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ACC/AHA PRACTICE GUIDELINE: PRACTICE GUIDELINE: FULL TEXT

ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease

A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease) Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons

Carole A. Warnes, MD, FRCP, FACC, FAHA, Co-Chair, WRITING COMMITTEE MEMBER, Roberta G. Williams, MD, MACC, FAHA, Co-Chair, WRITING COMMITTEE MEMBER, Thomas M. Bashore, MD, FACC, WRITING COMMITTEE MEMBER, John S. Child, MD, FACC, FAHA, WRITING COMMITTEE MEMBER, Heidi M. Connolly, MD, FACC, WRITING COMMITTEE MEMBER, Joseph A. Dearani, MD, FACC, WRITING COMMITTEE MEMBER^_*, Pedro del Nido, MD, WRITING COMMITTEE MEMBER, James W. Fasules, MD, FACC, WRITING COMMITTEE MEMBER, Thomas P. Graham, Jr, MD, FACC, WRITING COMMITTEE MEMBER, Ziyad M. Hijazi, MBBS, MPH, FACC, FSCAI, WRITING COMMITTEE MEMBER, Sharon A. Hunt, MD, FACC, FAHA, WRITING COMMITTEE MEMBER, Mary Etta King, MD, FACC, FASE, WRITING COMMITTEE MEMBER, Michael J. Landzberg, MD, FACC, WRITING COMMITTEE MEMBER, Pamela D. Miner, RN, MN, NP, WRITING COMMITTEE MEMBER, Martha J. Radford, MD, FACC, WRITING COMMITTEE MEMBER, Edward P. Walsh, MD, FACC, WRITING COMMITTEE MEMBER^|| and Gary D. Webb, MD, FACC, WRITING COMMITTEE MEMBER^¶

Key Words: ACC/AHA Practice Guidelines • congenital heart disease • cardiac defects • congenital heart surgery • unoperated/repaired heart defects • medical therapy • cardiac catheterization

	Task Force Members

Top Task Force Members Table of Contents Preamble 1. Introduction 2. Atrial Septal Defect 3. Ventricular Septal Defect 4. Atrioventricular Septal... 5. Patent Ductus Arteriosus 6. Left-Sided Heart Obstructive... 7. Right Ventricular Outflow... 8. Coronary Artery Abnormalities 9. Pulmonary... 10. Tetralogy of Fallot 11. Dextro-Transposition of the... 12. Congenitally Corrected... 13. Ebstein's Anomaly 14. Tricuspid Atresia/Single... Staff Appendixes Appendix 3 Appendix 4 References

	Table of Contents

Top Task Force Members Table of Contents Preamble 1. Introduction 2. Atrial Septal Defect 3. Ventricular Septal Defect 4. Atrioventricular Septal... 5. Patent Ductus Arteriosus 6. Left-Sided Heart Obstructive... 7. Right Ventricular Outflow... 8. Coronary Artery Abnormalities 9. Pulmonary... 10. Tetralogy of Fallot 11. Dextro-Transposition of the... 12. Congenitally Corrected... 13. Ebstein's Anomaly 14. Tricuspid Atresia/Single... Staff Appendixes Appendix 3 Appendix 4 References

 

Preamble.......e150

1. Introduction.......e150

1.1. Methodology and Evidence Review.......e150

1.2. Organization of Committee and Relationships With Industry.......e151

1.3. Document Review and Approval.......e151

1.4. Epidemiology and Scope of the Problem.......e151

1.5. Recommendations for Delivery of Care and Ensuring Access.......e151

1.5.1. Recommendations for Access to Care.......e154

1.5.2. Recommendations for Psychosocial Issues.......e155

1.5.3. Transition of Care.......e156

1.5.4. Exercise and Athletics.......e157

1.5.5. Employment.......e157

1.5.6. Insurability.......e157

1.5.7. Congenital Syndromes.......e158

1.5.8. Medical/Ethical/Legal Issues.......e158

1.6. Recommendations for Infective Endocarditis.......e159

1.7. Recommendations for Noncardiac Surgery.......e163

1.8. Recommendations for Pregnancy and Contraception.......e163

1.8.1. Contraception.......e164

1.9. Recommendations for Arrhythmia Diagnosis and Management.......e164

1.9.1. Management of Tachyarrhythmias: Wolff-Parkinson-White Syndrome.......e165

1.9.2. Intra-Atrial Reentrant Tachycardia or Atrial Flutter.......e165

1.9.3. Atrial Fibrillation.......e166

1.9.4. Ventricular Tachycardia.......e166

1.10. Management of Bradycardias.......e167

1.10.1. Sinoatrial Node Dysfunction.......e167

1.10.2. Atrioventricular Block.......e168

1.11. Cyanotic Congenital Heart Disease.......e168

1.11.1. Recommendations for Hematologic Problems.......e168

1.11.1.1 [EC] . Hemostasis.......e168

1.11.1.2 [EC] . Renal Function.......e169

1.11.1.3 [EC] . Gallstones.......e169

1.11.1.4 [EC] . Orthopedic and Rheumatologic Complications.......e169

1.11.1.5 [EC] . Neurological Complications.......e169

1.11.1.6 [EC] . Pulmonary Vascular Disease.......e169

1.12. Recommendations for General Health Issues for Cyanotic Patients.......e169

1.12.1. Hospitalization and Operation.......e169

1.12.2. Cardiac Reoperation and Preoperative Evaluation.......e169

1.13. Heart Failure in Adult Congenital Heart Disease.......e170

1.14. Recommendations for Heart and Heart/Lung Transplantation.......e172

2. Atrial Septal Defect.......e173

2.1. Definition.......e173

2.1.1. Associated Lesions.......e173

2.2. Clinical Course.......e173

2.2.1. Unrepaired Atrial Septal Defect.......e173

2.3. Recommendations for Evaluation of the Unoperated Patient.......e174

2.3.1. Clinical Examination.......e174

2.3.2. Electrocardiogram.......e174

2.3.3. Chest X-Ray.......e174

2.3.4. Echocardiography.......e174

2.3.5. Magnetic Resonance Imaging.......e174

2.3.6. Exercise Testing.......e175

2.4. Diagnostic Problems and Pitfalls.......e175

2.5. Management Strategies.......e175

2.5.1. Recommendations for Medical Therapy.......e175

2.5.2. Recommendations for Interventional and Surgical Therapy.......e175

2.5.3. Indications for Closure of Atrial Septal Defect.......e176

2.5.4. Catheter Intervention.......e176

2.5.5. Key Issues to Evaluate and Follow-Up.......e176

2.6. Recommendations for Postintervention Follow-Up.......e176

2.6.1. Endocarditis Prophylaxis.......e176

2.6.2. Recommendation for Reproduction.......e177

2.6.3. Activity.......e178

3. Ventricular Septal Defect.......e178

3.1. Definition.......e178

3.1.1. Associated Lesions.......e178

3.2. Clinical Course (Unrepaired).......e179

3.3. Clinical Features and Evaluation of the Unoperated Patient.......e179

3.3.1. Clinical Examination.......e179

3.3.2. Electrocardiogram.......e179

3.3.3. Chest X-Ray.......e179

3.3.4. Echocardiography.......e179

3.3.5. Magnetic Resonance Imaging/Computed Tomography.......e179

3.3.6. Recommendations for Cardiac Catheterization.......e180

3.4. Diagnostic Problems and Pitfalls.......e180

3.5. Management Strategies.......e180

3.5.1. Recommendation for Medical Therapy.......e180

3.5.2. Recommendations for Surgical Ventricular Septal Defect Closure.......e180

3.5.3. Recommendation for Interventional Catheterization.......e180

3.6. Key Issues to Evaluate and Follow-Up.......e181

3.6.1. Recommendations for Surgical and Catheter Intervention Follow-Up.......e181

3.6.2. Recommendation for Reproduction.......e181

3.6.3. Activity.......e181

4. Atrioventricular Septal Defect.......e181

4.1. Definition.......e181

4.2. Associated Lesions.......e181

4.3. Clinical Features and Evaluation.......e181

4.3.1. Clinical Examination.......e182

4.3.2. Electrocardiogram.......e182

4.3.3. Chest X-Ray.......e182

4.3.4. Echocardiography.......e182

4.3.5. Magnetic Resonance Imaging.......e182

4.3.6. Recommendation for Heart Catheterization.......e182

4.3.7. Exercise Testing.......e182

4.4. Management Strategies.......e182

4.4.1. Medical Therapy.......e182

4.4.2. Recommendations for Surgical Therapy.......e182

4.5. Key Issues to Evaluate and Follow-Up.......e183

4.5.1. Key Postoperative Issues.......e183

4.5.2. Evaluation and Follow-Up of the Repaired Patient.......e183

4.5.3. Electrophysiology Testing/Pacing Issues in Atrioventricular Septal Defects.......e183

4.5.4. Recommendations for Endocarditis Prophylaxis.......e183

4.6. Reproduction.......e183

4.6.1. Genetic Aspects.......e183

4.6.2. Recommendations for Pregnancy.......e183

4.7. Exercise.......e184

5. Patent Ductus Arteriosus.......e184

5.1. Definition and Associated Lesions.......e184

5.2. Presentation and Clinical Course.......e184

5.3. Recommendations for Evaluation of the Unoperated Patient.......e184

5.3.1. Clinical Examination.......e184

5.3.2. Electrocardiogram.......e184

5.3.3. Echocardiography.......e184

5.3.4. Chest X-Ray.......e184

5.3.5. Cardiac Catheterization.......e184

5.3.6. Magnetic Resonance Imaging/Computed Tomography.......e184

5.4. Problems and Pitfalls.......e184

5.5. Management Strategies.......e185

5.5.1. Recommendations for Medical Therapy.......e185

5.5.2. Recommendations for Closure of Patent Ductus Arteriosus.......e185

5.5.3. Surgical/Interventional Therapy.......e185

5.6. Key Issues to Evaluate and Follow-Up.......e185

6. Left-Sided Heart Obstructive Lesions: Aortic Valve Disease, Subvalvular and Supravalvular Aortic Stenosis, Associated Disorders of the Ascending Aorta, and Coarctation.......e185

6.1. Definition.......e185

6.2. Associated Lesions.......e186

6.3. Clinical Course (Unrepaired).......e186

6.4. Recommendations for Evaluation of the Unoperated Patient.......e186

6.4.1. Clinical Examination.......e187

6.4.2. Electrocardiogram.......e187

6.4.3. Chest X-Ray.......e187

6.4.4. Echocardiography.......e187

6.4.5. Magnetic Resonance Imaging/Computed Tomography.......e187

6.4.6. Stress Testing.......e187

6.4.7. Cardiac Catheterization.......e187

6.5. Problems and Pitfalls.......e188

6.6. Management Strategies for Left Ventricular Outflow Tract Obstruction and Associated Lesions.......e188

6.6.1. Recommendations for Medical Therapy.......e188

6.6.2. Catheter and Surgical Intervention.......e188

6.6.2.1 [EC] . Recommendations for Catheter Interventions for Adults With Valvular Aortic Stenosis .......e188

6.6.2.2 [EC] . Recommendations for Aortic Valve Repair/Replacement and Aortic Root Replacement.......e189

6.7. Recommendations for Key Issues to Evaluate and Follow-Up.......e190

6.7.1. Reproduction.......e190

6.7.2. Activity/Exercise.......e190

6.8. Isolated Subaortic Stenosis.......e190

6.8.1. Definition.......e190

6.8.2. Associated Lesions.......e191

6.8.3. Clinical Course With/Without Previous Intervention.......e191

6.8.4. Clinical Features and Evaluation.......e191

6.8.4.1 [EC] . Clinical Examination.......e191

6.8.4.2 [EC] . Electrocardiogram.......e191

6.8.4.3 [EC] . Chest X-Ray.......e191

6.8.4.4 [EC] . Echocardiography.......e191

6.8.5. Diagnostic Cardiac Catheterization.......e191

6.8.6. Problems and Pitfalls.......e191

6.8.7. Management Strategies.......e191

6.8.7.1 [EC] . Medical Therapy.......e191

6.8.7.2 [EC] . Recommendations for Surgical Intervention.......e191

6.8.8. Recommendations for Key Issues to Evaluate and Follow-Up.......e192

6.8.9. Special Issues.......e192

6.8.9.1 [EC] . Pregnancy.......e192

6.8.9.2 [EC] . Exercise and Athletics.......e192

6.9. Supravalvular Aortic Stenosis.......e192

6.9.1. Definition.......e192

6.9.2. Associated Lesions.......e192

6.9.3. Clinical Course (Unrepaired).......e192

6.10. Recommendations for Evaluation of the Unoperated Patient.......e193

6.10.1. Clinical Examination.......e193

6.10.2. Electrocardiogram.......e193

6.10.3. Chest X-Ray.......e193

6.10.4. Imaging.......e193

6.10.5. Stress Testing.......e193

6.10.6. Myocardial Perfusion Imaging.......e193

6.10.7. Cardiac Catheterization.......e193

6.11. Management Strategies for Supravalvular Left Ventricular Outflow Tract.......e193

6.11.1. Recommendations for Interventional and Surgical Therapy.......e193

6.11.2. Recommendations for Key Issues to Evaluate and Follow-Up.......e194

6.11.3. Special Issues.......e194

6.11.4. Exercise and Athletics.......e194

6.11.5. Recommendations for Reproduction.......e194

6.12. Aortic Coarctation.......e194

6.12.1. Definition.......e194

6.12.2. Associated Lesions.......e194

6.12.3. Recommendations for Clinical Evaluation and Follow-Up.......e194

6.13. Clinical Features and Evaluation of Unrepaired Patients.......e195

6.13.1. Electrocardiogram.......e195

6.13.2. Chest X-Ray.......e195

6.13.3. Echocardiography and Doppler.......e195

6.13.4. Stress Testing.......e195

6.13.5. Magnetic Resonance Imaging/Magnetic Resonance Angiography or Computed Tomography With 3-Dimensional Reconstruction.......e195

6.13.6. Catheterization Hemodynamics/Angiography.......e195

6.13.7. Problems and Pitfalls.......e195

6.14. Management Strategies for Coarctation of the Aorta.......e195

6.14.1. Medical Therapy.......e195

6.14.2. Recommendations for Interventional and Surgical Treatment of Coarctation of the Aorta in Adults.......e195

6.14.3. Recommendations for Key Issues to Evaluate and Follow-Up.......e196

6.14.4. Exercise and Athletics.......e196

6.14.5. Reproduction.......e197

6.14.6. Endocarditis Prophylaxis.......e197

7. Right Ventricular Outflow Tract Obstruction.......e197

7.1. Definition.......e197

7.2. Associated Lesions.......e197

7.3. Valvular Pulmonary Stenosis.......e198

7.3.1. Definition.......e198

7.4. Clinical Course.......e198

7.4.1. Unrepaired Patients.......e198

7.4.2. Noonan Syndrome Patients With Prior Repair.......e198

7.5. Recommendations for Evaluation of the Unoperated Patient.......e198

7.5.1. Clinical Examination.......e198

7.5.2. Electrocardiogram.......e198

7.5.3. Chest X-Ray.......e198

7.5.4. Echocardiography.......e199

7.5.5. Magnetic Resonance Imaging/Computed Tomography.......e199

7.5.6. Cardiac Catheterization.......e199

7.5.7. Relationship Between Peak Instantaneous Doppler Echocardiographic Pressure Gradients and Peak-to-Peak Cardiac Catheterization Gradients.......e199

7.6. Problems and Pitfalls.......e199

7.6.1. Dyspnea.......e199

7.6.2. Chest Pain.......e199

7.6.3. Enlarging Right Ventricle.......e199

7.6.4. Pulmonary Arterial Hypertension.......e200

7.6.5. Cyanosis.......e200

7.6.6. Systemic Venous Congestion.......e200

7.7. Management Strategies.......e200

7.7.1. Recommendations for Intervention in Patients With Valvular Pulmonary Stenosis.......e200

7.7.2. Percutaneous Balloon Pulmonary Valvotomy.......e200

7.7.3. Surgical Pulmonary Valvotomy or Valve Replacement.......e201

7.8. Recommendation for Clinical Evaluation and Follow-Up After Intervention.......e201

7.8.1. Reproduction.......e202

7.8.2. Endocarditis Prophylaxis.......e202

7.8.3. Exercise and Athletics.......e202

7.9. Right-Sided Heart Obstruction Due to Supravalvular, Branch, and Peripheral Pulmonary Artery Stenosis.......e202

7.9.1. Definition and Associated Lesions.......e202

7.9.2. Clinical Course.......e202

7.10. Clinical Features and Evaluation of the Unrepaired Patient.......e202

7.10.1. Electrocardiogram.......e203

7.10.2. Chest X-Ray.......e203

7.10.3. Echocardiography.......e203

7.10.4. Magnetic Resonance Imaging/Computed Tomography.......e203

7.10.5. Cardiac Catheterization.......e203

7.11. Recommendations for Evaluation of Patients With Supravalvular, Branch, and Peripheral Pulmonary Stenosis.......e203

7.11.1. Problems and Pitfalls.......e203

7.11.2. Management Strategies.......e203

7.11.2.1 [EC] . Medical Therapy.......e203

7.12. Recommendations for Interventional Therapy in the Management of Branch and Peripheral Pulmonary Stenosis.......e203

7.12.1. Recommendations for Evaluation and Follow-Up.......e204

7.13. Right-Sided Heart Obstruction Due to Stenotic Right Ventricular–Pulmonary Artery Conduits or Bioprosthetic Valves.......e204

7.13.1. Definition and Associated Lesions.......e204

7.13.2. Recommendation for Evaluation and Follow-Up After Right Ventricular–Pulmonary Artery Conduit or Prosthetic Valve.......e204

7.13.3. Clinical Examination.......e204

7.13.4. Electrocardiogram.......e204

7.13.5. Chest X-Ray.......e204

7.13.6. Echocardiography.......e204

7.13.7. Magnetic Resonance Imaging/Computed Tomography.......e204

7.13.8. Cardiac Catheterization.......e204

7.14. Recommendations for Reintervention in Patients With Right Ventricular–Pulmonary Artery Conduit or Bioprosthetic Pulmonary Valve Stenosis.......e204

7.14.1. Medical Therapy.......e205

7.14.2. Interventional Catheterization.......e205

7.14.3. Surgical Intervention.......e205

7.14.4. Key Issues to Evaluate and Follow-Up.......e205

7.15. Double-Chambered Right Ventricle.......e205

7.15.1. Definition and Associated Lesions.......e205

7.15.2. Clinical Features and Evaluation of the Unoperated Patient.......e205

7.15.3. Clinical Examination.......e206

7.15.4. Electrocardiogram.......e206

7.15.5. Echocardiography-Doppler Imaging.......e206

7.15.6. Magnetic Resonance Imaging.......e206

7.15.7. Cardiac Catheterization.......e206

7.16. Problems and Pitfalls.......e206

7.16.1. Multiple Levels of Right Ventricular Outflow Tract Obstruction.......e206

7.17. Management Strategies.......e206

7.17.1. Recommendations for Intervention in Patients With Double-Chambered Right Ventricle.......e206

7.18. Key Issues to Evaluate and Follow-Up.......e206

8. Coronary Artery Abnormalities.......e206

8.1. Definition and Associated Lesions.......e206

8.1.1. General Recommendations for Evaluation and Surgical Intervention.......e206

8.2. Recommendations for Coronary Anomalies Associated With Supravalvular Aortic Stenosis.......e207

8.2.1. Clinical Course (Unrepaired) .......e207

8.2.2. Clinical Features.......e207

8.3. Recommendation for Coronary Anomalies Associated With Tetralogy of Fallot.......e207

8.3.1. Preintervention Evaluation.......e207

8.3.2. Surgical and Catheterization-Based Interventions.......e207

8.4. Recommendation for Coronary Anomalies Associated With Dextro-Transposition of the Great Arteries After Arterial Switch Operation.......e207

8.4.1. Definition and Associated Lesions.......e207

8.4.2. Clinical Course.......e207

8.4.3. Clinical Features and Evaluation After Arterial Switch Operation.......e208

8.4.4. Surgical and Catheterization-Based Intervention.......e208

8.5. Recommendations for Congenital Coronary Anomalies of Ectopic Arterial Origin.......e208

8.5.1. Definition, Associated Lesions, and Clinical Course.......e208

8.5.2. Clinical Features and Evaluation of the Unoperated Patient.......e208

8.5.2.1 [EC] . Preintervention Evaluation.......e208

8.5.3. Management Strategies.......e208

8.5.3.1 [EC] . Surgical and Catheterization-Based Intervention.......e208

8.6. Recommendations for Anomalous Left Coronary Artery From the Pulmonary Artery.......e209

8.6.1. Definition and Associated Lesions and Clinical Course.......e209

8.7. Management Strategies.......e209

8.7.1. Surgical Intervention.......e209

8.7.2. Surgical and Catheterization-Based Intervention.......e209

8.8. Recommendations for Coronary Arteriovenous Fistula.......e209

8.8.1. Definition.......e210

8.8.2. Clinical Course.......e210

8.8.3. Preintervention Evaluation.......e210

8.9. Recommendations for Management Strategies.......e210

8.9.1. Surgical Intervention.......e210

8.9.2. Catheterization-Based Intervention.......e210

8.9.3. Preintervention Evaluation After Surgical or Catheterization-Based Repair.......e210

9. Pulmonary Hypertension/Eisenmenger Physiology.......e210

9.1. Definition.......e210

9.2. Clinical Course.......e211

9.2.1. Dynamic Congenital Heart Disease–Pulmonary Arterial Hypertension.......e211

9.2.2. Immediate Postoperative Congenital Heart Disease–Pulmonary Arterial Hypertension.......e211

9.2.3. Late Postoperative Congenital Heart Disease–Pulmonary Arterial Hypertension.......e211

9.2.4. Normal to Mildly Abnormal Pulmonary Vascular Resistance States.......e211

9.2.5. Eisenmenger Physiology.......e212

9.3. Problems and Pitfalls.......e212

9.4. Recommendations for Evaluation of the Patient With Congenital Heart Disease–Pulmonary Arterial Hypertension.......e212

9.4.1. Dynamic Congenital Heart Disease–Pulmonary Arterial Hypertension.......e212

9.4.2. Eisenmenger Physiology.......e213

9.5. Management Strategies.......e213

9.5.1. Recommendations for Medical Therapy of Eisenmenger Physiology.......e213

9.6. Key Issues to Evaluate and Follow-Up.......e214

9.6.1. Recommendations for Reproduction.......e214

9.6.2. Pregnancy.......e214

9.6.3. Other Interventions.......e215

9.6.4. Recommendations for Follow-Up.......e215

9.6.5. Endocarditis Prophylaxis.......e215

10. Tetralogy of Fallot.......e215

10.1. Definition and Associated Lesions.......e215

10.2. Clinical Course (Unrepaired).......e215

10.2.1. Presentation as an Unoperated Patient.......e215

10.2.2. Postsurgical Presentation.......e215

10.3. Clinical Features and Evaluation.......e215

10.3.1. Clinical Examination.......e215

10.3.2. Electrocardiogram.......e216

10.3.3. Chest X-Ray.......e216

10.3.4. Initial Surgical Repair.......e216

10.4. Recommendations for Evaluation and Follow-Up of the Repaired Patient.......e216

10.4.1. Recommendation for Imaging.......e217

10.5. Recommendations for Diagnostic and Interventional Catheterization for Adults With Tetralogy of Fallot.......e217

10.5.1. Branch Pulmonary Artery Angioplasty.......e217

10.5.2. Exercise Testing.......e218

10.5.3. Diagnostic Catheterization.......e218

10.6. Problems and Pitfalls in the Patient With Prior Repair.......e218

10.7. Management Strategy for the Patient With Prior Repair.......e218

10.7.1. Medical Therapy.......e218

10.8. Recommendations for Surgery for Adults With Previous Repair of Tetralogy of Fallot.......e218

10.8.1. Recommendations for Interventional Catheterization .......e219

10.9. Key Issues to Evaluate and Follow-Up .......e219

10.9.1. Recommendations for Arrhythmias: Pacemaker/Electrophysiology Testing.......e219

10.9.2. Reproduction.......e221

10.9.3. Exercise.......e221

10.9.4. Endocarditis Prophylaxis.......e221

11. Dextro-Transposition of the Great Arteries.......e221

11.1. Definition.......e221

11.2. Associated Lesions.......e221

11.3. Clinical Course: Unrepaired.......e221

11.4. Recommendation for Evaluation of the Operated Patient With Dextro-Transposition of the Great Arteries.......e221

11.4.1. Clinical Features and Evaluation of Dextro-Transposition of the Great Arteries After Atrial Baffle Procedure.......e222

11.4.2. Clinical Examination.......e222

11.4.3. Electrocardiogram.......e222

11.4.4. Imaging for Dextro-Transposition of the Great Arteries After Atrial Baffle Procedure.......e222

11.4.4.1 [EC] . Recommendations for Imaging for Dextro-Transposition of the Great Arteries After Atrial Baffle Procedure.......e222

11.4.5. Cardiac Catheterization.......e223

11.5. Clinical Features and Evaluation of Dextro-Transposition of the Great Arteries After Arterial Switch Operation.......e223

11.5.1. Clinical Examination.......e223

11.5.2. Electrocardiogram.......e223

11.5.3. Chest X-Ray.......e223

11.5.4. Recommendations for Imaging for Dextro-Transposition of the Great Arteries After Arterial Switch Operation.......e223

11.5.5. Recommendation for Cardiac Catheterization After Arterial Switch Operation.......e223

11.6. Clinical Features and Evaluation: Dextro-Transposition of the Great Arteries After Rastelli Operation.......e224

11.6.1. Electrocardiogram.......e224

11.6.2. Chest X-Ray.......e224

11.6.3. Imaging.......e224

11.7. Recommendations for Diagnostic Catheterization for Adults With Repaired Dextro-Transposition of the Great Arteries.......e224

11.7.1. Problems and Pitfalls.......e224

11.8. Management Strategies.......e224

11.8.1. Medical Therapy.......e224

11.8.2. Recommendations for Interventional Catheterization for Adults With Dextro-Transposition of the Great Arteries.......e224

11.8.2.1 [EC] . Interventional Catheter Options After Atrial Baffle.......e225

11.8.2.2 [EC] . Interventional Catheter Options After Arterial Switch Operation.......e225

11.8.2.3 [EC] . Interventional Catheter Options After Rastelli Repair.......e225

11.8.3. Recommendations for Surgical Interventions.......e225

11.8.3.1 [EC] . After Atrial Baffle Procedure (Mustard, Senning) .......e225

11.8.3.2 [EC] . After Arterial Switch Operation.......e225

11.8.3.3 [EC] . After Rastelli Procedure.......e225

11.8.3.4 [EC] . Reoperation After Atrial Baffle Procedure.......e226

11.8.3.5 [EC] . Reoperation After Arterial Switch Operation.......e226

11.8.3.6 [EC] . Reoperation After Rastelli Repair.......e226

11.8.3.7 [EC] . Other Reoperation Options.......e227

11.9. Recommendations for Electrophysiology Testing/Pacing Issues in Dextro-Transposition of the Great Arteries.......e227

11.10. Key Issues to Evaluate and Follow-Up.......e227

11.10.1. Recommendations for Endocarditis Prophylaxis.......e227

11.10.2. Recommendation for Reproduction.......e228

11.10.3. Activity and Exercise.......e228

12. Congenitally Corrected Transposition of the Great Arteries.......e228

12.1. Definition.......e228

12.2. Associated Lesions.......e228

12.3. Clinical Course.......e228

12.3.1. Presentation in Adulthood: Unoperated.......e228

12.4. Clinical Features and Evaluation of the Unoperated Patient.......e229

12.4.1. Clinical Examination.......e229

12.4.2. Electrocardiogram.......e229

12.4.3. Exercise Testing.......e229

12.4.4. Chest X-Ray.......e230

12.4.5. Two-Dimensional Echocardiography.......e230

12.4.6. Magnetic Resonance Imaging.......e230

12.4.7. Cardiac Catheterization.......e230

12.5. Recommendations for Evaluation and Follow-Up of Patients With Congenitally Corrected Transposition of the Great Arteries.......e230

12.6. Key Issues of Unoperated Patients.......e230

12.7. Management Strategies.......e231

12.8. Interventional Therapy.......e231

12.8.1. Recommendations for Catheter Interventions.......e231

12.8.2. Initial Surgical Repair.......e231

12.8.3. Recommendations for Surgical Intervention.......e231

12.8.4. Problems and Pitfalls.......e232

12.9. Arrhythmias/Pacemaker/Electrophysiology Testing.......e232

12.10. Recommendations for Postoperative Care.......e232

12.10.1. Recommendations for Endocarditis Prophylaxis.......e232

12.10.2. Recommendation for Reproduction.......e233

12.10.3. Activity.......e233

13. Ebstein's Anomaly.......e233

13.1. Definition.......e233

13.2. Clinical Course (Unoperated).......e233

13.2.1. Pediatric Presentation.......e233

13.2.2. Initial Adult Presentation.......e233

13.3. Clinical Features and Evaluation of the Unoperated Patient.......e233

13.4. Recommendation for Evaluation of Patients With Ebstein's Anomaly.......e234

13.4.1. Clinical Examination.......e234

13.4.2. Electrocardiogram.......e234

13.4.3. Chest X-Ray.......e234

13.4.4. Echocardiography.......e234

13.4.5. Magnetic Resonance Imaging/Computed Tomography.......e234

13.5. Recommendations for Diagnostic Tests.......e234

13.5.1. Cardiac Catheterization.......e235

13.5.2. Problems and Pitfalls.......e235

13.6. Management Strategies.......e235

13.6.1. Recommendation for Medical Therapy.......e235

13.6.2. Physical Activity.......e235

13.7. Recommendation for Catheter Interventions for Adults With Ebstein's Anomaly.......e235

13.7.1. Recommendation for Electrophysiology Testing/Pacing Issues in Ebstein's Anomaly.......e235

13.7.2. Recommendations for Surgical Interventions.......e235

13.7.3. Postoperative Findings.......e236

13.7.4. Expected Postoperative Course.......e236

13.8. Problems and Pitfalls.......e236

13.9. Recommendation for Reproduction.......e236

13.10. Recommendation for Endocarditis Prophylaxis.......e237

14. Tricuspid Atresia/Single Ventricle.......e237

14.1. Definition.......e237

14.2. Clinical Course (Unoperated and Palliated).......e237

14.3. Clinical Features and Evaluation of the Unoperated or Palliated Patient.......e237

14.3.1. Presentation.......e237

14.3.2. Clinical Examination.......e237

14.3.3. Electrocardiogram.......e237

14.3.4. Chest X-Ray.......e238

14.3.5. Echocardiography.......e238

14.3.6. Magnetic Resonance Imaging/Computed Tomography.......e238

14.3.7. Recommendation for Catheterization Before Fontan Procedure.......e238

14.4. Recommendation for Surgical Options for Patients With Single Ventricle.......e238

14.5. Recommendation for Evaluation and Follow-Up After Fontan Procedure.......e239

14.6. Clinical Features and Evaluation.......e240

14.6.1. Clinical Examination.......e240

14.6.2. Electrocardiogram.......e240

14.6.3. Chest X-Ray.......e240

14.6.4. Recommendation for Imaging.......e240

14.7. Recommendation for Diagnostic and Interventional Catheterization After Fontan Procedure.......e240

14.7.1. Evaluation of Patients With Protein-Losing Enteropathy.......e240

14.7.2. Problems and Pitfalls.......e241

14.8. Recommendations for Management Strategies for the Patient With Prior Fontan Repair.......e241

14.8.1. Recommendations for Medical Therapy.......e241

14.9. Recommendations for Surgery for Adults With Prior Fontan Repair.......e241

14.10. Key Issues to Evaluate and Follow-Up.......e242

14.10.1. Recommendations for Electrophysiology Testing/Pacing Issues in Single-Ventricle Physiology and After Fontan Procedure.......e242

14.10.2. Other Issues to Evaluate and Follow-Up.......e243

14.10.3. Recommendations for Endocarditis Prophylaxis.......e243

14.10.4. Activity.......e243

14.10.5. Recommendations for Reproduction.......e244

Appendix 1. Author Relationships With Industry and Other Entities.......e244

Appendix 2. Peer Reviewer Relationships With Industry and Other Entities.......e245

Appendix 3. Abbreviations List.......e247

Appendix 4. Definitions of Surgical Procedures for the Management of Adults With CHD.......e247

References.......e250

	Preamble

Top Task Force Members Table of Contents Preamble 1. Introduction 2. Atrial Septal Defect 3. Ventricular Septal Defect 4. Atrioventricular Septal... 5. Patent Ductus Arteriosus 6. Left-Sided Heart Obstructive... 7. Right Ventricular Outflow... 8. Coronary Artery Abnormalities 9. Pulmonary... 10. Tetralogy of Fallot 11. Dextro-Transposition of the... 12. Congenitally Corrected... 13. Ebstein's Anomaly 14. Tricuspid Atresia/Single... Staff Appendixes Appendix 3 Appendix 4 References

 
It is important that the medical profession play a central role in critically evaluating the use of diagnostic procedures and therapies introduced and tested for detection, management, or prevention of disease. Rigorous, expert analysis of the available data documenting absolute and relative benefits and risks of these procedures and therapies can produce guidelines that improve the effectiveness of care, optimize patient outcomes, and favorably affect the cost of care by focusing resources on the most effective strategies.

The American College of Cardiology Foundation (ACCF) and the American Heart Association (AHA) have jointly engaged in the production of guidelines in the area of cardiovascular disease since 1980. The American College of Cardiology (ACC)/AHA Task Force on Practice Guidelines is charged with developing, updating, and revising practice guidelines for cardiovascular diseases and procedures and directs this effort. Writing committees are charged with assessing the evidence as an independent group of authors to develop, update, or revise recommendations for clinical practice.

Experts in the subject under consideration have been selected from both organizations to examine subject-specific data and write guidelines in partnership with representatives from other medical practitioner and specialty groups. Writing committees are specifically charged to perform a formal literature review, weigh the strength of evidence for or against particular treatments or procedures, and include estimates of expected health outcomes where data exist. Patient-specific modifiers, comorbidities, and issues of patient preference that might influence the choice of tests or therapies are considered, as well as the frequency of follow-up and cost-effectiveness. When available, information from studies on cost is considered, but data on efficacy and clinical outcomes constitute the primary basis for recommendations in these guidelines.

The ACC/AHA Task Force on Practice Guidelines makes every effort to avoid actual, potential, or perceived conflicts of interest that might arise as a result of industry relationships or personal interests among the writing committee. Specifically, all members of the writing committee, as well as peer reviewers of the document, are asked to disclose all such relationships that might be perceived as real or potential conflicts of interest. Writing committee members are also strongly encouraged to declare previous relationships with industry that might be perceived as relevant to guideline development. If a writing committee member develops a new relationship with industry during their tenure, they are required to notify guideline staff in writing. These statements are reviewed by the parent task force, reported orally to all members at each meeting of the writing committee, and updated and reviewed by the writing committee as changes occur. Please refer to the methodology manual for ACC/AHA guideline writing committees for further description of the relationships with industry policy (1). See Appendix 1 for author relationships with industry and Appendix 2 for peer reviewer relationships with industry pertinent to this guideline.

Patient adherence to prescribed and agreed upon medical regimens and lifestyles is an important aspect of treatment. Prescribed courses of treatment in accordance with these recommendations are only effective if they are followed. Because lack of patient understanding and adherence may adversely affect outcomes, physicians and other healthcare providers should make every effort to engage the patient's active participation in prescribed medical regimens and lifestyles.

If these guidelines are used as the basis for regulatory or payer decisions, the goal is quality of care and serving the patient's best interest. The ultimate judgment regarding care of a particular patient must be made by the healthcare provider and the patient in light of all of the circumstances presented by that patient. There are circumstances in which deviations from these guidelines are appropriate.

The guidelines will be reviewed annually by the ACC/AHA Task Force on Practice Guidelines and considered current unless they are updated, revised, or withdrawn from distribution. The executive summary and recommendations are published in the December 2, 2008, issue of the Journal of the American College of Cardiology and December 2, 2008, issue of Circulation. The full-text guidelines are e-published in the same issue of these journals and posted on the ACC (www.acc.org) and AHA (htttp://my.americanheart.org) World Wide Web sites.

Sidney C. Smith, Jr, MD, FACC, FAHA

Chair, ACC/AHA Task Force on Practice Guidelines

	1. Introduction

Top Task Force Members Table of Contents Preamble 1. Introduction 2. Atrial Septal Defect 3. Ventricular Septal Defect 4. Atrioventricular Septal... 5. Patent Ductus Arteriosus 6. Left-Sided Heart Obstructive... 7. Right Ventricular Outflow... 8. Coronary Artery Abnormalities 9. Pulmonary... 10. Tetralogy of Fallot 11. Dextro-Transposition of the... 12. Congenitally Corrected... 13. Ebstein's Anomaly 14. Tricuspid Atresia/Single... Staff Appendixes Appendix 3 Appendix 4 References

 
1.1. Methodology and Evidence Review.   The recommendations listed in this document are, whenever possible, evidence-based. Unlike other ACC/AHA practice guidelines, there is not a large body of peer-reviewed published evidence to support most recommendations, which will be clearly indicated in the text. An extensive literature survey was conducted that led to the incorporation of 647 references. Searches were limited to studies, reviews, and other evidence conducted in human subjects and published in English. Key search words included but were not limited to adult congenital heart disease (ACHD), atrial septal defect, arterial switch operation, bradycardia, cardiac catheterization, cardiac reoperation, coarctation, coronary artery abnormalities, cyanotic congenital heart disease, Doppler-echocardiography, d-transposition of the great arteries, Ebstein's anomaly, Eisenmenger physiology, familial, heart defect, medical therapy, patent ductus arteriosus, physical activity, pregnancy, psychosocial, pulmonary arterial hypertension, right heart obstruction, supravalvular pulmonary stenosis, surgical therapy, tachyarrhythmia, tachycardia, tetralogy of Fallot, transplantation, tricuspid atresia, and Wolff-Parkinson-White. Additionally, the writing committee reviewed documents related to the subject matter previously published by the ACC and AHA. References selected and published in this document are representative and not all-inclusive.

The committee reviewed and ranked evidence supporting current recommendations with the weight of evidence ranked as Level A if the data were derived from multiple randomized clinical trials involving a large number of individuals. The committee ranked available evidence as Level B when data were derived from a limited number of trials involving a comparatively small number of patients or from well-designed data analyses of nonrandomized studies or observational data registries. Evidence was ranked as Level C when the consensus of experts was the primary source of the recommendation. In the narrative portions of these guidelines, evidence is generally presented in chronological order of development. Studies are identified as observational, randomized, prospective, or retrospective. The committee emphasizes that for certain conditions for which no other therapy is available, the indications are based on expert consensus and years of clinical experience and are thus well supported, even though the evidence was ranked as Level C. An analogous example is the use of penicillin in pneumococcal pneumonia where there are no randomized trials and only clinical experience. When indications at Level C are supported by historical clinical data, appropriate references (eg, case reports and clinical reviews) are cited if available. When Level C indications are based strictly on committee consensus, no references are cited. The final recommendations for indications for a diagnostic procedure, a particular therapy, or an intervention in ACHD patients summarize both clinical evidence and expert opinion. The schema for classification of recommendations and level of evidence is summarized in Table 1, which also illustrates how the grading system provides an estimate of the size of treatment effect and an estimate of the certainty of the treatment effect.

All members of the writing committee were required to disclose all relationships with industry relevant to the data under consideration (1).

1.3. Document Review and Approval.   This document was reviewed by 3 external reviewers nominated from both the ACC and the AHA, as well as reviewers from the American Society of Echocardiography, Canadian Cardiovascular Society, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, and Society of Thoracic Surgeons, and 20 individual content reviewers which included reviewers from the ACC Congenital Heart Disease and Pediatric Cardiology Committee and the AHA Congenital Cardiac Defects Committee. All reviewer relationships with industry information were collected and distributed to the writing committee and are published in this document (see Appendix 2 for details).

This document was approved for publication by the governing bodies of the ACCF and the AHA and endorsed by the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons.

1.4. Epidemiology and Scope of the Problem.   Remarkable improvement in survival of patients with congenital heart disease (CHD) has occurred over the past half century since reparative surgery has become commonplace. Since the advent of neonatal repair of complex lesions in the 1970s, an estimated 85% of patients survive into adult life. The 32nd Bethesda Conference report in 2000 estimated that there were approximately 800 000 adults with CHD in the United States (2,3). Given modern surgical mortality rates of less than 5%, one would expect that in the next decade, almost 1 in 150 young adults will have some form of CHD. In particular, there are a substantial number of young adults with single-ventricle physiology, systemic right ventricles (RVs), or complex intracardiac baffles who are now entering adult life and starting families. Young adults have many psychological, social, and financial issues that present barriers to proactive health management. The infrastructure that is provided to most pediatric cardiology centers, namely, case management by advanced practice nurses and social workers, is largely lacking within the adult healthcare system. Recognizing the compound effects of a complex and unfamiliar disease with an unprepared patient and healthcare system, the ACC/AHA ACHD Guideline Writing Committee has determined that the most immediate step it can take to support the practicing cardiologist in the care of ACHD patients is to provide a consensus document that outlines the most important diagnostic and management strategies and indicates when referral to a highly specialized center is appropriate. To provide ease of use, the writing committee constructed this document by lesion type and in each section included recommendations on topics common to all lesions (eg, infective endocarditis [IE] prophylaxis, pregnancy, physical activity, and medical therapy).

1.5. Recommendations for Delivery of Care and Ensuring Access.   Class I

1 The focus of current healthcare access goals for ACHD patients should include the following:

a Strengthening organization of and access to transition clinics for adolescents and young adults with CHD, including funding of allied healthcare providers to provide infrastructure comparable to that provided for children with CHD. (Level of Evidence: C)

b Organization of outreach and education programs for patients, their families, and caregivers to recapture patients leaving pediatric supervisory care or who are lost to follow-up. Such programs can determine when and where further intervention is required. (Level of Evidence: C)

c Enhanced education of adult cardiovascular specialists and pediatric cardiologists in the pathophysiology and management of ACHD patients. (Level of Evidence: C)

d A liaison with regulatory agencies at the local, regional, state, and federal levels to create programs commensurate with the needs of this large cardiovascular population. (Level of Evidence: C)

2 Health care for ACHD patients should be coordinated by regional ACHD centers of excellence that would serve as a resource for the surrounding medical community, affected individuals, and their families (Table 2).

a Every academic adult cardiology/cardiac surgery center should have access to a regional ACHD center for consultation and referral. (Level of Evidence: C)

b Each pediatric cardiology program should identify the ACHD center to which the transfer of patients can be made. (Level of Evidence: C)

c All emergency care facilities should have an affiliation with a regional ACHD center. (Level of Evidence: C)

3 ACHD patients should carry a complete medical "passport" that outlines specifics of their past and current medical history, as well as contact information for immediate access to data and counsel from local and regional centers of excellence. (Level of Evidence: C)

4 Care of some ACHD patients is complicated by additional special needs, including but not restricted to intellectual incapacities or psychosocial limitations that necessitate the inclusion of designated healthcare guardians in all medical decision making. (Level of Evidence: C)

5 Every ACHD patient should have a primary care physician. To ensure and improve communication, current clinical records should be on file with the primary care physician and a local cardiovascular specialist, as well as at a regional ACHD center; patients should also have copies of relevant records. (Level of Evidence: C)

6 Every cardiovascular family caregiver should have a referral relationship with a regional ACHD center so that all patients have geographically accessible care. (Level of Evidence: C)

A detailed integration of caregivers and support was suggested by the 32nd Bethesda Conference, from primary care to patient advocacy groups to the highest levels of subspecialty resources. The pediatric cardiology team should be paired with adult cardiologists to facilitate transition of care for affected individuals. It was recommended that all ACHD patients have a provider who constitutes the medical "home," as well as at least 1 overreaching visit with a cardiologist with advanced training and experience with ACHD patients (4). A pattern of visits, follow-up, surgical care, subspecialty (catheterization, electrophysiology) cardiac and noncardiac care, emergent medical access, data coordination and dissemination, referral guidance, and education (with recognized regional variation) was suggested for ACHD patients and their caregivers based on the degree of medical complexity. Improvement in patient outcome was stressed via extension of physician caregiving by team-based clinical care associates (midlevel practitioners) with expertise in the management of ACHD patients.

The 32nd Bethesda Conference described 3 levels of training of adult cardiovascular specialists in terms of experience in ACHD (5). Task Force 9 covered training in the care of adult patients with CHD and differentiated 3 levels of training and expected expertise. These levels were subsequently incorporated into the COCATS (Core Cardiology Training Symposium) III document (6). Level 1 training consists of basic exposure to CHD patients and organized educational material on CHD. To enable proper recognition of the problems of adults with CHD and to be cognizant of when specialized referral is needed, all medical cardiology fellows should achieve level 1 training in CHD. Level 1 trainees should be instructed by a faculty member with level 2 or 3 training or its equivalent. A pediatric cardiologist should also be involved in these training programs.

Level 2 training represents additional training for fellows who plan to care for adult patients with CHD so that they may acquire expertise in the clinical evaluation and management of such patients. Level 2 training generally requires 1 year of training in ACHD: either a 1-year formal program at a regional or tertiary care ACHD center or cumulative experience of 12 months through repetitive rotations or electives as a cardiology fellow with experienced ACHD cardiologists. This training should prepare the individual to be well-equipped for the routine care of even moderate to complex ACHD and to recognize when more advanced consultation or referral is advisable.

Level 3 training represents the level of knowledge needed by those graduates who wish to make a clinical and academic/research commitment to this field and not only become competent in the care of the entire spectrum of adult patients with CHD but also participate in the teaching and research of ACHD. Level 3 trainees generally require 2 years of training. These 24 months may either be consecutive or cumulative experience, and some recognition can be given to overall experience in CHD, be it pediatric, adolescent, or adult (eg, prior pediatric cardiology training or rotations). Such level 3 training would be sufficient to clinically manage the most complex ACHD patient in a regional or tertiary care center, to pursue an academic career, to train others in the field, or to direct an ACHD center program (6).

The 32nd Bethesda Conference report in 2000 highlighted the need for healthcare professionals, patients, and their families, together with regulatory agency representatives, to develop a strategic plan for organized advocacy for ACHD patients (3,4). This ACC/AHA Guideline Committee, working in parallel with but independently of a workgroup of the National Heart, Lung, and Blood Institute charged with recommending key research opportunities in ACHD patients, recognizes key actions that are currently and urgently required to improve care access for ACHD patients.

1.5.1. Recommendations for Access to Care
Class I

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 (Tables 3 and 4) (7) only in collaboration with level 2 or level 3 ACHD specialists. (4) (Level of Evidence: C)

2 For ACHD patients in the lowest-risk group (simple CHD;Table 5), cardiac follow-up at a regional ACHD center is recommended at least once to formulate future needs for follow-up. (Level of Evidence: C)

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. (Level of Evidence: C)

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. (4) (Level of Evidence: C)

5 Diagnostic and interventional procedures, including imaging (ie, echocardiography, magnetic resonance imaging [MRI], or computed tomography [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. (Level of Evidence: C)

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. (Level of Evidence: C)

7 ACHD patients should be transferred to an ACHD center for urgent or acute care of cardiac problems. (Level of Evidence: C)

8 Adult patients with complex or high-risk CHD should be transferred to an ACHD center for urgent or acute noncardiac problems. (Level of Evidence: C)

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. (Level of Evidence: C)

• Failure to have guided transition from pediatric to adult care

• Lack of sufficient numbers of specialty clinics and regional centers

• Inadequate access to or availability of insurance (10)

• Insufficient education of patients and caregivers regarding disease nature and follow-up (11,12)

• Inadequate system of management of patient's cognitive or psychosocial impairment

• Inadequate infrastructure for case management.

1.5.2. Recommendations for Psychosocial Issues
Class I

1 Individual and family psychosocial screening (including knowledge assessment of cardiac disease and management; perceptions about health and the impact of CHD; social functioning with family, friends, and significant others; employment and insurability status; and screening for cognitive, mood, and psychiatric disorders) should be part of the care of ACHD patients. Advanced practice nurses, physician assistants, psychologists, and social workers should play an integral role in assessing and providing for the psychosocial needs of ACHD patients. (Level of Evidence: C)

2 Informational tools should be developed before transfer from adolescent to adult care and used for patient/family education regarding CHD, including the following elements, to be provided in electronic format:

a Demographic data, including physician contact. (Level of Evidence: C)

b Description of CHD, surgeries, interventional procedures, and most recent diagnostic studies. (Level of Evidence: C)

c Medications. (Level of Evidence: C)

3 Additional health maintenance screening and information should be offered to ACHD patients as indicated during each visit to their ACHD healthcare provider, including the following:

a Endocarditis prophylaxis measures (refer to Section 1.6, Recommendations for Infective Endocarditis). (Level of Evidence: C)

b Exercise prescription, guidelines for exercise, and athletic participation for patients with CHD should reflect the published recommendations of the 36th Bethesda Conference report. (5) (Level of Evidence: C)

c Contraception and pregnancy information, including education regarding risk of CHD in offspring (for men and women). (Level of Evidence: C)

d General medical/dental preventive care (eg, smoking cessation, weight loss/maintenance, hypertension/lipid screening, oral care, and substance abuse counseling). (Level of Evidence: C)

e Recommended follow-up with cardiology. (Level of Evidence: C)

4 Vocational referral and health insurance information should be offered to ACHD patients during the transition period and refreshed at the time of their initial consultation in a tertiary referral center and intermittently as indicated by their social situation. (Level of Evidence: C)

5 A formal transition process should be used to provide optimal transfer of patients into ACHD care. This process should begin by 12 years of age and should be individualized on the basis of the patient's maturity level, with the goal being to transition and ultimately transfer the patient into adult care settings depending on the stability of the disease and psychosocial status. (Level of Evidence: C)

6 A psychological evaluation should be obtained if an adult's mental competency is in question and no appointed adult surrogate is available. (Level of Evidence: C)

7 All ACHD patients should be encouraged to complete an advance directive, ideally at a time during which they are not extremely ill or hospitalized, so that they can express their wishes thoughtfully in a less stressful setting and communicate these wishes to their families and caregivers. (Level of Evidence: C)

The degree of psychological impact caused by CHD remains ill defined. Results from decades of literature are divided with regard to the psychological functioning of ACHD patients. Methodologically, the challenges of controlling for medical, social, demographic, genetic, and cognitive variables that interact with psychological development make it difficult to draw general conclusions from studies (13,14); however, important clues regarding psychosocial outcomes have been useful in guiding medical therapy and thus form the foundation for comprehensive management of ACHD patients.

Early studies of psychosocial function dealt only with children and often reflected populations that confronted unrepaired CHD for longer periods of time. Thus, research focused on the "sick child" and recognized a recurrent theme of parental overprotection, as well as profiling the effect CHD had on the family unit (15,16). Maternal perceptions, accurate or not, were far more closely correlated to maladjustment in children than was medical severity of the child's illness (13,17). Intuitively, the psychopathology of children with CHD, imparted by physiological stress during early childhood, disruption of family dynamics, altered school and peer structure, and other unmet childhood milestones, may leave cognitive and psychological marks that carry over into adult life. Although there is evidence that argues for earlier reparative surgery to minimize childhood insecurities and morbidity (18), a correlation between the severity of CHD and psychological adjustment has not been substantiated (16,17,19–22). Moreover, new information is emerging about cognitive functioning in adolescents who underwent surgical repair in infancy with cardiopulmonary bypass that indicates some deficits in planning and self-management (23–27). Long-term behavioral outcome studies after the neonatal arterial switch operation (ASO) for transposition of the great arteries (TGA) have demonstrated highly specific disabilities that might impact the quality of self-care (28). Longer survival and decreasing morbidity among ACHD patients has made quality-of-life issues much more central to the understanding and management of this population (14,29–39). Some quality-of-life issues pertinent to ACHD patients, regardless of severity of disease, include independent living arrangements, education, employment, sports, health and life insurance acquisition, contraception, genetic counseling, and pregnancy concerns (40).

Circumstantial depression and anxiety are understandable in older adolescents and young adults with chronic health problems. One pilot study suggests that up to one third of ACHD patients may have a psychiatric disorder, with depression and anxiety being most prominent (41), whereas only 20% of the general population are afflicted with psychiatric illness (42). Accordingly, a careful assessment of depressive symptoms and their possible overlap with symptoms of medical illness or side effects of medications must be part of the clinical evaluation of ACHD patients (13,14).

1.5.3. Transition of Care
Physical and emotional maturity is the primary requirement for transfer of adolescent or young adult patients into adult care environments. The age at which this occurs varies and may range from the mid-teens to the mid-20s, depending on the patient. However, the process of transitioning, that is, preparing young patients for successful transfer to an adult healthcare provider at a later time, should begin by the age of 12 years (43).

Strategies for transfer of patients with CHD into adult care settings are well described (44,45) and use a stepwise approach to establishing autonomy and understanding one's cardiac problem and lifestyle issues important to long-term stability of CHD. Pediatric clinicians can reinforce autonomy by focusing their communication on the patient, so that the teen years serve as an ongoing "workshop" in which the ultimate goal is accepting ownership of and responsibility for one's cardiac disease. Parents should take an active role in fostering independence in their teenagers. The use of support groups and educational meetings geared toward parents and ACHD patients offers a prime opportunity for parents to discuss their fears and openly communicate reality-based strategies for approaching difficult topics with their children. National support organizations for CHD and ACHD patients now exist and provide resources for families (eg, the Congenital Heart Information Network and the Adult Congenital Heart Association). Regional tertiary centers for the care of ACHD patients may also provide conferences that serve this purpose. Some centers provide transitional support meetings so that adolescents and parents can familiarize themselves with the goals of ACHD care. Despite the availability of structured resources for parents, patients, and families with CHD, the ultimate responsibility still rests with clinicians to meet the educational needs of their young patients. Topics that should be discussed early in childhood and repeatedly through the teens, 20s, and beyond include a description of the cardiac defect and surgeries (including use of diagrams); medications; exercise prescription; risk modification; health maintenance and follow-up recommendations; vocational and educational recommendations; insurance information; and information about genetics, contraception, and pregnancy. This information should be given in verbal and written form and provided to the patient in an electronic or paper format (45,46). This is a reference tool that can be a constant resource for the patient long term and can assist healthcare providers who are not familiar with the patient. The use of advanced practice nurses and physician assistants in pediatric and ACHD settings optimizes the facilitation of the transition process from pediatrics to adult cardiology, identification of patient needs, screening and referral for psychosocial problems, and education and counseling of patients and families (47).

Pertinent medical records, including diagrams of cardiac defects and operations, operative and procedural reports, recent physical examination, electrocardiograms (ECGs), and echocardiograms, should be provided to all cardiologists involved in the care of a patient with CHD. In addition, once patients are properly educated and aware of basic terminology pertaining to their own cardiac status, they should be offered copies of their medical reports, which implies and imparts responsibility and autonomy regarding their condition.

1.5.4. Exercise and Athletics
Exercise restrictions correlate with internalization of fear in young people with CHD (48). The ability to exercise is a fundamental measure of quality of life, perceived capacity for social acceptance, employment, sexual relations, and procreation. Young people with CHD may experience exercise limitations for many reasons, including their underlying cardiac reserve, physical deconditioning, and lack of exercise experience in childhood; poor coordination related to coexisting disabilities; misperceptions about restrictions; lack of interest; and anxiety (43,44). Current symptoms only account for approximately 30% of all barriers to exercise. Recommendations regarding physical training, exercise, and athletics are core to the comprehensive patient education that should begin by early adolescence. An individual exercise prescription (one that accounts for physical limitations, developmental challenges, risk modification, health concerns such as obesity, and personal preferences) needs to be provided and updated regularly so that the beneficial utility of exercise is not lost among a list of restrictions. Guidelines for physical activity and exercise in patients with CHD are outlined in the 36th Bethesda Conference in "Eligibility: Recommendations for Competitive Athletes with Cardiovascular Abnormalities." (49) Currently, however, there are few data concerning activity guidelines for the nonathlete.

The finding of diminished aerobic capacity in all groups with CHD (50–58) validates the importance of comparative testing over time in patients until reference values can be researched further (54). However, improved oxygen uptake during exercise is only 1 parameter of the effect of training and cannot be used alone to determine whether the main goals of exercise have been achieved (59). Beyond improved oxygen consumption and tolerance of physical activity, physical training of children and adolescents can also result in decreased withdrawal and somatic complaints (60,61). This supports the need for organized exercise programs for young people with CHD, particularly adolescents who view physical activity as the defining focus of a healthy lifestyle despite restrictions from competitive athletics.

1.5.5. Employment
Entering the job market and establishing a career is arguably the most important developmental milestone of a young adult's life (30). Careful consideration of the individual's physical, mental, and psychological disposition will help in identifying the right career choice. This is a discussion that should include the cardiologist, so that realistic limitations are explored and misconceptions eliminated. Vocational planning should take place early in adolescence so that appropriate educational options can be pursued long before the patient enters the work force. Ideally, cognitive evaluation should be performed at or before 5 years of age so that the appropriate educational track can be determined. Early childhood intervention has been shown to result in improved employment status during adult life (62). The goal of clinicians caring for those with CHD should be to view every patient as employable and avoid the temptation to accept the status quo when a patient is receiving social security disability income or other disability assistance through Medicaid or Medicare. Governmental assistance programs may perpetuate long-term disability for those fearful of losing their health insurance coverage (63). Reports from more than a decade ago projected that approximately 10% of ACHD patients would be totally disabled (64). Furthermore, 8% to 13% of ACHD patients were receiving public assistance or living as a dependent with relatives (64,65). Important legislation has focused on bringing individuals with disabilities into the work force. Ultimately, with improving longevity in patients with CHD and better surgical outcomes, the proportion of physically disabled ACHD patients is expected to decrease.

Seeking assistance from the state employment development department (the names of these programs vary from state to state) can be an important step in finding jobs for adults with disabilities. These programs offer job and training referrals, counseling, and job search assistance and workshops. Federal programs provide for vocational rehabilitation for disabled individuals, as well as hiring and placement into jobs appropriate for their level of disability.

The Americans With Disabilities Act of 1990 prohibits discrimination with respect to hiring, promotion, or termination of employees on the basis of disability. Therefore, ACHD patients are not required to disclose their cardiac condition to a prospective employer unless physical restrictions imposed by their cardiac disease would limit their ability to satisfy the job description (63). The Family and Medical Leave Act of 1993 states that covered employers must grant eligible employees up to a total of 12 workweeks of unpaid leave during any 12-month period when the employee is unable to work because of a serious health condition or to take care of an immediate family member with a serious health condition (66).

The Work Incentives Improvement Act of 1999 (also known as the Medicaid Buy-In Program) is designed to promote employment and economic self-sufficiency for individuals with disabilities. Under this federal legislation, states can amend their Medicaid programs to enable individuals with disabilities to obtain coverage under Medicaid while giving incentives for these individuals to seek and maintain employment. Advocates in each ACC and AHA chapter should work with state Medicaid programs and state legislators to define appropriate health disabilities eligible for coverage (10).

1.5.6. Insurability
In the 1990s, studies indicated that up to 20% of ACHD patients were uninsured and that most health insurance policies were individual plans rather than group policies. The heterogeneity of CHD over the life span contributes to the difficulties faced by insurers when assigning risk. Regional tertiary centers specializing in the care of ACHD patients must collaborate in multicenter studies to define and publish survival data in a format amenable to life-table analysis (67). In addition, the use of clinical practice guidelines, such as those outlined in this ACC/AHA document, will further direct insurers, primary care providers, and cardiologists on the appropriate use of diagnostic testing, as well as the appropriate time for referral.

Today, the most affordable way to obtain health insurance is via a group policy, through one's employer, or with health management organizations. With national attention now focused on the need for regional tertiary care for patients with complex CHD, health maintenance organizations are being held accountable for finding appropriate specialized care for ACHD patients. Patients, with their physician's support, need to be their own advocates within the health maintenance organization system and demand referrals if they believe their cardiologist is ill equipped to manage their complex cardiac care. National patient support organizations such as the Adult Congenital Heart Association have made it their mission to educate adults via newsletters, the Internet, and the like about the need for specialized cardiac care in ACHD patients with moderate to severe disease, and they provide an extensive referral network of ACHD specialists.

Currently, at least 30 states offer high-risk health insurance plans through "risk pools." This provides a safety net for individuals who are denied health insurance because of a preexisting medical condition. More than 250 000 enrollees have been able to obtain comprehensive health insurance protection via these risk pools since the first pools were started in 1976. Risk pools are state created, are nonprofit, and usually do not require tax dollars for operational purposes. Eligible individuals must prove state residency and prove they have been rejected for similar health insurance with similar premiums by at least 1 insurer. Healthcare providers caring for ACHD patients should be aware of options available in their state. Healthcare providers need to give accurate health information to insurers in a prompt fashion so that a fair evaluation of the patient's risk status can be made.

Health and life insurance can be elusive to ACHD patients. Regardless of severity, ACHD patients face a significantly higher risk of being denied life insurance than their peers without CHD (10,40,68). Recent studies found that more than one third of ACHD patients were refused life insurance compared with 4% of age-matched peers without CHD, with no regard to severity of defect (40). A useful resource for state-by-state consumer guidelines about getting and keeping health insurance can be accessed via the Internet at www.healthinsuranceinfo.net.

1.5.7. Congenital Syndromes
Congenital syndromes, including coexisting neurological and cognitive deficits, can further complicate the psychological and social adjustment of ACHD patients. Approximately 18% of congenital heart defects are associated with a congenital syndrome or chromosomal abnormality (69). Among chromosomal abnormalities in infants with cardiovascular defects, 81% are Down syndrome, which has a CHD prevalence of 40%. Adults with Down syndrome represent a growing number of the patients seen in tertiary ACHD clinics and require careful attention to coexisting diseases and special care issues. Hypothyroidism, leukemia, Alzheimer's disease, depression, atlantoaxial subluxation, obesity, and sleep apnea are common in Down syndrome, and regular screening for these ailments should be performed. Often, sedation or general anesthesia is necessary for routine procedures, such as dental cleaning, Pap smears, or prolonged diagnostic procedures that require immobility. The risks of anesthesia and procedures need to be carefully reviewed by CHD specialists and discussed with the patient.

Approximately 15% of patients with tetralogy of Fallot and other conotruncal defects have chromosome 22q11.2 deletion, most commonly manifested as DiGeorge syndrome but also presenting as velocardiofacial (Shprintzen) syndrome and conotruncal anomaly face (Takao) syndrome (70). Patients with a history of type B interrupted aortic arch or truncus arteriosus also have a high incidence of DiGeorge syndrome. Many patients with this chromosome deletion show impairment in social function. Coexisting diseases associated with these overlapping syndromes include schizophrenia, mental disability, deafness, immune deficiencies, endocrinopathies, and clubbed foot (70).

Williams syndrome is a developmental disorder that involves connective tissue, the central nervous system, and supravalvular AS (SupraAS); it has been associated with a chromosome deletion in band 7q11.23 (70). Most Williams syndrome patients have a lack of social inhibition and some degree of mental disability that complicates planning and self-management. Adults with other syndromes, including Noonan and Turner syndromes, have varying degrees of cognitive deficits. Patients with Turner syndrome have a variety of noncardiovascular problems, including ovarian and thyroid disorders, inflammatory bowel disease, pigmented and melanotic nevi, and sensorineural deficits.

Because the cardiologist may be the only regular healthcare provider for adults who have congenital syndromes and chromosomal abnormalities in association with their CHD, careful screening and appropriate referrals (such as endocrinology, genetic counseling, psychiatry, and vocational rehabilitation) should take place. Because of the multiplicity of comorbidities and the potential for impact on cardiovascular management, there should be clarity about which healthcare provider is serving as the medical "home." Whenever possible, the ACHD specialist should work closely with a primary physician who accepts this responsibility. Although many of these patients are dependent on others for long-term care, some are able to live independently and require sensitive counseling regarding healthcare maintenance and risks. Advice regarding sexual activity and contraception is essential, even if the patient does not request it. Genetic counseling should be offered to all patients.

1.5.8. Medical/Ethical/Legal Issues
Some ACHD patients, especially those with associated syndromes, may be incapable of providing informed consent to the degree that meets ethical and legal standards of understanding their situation, understanding the risks associated with the decision at hand, and communicating a decision based on that understanding. Adults who may require a legal surrogate to facilitate informed consent are those who are cognitively impaired, such as those with Down syndrome, and those with impaired consciousness due to severe illness. If an adult's mental competency is in question, and no appointed adult surrogate is available, a psychological evaluation should be requested (63). The issue of legal guardianship in adults with significant mental disability becomes an ethical and legal challenge when 1 or both parents die or become incapacitated by illness with no accommodation for transfer of guardianship. Guidance in addressing these issues should be included as part of the transition education and reinforced thereafter.

Advance directives can assist family members and healthcare providers in understanding a patient's wishes if they are incapable of speaking for themselves (71). All ACHD patients should be encouraged to complete an advance directive, ideally at a time during which they are not morbidly ill or hospitalized, so that they can express their wishes in a less stressful setting.

1.6. Recommendations for Infective Endocarditis.   Class I

1 ACHD patients must be informed of their potential risk for IE and should be provided with the AHA information card with instructions for prophylaxis. (Level of Evidence: B)

2 When patients with ACHD present with an unexplained febrile illness and potential IE, blood cultures should be drawn before antibiotic treatment is initiated to avoid delay in diagnosis due to "culture-negative" IE. (Level of Evidence: B)

3 Transthoracic echocardiography (TTE) should be performed when the diagnosis of native-valve IE is suspected. (Level of Evidence: B)

4 Transesophageal echocardiography (TEE) is indicated if TTE windows are inadequate or equivocal, in the presence of a prosthetic valve or material or surgically constructed shunt, in the presence of complex congenital cardiovascular anatomy, or to define possible complications of endocarditis (eg, sepsis, abscess, valvular destruction or dehiscence, embolism, or hemodynamic instability). (72) (Level of Evidence: B)

5 ACHD patients with evidence of IE should have early consultation with a surgeon with experience in ACHD because of the potential for rapid deterioration and concern about possible infection of prosthetic material. (Level of Evidence: C)

Class IIa

1 Antibiotic prophylaxis before dental procedures that involve manipulation of gingival tissue or the periapical region of teeth or perforation of the oral mucosa is reasonable in patients with CHD with the highest risk for adverse outcome from IE, including those with the following indications:

a Prosthetic cardiac valve or prosthetic material used for cardiac valve repair. (Level of Evidence: B)

b Previous IE. (Level of Evidence: B)

c Unrepaired and palliated cyanotic CHD, including surgically constructed palliative shunts and conduits. (Level of Evidence: B)

d Completely repaired CHD with prosthetic materials, whether placed by surgery or by catheter intervention, during the first 6 months after the procedure. (Level of Evidence: B)

e Repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device that inhibits endothelialization. (Level of Evidence: B)

2 It is reasonable to consider antibiotic prophylaxis against IE before vaginal delivery at the time of membrane rupture in select patients with the highest risk of adverse outcomes. This includes patients with the following indications:

a Prosthetic cardiac valve or prosthetic material used for cardiac valve repair. (Level of Evidence: C)

b Unrepaired and palliated cyanotic CHD, including surgically constructed palliative shunts and conduits. (Level of Evidence: C)

Class III

1 Prophylaxis against IE is not recommended for nondental procedures (such as esophagogastroduodenoscopy or colonoscopy) in the absence of active infection. (Level of Evidence: C)

The clinical setting and presentation of endocarditis have changed over the last 50 years, in part owing to technical advances (eg, cardiac surgery, hemodialysis), the use of prosthetic devices and indwelling lines, the increasing prevalence of intravenous drug abuse, the emergence of resistant organisms, and the continued development of increasingly potent antibiotics (73–78). True surgical cures of congenital cardiovascular disorders are infrequent, and almost all patients who have undergone surgery are left with some form of residua or sequelae, many of which predispose to IE (73,74,77–82).

Epidemiological studies of IE have reported underlying CHD in 11% to 13% of cases (83,84). Li and Somerville reported that IE accounted for 4% of admissions to a specialized adult congenital heart service (85). Including pediatric data, certain unoperated and operated cardiac lesions may be more susceptible to infection (Table 6). Tetralogy of Fallot, TGA, unrepaired ventricular septal defect (VSD), patent ductus arteriosus (PDA), and bicuspid aortic valves (BAVs) with aortic valve stenosis or aortic regurgitation (AR) are susceptible to IE.(73,74,79,81,86–102) Patients who have had surgical palliation of CHD (eg, systemic–to–pulmonary artery shunts) or various types of reparative surgery (often requiring prosthetic materials or valves, conduit insertion, or conduit replacement) have major predisposing conditions for IE (74,79,81,103).

Li and Somerville (85) reported IE in the grown-up congenital heart population comprising 185 patients (214 episodes) during 2 periods, 1983 to 1993 and 1993 to 1996, divided into unoperated or palliated (group I) and operated definitive repair or valve repair/replacement (group II). They noted no IE in atrial septal defect (ASD), closed VSD, patent ductus, isolated PS, or unrepaired Ebstein's anomaly or after Fontan-type or Mustard operations. IE in group I was most commonly represented by VSD (24%), left ventricular outflow tract (LVOT) lesions (17%), and mitral valve disorders (13%) and in group II by LVOT lesions (35%), repaired tetralogy of Fallot (19%), and atrioventricular (AV) defects (14%). Of the 185 patients, 87 (47%) had a known predisposing event (dental procedure or sepsis in group I, 33%; cardiovascular surgery in group II, 50%). Diagnosis was delayed (from onset of symptoms to time of diagnosis) in group I by 60 days and in group II by 29 days.

Niwa et al in 2005 (105) reported IE in 170 pediatric and 69 adult patients from 1997 to 2001. They noted prior cardiac surgery in 199 patients with IE, 88 of whom had surgery for cyanotic cardiovascular malformations. IE was left-sided in 46% and right-sided in 51%; the most common organisms were streptococci (50%) and staphylococci (37%). Surgery during IE was needed in 26% for large vegetations (45%) and heart failure (29%). Complications were seen in 48.5%. Mortality was 8% for medical treatment alone and 11.1% for those who also required surgery. In 33.3% of patients, conditions and procedures associated with IE were identified that preceded IE; the most common were dental manipulation (37.2%) and cardiovascular surgery (25.6%), followed by pneumonia (14.1%). Of these cases, only 28.2% had received antibiotic prophylaxis.

Di Filippo et al reported in 2006 (106) on 153 episodes of IE in CHD diagnosed with the revised Duke criteria, showing an increasing rate with 81 episodes from 1966 to 1989 (3.5 per year) and 72 episodes from 1990 to 2001 (6 per year). During the second period, there were more adults (40% later versus 9% earlier). Of interest, CHD was known in 122 patients before IE but was unrecognized in 31. Of the 153 episodes of IE, 39 occurred in patients who had repaired lesions, 35 in patients who had palliation (usually complex disease), and 79 in patients who had unoperated CHD. Tetralogy of Fallot with IE decreased from 12% to 3%, and complex cyanotic disease increased from 14% to 28%; the proportion of aortic valve anomalies and small VSDs increased. Dental procedures as a presumed cause of IE were more common during the later period (33% versus 20% earlier), cutaneous infection rose to 17% (from 5% earlier), and postoperative infection appeared less frequently later (11%) than earlier (21%). The streptococcus group continued to represent the most prevalent organisms, followed by staphylococci. Their data emphasized that current targets of prevention of IE should include complex cyanotic lesions, lesions repaired with prosthetic material, and small VSDs.

The pathogenesis of IE in part requires damaged or traumatized endothelium and a portal of entry of bacteria into the bloodstream. Bacteria may bind to platelets in the blood pool and then be deposited at the site of vascular endothelial damage. The infective lesion usually occurs at the low-pressure end of a high-gradient lesion at the site of impact of a high-velocity jet. For example, vegetations in conjunction with aortic coarctation may occur in the downstream descending aorta. With aortic valve disease, not only may vegetations occur on the ventricular surface of the valve, but the regurgitant jet impacting on the mitral valve may cause secondary vegetation. Usual sites of vegetations with a restrictive VSD occur where the high-velocity left-to-right jet impacts on the right side of the heart (ie, tricuspid valve septal leaflet or mural RV endocardium). The consequences of the infective vegetation depend on the site or structure involved and the virulence of the organism. Valvular destruction with significant valvular regurgitation or fistulous connections can cause heart failure. Endarteritis (as with patent ductus and coarctation) can cause aneurysm formation with potential for rupture. Embolization of vegetative material can cause arterial obstruction (eg, stroke) and possible abscess formation, and right-sided embolization to the lung can mimic pneumonia. Immunologic reactions can trigger glomerulonephritis or vasculitis as a result of the deposition of circulating immune complexes in the small vessels in skin (Janeway lesion and Osler node) (75).

Many cases of clinically suspected IE are difficult to diagnose with certainty because of altered immune response, prior antibiotic exposure, or indolent organisms and in some patients with acute right-sided IE in whom the systemic and immune responses have not developed (73,75,76,80,81). Now widely accepted, Durack et al incorporated 2-dimensional echocardiography as a means of demonstration of vegetations (77,107). The Duke criteria defined 2 major criteria (positive blood culture with typical microorganisms and evidence of endocardial involvement, eg, a typical vegetation on an echocardiogram) and 6 minor criteria (ie, predisposition, fever, vascular phenomena, immunologic phenomena, suggestive microbiological evidence, and echocardiographic findings consistent with endocarditis but not meeting major echocardiographic criteria), with categories defined as definite, possible, and rejected (107). Echocardiography is crucial in the diagnosis of IE. In general, a TTE study is useful in confirming the presence of vegetation, but often, the sensitivity is too low to rule out its absence. If a TTE study is equivocal, or in the presence of a prosthetic valve or complex congenital cardiovascular anatomy in which transthoracic windows may be inadequate, TEE is indicated (73 to 79, 81, 108 to 112). TEE is particularly important in the search for IE in the adolescent and adult for evaluation of the thoracic aorta, ventricular outflow tracts, and valved conduits and for visualization of the entire ventricular septum. Given the complexity of many of the malformations and the wide array of surgical palliations and repairs, however, performance and interpretation of echocardiography must be done by those with expertise in the native and altered postoperative (109) anatomy (103,108,110–112).

A delay in diagnosis of IE carries the risk of significant morbidity and mortality. A high index of suspicion for IE in any patient with operated or unoperated CHD is a key to early diagnosis (74,78,79,81,103,113–115). Cardiac lesions and their relative risk of developing IE are listed in Table 7.

Additional issues more specific for patients with CHD and risk for IE may not be well recognized by many practitioners (72,74,78–80). Patients with unoperated cyanotic heart disease frequently have acne or have spongy, friable gums; appropriate care is necessary to diminish the risk of bacteremia. Epistaxis and hemoptysis are frequent in the cyanotic patient; nasal cautery may be a risk factor for IE. Nail biting is a problem, with the possibility of local infection being a focus for bacteremia. High-risk behaviors (eg, intravenous drug abuse, body piercing, or tattoos) not uncommon in adolescents and young adults in particular are well-known risk factors, especially for acute staphylococcal IE on the right side of the heart, and the patient should be informed of the risks of these activities. The use of intrauterine devices for female contraception is somewhat controversial because of concern about endocarditis, although the rate of infection is only approximately 1.4 times normal, provided that sexual relationships are monogamous. The AHA recommendations do not advise antibiotic prophylaxis for patients before genitourinary procedures, but because of the high risk for adverse outcome in patients with prosthetic cardiac valves and those with cyanotic CHD and the potential for infection in the setting of a complex vaginal delivery, this committee proposed that antibiotics might be considered in those high-risk patients at the time of membrane rupture (recognizing that proof of efficacy of prophylaxis is not available) (117).

In all cases of IE, cultures should be obtained to try to establish the causative organism before antibiotics are initiated (73,75,78,82,112). CHD patients who present with fever and potential IE should have blood cultures drawn before antibiotic therapy is initiated to avoid subsequent false-negative blood cultures (78,103,118). Recognizing that initial therapy is usually parenteral and usually intravenous, one should recall that cyanotic patients with right-to-left shunts have the possibility of "paradoxical" systemic embolization and, as such, risk of stroke. Air filters should be used on the line with meticulous attention to avoiding injection of air bubbles. Details of all aspects of medical and antimicrobial management of IE are beyond the scope of this review and are addressed by a separate AHA working group, as well as other authors (78,81,99,119,120). Collaboration with an infectious disease specialist is invaluable. Prompt referral of the adult patient with CHD to a specialized center is usually indicated because hemodynamic deterioration may be rapid, and surgical treatment of often complicated anatomy and/or reoperation may be required (78,82). Early consultation with a cardiac surgeon with experience treating adults with CHD is appropriate. Surgical intervention is considered in patients with uncontrolled congestive heart failure, continued emboli, medically uncontrolled infection, prosthetic material infection, and development of heart block (73,75,78,80,112–114,121). Management decisions regarding infected prosthetic valves or conduits in which the duration of preoperative antibiotic therapy must be balanced against the risk of reoperation must be made in collaboration with the surgeon. Ultimately, to reduce costs without risking efficacy, prolonged home parenteral antibiotics may be required after the initial inpatient hospitalization.

Prevention of IE includes nonchemotherapeutic and chemotherapeutic methods (74,75,78,80,81,103). Clinical judgment and discretion are required. It is always worthwhile to strive to provide evidence-based medical care. However, the Cochrane Collaboration did not provide evidence proving whether or not penicillin prophylaxis is effective protection against bacterial endocarditis in those with lesions considered at risk for development of IE and who were about to undergo an invasive dental procedure. They also noted that there is lack of evidence to support published guidelines in this area or to show whether the potential harm or cost of penicillin outweighs the benefit (122).

On the basis of a "revised" assessment regarding the risk of bacteremia-induced endocarditis, new guidelines for the prevention of endocarditis were published in 2006 by the Working Party of the British Society for Antimicrobial Chemotherapy (123). The 2006 guidelines recommend that antimicrobial prophylaxis for dental procedures be confined to those with (1) previous IE, (2) prosthetic cardiac valves, or (3) surgically constructed pulmonary shunts or conduits. In contrast, for bacteremic nondental procedures, the 2006 group expanded the "dental risk" list to also include (4) complex CHD (except not secundum ASD, which is presumably isolated and uncomplicated), (5) complex LVOT obstruction, including AS and BAVs, (6) acquired valvulopathy, and (7) mitral valve prolapse in the presence of echocardiographic "substantial leaflet pathology and regurgitation." The British Society for Antimicrobial Chemotherapy justifies its decisions by shifting the emphasis from "procedure-related bacteremia" to "cumulative bacteremia."

The 2007 AHA guidelines for the prevention of endocarditis have substantially changed the recommendations for antibiotic prophylaxis on the basis of a consensus of expert opinions (72). The new, simplified recommendations are based on the proposition that most bacteremia occurs during activities of daily living, that IE is more likely to result from long-term cumulative exposure to these daily random bacteremias than from procedural bacteremias, and that proof is lacking that prophylaxis prevents any (or at most a very small number) cases of IE. They posit that the risks of antibiotic adverse events in the patient (allergic reactions) and the emergence of resistant organisms exceed any proven benefit of antibiotic prophylaxis against IE.

The new AHA guidelines appropriately emphasize maintenance of oral health and hygiene to reduce daily bacteremia and underscore that this is more important than any dental antibiotic prophylaxis. Accordingly, the 2007 AHA writing committee for the updated guidelines on prevention of endocarditis concluded that antibiotic prophylaxis for dental procedures likely to induce procedural bacteremia (those that involve manipulation of gingival tissue or the periapical region of the teeth or perforation of the gingival mucosa) should be confined to cardiac conditions associated with the most significant adverse outcomes should IE develop (72). They included in this group those with previous IE; those with prosthetic cardiac valves or surgically constructed conduits or shunts; those with unrepaired cyanotic CHD or CHD repaired with prosthetic material or devices (until 6 months after the procedure); those with repaired CHD with residual defects at or adjacent to the site of a prosthetic patch or device; and cardiac transplant patients who develop valvulopathy. They specifically recommend no IE prophylaxis before gastrointestinal or genitourinary procedures, a major departure from previous guidelines. The new AHA guidelines have engendered some considerable controversy and may violate long-standing patient and provider expectations and practice. Concern has been expressed that changes in preexisting recommendations were not based on new data or randomized trials and that absence of proof of efficacy and safety cannot be used as proof of absence of efficacy and safety of antimicrobial prophylaxis (124). The present ACHD Guideline Committee understands that there may be reluctance to deviate from prior recommendations for patients with some forms of CHD. This reluctance may be especially true for patients with BAV or coarctation of the aorta. In select circumstances, the committee understands that some clinicians and some patients may still feel more comfortable continuing with IE prophylaxis. Accordingly, this committee recommends that healthcare providers discuss the rationale for these new changes with their patients, including the lack of scientific evidence demonstrating proven benefit for IE prophylaxis. In those settings, the clinician should determine that the risks associated with antibiotics are low before continuing a prophylaxis regimen. Over time, and with continuing education, the committee anticipates growing acceptance of the new guidelines among both provider and patient communities.

This ACHD writing committee proposes that the "high-risk" group in whom it is reasonable to give antibiotic prophylaxis before dental procedures would include the following: (1) those with a prosthetic cardiac valve; (2) those with prior IE; (3) those with unrepaired and palliated cyanotic CHD, including surgically constructed palliative shunts and conduits; (4) those with repaired CHD with prosthetic material or device, whether placed by surgery or by catheter intervention, during the first 6 months after the procedure; and (5) those with repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device that inhibit endothelialization.

We emphasize that nonchemotherapeutic methods are particularly important in the adolescent or young adult patient with CHD, among whom nail biting, acne, and problems with dental health are common. Oral prevention starts with meticulous oral care and routine preventive care by a dentist or oral hygienist. A patient with cyanotic heart disease often has spongy, friable gums, and a soft-bristle toothbrush must be used. Female contraception should be planned with the risks and benefits of intrauterine devices kept in mind.

The patient's knowledge of the need for and the type of IE prophylaxis is also an important issue (77,103,125). Caldwell et al noted that fewer than 50% of families knew about endocarditis prevention or precautions, and even fewer understood why prophylaxis was considered indicated (91). Cetta and Warnes reported in 1995 from their specialized ACHD clinic that those with CHD had inadequate knowledge about their cardiac lesion, about IE, and about prophylaxis (125). With aggressive education in their clinic, patient knowledge improved, but they emphasized that educational efforts need to be reinforced regularly. A patient should be given a detailed explanation of his or her diagnosis and the rationale for IE prevention, and the patient's specific regimen for dental procedures should be provided. Information about the signs and symptoms of IE should also be provided. At every subsequent visit, it should again be verified that the patient knows what is required for dental care and prophylaxis (74).

1.7. Recommendations for Noncardiac Surgery.   Class I

1 Basic preoperative assessment for ACHD patients should include systemic arterial oximetry, an ECG, chest x-ray, TTE, and blood tests for full blood count and coagulation screen. (Level of Evidence: C)

2 It is recommended that when possible, the preoperative evaluation and surgery for ACHD patients be performed in a regional center specializing in congenital cardiology, with experienced surgeons and cardiac anesthesiologists. (Level of Evidence: C)

3 Certain high-risk patient populations should be managed at centers for the care of ACHD patients under all circumstances, unless the operative intervention is an absolute emergency. High-risk categories include patients with the following:

a Prior Fontan procedure. (Level of Evidence: C)

b Severe pulmonary arterial hypertension (PAH). (Level of Evidence: C)

c Cyanotic CHD. (Level of Evidence: C)

d Complex CHD with residua such as heart failure, valve disease, or the need for anticoagulation. (Level of Evidence: C)

e Patients with CHD and malignant arrhythmias. (Level of Evidence: C)

4 Consultation with ACHD experts regarding the assessment of risk is recommended for patients with CHD who will undergo noncardiac surgery. (Level of Evidence: C)

5 Consultation with a cardiac anesthesiologist is recommended for moderate- and high-risk patients. (Level of Evidence: C)

Performance of any surgical procedure in ACHD patients carries a greater risk than in the normal population. Certain surgical procedures are frequently required in cyanotic patients, such as intervention for gallstones, scoliosis, and, less commonly, cerebral abscess. The risk for noncardiac surgery depends on the nature of the underlying CHD, the extent of the procedure, and the urgency of intervention. Table 7 lists lesions at moderate and high risk for noncardiac surgery.

A thorough evaluation of the patient with CHD should be undertaken before anticipated noncardiac surgery. Basic preoperative assessment includes an ECG, chest x-ray, TTE, and blood tests for full blood count and coagulation screen. It is recommended, when possible, that the preoperative evaluation and surgery be performed in an ACHD center by experienced surgeons and cardiac anesthesiologists. This allows close perioperative follow-up by an ACHD specialist. The specialist team should always be involved in the care of the complex and cyanotic adult patient with CHD, because this minimizes avoidable errors that can cause important morbidity or even death (126).

Select high-risk patient populations should be managed at centers for the care of ACHD patients under all circumstances, unless the operative intervention is an absolute emergency. These patients include those with prior Fontan procedure, severe PAH, cyanotic CHD, or complex CHD with residua such as heart failure, valve disease, or the need for anticoagulation.

Patients with cyanotic CHD, especially when associated with PAH, are at highest risk from noncardiac surgery (126). The bleeding risk can be reduced by preoperative phlebotomy if the hematocrit is more than 65% (127). Anesthetic management is critical, because a fall in systemic vascular resistance can worsen hypoxia, resulting in hemodynamic collapse. Long operations associated with hemodynamic instability and that require large-volume fluid replacement are associated with increased perioperative mortality. Fluid balance is critical in cyanotic and single-ventricle patients and those with heart failure because of occult renal failure in these patients.

Postoperatively, patients with CHD may need intensive care unit monitoring facilities even for relatively minor procedures. Nursing staff should be informed about the specific issues related to the CHD. Special issues that should be considered include administration of endocarditis prophylaxis, the need for anticoagulation around the time of the procedure, anticipation of special problems related to the underlying hemodynamics, filters for intravenous lines in cyanotic patients, prevention of venous thrombosis, monitoring of renal function, special care with drug administration, and the reduced arm blood pressure measurement in patients with prior classic Blalock-Taussig shunts. There is no evidence that cyanotic heart disease per se leads to liver disease (refer to Section 10, Tetralogy of Fallot, and Section 14, Tricuspid Atresia/Single Ventricle, for information regarding long-standing central venous hypertension leading to cardiac cirrhosis). There is increased prevalence of hepatitis C infection in adult patients who underwent CHD surgery before screening in 1992, and therefore these patients should be screened (128).

1.8. Recommendations for Pregnancy and Contraception.   Class I

1 Patients with CHD should have consultation with an ACHD expert before they plan to become pregnant to develop a plan for management of labor and the postpartum period that includes consideration of the appropriate response to potential complications. This care plan should be made available to all providers. (Level of Evidence: C)

2 Patients with intracardiac right-to-left shunting should have fastidious care of intravenous lines to avoid paradoxical air embolus. (Level of Evidence: C)

3 Prepregnancy counseling is recommended for women receiving chronic anticoagulation with warfarin to enable them to make an informed decision about maternal and fetal risks. (129–131) (Level of Evidence: B)

Class IIa

1 Meticulous prophylaxis for deep venous thrombosis, including early ambulation and compression stockings, can be useful for all patients with intracardiac right-to-left shunt. Subcutaneous heparin or low-molecular-weight heparin is reasonable for prolonged bed rest. Full anticoagulation can be useful for the high-risk patient. (Level of Evidence: C)

Class III

1 The estrogen-containing oral contraceptive pill is not recommended for ACHD patients at risk of thromboembolism, such as those with cyanosis related to an intracardiac shunt, severe PAH, or Fontan repair. (Level of Evidence: C)

Congenital malformations now represent the most common cause of maternal morbidity and mortality from heart disease in North America. Better assessment and management of this group of patients is likely to make a substantial improvement in outcomes for mother and baby (132).

Both men and women with ACHD should have a thorough understanding of the risks of transmitting CHD to their offspring. Counseling by an ACHD expert before pregnancy is important and should include genetic evaluation and, specifically for women, assessment of potential fetal risk, risk of prematurity or low birth weight in the offspring, review of medications that may be deleterious to the fetus, appropriate management of anticoagulation, and discussion of potential maternal complications (132). If pregnancy occurs, fetal echocardiography should be obtained and its consequences discussed (132).

The outcome of pregnancy is favorable in most women with CHD provided that functional class and systemic ventricular function are good. PAH presents a serious risk during pregnancy, particularly when the pulmonary pressure exceeds 70% of systemic pressure, irrespective of functional class. Events often occur after delivery (133). The need for full anticoagulation during pregnancy, although not a contraindication, poses an increased risk to both mother and fetus (134). The relative risks and benefits of the different anticoagulant approaches need to be discussed fully with the prospective mother. There is a small group of patients with complex CHD or high-risk disorders in whom pregnancy is either dangerous or contraindicated owing to the risk to mother or fetus. If pregnancy occurs and continues with any of these disorders, these high-risk patients should be managed and delivered in specialized centers with multidisciplinary expertise and experience in CHD, obstetrics, anesthesiology, and neonatology. A coordinated care pathway for supervision of delivery and the postpartum period needs to be developed and in place by the third trimester and made available to all caregivers and to the patient. A normal vaginal delivery or an assisted delivery is usually feasible and may be preferable for patients with CHD. Cesarean delivery is recommended in patients with CHD for obstetric reasons and for women fully anticoagulated with warfarin at the time of delivery due to the risk of fetal intracranial hemorrhage.

Medications should be used only when necessary in any pregnant patient. Certain medications are contraindicated during pregnancy; these medications include angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers. These medications cause congenital and renal disorders in the fetus when given during pregnancy; therefore, they should be discontinued before pregnancy occurs or early during pregnancy if possible (135). Warfarin should be used only after full discussion with the patient about the risks of using warfarin during pregnancy (136).

Although endocarditis is a recognized risk for maternal morbidity and mortality, endocarditis prophylaxis around the time of delivery is not universally recommended for patients with structural heart disease, because some believe that the risk of bacteremia is low. Others routinely administer antibiotics because it is not known in advance whether or not instrumentation will be required. Thus, there is no consensus on this point (117). Antibiotics should be considered for those at highest risk of an adverse outcome and, when appropriate, given as the membranes rupture. Intravenous amoxicillin and gentamicin should be considered for women with high-risk anatomy or previous history of endocarditis (see Section 1.6, Recommendations for Infective Endocarditis).

1.8.1. Contraception
It is the duty of the ACHD specialist to provide or otherwise make available informed advice on contraception, including discussion of risks. There are limited data on the safety of various contraceptive techniques in ACHD patients. The estrogen-containing oral contraceptive pill is generally not recommended in ACHD patients at risk of thromboembolism, such as those with cyanosis, prior Fontan procedure, atrial fibrillation, or PAH. In addition, this form of contraceptive therapy may upset anticoagulation control. However, medroxyprogesterone, the progesterone-only pills, and levonorgestrel may also cause fluid retention and should be used with caution in patients with heart failure. Depression and breakthrough bleeding may prevent the use of the progesterone-only pills, and there is a higher failure rate than with combined oral contraceptives.

Levonorgestrel, barrier methods, or tubal ligation are the recommended contraceptive methods for women with cyanotic CHD and PAH. The potential complications of the "morning after pill" (levonorgestrel "plan B") should be explained to those at risk of acute fluid retention. Tubal ligation, although the most secure method of contraception, can be a high-risk procedure in patients with complex CHD or those with PAH. Hysteroscopic sterilization (Essure) may be reasonable for high-risk patients (137). Sterilization of a male partner of a woman with CHD should only occur after full explanation of the prognosis to the patient. The specialist in the ACHD clinic needs to interact with both the general practitioner and the gynecologist to provide optimal advice regarding contraception. The risk of endocarditis with intrauterine devices in women with CHD is controversial, and recommendations should be individualized on the basis of discussions between the cardiologist and gynecologist.

Breast-feeding is safe in women with CHD. Women requiring cardiovascular medications should be aware that many of the medications will cross into breast milk and should clarify the potential effect of medications on the infant with a pediatrician.

1.9. Recommendations for Arrhythmia Diagnosis and Management.   Class I

1 Complete and appropriate noninvasive testing, as well as clear knowledge of the specific anatomy and review of all surgical and procedural records, is recommended before electrophysiological testing or device placement is attempted in ACHD patients. (Level of Evidence: C)

2 Decisions regarding tachycardia management in ACHD patients should take into account the broad cardiovascular picture, particularly repairable hemodynamic issues that might favor a surgical or catheter-based approach to treatment. (Level of Evidence: B)

3 Catheter ablation procedures for ACHD patients should be performed at centers where the staff is experienced with the complex anatomy and distinctive arrhythmia substrates encountered in congenital heart defects. (Level of Evidence: B)

4 Pacemaker and device lead placement (or replacement) in ACHD patients should be performed at centers where the staff is familiar with the unusual anatomy of congenital heart defects and their surgical repair. (Level of Evidence: B)

5 Epicardial pacemaker and device lead placement should be performed in all cyanotic patients with intracardiac shunts who require devices. (Level of Evidence: B)

Class IIa

1 It is reasonable to recommend the use of an implantable cardioverter defibrillator for any patient who has had a cardiac arrest or experienced an episode of hemodynamically significant or sustained ventricular tachycardia (VT). (Level of Evidence: C)

2 Pacemaker implantation can be beneficial in ACHD patients with bradyarrhythmias and may be helpful in overdrive pacing in patients with difficult-to-control tachyarrhythmias (see ACC/AHA/HRS 2008 Guidelines for Device-Based therapy of Cardiac Rhythm Abnormalities). (138) (Level of Evidence: B)

Class IIb

1 Pacemaker implantation may be beneficial for asymptomatic adult patients with resting heart rates of less than 40 beats per minute or abrupt pauses in excess of 3 seconds. (Level of Evidence: C)

Cardiac arrhythmias are a major source of morbidity and mortality for ACHD patients. Although rhythm disorders can often be observed in adults with unrepaired or palliated defects, the most difficult cases usually involve patients who have undergone prior intracardiac repairs, especially when this reparative surgery was performed relatively late in life (139,140). In this setting, the electrical pathology stems from the unique and complex myocardial substrates created by septal patches and suture lines in combination with cyanosis and abnormal pressure/volume status of variable duration. Virtually the entire spectrum of rhythm disturbances is manifested in these patients, including some disorders that are specific to the anatomic defect or the surgical technique used for repair (Table 8).

1.9.1. Management of Tachyarrhythmias: Wolff-Parkinson-White Syndrome
Accessory pathways can complicate certain forms of CHD, especially Ebstein's anomaly of the tricuspid valve (141). Tachycardia symptoms may begin in childhood but become increasingly problematic in adult years, when atrial dilation or surgical scars predispose the patient to atrial flutter or atrial fibrillation with potential for rapid conduction over an accessory pathway. An attempt at definitive therapy with catheter ablation has become the standard of care for these patients. However, compared with simple accessory pathway ablation in a structurally normal heart, the acute success rates are reported to be lower and the risk of recurrence higher in patients with anatomic defects (141–143). These differences appear to relate to the challenges of distorted anatomic landmarks, abnormal location for the AV node, and a high incidence of multiple pathways in the CHD population. Intraoperative accessory pathway ablation can be considered in the patient with Ebstein's anomaly referred for operative intervention for tricuspid valve disease. This approach has been demonstrated to be safe and effective (144).

1.9.2. Intra-Atrial Reentrant Tachycardia or Atrial Flutter
The most common form of tachycardia seen in the ACHD patient population is macroreentry within atrial muscle. This arrhythmia usually surfaces as a late postoperative disorder, and in children, it has been associated with chronotropic incompetence. Although it may arise after nearly any procedure that involves a right atriotomy (even simple closure of an ASD), the incidence is clearly highest after the Mustard, Senning, and Fontan operations, in which as many as 30% to 50% of patients can be expected to develop a symptomatic episode during extended follow-up (144,145). The term "intra-atrial reentrant tachycardia" (IART) has become the customary designation for this arrhythmia to distinguish it from classic atrial flutter seen in structurally normal hearts (146–148). Whereas typical atrial flutter involves a very predictable circuit around the tricuspid annulus that results in the familiar ECG appearance of sawtooth flutter waves at a rate of 300 beats per minute, IART can involve novel circuits around surgical scars and patches that generate a much wider spectrum of atrial rates and P-wave contours. Generally, IART tends to be slower than typical flutter, with atrial rates in the range of 170 to 250 beats per minute (144). In the setting of a healthy AV node, these rates will frequently allow a pattern of 1:1 AV conduction that may result in hemodynamic instability, syncope, or possibly death (149–151). Even if the ventricular response rate is safely titrated, sustained IART of long duration can be responsible for thromboembolic events.

Once IART is recognized, acute interruption is easily accomplished with either electrical cardioversion, overdrive pacing (150), or administration of certain class I or class III antiarrhythmic drugs (152). The far more difficult challenge is prevention of recurrence and adequate assessment of hemodynamic status that might predispose to recurrent tachycardia. Chronic antiarrhythmic drugs are still used in many cases, but the general experience with pharmacological therapy for this condition has been discouraging (149,153), which has led to a growing preference for nonpharmacological options at most centers.

Pacemaker implantation can be useful for those patients who have concomitant sinus node dysfunction as a prominent component of their clinical picture. Simply increasing the atrial rate to an appropriate level for the hemodynamic status can often result in marked reduction in IART frequency (150), while at the same time making it safer to prescribe medications that might aggravate bradycardia (138). Pacemakers with advanced programming features that incorporate atrial tachycardia detection and automatic burst pacing may also be beneficial in select cases (150,155) but carry the risk of accelerating the atrial rate and must thus be used cautiously in patients with robust AV conduction. Newer-generation implantable cardioverter defibrillators equipped with algorithms for both atrial tachycardia and VT detection and treatment, including atrial antitachycardia pacing and low-energy shocks for atrial tachycardia, have also been used successfully in a small number of ACHD patients with recurrent IART.

Catheter ablation has been adopted by many institutions as an early intervention for recurrent IART. The technique has evolved rapidly in terms of mapping accuracy and effectiveness, particularly since the introduction of 3-dimensional mapping technology for improved circuit localization (156,157) and irrigated-tip or large-tip ablation catheters for more effective lesion creation (158). With current technology, acute success rates of nearly 90% can be achieved with catheter ablation, although later tachycardia recurrence is still disappointingly common (159). The recurrence risk appears to be particularly high in the Fontan population of patients, who tend to have multiple IART circuits and the thickest/largest atrial dimensions. Although still far from perfect, ablation results for IART are likely to improve with continued advances in technology and even now are superior to the degree of control obtained with medications alone.

If the above measures fail to prevent IART recurrence, or if a patient with IART is returning to the operating room for hemodynamic reasons, consideration should be given to surgical ablation during a right atrial Maze operation. This procedure is used most commonly for the Fontan population with the most refractory variety of IART and is usually combined with revision of the Fontan connection or conversion from an older atriopulmonary anastomosis to a cavopulmonary connection. Results are encouraging, with very low rates of IART recurrence (160), but the surgical risks must be weighed against the electrophysiological benefit.

1.9.3. Atrial Fibrillation
Although far less common than IART in ACHD patients, atrial fibrillation is no less difficult to treat. It occurs most often in patients with congenital AS, mitral valve disease, or palliated single ventricles (161). Management principles are similar to atrial fibrillation encountered in other forms of heart disease, beginning with medical therapy for anticoagulation and ventricular rate control as needed, followed by electrical cardioversion. Class III antiarrhythmic agents may offer protection against recurrence of atrial fibrillation for some patients, but as in the case of IART, drug therapy has been only marginally successful for this group. Also similar to IART, pacemaker implantation may reduce atrial fibrillation episodes in patients with concomitant sinus node dysfunction. Successful elimination of atrial fibrillation has also been reported after combined right and left atrial Maze operations, which may be reasonable to consider if a patient requires cardiac surgery to address hemodynamic issues. Catheter ablation has not yet been extended in any systematic way to atrial fibrillation in the ACHD patient population.

1.9.4. Ventricular Tachycardia
There are several scenarios in which high-grade ventricular arrhythmias may develop in the ACHD patient. The most familiar involves macroreentrant VT as a late complication in postoperative patients who have undergone ventriculotomy and/or patching of a VSD, such as tetralogy of Fallot repair. In such cases, the reentry circuit is typically caused by narrow conduction corridors around regions of scar in the RV outflow tract (RVOT). The incidence of late VT or sudden death for repaired tetralogy has been estimated between 0.5% and 6.0% in various series (140,162,163). Some patients with slow organized VT may be hemodynamically stable at presentation, but VT tends to be rapid for the majority, producing syncope or cardiac arrest as the presenting symptom. The clinical picture is often confounded by the fact that symptomatic atrial tachycardias are also common in ACHD patients (164), which makes it difficult at times to tell whether an event was caused by VT, IART, or both.

Predicting which CHD patients will develop VT in advance of an episode remains a challenge. Studies seeking risk factors in the population with tetralogy of Fallot have identified older age at time of reparative surgery, advanced degrees of RV dilation, and prolonged QRS duration greater than 180 milliseconds as independent variables (140,165–167), although the predictive accuracy for each of these factors is imperfect. Holter monitoring and exercise testing have also been examined as screening tools with some degree of correlation between spontaneous ectopy and future VT events, but because ectopy on ambulatory monitoring is nearly ubiquitous in this population, the positive predictive value is diluted. Formal ventricular stimulation study can discriminate between high- and low-risk CHD patients (168,169) but remains too imperfect and too impractical to be recommended as a general screening tool. Intracardiac electrophysiology testing is usually reserved for selected patients with concerning symptoms or Holter findings when VT is suspected but not yet proven. At present, there is no generally accepted scheme for rhythm surveillance in asymptomatic patients with tetralogy of Fallot. Some combination of the above tests must be viewed in the context of the individual patient's history and general hemodynamic status to guide testing and treatment decisions whenever symptoms are minimal or absent. Symptoms of palpitations, dizziness, or unexplained syncope would obviously heighten the index of suspicion and should trigger a thorough and prompt diagnostic evaluation, which probably should include formal electrophysiological testing.

Although tetralogy of Fallot is typically cited as the archetypal lesion when VT in the ACHD patient population is discussed, serious ventricular arrhythmias may also develop in a number of other malformations, even in the absence of direct surgical scarring to ventricular muscle. Examples include congenital AS, dextro- or levo-TGA when the right ventricle supports the systemic circulation, severe Ebstein's anomaly, certain forms of single ventricle, and VSD with PAH. The appearance of ventricular arrhythmias in these cases commonly coincides with deterioration in overall hemodynamic status (165).

Therapy for VT in ACHD patients is complex and evolving. Similar to VT treatment in ischemic heart disease, sole reliance on pharmacological management has now been largely abandoned. Empirical beta blockade and class I or class III agents might still be prescribed in rare cases when the clinician remains ambivalent about a patient's VT risk after thorough testing, but no data support this approach once sustained VT or cardiac arrest has occurred. Drug therapy has now been replaced at most centers by more definitive interventions, such as implantable cardioverter defibrillator placement, catheter ablation, or arrhythmia surgery. Before deciding among these options, hemodynamic catheterization combined with comprehensive electrophysiology study should be obtained. Reparable hemodynamic issues may be identified that would favor a surgical strategy for therapy, such as closure of a residual septal defect or relief of valve regurgitation, combined with intraoperative VT mapping and ablation (170). In addition, IART may be identified as either a contributing or confounding factor for a patient's symptoms and can be addressed with either catheter or surgical ablation at the same setting. Finally, if VT can be induced that is slow enough to support the circulation during mapping, catheter ablation of the VT circuit may be considered on the basis of the risks and benefits to the individual patient (171,172). Although reports of ablation for VT in ACHD patients are still limited to small series, it appears that it can be accomplished with a reasonable degree of acute success (173,174); however, the risk of VT recurrence after ablation is now being more clearly defined and may exceed 20% (174). It seems wise to reserve ablation as isolated VT therapy for those CHD patients with superior hemodynamics and single circuits of slow tachycardia, and even then, to perform follow-up stimulation studies to ensure that the same or different circuits cannot be induced before dismissing the need for an implantable cardioverter defibrillator. Perhaps a more important role for catheter ablation may be as supplemental therapy to reduce the shock burden in patients with frequent VT recurrences who already have an implantable cardioverter defibrillator in place.

Most ACHD patients with documented or highly suspected VT are now managed with an implantable cardioverter defibrillator (175). Transvenous systems are possible in most cases, with the notable exceptions of single-ventricle patients, those with obstructed venous channels, and those with significant intracardiac shunts who would be at risk for systemic embolic events from an intravascular lead. Acute defibrillation thresholds in CHD patients are comparable to those encountered in acquired heart disease. What may differ during follow-up is the need for lead revision. There is now growing evidence that lead failure from insulation or conductor breaks is relatively high in this group (175), which possibly reflects a more active lifestyle for young ACHD patients than for an older population with ischemic disease.

1.10. Management of Bradycardias.   1.10.1. Sinoatrial Node Dysfunction
Although some rare forms of heterotaxy syndrome can be associated with congenital dysfunction or absence of the sinoatrial node, pathological sinus bradycardia in ACHD patients is more often an acquired problem related to cardiac surgery. Direct trauma to the sinoatrial node or its arterial supply occurs fairly frequently after the Mustard, Senning, Glenn, and Fontan operations (139,144,176,177). The likelihood of a patient developing IART or atrial fibrillation becomes significantly increased in this setting. Furthermore, patients with suboptimal hemodynamics may become symptomatic owing to chronotropic incompetence and the loss of AV synchrony. The updated guidelines for antibradycardia pacemaker implantation developed by the ACC and AHA (138) include information pertinent to CHD under the heading of "children and adolescents." These same guidelines can be applied reasonably well to ACHD patients. Implantation of an atrial or dual-chamber pacing system with activity responsiveness is recommended as a Class I indication in any symptomatic patient with sinoatrial node dysfunction. This will include most of those with tachy-brady syndrome and symptoms from recurrent atrial tachycardias, as well as any patient who is shown to have pause-dependent VT. Pacemaker implantation is also recommended as a Class IIb indication for asymptomatic adult patients with resting heart rates of less than 40 beats per minute or abrupt pauses in excess of 3 seconds. The possibility of developing ventricular dysfunction with apical ventricular pacing exists. Although dual-chamber pacing systems may be implanted, manipulation of pacing programming to maintain atrial pacing with intact AV conduction is desirable.

There are a number of unique technical considerations during pacemaker implantation in ACHD patients. Transvenous lead positions, for example, will often have to be modified in response to the cardiac lesion and vascular redirection imposed by surgical patches or anastomotic stenosis, as occurs after the Mustard or Senning operations. Transvenous leads may be impossible or ill advised in other CHD lesions, including in some postoperative Fontan patients or patients with intracardiac shunting, thereby necessitating epicardial lead placement. With either the endocardial or epicardial approach, it can be challenging to locate lead anchor points with proper pacing and sensing function due to fibrosis and patching, particularly for an atrial lead. Clear knowledge of the specific anatomy and review of all surgical records are essential before device placement is attempted in these patients.

1.10.2. Atrioventricular Block
Surgical repair of CHD may result in direct trauma to the AV conduction tissues. Although improved knowledge of the anatomy of the AV node and His bundle in various CHD lesions has lessened its occurrence (178), closure of some VSDs, surgery for left-sided heart outflow obstruction, and replacement or repair of an AV valve may still be complicated by AV block. Fortunately, in more than half of cases, this injury is a transient phenomenon, and conduction recovers within 7 to 10 days of the operation (179). Permanent pacemaker implantation is advised (138) as a Class I indication for any patient with postoperative advanced second- or third-degree AV block that is not expected to resolve or persists at least 7 to 10 days after cardiac surgery. A pacemaker is also recommended by some as a Class IIb indication when surgical AV block recovers but the patient is left with permanent bifascicular block.

The AV conduction tissues may also be congenitally abnormal in terms of their location and function in specific forms of CHD, notably congenitally corrected TGA (CCTGA), as well as AV septal defect (AVSD), particularly those with Down syndrome (180–182). These patients may be more susceptible to surgical or catheter-induced AV block but may also develop AV block spontaneously at any point in time ranging from fetal life to adulthood. Patients with these particular anatomic defects merit periodic assessment of AV conduction with serial ECGs and Holter monitoring, even if AV conduction was not directly affected by surgery.

1.11. Cyanotic Congenital Heart Disease.   Right-to-left intracardiac or extracardiac shunts result in hypoxemia, erythrocytosis, and cyanosis. Cyanotic ACHD patients should be seen at least annually by an ACHD specialist. Survival is determined by the type of underlying CHD and the medical complications of cyanosis.

1.11.1. Recommendations for Hematologic Problems
Class I

1 Indications for therapeutic phlebotomy are hemoglobin greater than 20 g per dL and hematocrit greater than 65%, associated with headache, increasing fatigue, or other symptoms of hyperviscosity in the absence of dehydration or anemia. (Level of Evidence: C)

Class III

1 Repeated routine phlebotomies are not recommended because of the risk of iron depletion, decreased oxygen-carrying capacity, and stroke. (Level of Evidence: C)

Cyanosis in patients with CHD has profound hematologic consequences that may affect many organ systems and need to be recognized and managed appropriately. The hematologic complications of chronic hypoxemia are erythrocytosis, iron deficiency, and bleeding diathesis (183). The increase in red blood cell mass that accompanies cyanosis is a compensatory response to improve oxygen transport. The white blood cell count is usually normal, and the platelet count may be normal or reduced.

The increased red blood cell mass may result in an increase in blood viscosity. However, the most likely cause of complications in adults with cyanotic CHD is aggressive phlebotomy or blood loss (184). Most cyanotic patients have compensated erythrocytosis with stable hemoglobin that requires no intervention. Therapeutic phlebotomy, therefore, is usually unnecessary unless the hemoglobin is more than 20 g/dL and the hematocrit is greater than 65% with associated symptoms of hyperviscosity and no evidence of dehydration. At these levels, patients may experience symptoms of headache and poor concentration. These symptoms may be relieved by removal of 1 unit of blood, always with an equal volume replacement of dextrose or saline. The purpose of the phlebotomy is to relieve hyperviscosity symptoms and occasionally, before elective operation, to improve coagulation. Repetitive phlebotomies deplete iron stores and may result in production of iron-deficient red blood cells. Iron deficiency, even in the face of erythrocytosis, is undesirable because of the reduced oxygen-carrying capacity and deformability of red blood cells (microcytes) and increased risk of stroke. A peripheral blood smear and serum ferritin or transferrin saturation will confirm the diagnosis.

The treatment for iron deficiency in a patient with destabilized erythropoiesis is challenging. Oral administration of iron frequently results in a rapid and dramatic increase in red cell mass; therefore, caution should be exercised and hemoglobin monitored. Once the serum ferritin and/or transferrin saturation is within the normal range, iron supplementation may be discontinued. Occasionally, patients are intolerant of oral iron and should be placed on pulses of intravenous iron supplementation instead.

1.11.1.1. Hemostasis
Hemostatic abnormalities have been documented in up to 20% of cyanotic patients. Platelet dysfunction and clotting factor deficiencies combine to produce a bleeding tendency in these patients. Epistaxis, gingival bleeding, menorrhagia, and pulmonary hemorrhage are the most common causes of bleeding. The use of anticoagulants and antiplatelet agents, therefore, is controversial and confined to well-defined indications with careful monitoring of the degree of anticoagulation. For a given concentration of citrate solution, the volume must be adjusted downward to correct for the lower plasma volume in those with high hematocrits.

1.11.1.2. Renal Function
In chronic cyanosis, the renal glomeruli are abnormal, frequently hypercellular, and congested and eventually become sclerotic (185). This results in a reduction of the glomerular filtration rate, increased creatinine levels, and proteinuria. This may cause problems with radiopaque contrast material and dehydration, leading to uremia, oliguria, and even anuria. Thus, patients should be hydrated before procedures that involve contrast media.

Abnormal urate clearance is common, and this in conjunction with an increased turnover of red blood cells leads to hyperuricemia and occasionally gout. Hyperuricemia without gout is usually well tolerated and rarely requires intervention (186). Symptomatic gout should be treated.

Medications that affect renal function, such as ACE inhibitors, diuretics, nonsteroidal antiinflammatory drugs, and select antibiotics, should be given with concern and cautious monitoring. As in all persons proceeding to catheterization, cyanotic patients should have an appropriate assessment of glomerular filtration rate (which may require more than measurement of serum creatinine), and the hydration state should be maximized within the constraints of appropriateness for a safe procedure. A low threshold for the use of renally protective strategies (N-acetylcysteine or bicarbonate administration) should be considered when indicated.

1.11.1.3. Gallstones
The increased breakdown of red blood cells in chronic cyanosis results in an increased risk of calcium bilirubinate gallstones. Surgical intervention is not recommended until patients become symptomatic (refer to Section 1.7, Recommendations for Noncardiac Surgery).

1.11.1.4. Orthopedic and Rheumatologic Complications
Hypertrophic osteoarthropathy with thickened, irregular periosteum occurs in the setting of cyanotic CHD. This may be accompanied by aching and tenderness, especially in the long bones of the legs.

Scoliosis occurs in a high percentage of patients with cyanotic CHD and is occasionally severe enough to compromise pulmonary function and require surgical intervention. Preoperative evaluation by an ACHD cardiologist and cardiac anesthesiologist is recommended before the operation for scoliosis is undertaken because of the recognized increased risk of surgery in cyanotic patients, especially those with PAH, for which this procedure may be contraindicated.

1.11.1.5. Neurological Complications
Neurological complications include an increased risk for paradoxical cerebral emboli. Brain abscess in cyanotic patients and thromboembolic events in patients with atrial tachycardia or atrial stasis associated with transvenous pacing leads can result in new neurological symptoms. These complications should be suspected in a cyanotic patient with headache, fever, and new neurological symptoms. Substantial cognitive and psychosocial issues are prevalent in this population, as discussed in Section 1.5.2, Recommendations for Psychosocial Issues.

1.11.1.6. Pulmonary Vascular Disease
Pulmonary vascular disease commonly accompanies cyanosis in patients with ACHD. The management of and concerns about pulmonary vascular disease and ACHD are discussed in more detail in a later section (Section 9, Pulmonary Hypertension/Eisenmenger Physiology).

1.12. Recommendations for General Health Issues for Cyanotic Patients.   Class I

1 Cyanotic patients should drink nonalcoholic and noncaffeinated fluids frequently on long-distance flights to avoid dehydration. (Level of Evidence: C)

Class IIb

1 Supplemental oxygenation may be considered for cyanotic patients during long-distance flights. (Level of Evidence: C)

Cyanotic patients should use only pressurized commercial airplanes. Oxygen therapy, although often unnecessary, may be suggested for prolonged travel. Similarly, residence at high altitude is detrimental for patients with cyanosis. Dehydration should be avoided by frequent fluid intake on long flights and during sports activities.

Competitive sports should be avoided in cyanotic patients (187). Cyanosis is a recognized handicap to fetal growth and development, and pregnancy outcome is impacted, with an increased risk of congestive heart failure, preterm delivery, intrauterine growth retardation, and miscarriage. Increased maternal and fetal mortality are also noted and correlate with the degree of cyanosis, ventricular dysfunction, and pulmonary pressures (117).

1.12.1. Hospitalization and Operation
Cyanotic patients are at high risk during any hospitalization or operation. When hospitalized for medical or surgical problems, these patients should be seen and followed up by an ACHD specialist. Management strategies that should be applied include those likely to reduce the risk of paradoxical emboli related to air in the intravenous lines. Medication adjustment may be needed, with cyanosis taken into account. Early ambulation may prevent venous stasis and thrombophlebitis.

1.12.2. Cardiac Reoperation and Preoperative Evaluation
Although some ACHD patients present without prior intervention, the majority will have undergone 1 or more prior repairs. Review of prior operative notes can provide important insight when a cardiac repair is planned. Repeat sternotomy may be associated with cardiac injury. The heart and great arteries may be closely adherent to the sternum because of the loss of pericardial integrity or presence of conduit material in the anterior mediastinum. In addition, right-sided heart structures may be enlarged or hypertensive, which also increases the potential for injury during sternotomy. Morphological abnormalities of the aorta, pulmonary artery, or ventricle–to–pulmonary artery conduit may also be at increased risk of injury. Peripheral vascular abnormalities may be present secondary to previous cardiac catheterization or operative procedures. For example, the radial pulse may be absent in patients with a prior classic Blalock-Taussig shunt. Femoral artery or vein occlusion may have occurred secondary to prior catheterization procedures or indwelling monitoring lines. Knowledge of the status of femoral or auxiliary vessels before reoperation may be particularly important if cannulation for establishment of cardiopulmonary bypass via these vessels is being planned.

To minimize the potential problems that may arise at the time of rerepair, additional preoperative studies may be necessary. The choice of the various supplemental imaging studies should be individualized on the basis of the surgeon's preference and institutional availability. Imaging studies that are frequently used include ultrasound, cine angiography, or MRI to document patency of cardiac anatomy (or occlusion) and status of femoral or axillary vessels. Coronary angiography or CT angiography is used to identify coronary anomalies or obstructive lesions. CT imaging of the chest may be helpful in identifying the relationship and proximity of the right ventricle, right atrium, aorta, pulmonary artery, or extracardiac conduit to the sternum or anterior chest wall. The importance of reviewing prior surgical notes when a repeat operation is planned cannot be overemphasized.

Men aged 35 years or older, premenopausal women 35 years or older with risk factors for atherosclerosis, and postmenopausal women should be evaluated by cardiac catheterization and coronary angiography to rule out associated coronary artery disease before they undergo reoperative cardiac surgery (112).

1.13. Heart Failure in Adult Congenital Heart Disease.   The New York Heart Association classification may be inadequate in ACHD patients, particularly if they are cyanotic. Respiratory physiology in cyanotic heart disease is well understood, and it is known that dyspnea may occur within the first 30 seconds of commencing exercise because of the arrival of hypoxemic and acidotic blood at the central receptors; thus, such dyspnea is not due to "pulmonary congestion," as is the case in heart failure (188–190). Therefore, cyanotic patients with ACHD may have dyspnea on exertion without having heart failure. It is preferable to use a functional ability or activity index (191). The patient with CHD who survives to adulthood will often have 1 or more substrates for developing the clinical syndrome of heart failure, which may either be right- or left-sided or involve both sides of the circulation. Typical ACHD substrates for late heart failure in ACHD patients are as follows:

• Severe AS and/or regurgitation BAV and variants, subvalvular or supravalvular pathology, superimposed coarctation

• Severe congenital mitral stenosis/regurgitation

• Unoperated ASD or partial AVSD

• CCTGA

• D-transposition after Mustard or Senning operation, in which the morphological right ventricle is the systemic ventricle

• Tetralogy of Fallot with early-era surgery, long-standing shunt, or severe pulmonary regurgitation

• Single-ventricle physiology

• Fontan surgery.

Many ACHD patients have experienced a combination of prolonged volume and pressure overload. Factors that predispose to the development of late heart failure include abnormal anatomy, surgical sequelae, and progression of underlying pathology. Myocardial damage during cardiac surgery was more common in patients who had operations during the earlier surgical era, but it may still occur in the present day with long cardiopulmonary bypass time, the need for large patches, or incisional scars (192). The reason for late heart failure in certain subsets of ACHD patients is of intense interest but not completely resolved. For example, anomalies in which the SV is a morphological right ventricle or there is a single ventricle have a higher incidence of myocardial dysfunction over time and with time may develop heart failure pathology (193). The presence of significant tricuspid (especially systemic AV valve) regurgitation is strongly associated with RV dysfunction and may be progressive (194). Inappropriate ventricular hypertrophy or myocardial oxygen supply-demand imbalance that results in myocardial ischemia has also been proposed to be a causative factor (195,196). In some forms of cardiac failure, there is evidence of biventricular interaction such that dysfunction of either ventricle negatively influences the other (ie, ventricular-ventricular dependence) (197,198). A straightforward example of adverse interventricular interaction is seen in right-sided heart volume overload in ASD, which results in changes in left ventricular (LV) shape, end-diastolic volume, and ejection fraction, which will normalize after closure of the defect (197). A recent special report on ventricular form and function, although targeted at the left ventricle, notes a shift from primary emphasis on contractile state and load to newer concepts of interaction and dynamic rearrangement of the myocardial layers, factors that may be altered considerably in CHD (199,200). To these substrates, other possible pathogenetic factors for heart failure can be added, such as the following:

• Prolonged cyanosis

• Prolonged pressure overload (eg, AS and subaortic stenosis [SubAS])

• Prolonged volume overload (eg, aortopulmonary shunt, AV semilunar valve regurgitation, or residual shunt)

• Poor myocardial intraoperative preservation

• Large ventricular septal patch

• Large ventricular incisions/scar

• Residual LVOT or RVOT obstruction (eg, PS/pulmonary regurgitation) or shunts (eg, VSD patch leak)

• Arrhythmias

• Obesity.

In addition, the following superimposed diseases or conditions unrelated to ACHD patients that become common in adulthood can contribute to or "tip the balance" toward development of heart failure:

• Acquired valvular heart disease

• Coronary artery disease

• Systemic hypertension

• Diabetes mellitus

• Pregnancy

• Endocarditis

• Chronic respiratory disease

• Cardiotoxic chemotherapy/mediastinal irradiation

• Illicit drug use

• Acquired renal or liver disease

• Obstructive sleep apnea

• Hyperthyroidism or hypothyroidism.

One concept that deserves more attention in the field of heart failure and ACHD is that of "ventriculoarterial coupling." It is well known that increased systemic arterial pressure or isolated systolic hypertension occurs in many individuals as they age and that this has detrimental effects. Changes in aortic diameter, stiffness, and wave reflection increase with age, which leads to an increase in ventricular afterload and may adversely affect late systolic ejection and/or early diastolic relaxation. Such aging changes may be detrimental to a systemic right or single ventricle that is ill prepared for any additional afterload. In addition, a combination of ventricular hypertrophy and arterial stiffening may lead to diastolic heart failure in the presence of preserved ventricular ejection fraction.

Signs of heart failure in ACHD patients may vary from the usual findings in patients with acquired heart disease and heart failure. Cardiorespiratory and ventilatory responses to exercise after a Fontan procedure, for example, are subnormal, including lower than expected o₂ max, subnormal cardiac output and heart rate responses to exercise, and an abnormal reduction of resting arterial O₂ saturation at peak exercise. After a Fontan or Glenn procedure, interpretation of the jugular venous pressure loses its usual meaning.

The ACC/AHA 2005 Guideline Update for the Diagnosis and Management of Chronic Heart Failure in the adult appropriately notes that critical assessment of ventricular function is needed in the patient with heart failure (201). It is desirable that assessments of function include quantitative measurements (eg, cardiopulmonary exercise testing with determination of oxygen consumption or cardiac function assessed by echocardiography with specific measures of systolic and diastolic function). Cardiac MRI to assess ventricular anatomy and function, dimensions, myocardial perfusion, and ischemia in adults with unoperated or operated CHD (eg, after atrial switch procedures) may be helpful (202–204). MRI studies of systemic right ventricles and single ventricles may show abnormalities of myocardial twist, torsion, radial motion, shortening, and strain relations (205,206). The frequent presence of abnormal ventricular anatomy warrants the addition of a Doppler echocardiography–derived index of myocardial performance index (or Tei index) or measurement of blood levels of brain natriuretic peptide (BNP) (192–194,207,208).

BNP production is affected by ventricular wall stress (eg, pressure overload such as AS, in which BNP appears to be influenced by both systolic and diastolic load) (209). BNP has been shown to be elevated not only in patients with heart failure and LV systolic dysfunction but also in patients with diastolic dysfunction and in RV dysfunction (198). However, BNP can be elevated in cyanotic heart disease without evidence of heart failure or myocardial dysfunction (210). BNP levels overall have been shown to be predictors of cardiac events (211) and have been shown to aid in emergency department diagnosis of heart failure when the cause of a patient's dyspnea is unclear (212), but their role in outpatient diagnosis and clinical follow-up of heart failure and ACHD remains under investigation. Serial measurement of BNP in patients at risk for the development of heart failure, such as patients with single-ventricle anatomy, may prove useful in guiding intervention.

Many current therapeutic strategies for the treatment of heart failure are directed at blocking activation of the neurohormonal system. The role of such medical treatments (eg, ACE inhibitors, angiotensin receptor blockers, and beta blockers) in the prevention or treatment of heart failure has only been studied in small numbers of ACHD patients. One such report on the use of ACE inhibitors in adults after the Mustard procedure showed no significant change in MRI parameters of RV volumes and ejection fractions or of measured exercise capacity (o₂ max, exercise duration, and blood pressure response) for the group as a whole, although there was improvement in some patients; the authors recommended a multiinstitutional prospective trial (53).

Established medical therapy for those with acquired heart disease and heart failure now incorporates medications directed at the renin-angiotensin-aldosterone system and sympathetic nervous systems. Although there exist multiple large, randomized, controlled clinical trials of drugs and other therapeutic interventions for heart failure in acquired heart disease, none have included the ACHD population (213). Thus, one should extrapolate cautiously from heart failure trials in acquired heart disease (214–218).

Aldosterone blockade with spironolactone has been shown in a small number of Fontan patients to improve the protein-losing enteropathy (PLE) syndrome (219). Few clinical trials have addressed the effect of angiotensin receptor blockers on outcomes in any adult patients with CHD. The role of the central and peripheral autonomic nervous system in ACHD patients has received some attention but needs further investigation (220–226). For example, in patients with previously operated tetralogy of Fallot, RVOT reconstruction may have affected cardiac autonomic nervous activity, which may also affect exercise hemodynamics, in part via heart rate recovery, altered respiratory physiology, and a decreased systolic blood pressure response with reduced cardiac output reserve. Critical investigation of various medications and other interventions for the possible treatment or prevention of heart failure in patients after tetralogy of Fallot repair, in patients with diminished systemic right or single-ventricle function, and in patients after a Fontan procedure is needed to optimize outcomes for these patients.

The role of pacemaker therapy in the treatment of cardiac failure is evolving rapidly (71,227). The need for pacing often coincides with worsening hemodynamic status, and it is not always possible to separate cause and effect. Regardless, it is well known that abnormal activation sequences (eg, from RV pacing) may cause a reduction in ventricular function (228,229).

Intraventricular or interventricular dyssynchrony may exacerbate chronic heart failure. Cardiac resynchronization therapy is an accepted means of improving ventricular function in conditions with normal 2-ventricle morphology and is now being proposed for treatment of heart failure in patients with a systemic RV (230). At present, there is no evidence to support its use in any patient with single-ventricle morphology. Current criteria for cardiac resynchronization therapy implantation in patients with normal (2-ventricle) morphology and heart failure include persistent heart failure symptoms despite appropriate medical therapy, QRS duration greater than or equal to 120 milliseconds with left bundle-branch block morphology, and the presence of sinus rhythm.

1.14. Recommendations for Heart and Heart/Lung Transplantation.   Class I

1 Patients with CHD and heart failure who may require heart transplantation should be evaluated and managed in tertiary care centers with medical and surgical personnel with experience and expertise in the management of both CHD and heart transplantation. (Level of Evidence: C)

2 Patients with CHD and heart or respiratory failure who may require lung or heart/lung transplantation should be evaluated and managed in tertiary care centers with medical and surgical personnel with experience and expertise in the management of CHD and lung or heart/lung transplantation. (Level of Evidence: C)

In ACHD patients, postoperative ventricular failure may occur early after operation but more commonly develops late after operation, often in adulthood. Late systemic ventricular failure can be associated with many congenital diagnoses.

The pretransplantation evaluation involves a multidisciplinary approach that addresses assessment of cardiopulmonary, renal, neurological, hepatic, infectious disease, socioeconomic, and psychological issues. In addition to history and physical examination, diagnostic studies include ECG, echocardiography, chest x-ray, and Holter monitoring. Cardiac catheterization is required to assess pulmonary vascular resistance (PVR) and transpulmonary gradient (231). In addition to cardiac catheterization, MRI or CT angiography is often performed to delineate the anatomy in patients with complex CHD (eg, patients with malposition of the great arteries and/or substernal position of an extracardiac conduit, abnormalities of systemic venous return, and situs abnormalities).

Many patients with long-standing heart failure may have elevated PVR. Consequently, donor right-sided heart failure may result when the heart is abruptly placed proximal to such a high-resistance pulmonary vascular bed. Pharmacological modulation of pulmonary hemodynamics with pulmonary vasodilators during cardiac catheterization helps predict outcome after heart transplantation (232,233). In most centers, a fixed PVR index of 6 units or more or a transpulmonary gradient greater than 15 mm Hg that does not respond to vasodilator therapy (oxygen, nitric oxide, milrinone, or dobutamine) is a contraindication to cardiac transplantation alone, although transplantation from a rare donor with PAH in a Domino procedure with heart/lung transplantation in 1 recipient followed by transplantation of the recipient's heart into another recipient may still be successful.

Contraindications to cardiac transplantation include the following:

• Active infection

• Positive serology for human immunodeficiency virus or hepatitis C infections

• Severe metabolic disease

• Multiple other severe congenital anomalies

• Multisystem organ failure

• Active malignancy

• Cognitive or behavioral disability that interferes with compliance.

Heart/lung transplantation is usually reserved for patients with uncorrectable or previously repaired or palliated CHD associated with significant pulmonary vascular obstructive disease, such as single-ventricle physiology with pulmonary vascular disease or LV dysfunction with associated pulmonary vascular disease. When a simple cardiac defect is present, such as ASD, VSD, or PDA, the cardiac defect can often be repaired at lung transplantation (234). In the presence of more complex intracardiac abnormalities, combined heart/lung transplantation is usually most appropriate.

Previous thoracotomies are not an absolute contraindication to transplantation, but in the presence of chronic cyanosis, vascular collaterals may lead to fatal hemorrhagic complications. The absence of detectable recurrence of malignancy for 5 years may permit successful transplantation. Obesity is a relative contraindication to transplantation.

Survival rates after heart transplantation have improved over the years, and the current predicted posttransplantation half-life (the time at which 50% of those with transplanted organs remain alive, or median survival) for the entire cohort of pediatric and adult heart recipients is 10 years, with a half-life of 13 years for those who survive the first year; however, having ACHD as an indication for transplant increases that risk during the first year by 2-fold (235).

Advances in selection, technique, and management of patients undergoing lung or heart/lung transplant have resulted in significant improvement in survival. Overall, survival after pediatric lung transplantation as reported by the International Society of Heart and Lung Transplant registry is approximately 75% at 1 year and 60% at 2 years (236). The most common cause of mortality in the first month after lung transplantation is acute graft failure; from 1 month to 1 year after transplantation, infection is the leading cause of death. From 1 to 3 years after lung transplantation, chronic rejection or bronchiolitis obliterans is the leading cause of death. Beyond this time frame, main causes of death include chronic rejection and infection. The outcome for heart/lung transplantation is similar to that for lung transplantation.

Actuarial survival at 10 years after heart/lung transplantation is 20%. Results of lung and heart/lung transplantation for PAH and ACHD are comparable to those reported for children, with an increased risk of early mortality related to perioperative complications and complexity compared with transplantation for obstructive pulmonary disease or cystic fibrosis. Outcomes for lung transplantation and cardiac repair are comparable to those for heart/lung transplantation in the treatment of PAH and CHD (237).

	2. Atrial Septal Defect

Top Task Force Members Table of Contents Preamble 1. Introduction 2. Atrial Septal Defect 3. Ventricular Septal Defect 4. Atrioventricular Septal... 5. Patent Ductus Arteriosus 6. Left-Sided Heart Obstructive... 7. Right Ventricular Outflow... 8. Coronary Artery Abnormalities 9. Pulmonary... 10. Tetralogy of Fallot 11. Dextro-Transposition of the... 12. Congenitally Corrected... 13. Ebstein's Anomaly 14. Tricuspid Atresia/Single... Staff Appendixes Appendix 3 Appendix 4 References

 
2.1. Definition.   One of the most common adult congenital heart defects, an ASD is a persistent communication between the atria. There are several different types of ASD: the secundum ASD in the region of the fossa ovalis (75% of cases), the primum ASD (15% to 20%) positioned inferiorly near the crux of the heart, the sinus venosus ASD (5% to 10%) located superiorly near the superior vena caval entry or inferiorly near the inferior vena caval entry, and the uncommon coronary sinus septal defect (less than 1%), which causes shunting through the ostium of the coronary sinus (238). The patent foramen ovale (PFO) is a flaplike communication in which the septum primum covering the fossa ovalis overlaps the superior limbic band of the septum secundum. In some patients, the septum primum or secundum is aneurysmal and may have multiple small fenestrations.

2.1.1. Associated Lesions
ASD can be associated with additional malformations in nearly 30% of cases (Table 9) (239). As a form of AVSD, the primum ASD is nearly always accompanied by a cleft in the anterior mitral valve leaflet. Discrete SubAS may develop postoperatively. Sinus venosus defects frequently have partial anomalous venous drainage of the right pulmonary veins. This association is present in a small number of patients with secundum ASDs as well. Mitral valve prolapse is frequently seen in patients with ASD. Valvular pulmonic stenosis is frequently described in association with ASD, but in some cases, there is a mild RV outflow gradient that is caused by increased flow but not a structural valve abnormality (240,241).

2.2. Clinical Course.   2.2.1. Unrepaired Atrial Septal Defect
The consequence of left-to-right shunt across an ASD is RV volume overload and pulmonary overcirculation. Large atrial shunts lead to symptoms from excess pulmonary blood flow and right-sided heart failure, including frequent pulmonary infections, fatigue, exercise intolerance, and palpitations. Atrial arrhythmias—atrial flutter, atrial fibrillation, and sick sinus syndrome—are a common result of long-standing right-sided heart volume and pressure overload. Flow-related PAH accompanies large left-to-right shunts, and pulmonary vascular obstructive disease may develop in adult years but occurs much later with ASD than with high-pressure left-to-right shunts such as VSD or PDA. Paradoxical embolism from peripheral venous or pelvic vein thromboses, atrial arrhythmias, unfiltered intravenous infusions, or indwelling venous catheters is a risk for all defects regardless of size (242–244).

The initial presentation in adulthood most commonly includes symptoms of dyspnea and palpitations (245,246). Other modes of presentation in the previously undiagnosed adult with an ASD include cardiomegaly on routine chest x-ray, a more audible murmur during pregnancy, new onset of atrial flutter/fibrillation, or a paradoxical embolic event. Patients with small defects (less than 10 mm) may remain asymptomatic well into the fourth and fifth decade of life (236,246); however, symptoms may develop with increasing age even with small defects owing to an increase in shunting caused by a decrease in LV compliance secondary to coronary artery disease, acquired valvular disease, or hypertension.

2.3. Recommendations for Evaluation of the Unoperated Patient.   Class I

1 ASD should be diagnosed by imaging techniques with demonstration of shunting across the defect and evidence of RV volume overload and any associated anomalies. (Level of Evidence: C)

2 Patients with unexplained RV volume overload should be referred to an ACHD center for further diagnostic studies to rule out obscure ASD, partial anomalous venous connection, or coronary sinoseptal defect. (Level of Evidence: C)

Class IIa

1 Maximal exercise testing can be useful to document exercise capacity in patients with symptoms that are discrepant with clinical findings or to document changes in oxygen saturation in patients with mild or moderate PAH. (Level of Evidence: C)

2 Cardiac catheterization can be useful to rule out concomitant coronary artery disease in patients at risk because of age or other factors. (Level of Evidence: B)

Class III

1 In younger patients with uncomplicated ASD for whom imaging results are adequate, diagnostic cardiac catheterization is not indicated. (Level of Evidence: B)

2 Maximal exercise testing is not recommended in ASD with severe PAH. (Level of Evidence: B)

The diagnostic workup for a patient with a suspected ASD is directed at defining the presence, size, and location of the ASD; the functional effect of the shunt on the right and left ventricles and the pulmonary circulation; and any associated lesions.

2.3.1. Clinical Examination
Clinical findings include a precordial lift, systolic pulmonary flow murmur, and fixed splitting of the second heart sound (although fixed splitting is not invariable). With large shunts, a diastolic flow rumble across the tricuspid valve is present.

2.3.2. Electrocardiogram
The ECG often shows right-axis deviation, right atrial enlargement, incomplete right bundle-branch block (secundum ASD), superior left-axis deviation (primum ASD), or an abnormal P-wave axis (superiorly located sinus venosus ASD). Complete heart block may be present in association with familial ASD (247). The superior left axis with RV conduction delay seen in primum ASD is due to the anatomic position of the conduction bundles and should not be confused with bifascicular block.

2.3.3. Chest X-Ray
The chest x-ray may show RV and right atrial enlargement, a prominent pulmonary artery segment, and increased pulmonary vascularity.

2.3.4. Echocardiography
A TTE is the primary diagnostic imaging modality for ASD. The study should include 2-dimensional imaging of the atrial septum from the parasternal, apical, and subcostal views with color Doppler demonstration of shunting. Subcostal views with deep inspiration and high right parasternal views can be particularly helpful for imaging ASD in adults. The entire atrial septum from the orifice of the superior vena cava to the orifice of the inferior vena cava should be visualized to detect sinus venosus defects or the extension of large secundum defects in these regions. A TEE may be necessary to identify the connection of all pulmonary veins in patients with ASD. In adults with poor-quality transthoracic images, TEE may be necessary to adequately image the atrial septum (248–251), because it provides exact localization and sizing of the ASD, as well as measurement of septal rims, each of which is important for decision making.

A large coronary sinus orifice with evidence of atrial shunting may indicate a defect in the roof of the coronary sinus (eg, sinoseptal defects). Thus, the entire coronary sinus roof should be imaged when this is suspected. When a coronary sinoseptal defect is associated with lesions that cause right-to-left shunting, the orifice of the coronary sinus may not be enlarged and the defect not recognized until after definitive surgery, at which time a left-to-right shunt may occur. With PAH, the low velocity of the shunt flow across the coronary sinoseptal defect may be difficult to distinguish from other low-velocity flow within the atria.

Right atrial and RV enlargement with diastolic flattening and paradoxical motion of the interventricular septum are evidence of RV volume overload and a significant left-to-right shunt. The RV systolic pressure should be estimated from the peak velocity of the tricuspid regurgitant jet if present. Two-dimensional imaging should assess associated lesions such as mitral valve prolapse, cleft mitral valve, anomalous pulmonary veins, and PS, and their functional significance should be determined by color and spectral Doppler.

Contrast echocardiography with intravenous agitated saline injection is used to confirm the presence of a right-to-left atrial shunt if imaging and color Doppler are not conclusive (252). Additionally, the presence of negative contrast in the right atrium may be helpful in identifying a left-to-right shunt. If a left-to-right shunt or RV volume overload is recognized but unexplained, the patient should be referred to an ACHD center for further imaging studies.

2.3.5. Magnetic Resonance Imaging
MRI provides an additional noninvasive imaging modality if findings by echocardiography are uncertain. Direct visualization of the defect and pulmonary veins is possible, RV volume and function can be quantified, and estimates of shunt size can also be obtained (253–255). Contrast-enhanced ultrafast cine CT can also provide diagnostic information, although the radiation exposure limits its utility in most cases (256).

Diagnostic cardiac catheterization is not required for uncomplicated ASDs in younger patients with adequate noninvasive imaging (257,258). It is generally reserved for investigation of coronary artery disease in those patients at risk by virtue of age or family history and for whom surgical intervention is planned and to assess PVR and reactivity in patients with significant PAH. Catheterization may also be required to evaluate ASD size, pulmonary venous return, and associated valvular disease if noninvasive methods have been unable to provide this information. In most instances, catheterization is now performed in conjunction with device closure of the defect.

2.3.6. Exercise Testing
Exercise testing can be useful to document exercise capacity in patients with symptoms that are discrepant with clinical findings or to document changes in oxygen saturation in patients with PAH. Maximal exercise testing is not recommended in ASD with severe PAH, however.

2.4. Diagnostic Problems and Pitfalls.   The gradual onset of symptoms and the subtlety of the physical findings with ASDs often lead to late diagnosis, which puts the patient at greater risk for developing PAH, arrhythmia, and paradoxical embolism. False-positive diagnosis of ASD can result from either apparent septal dropout on 2-dimensional echocardiography images or misinterpretation by color Doppler of vena caval inflow as shunt flow. The use of contrast echocardiography or TEE will prevent false-positive interpretations. Patients with partial anomalous pulmonary venous drainage without an ASD will have RV volume overload and may be erroneously presumed to have an ASD.

False-negative diagnoses are relatively common in adults with poor-quality transthoracic images, especially patients with sinus venosus ASD. Because of its superior location, the superior sinus venosus defect is most often missed by TTE (248). Patients with an unexplained RV volume overload by TTE should be studied by TEE or another imaging modality to fully evaluate the atrial septum and pulmonary veins and to rule out defects in the roof of the coronary sinus.

2.5. Management Strategies.   2.5.1. Recommendations for Medical Therapy
Class I

1 Cardioversion after appropriate anticoagulation is recommended to attempt restoration of the sinus rhythm if atrial fibrillation occurs. (Level of Evidence: A)

2 Rate control and anticoagulation are recommended if sinus rhythm cannot be maintained by medical or interventional means. (Level of Evidence: A)

Patients with small shunts and normal RV size are generally asymptomatic and require no medical therapy. Routine follow-up of the patient with a small ASD without evidence of RV enlargement or PAH should include assessment of symptoms, especially arrhythmias, and possible paradoxical embolic events. A repeat echocardiogram should be obtained every 2 to 3 years to assess RV size and function and pulmonary pressure. Reductions in LV compliance related to hypertension, coronary artery disease, or acquired valvular disease increase the degree of left-to-right shunt across an existing ASD.

Atrial arrhythmias should be treated to restore and maintain sinus rhythm if possible (259). If atrial fibrillation occurs, both antiarrhythmic therapy and anticoagulation should be recommended.

ASDs that are large enough to cause PAH should be closed provided there is evidence of pulmonary vascular reactivity and a net left-to-right shunt. Medical therapy for PAH is indicated only for those patients who are considered to have irreversible PAH and therefore are not eligible for ASD closure (refer to Section 9, Pulmonary Hypertension/Eisenmenger Physiology, for more extensive discussion of the treatment of PAH).

2.5.2. Recommendations for Interventional and Surgical Therapy
Class I

1 Closure of an ASD either percutaneously or surgically is indicated for right atrial and RV enlargement with or without symptoms. (Level of Evidence: B)

2 A sinus venosus, coronary sinus, or primum ASD should be repaired surgically rather than by percutaneous closure. (Level of Evidence: B)

3 Surgeons with training and expertise in CHD should perform operations for various ASD closures. (Level of Evidence: C)

Class IIa

1 Surgical closure of secundum ASD is reasonable when concomitant surgical repair/replacement of a tricuspid valve is considered or when the anatomy of the defect precludes the use of a percutaneous device. (Level of Evidence: C)

2 Closure of an ASD, either percutaneously or surgically, is reasonable in the presence of:

a Paradoxical embolism. (Level of Evidence: C)

b Documented orthodeoxia-platypnea. (Level of Evidence: B)

Class IIb

1 Closure of an ASD, either percutaneously or surgically, may be considered in the presence of net left-to-right shunting, pulmonary artery pressure less than two thirds systemic levels, PVR less than two thirds systemic vascular resistance, or when responsive to either pulmonary vasodilator therapy or test occlusion of the defect (patients should be treated in conjunction with providers who have expertise in the management of pulmonary hypertensive syndromes). (Level of Evidence: C)

2 Concomitant Maze procedure may be considered for intermittent or chronic atrial tachyarrhythmias in adults with ASDs. (Level of Evidence: C)

Class III

1 Patients with severe irreversible PAH and no evidence of a left-to-right shunt should not undergo ASD closure. (Level of Evidence: B)

Surgical closure has been the "gold standard" form of treatment, with excellent late outcome. A surgeon not trained in CHD should be cautious when planning to close a secundum ASD, because the intraoperative discovery of an unexpected primum ASD or partial anomalous pulmonary venous drainage can present challenges.

Primary operation includes pericardial patch closure or direct suture closure. Tricuspid valve repair should be performed for significant tricuspid regurgitation (TR). Anomalous pulmonary venous drainage should be repaired. The Warden procedure (translocation of the superior vena cava to the right atrial appendage) may be applied to the sinus venosus ASD when the anomalous pulmonary venous drainage enters the mid or upper superior vena cava. A concomitant Maze procedure may be performed for intermittent/chronic atrial fibrillation/flutter. The surgical approach can be by right thoracotomy or sternotomy, and more limited incisions are feasible with either approach.

Early mortality is approximately 1% in the absence of PAH or other major comorbidities. Long-term follow-up is excellent, and preoperative symptoms decrease or abate. The incidence of atrial fibrillation/flutter is reduced when concomitant antiarrhythmic procedures (eg, Maze) are performed; however, atrial arrhythmias may occur de novo after repair.

The need for reoperation of residual/recurrent ASD is uncommon. Superior vena cava stenosis or pulmonary vein stenosis may occur after closure of sinus venosus ASD.

2.5.3. Indications for Closure of Atrial Septal Defect
Small ASDs with a diameter of less than 5 mm and no evidence of RV volume overload do not impact the natural history of the individual and thus may not require closure unless associated with paradoxical embolism. Larger defects with evidence of RV volume overload on echocardiography usually only cause symptoms in the third decade of life, and closure is usually indicated to prevent long-term complications such as atrial arrhythmias, reduced exercise tolerance, hemodynamically significant TR, right-to-left shunting and embolism during pregnancy, overt congestive cardiac failure, or pulmonary vascular disease that may develop in up to 5% to 10% of affected (mainly female) individuals.

2.5.4. Catheter Intervention
The development of percutaneous transcatheter closure techniques has provided an alternative method of closure for uncomplicated secundum ASDs with appropriate morphology (260–262). Currently, the majority of secundum ASDs can be closed with a percutaneous catheter technique. When this is not feasible or is not appropriate, surgical closure is recommended.

Sinus venosus, coronary sinus, and primum defects are not amenable to device closure. An ASD with a large septal aneurysm or a multifenestrated atrial septum requires careful evaluation by and consultation with interventional cardiologists before device closure is selected as the method of repair.

2.5.5. Key Issues to Evaluate and Follow-Up
Key issues to evaluate and monitor in adults with ASD are listed in Table 10.

1 Early postoperative symptoms of undue fever, fatigue, vomiting, chest pain, or abdominal pain may represent postpericardiotomy syndrome with tamponade and should prompt immediate evaluation with echocardiography. (Level of Evidence: C)

2 Annual clinical follow-up is recommended for patients postoperatively if their ASD was repaired as an adult and the following conditions persist or develop:

a PAH. (Level of Evidence: C)

b Atrial arrhythmias. (Level of Evidence: C)

c RV or LV dysfunction. (Level of Evidence: C)

d Coexisting valvular or other cardiac lesions. (Level of Evidence: C)

3 Evaluation for possible device migration, erosion, or other complications is recommended for patients 3 months to 1 year after device closure and periodically thereafter. (Level of Evidence: C)

4 Device erosion, which may present with chest pain or syncope, should warrant urgent evaluation. (Level of Evidence: C)

Follow-up for patients after device closure requires clinical assessment of symptoms of arrhythmia, chest pain, or embolic events and echocardiographic surveillance for device position, residual shunting, and complications such as thrombus formation or pericardial effusion. The frequency of echocardiographic follow-up is usually at 24 hours, 1 month, 6 months, and 1 year and at regular intervals thereafter.

Pericardial effusions and cardiac tamponade may occur up to several weeks after surgical repair of ASDs and should be evaluated by clinical examination and echocardiography before hospital discharge and at the early postoperative visits. Patients and their primary care physicians should be instructed to report fever or unusual symptoms of chest or abdominal pain and vomiting or undue fatigue in the first weeks after surgery, because they might represent early signs of cardiac tamponade. Assessment of pulmonary pressure, RV function, and residual atrial shunting should also be made during follow-up echocardiography. Clinical and ECG surveillance for recurrent or new-onset arrhythmia is an important feature of postoperative evaluation. Periodic long-term clinical follow-up is required for patients postoperatively if their ASD was repaired as an adult, if PAH was present preoperatively, if there were atrial arrhythmias either preoperatively or postoperatively, if there was RV or LV dysfunction preoperatively or postoperatively, or if there are coexisting valvular or other cardiac lesions. Patients with ASD who have undergone surgical closure in childhood are generally free of late complications.

2.6.1. Endocarditis Prophylaxis
Endocarditis does not occur in patients with isolated ASDs and is usually associated with concomitant valvular lesions, such as a cleft mitral valve (94). Endocarditis prophylaxis is therefore not indicated for isolated ASDs before or after surgery except for the first 6 months after closure (refer to Section 1.6, Recommendations for Infective Endocarditis, for additional information).

2.6.2. Recommendation for Reproduction
Class III

1 Pregnancy in patients with ASD and severe PAH (Eisenmenger syndrome) is not recommended owing to excessive maternal and fetal mortality and should be strongly discouraged. (Level of Evidence: A)

Pregnancy in patients with ASDs is generally well tolerated, with no maternal mortality and no significant maternal or fetal morbidity. Although the left-to-right shunt may increase with the increase in cardiac output during pregnancy, this is counterbalanced by the decrease in peripheral resistance.

Women with large shunts and PAH may experience arrhythmias, ventricular dysfunction, and progression of PAH. Pregnancy in patients with ASD and severe PAH (Eisenmenger syndrome) is contraindicated owing to excessive maternal and fetal mortality and should be strongly discouraged (263,264). Paradoxical embolism may occasionally be encountered in small and large ASDs (134,265).

Familial occurrence of secundum ASDs is well recognized, and in some kindreds, a defect has been localized to chromosome 5 (266). Familial ASD with AV conduction defect is an autosomal dominant trait, with mutations in the cardiac homeobox transcription factor gene NKX2–5 (267,268).

The risk of transmission of CHD to offspring of women with sporadic ASD is estimated at 8% to 10% (133,269). Genetic syndromes with skeletal abnormalities associated with ASD include a variety of heart-hand syndromes, of which Holt-Oram syndrome is best known (270–272). Both secundum and primum ASDs are associated with trisomy 21 (Down syndrome). Because of the possibility of familial occurrence, a careful family history should be taken in patients with ASD, and parents and offspring should be evaluated clinically for possible septal defect, conduction disturbances, and skeletal anomalies.

2.6.3. Activity
Patients with small ASDs and without PAH have normal exercise capacity and do not need any limitation of physical activity. In those patients with large left-to-right shunts, exercise is often self-limited owing to decreased cardiopulmonary function (273). Symptomatic supraventricular or ventricular arrhythmias may also compromise exercise capacity and impose limitations on engagement in competitive sports. Patients with significant PAH (peak systolic pulmonary artery pressure greater than 40 mm Hg) should limit their activity to low-intensity sports. Severe PAH with right-to-left shunting is usually self-limiting, but participation in athletics or active physical effort should be avoided (274).

	3. Ventricular Septal Defect

Top Task Force Members Table of Contents Preamble 1. Introduction 2. Atrial Septal Defect 3. Ventricular Septal Defect 4. Atrioventricular Septal... 5. Patent Ductus Arteriosus 6. Left-Sided Heart Obstructive... 7. Right Ventricular Outflow... 8. Coronary Artery Abnormalities 9. Pulmonary... 10. Tetralogy of Fallot 11. Dextro-Transposition of the... 12. Congenitally Corrected... 13. Ebstein's Anomaly 14. Tricuspid Atresia/Single... Staff Appendixes Appendix 3 Appendix 4 References

 
3.1. Definition.   VSD is the most common congenital heart defect at birth (275) and presents in approximately 3.0 to 3.5 infants per 1000 live births. Because there is a high incidence of spontaneous closure of small VSDs, the incidence is much less in older infants and particularly in adults (276,277).

There are 4 anatomic types of VSDs (278–280), with multiple synonyms for each type. In an effort to establish a unified reporting system, the Society for Thoracic Surgery's Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery developed a classification scheme, as shown in Table 11.

Type 2 or perimembranous VSDs are the most common defects, and almost 80% of defects are in this location. This defect is in the membranous septum and is adjacent to the septal leaflet of the tricuspid valve, which can become adherent to the defect, thus forming a pouch or "aneurysm" of the ventricular septum. This pouch will limit left-to-right shunting and can result in partial or complete closure of the defect. On the LV side of the septum, the defect is adjacent to the aortic valve.

Type 3 or inlet VSDs occur in the lower part of the right ventricle and adjacent to the tricuspid valve (278–280). These defects typically occur in patients with Down syndrome.

Type 4 or muscular VSDs can be located centrally (midmuscular), apically, or at the margin of the septum and RV free wall. They can be multiple in number. Spontaneous closure is common, and although these defects can account for up to 20% of VSDs in infants, the incidence is much lower in adults (276–278).

3.1.1. Associated Lesions
Although VSD is most often an isolated lesion, it is a common component of complex abnormalities such as conotruncal defects (eg, tetralogy of Fallot, TGA). VSD can also be associated with left-sided obstructive lesions such as SubAS and coarctation of the aorta. A subpulmonary (supracristal) VSD is often associated with progressive aortic valve regurgitation caused by prolapse of the aortic cusp (usually right) through the defect.

3.2. Clinical Course (Unrepaired).   It is unlikely for an adult with an isolated VSD to present with no prior workup/diagnosis. Possible scenarios include the following:

• An asymptomatic patient with a systolic murmur previously thought to be an innocent murmur

• Fever and bacteremia secondary to IE

• A new diastolic murmur of AR secondary to aortic valve prolapse

• Cyanosis and exercise intolerance secondary to the progressive development of pulmonary vascular disease.

Clinical presentation in an isolated VSD depends largely on defect size and PVR. Small defects that are less than or approximately equal to 25% the size of the aortic annulus diameter have small left-to-right shunts, no left ventricle volume overload, and no PAH and present as systolic murmurs.

VSDs that are more than 25% but less than 75% of the aortic diameter can be classified as moderate in size, with small to moderate left-to-right shunts, mild to moderate LV volume overload, and mild or no PAH. Patients may remain asymptomatic or develop symptoms of mild congestive heart failure. Symptoms usually abate with medical treatment and with time as the size of the VSD decreases in absolute terms or relative to increasing body size.

If the defect is large (greater than or equal to 75% of the aortic diameter), there is usually a moderate to large left-to-right shunt, LV volume overload, and PAH. Most adult patients with large VSDs will have a history of congestive heart failure in infancy. Rarely, patients with large VSDs do not develop large left-to-right shunts and do not have the normal postnatal fall in PVR. They can present with right-to-left shunting and Eisenmenger syndrome later in childhood or as adolescents or young adults. Key issues to follow in patients with VSD are summarized in Table 12. Patients with a small VSD who develop endocarditis may present with pulmonary embolism or cerebral abscess. Spontaneous closure of small defects can occur at any age but most commonly occurs in infancy (277,281,282). Postsurgical presentations include signs and symptoms associated with IE, AR, heart block, LV dysfunction, PAH, TR, recurrent VSD, and ventricular arrhythmias.

3.3.2. Electrocardiogram
In patients with large VSD and significant PAH, the ECG will show biventricular hypertrophy or isolated RV hypertrophy, depending on the extent to which the LVOT has diminished in response to the reduction in left-to-right shunt.

3.3.3. Chest X-Ray
Patients with a small VSD will have a normal chest x-ray. The presence of a significant left-to-right shunt will create the appearance of left atrial and LV enlargement and increased pulmonary vascular markings. Patients with significant PAH will not demonstrate LV enlargement but will have a prominent pulmonary artery segment and diminished pulmonary vascular markings at the periphery of the lung.

3.3.4. Echocardiography
Echocardiography-Doppler is the mainstay of modern diagnosis. Transthoracic echocardiographic studies are almost always diagnostic in children and adolescents and in most adults with good echocardiographic windows. Data to be obtained include the number of defects, location of defect(s), chamber sizes, ventricular function, presence or absence of aortic valve prolapse and/or regurgitation, presence or absence of RV or LV outflow obstruction, and presence or absence of TR. Estimation of RV systolic pressure from TR jet, VSD jet, and/or septal configuration should be a part of the study. In adults with poor echocardiographic windows, TEE may be necessary.

Echocardiography-Doppler of postoperative patients should focus on the presence or absence and location of residual shunting and the evaluation of pulmonary artery pressure by TR or pulmonary regurgitation jet velocity. In addition, patients should be evaluated for AR, ventricular function, and RV or LV outflow obstruction.

3.3.5. Magnetic Resonance Imaging/Computed Tomography
MRI or CT may be useful, if local expertise in cardiac studies is available, for the following:

• Assessment of pulmonary artery, pulmonary venous, and aortic anatomy if there are coexisting lesions

• To confirm the anatomy of unusual VSDs such as inlet or apical defects not well seen by echocardiography.

3.3.6. Recommendations for Cardiac Catheterization
Class I

1 Cardiac catheterization to assess the operability of adults with VSD and PAH should be performed in an ACHD regional center in collaboration with experts. (Level of Evidence: C)

Class IIa

1 Cardiac catheterization can be useful for adults with VSD in whom noninvasive data are inconclusive and further information is needed for management. Data to be obtained include the following:

a Quantification of shunting. (Level of Evidence: B)

b Assessment of pulmonary pressure and resistance in patients with suspected PAH. Reversibility of PAH should be tested with various vasodilators. (Level of Evidence: B)

c Evaluation of other lesions such as AR and double-chambered right ventricle. (Level of Evidence: C)

d Determination of whether multiple VSDs are present before surgery. (Level of Evidence: C)

e Performance of coronary arteriography is indicated in patients at risk for coronary artery disease. (Level of Evidence: C)

f VSD anatomy, especially if device closure is contemplated. (Level of Evidence: C)

3.4. Diagnostic Problems and Pitfalls.   Problems and pitfalls in the diagnosis of adults with VSDs include the following:

• Patients with loud murmur of a known small VSD may develop double-chambered right ventricle or SubAS with little appreciable change in murmur.

• Patients with a small VSD and aortic valve prolapse may develop progressive AR.

• Patients with unrecognized RV outflow obstruction associated with a VSD may have a high-velocity TR jet and may be assumed to have PAH.

• A VSD jet may be mistaken for a TR jet in a patient with normal pulmonary pressure assumed to have PAH.

3.5. Management Strategies.   3.5.1. Recommendation for Medical Therapy
Class IIb

1 Pulmonary vasodilator therapy may be considered for adults with VSDs with progressive/severe pulmonary vascular disease (refer to Section 9, Pulmonary Hypertension/Eisenmenger Physiology). (Level of Evidence: B)

3.5.2. Recommendations for Surgical Ventricular Septal Defect Closure
Class I

1 Surgeons with training and expertise in CHD should perform VSD closure operations. (Level of Evidence: C)

2 Closure of a VSD is indicated when there is a Qp/Qs (pulmonary–to–systemic blood flow ratio) of 2.0 or more and clinical evidence of LV volume overload. (Level of Evidence: B)

3 Closure of a VSD is indicated when the patient has a history of IE. (Level of Evidence: C)

Class IIa

1 Closure of a VSD is reasonable when net left-to-right shunting is present at a Qp/Qs greater than 1.5 with pulmonary artery pressure less than two thirds of systemic pressure and PVR less than two thirds of systemic vascular resistance. (Level of Evidence: B)

2 Closure of a VSD is reasonable when net left-to-right shunting is present at a Qp/Qs greater than 1.5 in the presence of LV systolic or diastolic failure. (Level of Evidence: B)

Class III

1 VSD closure is not recommended in patients with severe irreversible PAH. (Level of Evidence: B)

Primary operation for isolated VSD includes patch closure, usually with a synthetic material (eg, Dacron, polytetrafluoroethylene [Gore-Tex]), and, rarely, primary closure. Careful intraoperative inspection of the muscular septum by TEE is indicated to rule out associated VSDs that might manifest by shunting only after closure of the dominant VSD. Associated RV outflow obstruction should be treated with resection or RV outflow patch enlargement, AR by aortic valve replacement (AVR), and SubAS usually by resection of a subaortic membrane and rarely by a Konno procedure tricuspid valve repair if there is associated significant TR.

Early mortality is approximately 1% in the absence of elevated PVR. Late survival is excellent when ventricular function is normal. PAH may regress, progress, or remain unchanged. Atrial fibrillation may occur and is more likely if there has been chronic volume overload resulting in left atrial dilatation. Complete heart block may occur early or late after surgical repair. Ventricular arrhythmias are uncommon unless repair is performed late in life. The need for reoperation for a residual VSD is uncommon. Late reoperation is occasionally required for TR or AR.

3.5.3. Recommendation for Interventional Catheterization
Class IIb

1 Device closure of a muscular VSD may be considered, especially if the VSD is remote from the tricuspid valve and the aorta, if the VSD is associated with severe left-sided heart chamber enlargement, or if there is PAH. (Level of Evidence: C)

Indications for catheter device closure of VSD include residual defects after prior attempts at surgical closure, restrictive VSDs with a significant left-to-right shunt, trauma, or iatrogenic artifacts after surgical replacement of the aortic valve. Indications for closure of restrictive VSDs in the adult population include a history of bacterial endocarditis or a hemodynamically significant left-to-right shunt (Qp/Qs greater than 1.5:1).

Percutaneous closure of VSD offers an attractive alternative to surgical management in patients with increased surgical risk factors, multiple previous cardiac surgical interventions, poorly accessible muscular VSDs, or "Swiss cheese"–type VSDs. At the time of this writing, US Food and Drug Administration approval for device closure of VSDs in the United States is limited to closure of muscular VSDs. Experience with percutaneous closure of other types of VSDs has been obtained at centers outside the United States or in centers with investigational protocols.

Complications have been reported in as many as 10.7% of patients and most frequently include rhythm and conduction abnormalities, as well as hypotensive episodes or blood loss (283); however, complications are significantly associated with a lower patient weight (below 10 kg), and therefore the adult population is likely to represent a lower-risk group for percutaneous closure of muscular VSDs. Complications after closure of perimembranous VSDs predominantly include rhythm and conduction abnormalities, as well as the potential for new or increased AR or TR, which is usually of a trivial or mild degree.

Success rates of the procedure are high, with closure rates with the membranous device of up to 92% at 15 minutes after device implantation and a 92% rate of complete closure at the 12-month follow-up for device closure of muscular VSD. These results, unfortunately, are not matched in patients undergoing closure of a postinfarct VSD because of the often moribund status of these patients and the tendency of the VSD to enlarge over time owing to ongoing necrosis.

3.6. Key Issues to Evaluate and Follow-Up.   3.6.1. Recommendations for Surgical and Catheter Intervention Follow-Up
Class I

1 Adults with VSD with residual heart failure, shunts, PAH, AR, or RVOT or LVOT obstruction should be seen at least annually at an ACHD regional center. (Level of Evidence: C)

2 Adults with a small residual VSD and no other lesions should be seen every 3 to 5 years at an ACHD regional center. (Level of Evidence: C)

3 Adults with device closure of a VSD should be followed up every 1 to 2 years at an ACHD center depending on the location of the VSD and other factors. (Level of Evidence: C)

Adults with no residual VSD, no associated lesions, and normal pulmonary artery pressure do not require continued follow-up at a regional ACHD center except on referral from their cardiologist or physician. Patients who develop bifascicular block or transient trifascicular block after VSD closure are at risk in later years for the development of complete heart block and should be followed up yearly by history and ECG and have periodic ambulatory monitoring and/or exercise testing.

3.6.2. Recommendation for Reproduction
Class III

1 Pregnancy in patients with VSD and severe PAH (Eisenmenger syndrome) is not recommended owing to excessive maternal and fetal mortality and should be strongly discouraged. (Level of Evidence: A)

Women with small VSDs, no PAH, and no associated lesions have no increased cardiovascular risk for pregnancy. Women with PAH should be counseled against pregnancy (refer to Section 9, Pulmonary Hypertension/Eisenmenger Physiology).

Pregnancy is generally well tolerated, with no maternal mortality and no significant maternal or fetal morbidity. Although the left-to-right shunt may increase with the increase in cardiac output during pregnancy, this is counterbalanced by the decrease in peripheral resistance. Women with large shunts and PAH may experience arrhythmias, ventricular dysfunction, and progression of PAH.

3.6.3. Activity
No activity restrictions are indicated for patients with small VSDs, no associated lesions, and normal ventricular function. If pulmonary vascular disease is present, activity is usually self-restricted, but patients should be advised against strenuous exercise or travel to altitudes above 5000 feet. Long-distance air travel should be approached with caution to avoid dehydration, with specific recommendation by an ACHD specialist concerning the need for supplemental oxygen (refer to Section 9, Pulmonary Hypertension/Eisenmenger Physiology).

	4. Atrioventricular Septal Defect

Top Task Force Members Table of Contents Preamble 1. Introduction 2. Atrial Septal Defect 3. Ventricular Septal Defect 4. Atrioventricular Septal... 5. Patent Ductus Arteriosus 6. Left-Sided Heart Obstructive... 7. Right Ventricular Outflow... 8. Coronary Artery Abnormalities 9. Pulmonary... 10. Tetralogy of Fallot 11. Dextro-Transposition of the... 12. Congenitally Corrected... 13. Ebstein's Anomaly 14. Tricuspid Atresia/Single... Staff Appendixes Appendix 3 Appendix 4 References

 
4.1. Definition.   The terms AVSD, AV canal defect, and endocardial cushion defect can be used interchangeably to describe this group of defects. The basic morphology of AVSD includes a large, central defect that may lie above the AV valve (refer to Section 2, Atrial Septal Defect) or may extend to variable degrees above and below the AV valve; therefore, the interventricular communication can range from large to small. There is a common AV valve annulus that stretches across both ventricles. There may be a common superior leaflet, or the superior leaflet may be separated at its distal margin into right and left components. The AV valve may be misaligned with respect to the ventricles, in association with hypoplasia of the right or left ventricle. The left AV valve is a trileaflet valve made of superior and inferior bridging leaflets separated by a mural leaflet. There may be abnormal lateral rotation of the posteromedial papillary muscle. Most complete AVSDs are in Down syndrome patients (more than 75%). Most partial AVSDs occur in non–Down syndrome patients (more than 90%).

4.2. Associated Lesions.   Tetralogy of Fallot and other conotruncal anomalies and heterotaxy syndromes also occur in association with AVSD.

4.3. Clinical Features and Evaluation.   Most patients will have had surgery in childhood. The unrepaired adult may be asymptomatic or may present with congestive heart failure, exertional limitation, PAH and cyanosis, IE, or atrial flutter/fibrillation. Patients with partial AVSD are likely to become symptomatic at a younger age if significant left AV valve regurgitation is present.

4.3.1. Clinical Examination
Physical examination of the unoperated patient may show findings of an ASD, a VSD, AV valve regurgitation, LVOT obstruction, or PAH with cyanosis. A patient with severe PAH may have no murmur, a single loud second heart sound, and cyanosis/clubbing.

The typical repaired patient will have a normal examination apart from an apical systolic murmur if there is residual mitral regurgitation or subaortic obstruction. Subaortic obstruction may occur naturally in association with abnormal AV valve attachments or may be the consequence of surgery. In addition, the surgical repair may have created AV valve stenosis. Cyanosis should not be present in the absence of Eisenmenger syndrome or RV outflow obstruction.

4.3.2. Electrocardiogram
The typical ECG shows superior left-axis deviation with a counterclockwise loop in the frontal plane. First-degree AV block may be present. Atrial flutter or fibrillation may develop in the older patient. Left atrial enlargement and LV hypertrophy may be present if there is significant left AV valve regurgitation. RV hypertrophy may predominate if there is PAH or associated RVOT obstruction.

4.3.3. Chest X-Ray
Cardiomegaly may be present due to dilation of the right or left AV heart chambers, depending on the degree and direction of AV valve regurgitation and the degree and level of left-to-right shunting. Increased pulmonary vascular markings are present when there is a significant left-to-right shunt. Pulmonary venous congestion may be seen when there is long-standing mitral regurgitation. In patients with PAH, a prominent main pulmonary artery segment and pruning of distal pulmonary vessels may be present.

4.3.4. Echocardiography
In the patient with a partial and unrepaired AVSD, TTE is the primary imaging modality and should include demonstration of the borders of the primum ASD, a VSD (if present), the morphology and function of the AV valve, ventricular size and shunting, and SubAS (if present). In the patient with a complete and unrepaired AVSD, this will include the presence and size of the septal defect, the morphology and function of the common AV valve, and ventricular size and function. When the ventricular portion of the septal defect is large, the ventricular septum may be deficient apically and inferiorly. Pulmonary artery pressures (expected to be very high in complete AVSD) should be evaluated by measuring TR and pulmonary regurgitation jet velocity with simultaneous systemic blood pressure measurement. Evidence of subaortic obstruction, caused by AV valve attachments to the crest of the interventricular septum, should be sought by imaging and Doppler. In the postrepair patient, residua may include left AV valve dysfunction, SubAS, VSD patch leak, and PAH. It may be difficult to distinguish residual LV to right atrial shunt from TR with RV hypertension. The failure to distinguish these may result in erroneous diagnosis of PAH.

4.3.5. Magnetic Resonance Imaging
MRI may be useful to evaluate venous and arterial anatomy when associated lesions are suspected. Three-dimensional MRI is sometimes helpful in delineating leaflet morphology and outflow anatomy.

4.3.6. Recommendation for Heart Catheterization
Class IIa

1 Cardiac catheterization is reasonable to assess PAH and test vasoreactivity in patients with repaired or unrepaired AVSD. (Level of Evidence: B)

Heart catheterization has a limited role in the assessment of these patients unless noninvasive findings are equivocal. Evaluation of PAH and coronary anatomy may be needed when reoperation is being considered. Hemodynamic data may also be needed when noninvasive studies have not been able to provide this information.

4.3.7. Exercise Testing
Exercise testing may be used to objectively assess functional capacity.

4.4. Management Strategies.   4.4.1. Medical Therapy
Most patients need no regular medication in the absence of specific problems. ACE inhibitors and/or diuretics may be used in patients with AV valve regurgitation and symptoms of chronic heart failure. Pulmonary vasodilation therapy may be indicated in patients with PAH and no significant left-to-right shunt who are deemed to be at high risk for surgical repair, but this should be approached with caution because of the potential for producing a significant right-to-left shunt.

4.4.2. Recommendations for Surgical Therapy
Class I

1 Surgeons with training and expertise in CHD should perform operations for AVSD. (Level of Evidence: C)

2 Surgical reoperation is recommended in adults with previously repaired AVSD with the following indications:

a Left AV valve repair or replacement for regurgitation or stenosis that causes symptoms, atrial or ventricular arrhythmias, a progressive increase in LV dimensions, or deterioration of LV function. (Level of Evidence: B)

b LVOT obstruction with a mean gradient greater than 50 mm Hg or peak instantaneous gradient greater than 70 mm Hg, or a gradient less than 50 mm Hg in association with significant mitral regurgitation or AR. (Level of Evidence: B)

c Residual/recurrent ASD or VSD with significant left-to-right shunting (refer to Section 2, Atrial Septal Defect, and Section 3, Ventricular Septal Defect). (Level of Evidence: B)

Primary operation is rarely recommended for complete AVSD in adults because of pulmonary vascular obstructive disease. Unoperated partial or transitional AVSD, also known as partial or transitional AV canal, may not be identified until adulthood. Primary repair is generally recommended provided there is no fixed PAH.

Complete AVSD is usually repaired during infancy because of the risk of accelerated pulmonary vascular disease. Partial AVSD, also known as partial AV canal, is usually repaired in early childhood. The timing of repair of intermediate or transitional AVSD depends on the size of the VSD and the degree of shunting. Complete repair usually includes patch closure of the septal defect. Suture of the cleft in the left AV valve is dependent on leaflet morphology and surgical choice. Pulmonary artery banding of complete AVSD is rarely performed and is reserved for complex lesions.

Rerepair includes valve repair or replacement for left AV regurgitation or stenosis. LVOT obstruction is treated most commonly by resection of the fibrous stenosis/membrane, modified Konno procedure, or Konno-Rastan procedure. Suture or patch closure is performed for residual/recurrent ASD or VSD. A concomitant Maze procedure may be performed for intermittent or chronic atrial fibrillation/flutter. Management of patients should be in tertiary CHD centers or children's hospitals with experienced medical and surgical personnel.