pathway. In a group of, 72 unoperated adults aged 25 years or older followed for 8, years, all but 5 patients were initially in New York Heart, Association functional class I or II.A23 Atrial arrhythmia was, the most common clinical presentation in 51%, with flutter, and fibrillation accounting for about half of these. Preexcitation was present in 44% on electrocardiogram. Onset of atrial, arrhythmia commonly causes patients to progress to a worse, functional class. Occasionally, adults present with untreated, severe disease manifested by severe exercise intolerance, cyanosis, low cardiac output due to right heart failure, hepatic, and renal compromise, and uncontrolled atrial and ventricular arrhythmias. Sudden death occurs and is associated with, arrhythmias. Overall prognosis is poor in this subset of, patients (Fig. 29-40)., Adults with previously diagnosed relatively mild disease, will not have had prior surgery. These patients may present, in adulthood with progression of cyanosis, progression of, , 1.0, , Section XIV, , Ebstein Anomaly, , DEFINITION, Definition, morphology, and basic physiology of Ebstein, anomaly are described in Chapter 42. Of adults presenting, with congenital heart disease, Ebstein anomaly is uncommon because it accounts for only about 1% of all congenital, heart disease,P8 and about 50% of those born with this, anomaly do not survive to adulthood, especially those with, a critical neonatal presentation.C10,Y5 About 10% of patients, will present for the first time in adulthood.C9 Reflecting the, wide spectrum of physiologic derangement, the presentation, in adults may be newly diagnosed primary disease, previously, diagnosed primary disease with benign physiology, or secondary disease following earlier surgery. Some patients with, severe Ebstein anomaly enter a single-ventricle surgical management strategy in childhood. They will not be discussed, in this section., , Probability of death, , .8, , .6, Overall, group, , .4, , Echo group, 3, 2, 1, , .2, , 0.0, 0, , 5, , 10, Years of follow-up, , 15, , 20, , Figure 29-40 Probability of death according to severity of tricuspid, valve deformity in 72 adult patients with Ebstein anomaly. Tricuspid, valve deformity was estimated by echocardiographic measurement, of amount of displacement of septal leaflet from true valve anulus., Patients were then placed in Group 1 (mild deformity), Group 2, (moderate), or Group 3 (severe). (From Attie and colleagues.A23), , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1119

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1120, , PART VI, , Congenital Heart Disease in the Adult, , right heart failure symptoms, new-onset arrhythmias, or occasionally, paradoxical embolism., Adults with prior surgery that included placing a bioprosthetic valve in the tricuspid position usually present with valve, degeneration, manifested by a combination of predominant, stenosis and some regurgitation. Adults with prior surgery, that included tricuspid valve repair may also present with, progressive tricuspid valve disease; however, recurrent regurgitation predominates, although stenosis after aggressive, repair can also occur. Cyanosis in previously repaired patients, is unusual because most surgical protocols involve closure of, atrial-level shunts. Many repaired patients may have received, a bidirectional superior cavopulmonary anastomosis as part of, initial repair; upper body swelling, headache, and superior, caval aneurysm may develop., Some patients with complex disease have also received, right ventricle–to–pulmonary trunk reconstructions in childhood. This will have important implications during adult life,, creating sequelae such as pulmonary valve regurgitation,, recurrent pulmonary stenosis, or conduit degeneration., Patients with associated anomalies such as pulmonary stenosis or atresia, and left ventricular myocardial abnormalities, such as noncompaction, typically have symptoms early in life, and thus do not present in adulthood without prior surgery., Adults with prior surgery may also present with new-onset, atrial or ventricular arrhythmias and right heart failure., , Electrophysiologic evaluation should be considered in all, adults presenting with Ebstein anomaly and is strictly indicated if there is evidence of arrhythmia by history, ECG, or, Holter monitoring., , NATURAL HISTORY, Adults with mild deformity of the tricuspid valve may, be asymptomatic and have normal survival (see Fig., 29-40).A23,S20 They typically have normal systemic arterial, oxygen saturation, either without an atrial septal defect or, with an atrial septal defect and left to right shunting. Patients, with cyanosis from right to left shunting typically present, earlier in life, partly because the cyanosis is obvious, but, also because cyanosis is associated with a more severe form, of the disease., , TECHNIQUE OF OPERATION, Surgical techniques used in adults with Ebstein anomaly, are the same as those used in children (see Chapter 42)., Surgery in adults with unrepaired Ebstein anomaly may, include the following core procedures in various combinations, with the specific combination of procedures applied to, an individual patient depending on the details of that patient’s, physiologyM5:, ■, , Diagnosis, , ■, , The echocardiogram may show preexcitation due to a rightsided accessory bypass tract,S39 prominent P waves, and right, bundle branch block. The chest radiogram will show a variable degree of cardiomegaly due to right atrial enlargement,, depending on severity of the morphologic and physiologic, derangement. Echocardiography is diagnostic and in many, cases is sufficient to proceed to surgery.R5,S43,T27 It shows the, structural alterations in the tricuspid valve, including apical, displacement of the septal and posterior leaflets and the large, abnormal anterior leaflet, the atrialized portion of the right, ventricle, enlarged right atrium, and status of the atrial, septum. It also provides physiologic information, including, amount of tricuspid regurgitation, status of right ventricular, function, and presence and direction of atrial-level shunting., Other associated anomalies, such as pulmonary stenosis or, regurgitation, ventricular septal defect, and left ventricular, noncompaction, can also be identified. Cardiac catheterization may be indicated if there is unexpected ventricular dysfunction or concern for arteriosclerotic coronary artery, disease; assessment includes measurement of ventricular enddiastolic pressure and imaging of the coronary arteries. It is, also indicated to calculate pulmonary vascular resistance if a, bidirectional cavopulmonary anastomosis is being considered, as part of the repair. Magnetic resonance imaging is capable, of providing definitive information for diagnosis of Ebstein, anomaly, including demonstration of all pertinent structural, alterations discussed under echocardiography; additionally, it, may provide quantitative functional information superior to, that provided by echocardiography (Fig. 29-41).E7, Formal exercise testing may be helpful. In addition to measuring functional capacity, pulse oximetry can demonstrate, onset or worsening of cyanosis with exercise. This information may be helpful in determining the best operative approach, to the patient, as well as response to therapy., , ■, ■, ■, ■, ■, ■, , Tricuspid valve repair, Tricuspid valve replacement, Closure of atrial septal defect, Right atrial reduction plasty, Bidirectional cavopulmonary anastomosis, Maze procedure, Targeted cryoablation, In adults presenting with unsalvageable right heart dysfunction, the Fontan operation can be consideredP16, , In general, decision making with respect to various core, procedures that should be incorporated into the operation in, an individual patient is similar to but not exactly the same as, that in children. Repair of the tricuspid valve is preferred, with, replacement the alternative if repair is not possible. This, preference, however, is more difficult to justify in adults than, it is in children because growth and rapid calcification of, bioprostheses resulting from active calcium metabolism are, not of concern. In adults with severely deformed tricuspid, valves, choice between a marginally adequate repair and a, bioprosthesis may favor a bioprosthesis. Interestingly, Dearani, and colleagues have shown that in 294 adults (mean age 20, years), 12-year freedom from reoperation was identical in, patients with initial valve repair versus replacement (Fig., 29-42).D9 Both bioprosthetic and mechanical valves have, been used when valve replacement is required; however,, long-term survival is much better when bioprosthetic valves, are used.B32, Surgical electrophysiologic procedures are more likely, to be part of the operation in adults than in children because, atrial electrical degeneration becomes more frequent over, time. A bidirectional superior cavopulmonary anastomosis, is less likely to be part of the operation in adults, because, individuals who reach adulthood without surgery generally, have less severe structural changes and thus a more functional, right heart., , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, , A, , B, , Figure 29-41 Magnetic resonance imaging (MRI) in 47-year-old, man with unrepaired Ebstein anomaly. A, Gated Tl-weighted, spin-echo four-chamber view demonstrating right atriomegaly, and inferior insertion of tricuspid valve leaflets within atrialized, right ventricular cavity. B, Four-chamber view from cine-MRI, demonstrating systolic flow dephasing (arrow) arising within, right ventricular cavity, suggestive of Ebstein anomaly. C, Sagittal, oblique view showing use of a selective saturation pulse through, right atrium parallel to atrial septum (curved arrow). Signal loss, (arrow) indicates right-to-left shunting through a small atrial, septal defect. (From Eustace and colleagues.E7), , C, , 100, 80, Survival (%), , Congenital Heart Disease in the Adult, , 60, 40, 20, 539, , 0, , 332, , 0, , 5, , 200, , 121, , 54, , 10, , 15, , 20, , Years, , Figure 29-42 Survival of 539 Ebstein anomaly patients (mean age, 24 years at time of surgery) after surgical correction. (From Brown, , and colleagues.B31), , Occasionally in mild cases of Ebstein anomaly, atrial septal, defect may be the dominant structural anomaly. If there is a, left-to-right shunt with both rest and exercise, catheter-based, intervention and closure is appropriate. If cyanosis at rest, is present, catheter-based intervention is contraindicated;, surgery with a more extensive procedure than atrial septal, defect closure alone is required and commonly will include, addressing the tricuspid valve and performing a bidirectional, cavopulmonary anastomosis. If there is left-to-right shunting, at rest but right-to-left shunting with exercise, surgery is, preferred, but catheter-based intervention may be considered, in selected cases., , RESULTS, Early mortality following initial surgery to correct Ebstein, anomaly in adults ranges from 0% to 5%.D3,T1,W28 In the largest, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1121

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PART VI, , Congenital Heart Disease in the Adult, , series of adult patients (539 patients, mean age 24 years),, early mortality was 5.9% (CL 4.9%-7.2%); however, this experience dates back to 1972.B31 For patients undergoing surgery, between 2001 and 2006 in this series, early mortality was, 2.7%. Twenty-year survival was 71% (Fig. 29-43). Preoperative risk factors for overall mortality were left or right ventricular systolic dysfunction, increased hemoglobin, male sex,, right ventricular outflow tract obstruction, and hypoplastic, pulmonary arteries., In another series of 40 patients undergoing initial surgical, correction at a mean age of 41 years, early mortality was 5%, (CL 1.7%-11%), and 5-year survival was 84%.A11 Survival was, similar for patients receiving a concomitant superior bidirectional cavopulmonary anastomosis (23/40) and those who, did not (17/40) (Fig. 29-44). Importantly in this study, the, decision to include the cavopulmonary anastomosis was based, on preoperative assessment of high operative risk (based on, tricuspid valve and right ventricular characteristics), and thus,, the authors inferred that the cavopulmonary anatomosis had, , 100, Free of reoperation (%), , 83%, 80, , 82%, , 60, 40, Tricuspid repair (n130), Tricuspid replacement (n164), PNS, , 20, 0, , 0, , 2, , 4, , 6, , 8, , 10, , 12, , Years since surgery, , Figure 29-43 Freedom from reoperation after initial tricuspid valve, replacement compared with that after initial tricuspid valve repair, in 294 patients (mean age 20 years at surgery) with Ebstein anomaly., (From Dearani and colleagues.D9), , 1.0, .8, , Survival, , 1122, , .6, .4, .2, 0.0, 0, , Numbers at risk, With CPS 20, Without CPS 16, , 1, , 2, 3, 4, 5, Follow-up after surgery (years), 18, 15, , 17, 14, , 6, 17, 14, , Figure 29-44 Comparison of survival between patients with (red, line) and without (blue line) superior cavopulmonary shunts (CPS), in 36 adults undergoing surgical correction of Ebstein anomaly., (From Al-Najashi and colleagues.A11), , a beneficial effect on survival. Chauvaud and colleagues prospectively assigned high-risk patients to two groups, those, receiving a superior cavopulmonary anastomosis and those, who did not.C15 They showed a substantial early survival, advantage for the group receiving the cavopulmonary anastomosis. Marianeschi and colleagues also showed that use, of the superior bidirectional cavopulmonary anastomosis, reduced the need for tricuspid valve replacement.M10, Early mortality in adults undergoing initial surgical repair, is lower than that in neonates and infants, and probably lower, than that in children. The reason is that a relatively mild form, of the disease usually exists in patients who initially present, in adulthood. This advantage is partially offset, however, by, an increase in associated degenerative changes over time in, the myocardium and electrical system.C9, Functional status, substantially below normal in unrepaired adults, improves following surgery, as measured both, by subjective patient response and objective exercise, testing.M45,T21, Khositseth and colleagues reported on 83 Ebstein patients, (mean age 28 years) with preoperative indication for concomitant arrhythmia surgery.K12 A variety of surgical arrhythmia procedures were performed, including ablation of, accessory pathways, perinodal ablation for atrioventricular, nodal reentrant tachycardia, and the maze procedure for atrial, flutter or fibrillation. At a mean follow-up of 34 months,, there was no recurrence of accessory pathway tachycardia or, reentrant tachycardia, and a 25% recurrence of atrial flutter, or fibrillation. Early mortality for concomitant arrhythmia, procedures was 4.8% (CL 2.5%-8.6%)., Freedom from reoperation after bioprosthetic tricuspid, valve replacement was 81% at 15 years in a series of 158, patients (mean age 19 years at surgery).K17, , INDICATIONS FOR OPERATION, In unrepaired adult patients, cyanosis is an indication for, surgical intervention. Progressive cardiomegaly due to tricuspid regurgitation, with right atrial and ventricular enlargement, is a relative indication for surgery, even if there are no, symptoms. The natural history of the disease suggests that, development of atrial flutter or fibrillation is likely if these, findings are present. New onset of atrial arrhythmias in previously asymptomatic patients with relatively mild structural, disease is an indication for surgery. Any patient with important symptoms or reduced functional capacity should undergo, surgery. These specific indications are no different from those, in younger patients with Ebstein anomaly, described in, Chapter 42., In adult patients with prior repair, the preceding indications all apply. Progressive regurgitation of a previously, repaired valve has the same sequelae as an unrepaired regurgitant valve. Patients with prior tricuspid valve replacement, will eventually present with a degenerated bioprosthesis., Most commonly, stenosis will predominate, but mixed stenosis and regurgitation is almost always present. Occasionally,, regurgitation will predominate. Predominant prosthetic valve, regurgitation presents like native valve regurgitation, and, indications for intervention are similar. Predominant prosthetic valve stenosis is an indication for surgery if the mean, gradient is 10 mmHg or greater or if symptoms of reduced, functional capacity, peripheral edema, or hepatic congestion, are evident., , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, In adult patients with prior tricuspid valve repair, repeat, valve repair may be indicated. However, there is a higher, chance that valve replacement will be required than in primary, surgery cases. In adult patients with degenerated prostheses,, re-replacement is the only option., , Congenital Heart Disease in the Adult, , may occur at the ostium, or even in the sinus, or the problem, may be more distal because of arteriopathy., , CLINICAL FEATURES AND DIAGNOSTIC CRITERIA, Presentation, , Coronary Artery, Problems Related to Congenital, Heart Disease, Section XV, , DEFINITION, Definition, morphology, and basic physiology of congenital, coronary artery anomalies are described in Chapter 46. Presentation of coronary artery problems related to congenital, heart disease in the adult may be as newly diagnosed primary, disease (primary congenital coronary anomalies), previously, diagnosed primary disease with benign physiology (primary, congenital coronary anomalies), or secondary disease (following congenital heart surgery for other anomalies). Coronary, artery problems in the adult may also result from sequelae of, acquired pediatric diseases in otherwise structurally normal, hearts (Kawasaki disease).K7, , MORPHOLOGY, Coronary artery problems related to congenital heart disease, in the adult are uncommon and can be categorized into two, main groups. One category includes the primary congenital, coronary anomalies. The most important of these are coronary artery arising from the wrong aortic sinus, coronary, artery arising from the pulmonary artery, and coronary artery, fistula. Coronary artery fistulas are the most common of, these, accounting for about half of all anomalies in adults. A, more complete listing of coronary artery anomalies is shown, in Box 29-5.A14 These anomalies can be grouped into a fourtiered clinical-significance–based classification, as shown in, Table 29-12. Table 29-13 lists the 62 anomalies (1.2%) found, in one study of 5100 coronary angiograms. Mean age of, the patients was 65 years. During a 5-year follow-up period,, survival was 83%, and 71% of all cardiac events and 100%, of all deaths occurred in patient with class III and IV, anomalies., The second category of coronary artery problems related, to congenital heart disease encompasses otherwise normal, coronary arteries that are put at risk, either as a result of surgical procedures used to repair other congenital heart anomalies, (e.g., arterial switch operation, Ross operation) or as the, natural progression of certain congenital heart diseases (e.g.,, supravalvar aortic stenosis). Accelerated obstructive arteriosclerotic coronary artery disease, which can develop in patients, with left-sided obstructive problems such as aortic coarctation, will not be discussed further in this section (see Section, X earlier in this chapter). In patients with a postsurgical etiology, coronary obstruction is usually ostial or very proximal, and results from manipulation and translocation of the coronary arteries, which cause some combination of malposition,, scarring, torsion, tension, ischemia, or thrombosis. In the, cases that occur in supravalvar aortic stenosis, obstruction, , Patients with coronary anomalies of any kind may present, with classic signs and symptoms of coronary insufficiency,, including angina, ventricular arrhythmia, sudden death, left, ventricular dysfunction, or mitral regurgitation, or these, anomalies may be more occult if coronary obstruction, or insufficiency is less severe, completely absent, or develops, slowly, allowing coronary artery collaterals to develop., Many of the more minor coronary anomalies are found, incidentally., Patients with coronary artery fistulas present with chest, pain or dyspnea in 71% of cases, and with either fatigue,, palpitations, heart failure, or syncope in another 20%.W4 Only, 9% are asymptomatic. Symptoms are much more likely in, adults than in children, who are asymptomatic 79% of the, time. In adults, spontaneous closure occurs in 3%, rupture in, 4%, and aneurysm in 14%. Endocarditis has been reported., Fistulas are single in 74% to 90% of cases., Anomalous origin of a coronary artery from the pulmonary, trunk presenting as newly diagnosed disease in the adult will, rarely involve the left main coronary artery because the natural, history does not allow asymptomatic survival unless there is, associated stenosis of the anomalous ostium or pulmonary, hypertension. When it does present in the adult, severe left, ventricular failure is present (see Chapter 46).W4 Isolated, anomalous origin of the left anterior descending, circumflex,, or right coronary artery is more likely to present in the adult, (Figs. 29-45 and 29-46)., The presentation of a coronary artery arising from the, wrong aortic sinus is reviewed in detail in Chapter 46. Both, the left main coronary artery arising from the right aortic, sinus and the right coronary artery arising from the left, aortic sinus can be associated with ischemia, and particularly, with sudden death in young adult athletes either during or, immediately after exertion.W4, Kawasaki disease acquired in childhood may result in, chronic progressive degenerative changes in the coronary, arteries, leading to coronary ectasia, aneurysm formation,, stenosis, and occlusion. Aneurysms have a tendency to, thrombose and to develop stenosis at both their proximal and, distal ostia., Diagnosis, The electrocardiogram may be normal or may show changes, consistent with ischemia or myocardial infarction. Echocardiography and cardiac catheterization are indicated to establish etiology. Echocardiography may confirm the diagnosis,, especially if the etiology is a primary coronary anomaly (coronary artery arising from the wrong aortic sinus or from the, pulmonary trunk); however, more definitive imaging is almost, always required (see Chapter 46). Echocardiography is also, performed to assess function of the myocardium and valves., Cardiac catheterization with coronary angiography confirms, or establishes the diagnosis, providing important confirmation of the abnormality, whether that is an anomalous coronary origin, a fistula, an aneurysm, or a coronary obstruction, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1123

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1124, , PART VI, , Congenital Heart Disease in the Adult, , Box 29-5, , Classification of Coronary Anomalies Observed in Normal Human Hearts, , Anomalies of Origination and Course, , Absent left main trunk (split origination of LCA), Anomalous location of coronary ostium within aortic root or, near proper aortic sinus of Valsalva for each artery:, High, Low, Commissural, Anomalous location of coronary ostium outside normal, coronary aortic sinuses:, Right posterior aortic sinus, Ascending aorta, Left ventricle, Right ventricle, Pulmonary artery—variants:, LCA arising from posterior facing sinus (ALCAPA), Cx arising from posterior facing sinus, LAD arising from posterior facing sinus, RCA arising from anterior right facing sinus, Ectopic location (outside facing sinuses) of any coronary, artery from pulmonary artery:, From anterior left sinus, From pulmonary trunk, From pulmonary branch, Aortic arch, Brachiocephalic artery, Right carotid artery, Internal thoracic artery, Bronchial artery, Subclavian artery, Descending thoracic aorta, Anomalous origination of coronary ostium from opposite,, facing coronary sinus (which may involve joint origination or, adjacent double ostia)—variants:, RCA arising from left anterior sinus, with anomalous, course:, Posterior atrioventricular groovea or retrocardiac, Retroaortica, Between aorta and pulmonary arterya, Intraseptala, Anterior to pulmonary outflowa or precardiac, Posteroanterior interventricular groovea, LAD arising from right anterior sinus, with anomalous, course:, Between aorta and pulmonary artery, Intraseptal, Anterior to pulmonary outflow or precardiac, Posteroanterior interventricular groove, Cx arising from right anterior sinus, with anomalous, course:, Posterior atrioventricular groove, Retroaortic, , LCA arising from right anterior sinus, with anomalous, course:, Posterior atrioventricular groovea or retrocardiac, Retroaortica, Between aorta and pulmonary arterya, Intraseptala, Anterior to pulmonary outflowa or precardiac, Posteroanterior interventricular groovea, Single coronary artery, , Anomalies of Intrinsic Coronary Arterial Anatomy, , Congenital ostial stenosis or atresia (LCA, LAD, RCA, Cx), Coronary ostial dimple, Coronary ectasia or aneurysm, Absent coronary artery, Coronary hypoplasia, Intramural coronary artery (muscular bridge), Subendocardial coronary course, Coronary crossing, Anomalous origination of posterior descending from anterior, descending branch or septal penetrating branch, Absent posterior descending or split RCA:, Proximal distal posterior descendings, arising from, separate RCA sources, Proximal posterior descending arising from RCA, distal, posterior descending arising from LAD, Proximal posterior descending arising from RCA, distal, posterior descending arising from Cx, Absent LAD or split LAD:, Large first septal branch and small distal LAD, Double LAD, Ectopic origination of first septal branch, , Anomalies of Coronary Termination, , Decreased number of arteriolar/capillary ramifications (?), Fistulas from RCA, LCA, or infundibular artery to:, Right ventricle, Right atrium, Coronary sinus, Superior vena cava, Pulmonary artery, Pulmonary vein, Left atrium, Left ventricle, Multiple, right and/or left ventricles, , Anomalous Collateral Vessels, , Modified from Angelini and colleagues.A14, If a single common ostium is present, the pattern is considered to represent “single” coronary artery., Key: Cx, Circumflex; LAD, left anterior descending coronary artery; LCA, left coronary artery; PD, posterior descending branch; RCA, right coronary artery., , a, , (see Fig. 29-45). Magnetic resonance imaging and computed, tomography with angiography may add important details, regarding the morphology (see Fig. 29-46)., Asymptomatic adults known to have unrepaired coronary, artery origin from the wrong sinus, coronary arising from the, pulmonary trunk, or coronary fistula should be evaluated as, described in the previous paragraph. If the anomaly involves, origin of a coronary artery from the wrong sinus, exercise, stress testing or other tests designed to provoke ischemia are, , at best not helpful and may actually be misleading. Provocative tests for ischemia are not particularly helpful for coronary, artery arising from the pulmonary artery because the diagnosis alone is an indication for repair. Provocative tests may be, helpful in determining whether closure is indicated for small, or moderate-sized coronary fistulas., Asymptomatic adults with known history of previous, surgery or another condition that puts the coronary arteries, at risk should be managed with a high index of suspicion., , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, , Congenital Heart Disease in the Adult, , Table 29-12 Clinical Significance–Based Classification of, Coronary Artery Anomalies in the Adult, Class, , Coronary Artery Anomaly, , I. Benign, , Ectopic origin of LCx from right, sinus, Separate origin of LCx and LAD, Ectopic origin of LCx from RCA, Dual LAD types I-IV, Myocardial bridge (score < 5), , II. Relevant, , Coronary artery fistula, , Related to myocardial, ischemia, , LCx origin, , Single coronary artery R-L, I-II-III,, A-P, Ectopic origin of LCA from PA, Atretic coronary artery, Hypoplastic coronary artery, , III. Severe, , LCx, , Ectopic origin of LCA from right, sinus, , Potentially related to, sudden death, , Single coronary artery R-L, I-II-III,, A-P, Ectopic origin of LCA from PA, Atretic coronary artery, Hypoplastic coronary artery, , IV. Critical, , Class II and superimposed CAD, , Related to sudden death/, myocardial ischemia, and associated with, superimposed CAD, , A, , LAD, , Class III and superimposed CAD, , LCx origin, , From Rigatelli and colleagues.R12, Key: CAA, Coronary artery anomaly; CAD, coronary artery disease; LAD, left, anterior descending coronary artery; LCA, left coronary artery; LCx, left, circumflex coronary artery; PA, pulmonary artery; RCA, right coronary artery., , LCx, , Table 29-13 Occurrence of Different Types of Coronary Artery, Anomalies in 5100 Angiograms, Coronary Anomaly, , n, , Prevalence %, , Ectopic origin of LCA from right sinus, , 5, , 0.098, , 13, , 0.25, , Ectopic origin of LCx from right sinus, , Ectopic origin of LCx from RCA, , 7, , 0.13, , Atretic LCx, , 2, , 0.039, , Ectopic origin of RCA from pulmonary artery, , 1, , 0.020, , Ectopic origin of LCA from pulmonary artery, , 1, , 0.020, , Dual LAD, , 2, , 0.039, , Single coronary artery, , 5, , 0.098, , Ectopic origin of the RCA from left sinus, , 2, , 0.039, , Separated origin of LAD and LCx, Coronary artery fistula, Myocardial bridge, TOTAL, R12, , 16, , 0.31, , 2, , 0.039, , 6, , 0.11, , 62, , 1.21, , From Rigatelli and colleagues., Key: CAA, Coronary artery anomaly; CAD, coronary artery disease; LAD, left, anterior descending coronary artery; LCA, left coronary artery; LCx, left, circumflex coronary artery; RCA, right coronary artery., , RCA, , B, , Left anterior oblique (~35°), , Figure 29-45 Coronary angiography showing retrograde filling of, an anomalous left circumflex coronary artery (LCx) though collateral, vessels after injection of contrast into (A) left anterior descending, coronary artery (LAD) and (B) right coronary artery (RCA). Anomalous origin of LCx from right pulmonary artery is demonstrated., (From Korosoglou and colleagues.K23), , Patients who have had arterial switch surgery should undergo, noninvasive testing for myocardial ischemia every 3 to 5, years.W13 This recommendation is based on the established, occurrence of coronary artery occlusion and stenosis found, incidentally at cardiac catheterization after this operation.T2, If noninvasive testing is positive, further coronary imaging as, described earlier in this section is indicated. Asymptomatic, patients who have undergone a Ross procedure should have, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1125

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1126, , PART VI, , Congenital Heart Disease in the Adult, arteriopathy and known occurrence of coronary events associated with this anomaly.W13, , TECHNIQUE OF OPERATION, , Right pulmonary, artery, , Surgical techniques used to treat adults with primary congenital anomalies of the coronary arteries are described in, Chapter 46. Surgical techniques used for coronary artery, obstruction in adult patients who have undergone surgery for, a congenital heart defect are described in Chapter 7., , RESULTS, LCx, LCx origin, , A, , Early mortality following surgery for congenital anomalies, and congenitally associated anomalies of the coronary artery, system in adults is not well documented. Based on outcomes, for similar problems in children, and on outcomes for adults, with arteriosclerotic coronary artery disease and good ventricular function, a mortality of 1% or less represents a reasonable estimate. Long-term outcome in adults is not available., Early mortality for ligation of coronary artery fistulas in adults, ranges from 0% to 4%.C4,S3,W4, , INDICATIONS FOR OPERATION, , RCA, , LAD, , B, Figure 29-46 Multiplanar reformatted cardiac magnetic resonance images (MRI) depict exact origin of (A) anomalous left circumflex coronary artery (LCx) from right pulmonary artery and, confirm normal origin and course of (B) left anterior descending, coronary artery (LAD) and right coronary artery (RCA) in a 54-yearold man. (From Korosoglou and colleagues.K23), , imaging of their coronary arteries at least once during adult, life.W13 Often this can be timed to be part of the preoperative, evaluation in preparation for right ventricular outflow tract, conduit revision. Asymptomatic patients with a history of, repaired or unrepaired supravalvar aortic stenosis should, undergo noninvasive testing for myocardial ischemia as adults, every 1 to 2 years, based on the known association of diffuse, , Surgical correction is indicated for all coronary arteries, arising from the pulmonary trunk, for all symptomatic, patients with coronary arteries arising from the wrong sinus,, and for asymptomatic patients age 50 years or younger with, coronary arteries arising from the wrong sinus if the coronary artery passes between the great arteries, is intramural,, or has evidence of angiographic narrowing (see Chapter 46)., Coronary artery fistula closure, either by surgery or by, catheter-based intervention, is indicated for patients with, symptoms attributable to the fistula, regardless of the size of, the left-to-right shunt, and for asymptomatic patients if the, fistula causes a large left-to-right shunt. Catheter-based, closure of a coronary artery fistula can be effectiveA18,M18 and, is indicated if morphologic details allow occlusion without, interruption of nutrient branches of the affected coronary, artery. Currently, this is possible in a minority of cases. In a, literature review of coronary artery fistulas in adults covering, 1993 and 2004, 107 fistulas were identified.S3 Definitive, management was noted in 67%, with 38% closed surgically,, 24% medically managed, and 5% closed by percutaneous, embolization., If coronary abnormalities are detected in patients with a, history of supravalvar aortic stenosis, management will depend, on the nature and severity of the abnormality. Obstructive, lesions should be managed like other more common arteriosclerotic lesions, with either coronary artery bypass grafting, or catheter-based dilatation or stenting (see Chapter 7)., Ostial reconstruction for ostial occlusion, more commonly, recommended in children, remains an attractive option in the, adult, along with bypass grafting., Indications for intervention for coronary obstructive, lesions identified in patients who have undergone an arterial, switch operation or Ross procedure are:, ■, , ■, , Any obstructive lesion associated with symptoms of, ischemia, Asymptomatic lesions with 50% or greater luminal, narrowing, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, ■, , Asymptomatic lesions with demonstrable flow dis­, turbance, , Intervention may be by surgery or catheter-based balloon, dilatation or stenting, depending on the position and nature, of the lesion (see Chapter 7).A1,H10,R2, Surgical management of coronary involvement in Kawasaki disease is limited and decision making complex. If, ischemia is present, bypass grafting or catheter balloon dilatation and stenting may be indicated. Large saccular aneurysms, may be asymptomatic but carry a risk of rupture, thrombosis,, and occlusion of adjacent coronary arteries. Aneurysm resection and coronary reconstruction or bypass grafting may be, indicated., , Section XVI, , Single Ventricle, , DEFINITION, Definition and details of single-ventricle physiology are, described in Chapter 41., , MORPHOLOGY, A spectrum of morphologic entities falls under the category, of single-ventricle physiology. The most common is hypoplastic left heart syndrome (see Chapter 49), followed by, tricuspid atresia (see Chapter 41) and then by less common, entities, such as double inlet ventricle and common ventricle, (see Chapter 56), unbalanced atrioventricular septal defect, (see Chapter 58), certain forms of Ebstein anomaly (see, Chapter 42), and certain forms of pulmonary atresia with, intact ventricular septum (see Chapter 40). In addition, some, patients are managed as having single-ventricle physiology, even though their structural anomalies are characterized by, two well-formed ventricles and two well-formed atrioventricular valves, because the surgical reconstruction that would, be required to septate such hearts is either impossible or, inadvisable. An example would be some forms of double, outlet right ventricle with noncommitted ventricular septal, defect (see Chapter 53). Adults presenting with congenital, heart disease uncommonly will have single-ventricle physiology. This is because all morphologic entities that result in, single-ventricle physiology combined account for only a small, fraction of congenital heart disease. Furthermore, mortality, rates are high in infancy and childhood, with fewer than 50%, of patients surviving to adulthood. Nevertheless, the palliated, state of all survivors dictates that this small group of patients, will demand an inordinate amount of resources in the adult, congenital heart disease program., Adults with single-ventricle physiology rarely present with, newly diagnosed disease, previously diagnosed disease with, benign physiology, or previously diagnosed disease thought, to be too complex for intervention; only 13 adult patients, with mixed circulation were evaluated over a 10-year period, in one large adult congenital referral center.A12 The great, majority of patients will present in adulthood with secondary, disease following prior surgery. The prior surgery will by, necessity be palliative and will result in one of three basic, physiologic states:, , ■, ■, , ■, , Congenital Heart Disease in the Adult, , Palliation with separated circulations (Fontan cir­culation), Palliation with partially separated circulation (superior, cavopulmonary anastomosis, or Glenn, circulation), Palliation with completely mixed circulation and, controlled pulmonary blood flow (mixed circulation, with systemic-to–pulmonary artery shunt or pulmonary, trunk band), , The general goal in infancy and childhood is to achieve, Fontan circulation in all patients with single-ventricle physiology. Some patients will not meet criteria required to undergo, the Fontan and will reach final palliation with a superior, cavopulmonary anastomosis. Still others will not meet criteria, for a superior cavopulmonary anastomosis and will reach final, palliation with a systemic-to–pulmonary artery shunt or a, pulmonary trunk band., A deeper level of complexity exists in this patient population. Patients with Fontan circulation may have an atriopulmonary Fontan, a lateral tunnel Fontan, or an extracardiac, conduit Fontan. Additionally, the circulations may not be, fully separated if a fenestration was created as part of the, Fontan operation. Patients with Glenn circulation may have, a bidirectional cavopulmonary connection or a unilateral, cavopulmonary connection, and may or may not have additional sources of pulmonary blood flow through a surgically, created systemic-to–pulmonary artery shunt or pulmonary, trunk band, or through a congenitally stenotic pulmonary, valve. Patients with completely mixed circulation may, have a single source of pulmonary blood flow through a, shunt or band, or may have multiple sources of pulmonary, blood flow through various combinations of shunts and, bands. All these variations have particular importance in the, adult with respect to the nature of sequelae that will be, encountered., As a final level of complexity, single-ventricle patients with, any of these palliated states may have had arch reconstruction,, repair of anomalous pulmonary veins, valve repairs or replacements, or subvalvar ventricular outflow tract muscle resections. Some patients may have their morphologic tricuspid, valve, pulmonary valve, and morphologic right ventricle functioning in the systemic circulation. All these factors will have, management implications in the adult., Presentation, Single-ventricle patients may undergo evaluation for surgical, therapy in a number of circumstances:, ■, , ■, , ■, , ■, , ■, , Structural problems such as arch obstruction, bulboventricular foramen obstruction, valve regurgitation, or pulmonary artery obstruction regardless of mixed, partially, separated, or separated circulation, Systemic-to-pulmonary shunt or pulmonary trunk band, revision (or rarely, initial placement) in mixed circulation, patients, Advancement of mixed circulation patients to partially, separated circulation by performing a superior cavopulmonary anastomosis, Advancement of patients with partially separated circulation to separated circulation with a Fontan operation, Addition of a second source of pulmonary blood flow, in cyanotic patients with a superior cavopulmonary, anastomosis, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1127

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1128, , PART VI, ■, , Congenital Heart Disease in the Adult, , Conversion of an atriopulmonary connection Fontan, to an extracardiac conduit or lateral tunnel connection, Fontan, , Adult patients with mixed circulation can present in a, variety of ways. Progressive cyanosis is common, especially, in patients with a systemic-to–pulmonary artery shunt. This, often is accompanied by increasing exercise intolerance., Heart failure resulting from chronic cyanosis and volume, overload is also common. This may be accompanied by onset, of arrhythmias, atrioventricular valve regurgitation, and, increasing exercise intolerance as well. Patients with chronic, unrestricted pulmonary blood flow present with pulmonary, hypertension with Eisenmenger physiology. Infective endocarditis may develop, either at the shunt or band site or at an, abnormal valve. Paradoxical embolism may occur, resulting, in cerebrovascular events or other end-organ dysfunction., Thrombotic complications may develop as a result of increased, blood viscosity related to elevated hematocrit. Brain abscess, is also described in this population., Adult patients with partially separated circulation—those, with a bidirectional superior cavopulmonary anastomosis—, typically present with progressive cyanosis as upper body–, to–lower body venovenous collaterals and pulmonary, arteriovenous malformations develop. Because of the cyanosis, and partial mixing of the circulations, all of the sequelae, described for mixed circulation patients can also occur. Unless, there is an additional source of pulmonary blood flow, superior cavopulmonary anastomosis patients will not have a, volume-loaded ventricle, and thus ventricular failure is less, common than in mixed circulation patients., Adult patients with separated circulation—those with, a Fontan—also present in a variety of ways. At baseline,, they have greatly diminished lung function and aerobic, capacity.F16,H8 Those with an atriopulmonary Fontan connection inevitably develop atrial dilatation, which is associated, with atrial arrhythmias, atrial thrombus with possible pulmonary embolism, and right pulmonary venous obstruction., Low cardiac output commonly develops in adult patients,, regardless of the Fontan connection, and is associated with, exercise intolerance, protein-losing enteropathy, hepatic, congestion and dysfunction, peripheral edema, pleural effusion and ascites, and plastic bronchitis. Obstruction in the, Fontan pathway may accelerate these signs and symptoms,, as may progressive elevation of pulmonary vascular resistance, from chronic pulmonary emboli. Atrial shunting resulting, in cyanosis is unusual; however, venovenous connection, from the systemic circulation to the pulmonary veins may be, a cause of cyanosis. Patients with lateral tunnel and extra­, cardiac conduit Fontan connections do not develop atrial, dilatation and thus are less likely to develop atrial thrombus, with chronic pulmonary emboli and atrial arrhythmias.D11,M7, Atrial arrhythmias, however, such as flutter, fibrillation, and, sinus node disease leading to junctional rhythm, can occur., Low cardiac output, and its sequelae as described for atriopulmonary Fontan connection patients, also develops, but, more slowly., Diagnosis, The main utility of the electrocardiogram (ECG) is to identify, atrial and ventricular arrhythmias. Holter monitoring and, electrophysiologic evaluation may be indicated based on the, , ECG or symptoms. The chest radiograph is particularly, helpful in identifying and following pleural effusions in, Fontan patients. Effusions suggest low cardiac output and, failing Fontan circulation, and all of the sequelae associated, with it, including protein-losing enteropathy.M29 Echocar­, diography is the mainstay for evaluating and following, single-ventricle patients. The usual intracardiac structural and, functional evaluation of the ventricle, valves, aortic arch, and, arterial and venous connections is performed. In Fontan, patients, additional evaluation of the Fontan pathway is performed, looking for obstruction at the inferior caval and, pulmonary artery ends, for thrombus and low flow with spontaneous contrast formation, for flow characteristics and severity of dilatation of the inferior vena cava and hepatic veins,, and for the status of a fenestration if one is present., Cardiac catheterization is indicated in any symptomatic, single-ventricle patient to assess a number of variables,, including:, ■, ■, ■, , ■, ■, ■, ■, ■, , Ventricular hemodynamics, Coronary arteries, Pulmonary vascular morphology, pressure, and resistance, Cardiac output, ,  ratio, Qp/Qs, Status of surgical shunts, Bands and cavopulmonary connections, Assessment of pulmonary arteriovenous malformations,, venovenous abnormal connections, and systemic-to–, pulmonary artery collateral formation, , Cardiac catheterization is a necessity in any single-ventricle, patient with previously operated or unoperated mixed circulation, or partially separated circulation, who is being considered for Fontan completion surgery (see Chapter 41 for, criteria for proceeding to the Fontan). It is also a necessity, for any patient being evaluated for conversion from an atriopulmonary Fontan to either an extracardiac conduit or a, lateral tunnel Fontan., Magnetic resonance imaging (MRI) and computed tomography with angiography may be useful in single-ventricle, patients, usually to augment the structural and functional, echocardiographic evaluation, particularly in the pulmonary, arteries and veins. MRI is also useful for functional evaluation, of the ventricular myocardium., , NATURAL HISTORY, The best prognosis is for patients with S,L,L double inlet left, ventricle with moderate pulmonary stenosis. Survival into the, sixth decade is possible.A12, Ten-year survival after lateral tunnel and extracardiac, conduit Fontan connection, the predominant Fontan operations performed over the last 20 years, is 90% or greater,, much better than that observed after the atriopulmonary, Fontan.S44 Once low output and its sequelae develop, unless, there is a structurally correctable problem such as Fontan, pathway obstruction, valve regurgitation, or systemic ventricular outlet obstruction or coarctation, survival is limited,, with 5-year survival of about 50%. Survival in Fontan patients, nevertheless is much better than the approximately 50%, 20-year survival for patients with mixed circulation and partially separated circulation.W13, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. 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Chapter 29, The chronicity of the abnormal hemodynamic state associated with Fontan circulation leads to additional challenges for, adults not generally considered in younger patients. Prevalence and severity of chronic lower extremity venous insufficiency are much greater than in the normal population.V1, Also, intracardiac thrombus formation is noted in 12% of, adult Fontan patients when it is looked for, and is attended, by significant morbidity.T23 Atrial arrhythmias occur with, increasing frequency in adults, noted in 41% of Fontan, patients in one study, and arrhythmias were associated with, atrial thrombus.G13 In an analysis of all adult Fontan patients, presenting to a large clinic, 17% were found to have silent, pulmonary emboli.V11, , TECHNIQUE OF OPERATION, Surgical techniques, including systemic-to–pulmonary, artery shunt, pulmonary artery band, bidirectional superior, cavopulmonary anastomosis, and extracardiac conduit and, lateral tunnel Fontan operations, are described in, Chapter 41., Therapeutic catheter-based procedures may be used to, relieve various obstructions and to occlude abnormal, , A, , Congenital Heart Disease in the Adult, , venovenous connections, systemic-to–pulmonary artery, collaterals, surgical shunts, and Fontan fenestrations., The maze procedure is modified as appropriate for patients, with the variable morphologic features associated with the, spectrum of single-ventricle anomalies. Fig. 29-47 shows the, cryolesions for a right and left atrial maze procedure applied, to a patient with tricuspid atresia who is about to undergo, an extracardiac conduit Fontan., , RESULTS, Systemic-to–pulmonary artery shunt and pulmonary trunk, band procedures have a low surgical mortality in adults, without important comorbid conditions, similar to the mortality of these same procedures in children (see Chapter, 41). For shunt procedures it is about 5%, and for bands less, than 1%., Early mortality for the superior bidirectional cavopulmonary anastomosis and Fontan operation in adults is not well, documented, but should not differ from outcomes in younger, patients, about 2% to 5% for each, as long as selection criteria, are met. Observed mortality in most series is higher than this,, however, because these series span many decades, and the, , B, , Figure 29-47 Cryoablation left and right atrial maze (Cox-maze III) procedure in patient with tricuspid atresia undergoing an extracardiac, conduit Fontan conversion. A, Superior (SVC) and inferior vena cavae (IVC) have been detached from right atrium in preparation for extracardiac conduit Fontan, and cardiac stumps of SVC and IVC have been oversewn. Cryolesions are placed in four locations in the right atrium, at −60°C for 90 seconds each. In this patient with tricuspid atresia, a linear cryoprobe is used to ablate tissue between atrial septal defect, (ASD) and SVC stump, ASD and lateral edge of atriotomy, coronary sinus os and IVC stump, and area of atretic tricuspid valve anulus and, IVC stump. B, Left-sided cryolesions are placed for patients with preoperative atrial fibrillation. They are placed around the pulmonary veins,, at orifice of left atrial appendage, and between inferior pulmonary veins and mitral valve anulus. Appropriate adjustments, while adhering, to the basic principles of the maze, must be made in placing the cryolesions for other single-ventricle morphology, for example, in patients, with unbalanced atrioventricular septal defect or hypoplastic left heart. (From Weinstein and colleagues.W18), , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1129

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PART VI, , Congenital Heart Disease in the Adult, , outcomes often reflect the era. In the largest single-institution, study, 132 adults (mean age 23 years) underwent the Fontan, procedure between 1973 and 2001, with an early mortality, of 8.3% (CL 5.8%-12%).B34 This was similar to Fontan mortality in children over the same time period at the same institution. Early mortality improved in later years of the study. Late, survival is shown in Fig. 29-48, and causes of late mortality, are shown in Table 29-14. Risk factors for late mortality, included male gender, pulmonary artery pressure greater than, 15 mmHg, and age greater than 30 years., In the next largest series, Veldtman and colleagues reported, 61 cases (median age 36 years) with an early mortality of 13%, (CL 8.6%-19%); 15-year survival was 67%.V12 In this study,, when the Fontan group was compared with a group of adult, single-ventricle patients without Fontan circulation, the, Fontan group had better functional status, fewer arrhythmias,, better ventricular function, and less atrioventricular valve, regurgitation. Many reports include small numbers, and thus, mortality statistics vary widely. Van den Bosch reported on, seven adults undergoing the Fontan with one early death, (14%; CL 2.3%-41%), and in a mean follow-up period of 15, years, there were two additional deaths and one cardiac, transplantation.V3 Gates and colleagues reported on 21, patients with an early mortality of 4.8% (CL 0.8%-15%), with, 100, 80, Alive (%), , 1130, , (94), , 68%, , (63), , 60, , (27), , 40, 20, 0, , 0, , 5, , 10, , 15, , Years since surgery, , Figure 29-48 Survival, excluding early mortality, of 121 adult, patients after Fontan operation. Numbers in parentheses represent, patients remaining at risk. (From Burkhart and colleagues.B34), , Table 29-14 Causes of Late Mortality in 121 Adults Surviving, Hospital Discharge after Fontan Operation, Cause, , n, , Congestive heart failure, , 6, , Sudden/arrhythmia, , 5, , Protein-losing enteropathy, , 3, , Sepsis, , 3, , Pulmonary embolism, , 1, , Inferior vena cava thrombosis, , 1, , Tuberculosis, , 1, , Appendectomy complication, , 1, , Accident, , 1, , Unknown, , 10, , TOTAL, , 32, , From Burkhart and colleagues., , B34, , one late death over a mean follow-up time of 7.4 years.G3, Other small series show similar outcomes.F18,M43,O10, Podzolkov and colleagues reported an early mortality of, 6.9% (2/29; CL 2.4%-16%) for the superior bidirectional, cavopulmonary anastomosis in adults, and 8.3% for the, Fontan in adults. Both values were similar to their outcomes, in children.P13, Early mortality after conversion of the atriopulmonary, Fontan to the cavopulmonary Fontan ranges from 0% to, 25%.M8,M16,M29 The wide range of mortality is likely related to, the selection criteria used in individual series. In one of, the largest series (containing both children and adults),, Mavroudis and colleagues reported no early mortality, (CL 0%-4.6%) and one transplant in 40 conversions and, concomitant maze procedures (mean age 19 ± 9.0 years at, conversion).M17 At a mean follow-up of 2.5 years, functional, status was improved, and there was one death and two additional transplants. In another study of 27 conversions in, adults (mean age 30 years), there were two early deaths, (7.4%; CL 2.6%-17%). Both patients who died had liver cirrhosis and had been evaluated and turned down for transplantation. Functional status improved following conversion., Most patients in this series (78%) also had concomitant maze, arrhythmia surgery for atrial flutter or fibrillation, with late, arrhythmia recurrence of 14% at 4.2-year follow-up.A2 Another, study of 10 adults (mean age 21 years) undergoing conversion and concomitant cryoablation showed one early death, (10%; CL 1.6%-30%) and a second midterm death.W18, At follow-up, all survivors improved functionally, and, arrhythmia recurrence was 10%., Sheikh and colleagues reported 15 conversions (mean age, 20 years) with no early (CL 0%-12%) or late mortality and, improved functional status at a mean follow-up of 43 months., Eleven of the 15 patients had arrhythmia surgery as well, with, reduced requirement for medications at follow-up.S23 Mott, and colleagues reported no early (CL 0%-12%) or late deaths, in 15 conversions with concomitant arrhythmia surgery, (mean age 23 years) at 30-month follow-up.M43, , INDICATIONS FOR OPERATION, In adults with mixed circulation (usually previously operated,, but rarely with no surgical history) and unclear physiology,, full evaluation, including cardiac catheterization, will determine whether criteria are met to advance to a state of separated circulation. Criteria for advancing to a superior, cavopulmonary anastomosis are the same as those for infants, and children (see Chapter 41). If these criteria are met,, however, in contrast to the situation in young patients, a, superior cavopulmonary anastomosis will rarely be adequate, as the sole source of pulmonary blood flow, because of severe, cyanosis. A secondary source, either from a new or existing, systemic–pulmonary artery shunt or pulmonary trunk band,, or from native pulmonary blood flow across a naturally stenotic outflow tract to the pulmonary arteries, will be required., If the criteria are not met to advance to a separated circulation in the adult with mixed circulation, and the patient is, cyanotic due to reduced pulmonary blood flow, then further, surgical management with a systemic-to–pulmonary artery, shunt, or possibly a pulmonary trunk band revision (if one is, in place already), is indicated. If the patient is cyanotic because, of pulmonary hypertension, evaluation for heart-lung transplantation is the only surgical option., , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, If pulmonary overcirculation is present and pulmonary, vascular obstructive disease is not, then shunt revision or, pulmonary trunk band placement or tightening is indicated, as appropriate. Such patients can be further evaluated with, catheterization approximately 6 months after shunt or band, revision to assess criteria for advancing to a separated circulation. If pulmonary vascular obstructive disease is present in, the overcirculated patient, the only surgical option is heartlung transplantation., In adult patients with partially separated circulation, if, criteria are met at cardiac catheterization (see Chapter 41),, advancement to a separated circulation with a completion, Fontan operation is indicated. If the patient does not meet, criteria for a Fontan and is symptomatic from cyanosis, creation of an additional source of pulmonary blood flow (usually, a systemic-to–pulmonary artery shunt) is indicated. If pulmonary arteriovenous malformations are partially the cause of, cyanosis, the shunt should be placed to direct the systemic, flow primarily to the lung with the dominant malformations., Rarely, large isolated arteriovenous malformations can be, closed using percutaneous catheter-based methodology., In adult patients with separated circulation, surgical intervention may be indicated in several circumstances. Any significant structural cardiac problem or systemic arterial,, pulmonary arterial, systemic venous, pulmonary venous, or, Fontan pathway obstruction is an indication for surgical correction if catheter-based intervention is contraindicated or, unsuccessful. In symptomatic patients with low cardiac output, due to elevated pulmonary vascular resistance, a surgical fenestration may be indicated in selected cases. Adult Fontan, patients who are fully or nearly fully saturated, however, may, not tolerate the increase in cyanosis that attends fenestration., All adult atriopulmonary Fontan patients with acceptable, ventricular function and low pulmonary vascular resistance, should be considered for conversion to an extracardiac, conduit or lateral tunnel Fontan connection. Conversion is, strictly indicated if atrial flutter or fibrillation, pulmonary, venous compression, atrial thrombus, or structural problems, such as obstruction in the Fontan pathway are present.M8,M16, In all single-ventricle patients with severe ventricular, failure, regardless of mixed, partially separated, or separated, circulation, and normal pulmonary vascular resistance, the, only surgical option is heart transplantation.G2, If atrial flutter or fibrillation is present in single-ventricle, patients undergoing surgery to correct structural problems,, to advance to a more separated circulation, or to convert from, an atriopulmonary to a cavopulmonary Fontan, a maze procedure or one of its modifications is indicated as a concomitant procedure.M16, , Congenital Heart Disease in the Adult, , The reasons for this are that the valve obstruction is often, mild and self-limited, and percutaneous catheter-based valvotomy is often effective in those who need treatment., Morphology, Two common morphologic subtypes of isolated pulmonary, stenosis are recognized. The predominant type is the, doming valve with three fused commissures but underlying, well-developed valve cusp tissue. The less common type, (≈20%) is the dysplastic valve, defined by three cusps with, fully formed commissures but thickened immobile myxomatous cusp tissue. This type of valve is associated with Noonan, syndrome.N11 Occasionally, isolated pulmonary valve stenosis, will be caused by a bicuspid valve., Technique of Operation, Because surgery for pulmonary stenosis in the current era is, relegated to a second-choice role behind balloon valvotomy,, most patients presenting for surgery will have unfavorable, morphology and will likely require pulmonary valve replacement. The occasional patient with a doming valve that does, not respond to catheter-based balloon dilatation, and the, occasional patient with a doming valve who is undergoing, cardiac surgery for another anomaly, are candidates for, surgical commissurotomy. Most surgical cases, however, have, either dysplastic morphology or one or more associated conditions, such as anular hypoplasia or valve regurgitation, and, will require valve replacement. Occasionally, a dysplastic valve, without regurgitation and with adequate anular size will, respond to surgical valvuloplasty involving shaving of the, thickened cusps., Currently, the replacement option of choice is a bioprosthetic valve placed orthotopically. It may be necessary to, patch augment the anular and supravalvar component of the, right ventricular outflow tract or to resect muscle in the subvalvar component of the right ventricular outflow tract as, well. Surgical reduction of the pulmonary trunk may be considered when there is associated marked post-stenotic dilatation of the pulmonary trunk, but this procedure is truly, indicated only in the rare case when there are symptoms of, compression of other mediastinal structures. Dissection, true, aneurysm formation, and rupture of the pulmonary trunk are, not described when pulmonary artery pressure is normal, and, these potential complications should not be of concern., The typical doming valve is managed reasonably well by, catheter intervention in the adult., Results, , Other Anomalies, Rarely Requiring Surgery, in the Adult, , Section XVII, , ISOLATED PULMONARY STENOSIS, Definition, Isolated pulmonary stenosis is a common congenital heart, anomaly; however, it rarely requires surgery in the adult.H11, , In one series of 40 patients (age range 18-56 years),, the gradient was reduced by catheter intervention but not, eliminated (persistent gradient of 37 ± 14 mmHg).K8 All, patients had mild or less regurgitation, and there was a tendency for subpulmonic gradients to regress at 24-month, mean follow-up (Fig. 29-49). Other studies with followup of up to 10 years after balloon dilatation show similar, findings.C16,F3,F4,J2,K3,S22,S46,T7 Dysplastic valves are not as effectively treated by catheter-based balloon valvotomy, with generally unacceptable residual gradients.J2,S46, Balloon valvotomy for the doming valve is currently, the first line of therapy. Two studies comparing balloon, , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1131

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PART VI, , Congenital Heart Disease in the Adult, from reoperation are also extremely favorable, with no late, mortality and 95% freedom from reintervention at 25 years.P12, When valve replacement is necessary, long-term outcomes are, similar to those for other patients with bioprosthetic valves, in the pulmonary position., , 160, , Indications for Operation, There are several indications for surgical intervention for, isolated pulmonary stenosis:, , PG (mmHg), , 100, , ■, , ■, , ■, , 0, Before, , After, , Follow-up, , Figure 29-49 Peak pulmonary systolic gradient (PG) by echocardiography from right ventricle to pulmonary artery before, immediately after, and at mean follow-up of 24 months after balloon, pulmonary valvuloplasty in 32 adult patients. Bars represent mean, ± standard deviation. (From Kaul and colleagues.K8), , After catheter-based balloon dilatation with a residual, peak echocardiographic Doppler gradient of greater, than 60 mmHg in asymptomatic patients or a residual, peak gradient of more than 50 mmHg in symptomatic, patients, In any patient with associated severe pulmonary regurgitation, anular hypoplasia, fixed subvalvar stenosis,, supravalvar stenosis, or dysplastic valve morphology, If other cardiac anomalies requiring cardiac surgery, such, as coronary artery disease, ventricular septal defect, or, tricuspid regurgitation, are presentP12, , If there is an associated atrial septal defect, combined catheterbased closure and balloon pulmonary valvotomy may be, performed.M25,W2, , TOTAL ANOMALOUS PULMONARY, VENOUS CONNECTION, Definition, , (54), , Freedom from reintervention, , 1132, , 1.0, .9, .8, .7, .6, .5, .4, .3, .2, .1, 0, , (52), , (81), , (51), , (79), (63), , 0, , Surgical, , 10, , Balloonplasty, , Time (years), , 20, , Definition, morphology, and basic physiology of total anomalous pulmonary venous connection (TAPVC) are described, in Chapter 31., Morphology, , 30, , Figure 29-50 Freedom from reintervention in adults with isolated, pulmonary valve stenosis managed by surgery (n = 54) or balloon, valvuloplasty (n = 92). Numbers represent patients remaining at risk., (From Peterson and colleagues.P12), , treatment and surgery suggest that gradient relief and, freedom from reintervention are better with surgery (Fig., 29-50); however, although these differences are important,, they are small, and thus they are offset by a higher occurrence of important regurgitation, more ventricular ectopy,, greater cost, and increased invasiveness associated with, surgery.O1,P12, Surgery (pulmonary valvotomy) for unselected cases, of isolated pulmonary stenosis in the adult has an early mortality approaching zero.P12 Long-term survival and freedom, , It is rare for adults with TAPVC to survive into adulthood, unrepaired. A few isolated cases are reported. Characteristics, of individuals who survive include unobstructed supracardiac, form of the disease, large intraatrial communication, and low, pulmonary vascular resistance with increased pulmonary, blood flow, resulting in only mild cyanosis.A17, Clinical Features and Diagnostic Criteria, Clinical presentation is characterized by varying severity, of cyanosis and signs and symptoms of right heart and, pulmonary overcirculation. Echocardiography is diagnostic;, however, cardiac catheterization is mandatory to assess the, status of the pulmonary vascular bed. Magnetic resonance, imaging (MRI) and computed tomography (CT) may be, helpful., Natural History, Recurrent pulmonary vein stenosis presenting in adults with, a history of infant repair of TAPVC is rare. Recurrent obstruction following infant repair occurs in about 10% of cases in, which the total anomalous connection was an isolated lesion, (it is higher in single-ventricle and heterotaxy patients)., , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.

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Chapter 29, Furthermore, recurrence almost always occurs in the first 6, months after initial infant repair.H28,L2,R11, Technique of Operation, Surgical techniques used to repair this anomaly in adults are, the same as those used in infants (see Chapter 31)., Results, Early mortality approaches zero. Arikawa and colleagues, report two adult cases, aged 43 and 51 years, without, mortality and with uneventful postoperative courses and, improved functional status.A17 These authors also review the, Japanese literature and report on six additional patients over, age 40 years, with similar outcomes. John, Sukumar and, their colleagues note that presentation in late childhood and, adulthood implies a better prognosis than presentation in, infancy. They report no early or late mortality in the former, groups.J8 Berg and colleagues report on three patients, aged, 22, 29, and 33 years, all with minimal cyanosis at rest,, dyspnea, mild heart failure, and mild to moderate pulmonary, hypertension. One patient had gone through an uneventful, pregnancy. Another had concomitant important mitral, regurgitation that was surgically repaired. There was no early, or late mortality after repair, and all patients were functionally improved.B17, , VASCULAR RING AND ASSOCIATED, ARCH ANOMALIES, Definition, Definition, morphology, and basic physiology of vascular, ring are described in Chapter 51. Vascular rings rarely present, in adults. Most reported cases are single case reports., Morphology, In adults, the most common anomaly is double aortic arch, (≈45%), followed by right aortic arch with anomalous left, subclavian artery with ligamentum arteriosum (≈30%).G20, Clinical Features and Diagnostic Criteria, Vascular rings in adults may be found incidentallyK1,P3 or may, present with symptoms of dysphagia lusoria or upper respiratory obstruction.F1,G20,G21,M39,N4,S34 It is more common for dysphagia to be the presenting symptom in adults than in, children, although respiratory obstruction is still the most, common presentation in adults (42% present with respiratory, symptoms, 33% with dysphagia). Diagnosis can often be, made by chest radiography, contrast esophagogram, and echocardiography; however, MRI or CT are critical, allowing more, precise imaging of the vascular anomaly and also imaging of, the vasculature relationship to adjacent structures, including, the trachea and esophagus., Pulmonary artery sling is rare in adults. The literature, consists of case reports only. Most are asymptomatic, presenting as an incidental findingP21,S49; however, some present with, esophageal or tracheal obstructive symptoms, or both.L1, Diagnostic evaluation and indications for surgery are similar, to those for vascular rings., , Congenital Heart Disease in the Adult, , Natural History, Presentation in adulthood after vascular ring repair in childhood is rare. Ruzmetov and colleagues followed 183 childhood repairs for up to 35 years with no reports of late, complications.R24 There is a single case report of late presentation of subclavian steal syndrome in an adult who underwent, vascular ring repair in infancy.C30, Technique of Operation, All rings can be approached effectively through a median, sternotomy. However, the more traditional approach is, through a lateral thoracotomy (see Chapter 51).M39, Surgical management of pulmonary artery sling is described, in Chapter 51. In adults, in contrast to infants, tracheal, reconstruction is usually not necessary., Results, There are no reports of perioperative deaths in the modern, literature. Symptomatic improvement occurs reliably in, patients presenting with dysphagia, but less reliably in those, presenting with respiratory symptoms. In the case of late, presentation of subclavian steal syndrome, the original repair, involved division of the ligamentum and left subclavian artery,, without subclavian artery reimplantation.C30, Indications for Operation, Asymptomatic anomalies may be followed. Surgical repair is, recommended in the presence of important symptoms., , COR TRIATRIATUM, Definition, There are sporadic reports of cor triatriatum sinister pre­, senting in the adult, either in the form of isolated case, reports or included in single-institution (mostly) pediatric, series.C17,H22,K10,K26,O4,S5,V7 Cor triatriatum dexter has been, reported in adults, usually as an asymptomatic incidental, finding.M31,S6,Y4 When symptoms are present, they mimic right, heart failure., Morphology, In contrast to children (see Chapter 32), most adults do not, have complex associated cardiac anomalies; however, associated cardiac structural anomalies have been found in some, patients presenting in adulthood.C17, Clinical Features and Diagnostic Criteria, Presentation of isolated cor triatriatum sinister in the adult is, identical to that of mitral stenosis, including dyspnea, pulmonary hypertension, atrial fibrillation, right heart failure, and, hemoptysis. In mild cases, presentation may be incidental., Thrombus was present in the accessory chamber in one, adult.K10 Diagnosis can be made by echocardiography; however,, MRI and CT define the morphologic details with more, clarity. Cardiac catheterization is indicated to assess the state, of the pulmonary vasculature., , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved., , 1133

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1134, , PART VI, , Congenital Heart Disease in the Adult, , Cor triatriatum dexter has been reported in adults, usually, as an asymptomatic incidental finding.M31,S6,Y4 When symptoms are present, they mimic right heart failure., Technique of Operation, Surgical techniques are similar to those used in children (see, Chapter 32). If chronic atrial fibrillation or flutter is present,, a concomitant maze procedure should be performed., Results, In the few case reports documented in the literature, surgical, repair has been performed without early mortality and with, improvement in symptoms and reduction in pulmonary artery, pressure., Indications for Operation, Surgical repair is indicated if symptoms are present or if, other changes, such as thrombus formation, develop. Surgical, excision of cor triatriatum dexter is indicated if symptoms, are present., , SINUS OF VALSALVA ANEURYSM, Definition, Definition, morphology, and basic physiology of sinus of, Valsalva aneurysm are described in Chapter 36., Morphology, These anomalies are rare in adults. When they occur, they are, often associated with unicuspid or bicuspid aortic valves. The, aneurysm occurs most frequently in the noncoronary and, right coronary sinuses, but can occur in the left., Clinical Features and Diagnostic Criteria, Sinus of Valsalva aneurysms are generally asymptomatic until, they rupture. However, they may cause problems from a mass, effect without rupture, including coronary obstruction, aortic, regurgitation, right ventricular outflow obstruction, and ventricular tachycardia. Thrombus formation with subsequent, embolism can also occur. When rupture occurs, it is usually, into a right-sided chamber, creating an aortic to right atrial, or right ventricular fistula. Rupture may also cause cardiac, tamponade. Rupture usually presents as an emergency with, extreme hemodynamic instability. Sinus of Valsalva aneurysms are often detected incidentally or during imaging of, abnormal aortic valves., Natural History, The natural history of small and moderate aneurysms is, unknown, and the risk of rupture of large aneurysm, although, well documented, is not quantified., Technique of Operation, The appropriate surgical procedure will vary from simple, plication, to resection and sinus patch reconstruction,, , to aortic root replacement with coronary artery reimplanta­, tion.D10,H3,P11,R10,U1, Indications for Operation, Treatment options are well defined. If surgery is required for, aortic valve disease or any other cardiac problem, sinus of, Valsalva aneurysms of any size should be addressed. If no, other surgery is indicated, surgical correction of large and, moderate-sized aneurysms is the best approach to prevent, rupture and other sequelae. Small aneurysms should be, followed carefully., , DOUBLE-CHAMBERED RIGHT VENTRICLE, Definition, This anomaly is rare in adulthood in developed countries., Two series with multiple patients are reported in the, literatureL6,M19; otherwise, there are only occasional individual, case reports or mention of an occasional adult in series, that include predominantly children.G1,S32 In the study by, McElhinney and colleagues, it is reported that a literature, review identified six case reports.M19 Larger series of adult, patients are reported from countries with underdeveloped, healthcare systems where this anomaly may go undiagnosed, until adulthood.S33, Morphology, In adulthood, as in childhood, there are almost always associated structural anomalies. In one series of 11 patients, 10 had, associated anomalies, usually a ventricular septal defect, and, in a series of 3 adults, all had associated anomalies, either, atrial or ventricular septal defects.L6,M19, Clinical Features and Diagnostic Criteria, Symptoms in adults are different from those in children., They include syncope, chest pain, and exertional dys­, pnea.L6,M19 New York Heart Association functional class, ranges from II to IV in most patients. Diagnosis in the, adult may be difficult. Electrocardiography (ECG) is nonspecific, showing evidence of right ventricular hyper­trophy,, right atrial enlargement, and diffuse ST-wave changes., Transthoracic echocardiography often fails to make the, diagnosis.L6 The incorrect diagnosis is often tetralogy of, Fallot, ventricular septal defect, or pulmonary valve stenosis., Transesophageal echocardiography may be more accurate.G1, MRI has been reported to establish the diagnosis and may, supplement echocardiography.N5 Cardiac catheterization, should be performed in all cases and establishes the diagnosis in all. It also should always be performed to assess, coronary arteries and overall hemodynamics.G1, Technique of Operation, Surgical techniques are similar to those used in children., Usually a transatrial–transpulmonary artery approach, will be effective, with a formal right ventriculotomy unnecessary. Associated anomalies should be addressed, as well., , Downloaded for Abhishek Srivastava ([email protected]) at Fortis Escorts Heart Institute and Research Centre from ClinicalKey.com by, Elsevier on November 08, 2021. For personal use only. No other uses without permission. Copyright ©2021. Elsevier Inc. All rights reserved.