ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease
2008; Lippincott Williams & Wilkins; Volume: 118; Issue: 23 Linguagem: Inglês
10.1161/circulationaha.108.190690
ISSN1524-4539
AutoresCarole A. Warnes, Roberta G. Williams, Thomas M. Bashore, John Child, Heidi M. Connolly, Joseph A. Dearani, Pedro J. del Nido, James W. Fasules, T.P. Graham, Ziyad M. Hijazi, Sharon A. Hunt, Mary Etta King, Michael J. Landzberg, Pamela D. Miner, Martha J. Radford, Edward P. Walsh, Gary D. Webb,
Tópico(s)Coronary Artery Anomalies
ResumoHomeCirculationVol. 118, No. 23ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessReview ArticlePDF/EPUBACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart DiseaseA 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, WRITING COMMITTEE:, MD, FRCP, FACC, FAHA, Co-Chair, Roberta G. Williams, MD, MACC, FAHA, Co-Chair, Thomas M. Bashore, MD, FACC, John S. Child, MD, FACC, FAHA, Heidi M. Connolly, MD, FACC, Joseph A. Dearani, MD, FACC, Pedro del Nido, MD, James W. Fasules, MD, FACC, Thomas P. GrahamJr, MD, FACC, Ziyad M. Hijazi, MBBS, MPH, FACC, FSCAI, Sharon A. Hunt, MD, FACC, FAHA, Mary Etta King, MD, FACC, FASE, Michael J. Landzberg, MD, FACC, Pamela D. Miner, RN, MN, NP, Martha J. Radford, MD, FACC, Edward P. Walsh, MD, FACC and Gary D. Webb, MD, FACC Carole A. WarnesCarole A. Warnes Search for more papers by this author , Roberta G. WilliamsRoberta G. Williams Search for more papers by this author , Thomas M. BashoreThomas M. Bashore Search for more papers by this author , John S. ChildJohn S. Child Search for more papers by this author , Heidi M. ConnollyHeidi M. Connolly Search for more papers by this author , Joseph A. DearaniJoseph A. Dearani Search for more papers by this author , Pedro del NidoPedro del Nido Search for more papers by this author , James W. FasulesJames W. Fasules Search for more papers by this author , Thomas P. GrahamJrThomas P. GrahamJr Search for more papers by this author , Ziyad M. HijaziZiyad M. Hijazi Search for more papers by this author , Sharon A. HuntSharon A. Hunt Search for more papers by this author , Mary Etta KingMary Etta King Search for more papers by this author , Michael J. LandzbergMichael J. Landzberg Search for more papers by this author , Pamela D. MinerPamela D. Miner Search for more papers by this author , Martha J. RadfordMartha J. Radford Search for more papers by this author , Edward P. WalshEdward P. Walsh Search for more papers by this author and Gary D. WebbGary D. Webb Search for more papers by this author Originally published7 Nov 2008https://doi.org/10.1161/CIRCULATIONAHA.108.190690Circulation. 2008;118:e714–e833Other version(s) of this articleYou are viewing the most recent version of this article. Previous versions: November 7, 2008: Previous Version 1 Preamble…e7211. Introduction…e721 1.1. Methodology and Evidence Review…e721 1.2. Organization of Committee and Relationships With Industry…e722 1.3. Document Review and Approval…e722 1.4. Epidemiology and Scope of the Problem…e722 1.5. Recommendations for Delivery of Care and Ensuring Access…e722 1.5.1. Recommendations for Access to Care…e725 1.5.2. Recommendations for Psychosocial Issues…e726 1.5.3. Transition of Care…e727 1.5.4. Exercise and Athletics…e728 1.5.5. Employment…e728 1.5.6. Insurability…e728 1.5.7. Congenital Syndromes…e729 1.5.8. Medical/Ethical/Legal Issues…e729 1.6. Recommendations for Infective Endocarditis…e730 1.7. Recommendations for Noncardiac Surgery…e734 1.8. Recommendations for Pregnancy and Contraception…e734 1.8.1. Contraception…e735 1.9. Recommendations for Arrhythmia Diagnosis and Management…e735 1.9.1. Management of Tachyarrhythmias: Wolff-Parkinson-White Syndrome…e736 1.9.2. Intra-Atrial Reentrant Tachycardia or Atrial Flutter…e736 1.9.3. Atrial Fibrillation…e737 1.9.4. Ventricular Tachycardia…e737 1.10. Management of Bradycardias…e738 1.10.1. Sinoatrial Node Dysfunction…e738 1.10.2. Atrioventricular Block…e739 1.11. Cyanotic Congenital Heart Disease…e739 1.11.1. Recommendations for Hematologic Problems…e739 1.11.1.1. Hemostasis…e739 1.11.1.2. Renal Function…e740 1.11.1.3. Gallstones…e740 1.11.1.4. Orthopedic and Rheumatologic Complications…e740 1.11.1.5. Neurological Complications…e740 1.11.1.6. Pulmonary Vascular Disease…e740 1.12. Recommendations for General Health Issues for Cyanotic Patients…e740 1.12.1. Hospitalization and Operation…e740 1.12.2. Cardiac Reoperation and Preoperative Evaluation…e740 1.13. Heart Failure in Adult Congenital Heart Disease…e741 1.14. Recommendations for Heart and Heart/Lung Transplantation…e7432. Atrial Septal Defect…e744 2.1. Definition…e744 2.1.1. Associated Lesions…e744 2.2. Clinical Course…e744 2.2.1. Unrepaired Atrial Septal Defect…e744 2.3. Recommendations for Evaluation of the Unoperated Patient…e744 2.3.1. Clinical Examination…e745 2.3.2. Electrocardiogram…e745 2.3.3. Chest X-Ray…e745 2.3.4. Echocardiography…e745 2.3.5. Magnetic Resonance Imaging…e745 2.3.6. Exercise Testing…e745 2.4. Diagnostic Problems and Pitfalls…e745 2.5. Management Strategies…e746 2.5.1. Recommendations for Medical Therapy…e746 2.5.2. Recommendations for Interventional and Surgical Therapy…e746 2.5.3. Indications for Closure of Atrial Septal Defect…e747 2.5.4. Catheter Intervention…e747 2.5.5. Key Issues to Evaluate and Follow-Up…e747 2.6. Recommendations for Postintervention Follow-Up…e747 2.6.1. Endocarditis Prophylaxis…e747 2.6.2. Recommendation for Reproduction…e747 2.6.3. Activity…e7483. Ventricular Septal Defect…e749 3.1. Definition…e749 3.1.1. Associated Lesions…e749 3.2. Clinical Course (Unrepaired)…e749 3.3. Clinical Features and Evaluation of the Unoperated Patient…e750 3.3.1. Clinical Examination…e750 3.3.2. Electrocardiogram…e750 3.3.3. Chest X-Ray…e750 3.3.4. Echocardiography…e750 3.3.5. Magnetic Resonance Imaging/Computed Tomography…e750 3.3.6. Recommendations for Cardiac Catheterization…e750 3.4. Diagnostic Problems and Pitfalls…e751 3.5. Management Strategies…e751 3.5.1. Recommendation for Medical Therapy…e751 3.5.2. Recommendations for Surgical Ventricular Septal Defect Closure…e751 3.5.3. Recommendation for Interventional Catheterization…e751 3.6. Key Issues to Evaluate and Follow-Up…e752 3.6.1. Recommendations for Surgical and Catheter Intervention Follow-Up…e752 3.6.2. Recommendation for Reproduction…e752 3.6.3. Activity…e7524. Atrioventricular Septal Defect…e752 4.1. Definition…e752 4.2. Associated Lesions…e752 4.3. Clinical Features and Evaluation…e752 4.3.1. Clinical Examination…e752 4.3.2. Electrocardiogram…e752 4.3.3. Chest X-Ray…e753 4.3.4. Echocardiography…e753 4.3.5. Magnetic Resonance Imaging…e753 4.3.6. Recommendation for Heart Catheterization…e753 4.3.7. Exercise Testing…e753 4.4. Management Strategies…e753 4.4.1. Medical Therapy…e753 4.4.2. Recommendations for Surgical Therapy…e753 4.5. Key Issues to Evaluate and Follow-Up…e753 4.5.1. Key Postoperative Issues…e753 4.5.2. Evaluation and Follow-Up of the Repaired Patient…e754 4.5.3. Electrophysiology Testing/Pacing Issues in Atrioventricular Septal Defects…e754 4.5.4. Recommendations for Endocarditis Prophylaxis…e754 4.6. Reproduction…e754 4.6.1. Genetic Aspects…e754 4.6.2. Recommendations for Pregnancy…e754 4.7. Exercise…e7545. Patent Ductus Arteriosus…e754 5.1. Definition and Associated Lesions…e754 5.2. Presentation and Clinical Course…e754 5.3. Recommendations for Evaluation of the Unoperated Patient…e755 5.3.1. Clinical Examination…e755 5.3.2. Electrocardiogram…e755 5.3.3. Echocardiography…e755 5.3.4. Chest X-Ray…e755 5.3.5. Cardiac Catheterization…e755 5.3.6. Magnetic Resonance Imaging/Computed Tomography…e755 5.4. Problems and Pitfalls…e755 5.5. Management Strategies…e755 5.5.1. Recommendations for Medical Therapy…e755 5.5.2. Recommendations for Closure of Patent Ductus Arteriosus…e756 5.5.3. Surgical/Interventional Therapy…e756 5.6. Key Issues to Evaluate and Follow-Up…e7566. Left-Sided Heart Obstructive Lesions: Aortic Valve Disease, Subvalvular and Supravalvular Aortic Stenosis, Associated Disorders of the Ascending Aorta, and Coarctation…e756 6.1. Definition…e756 6.2. Associated Lesions…e756 6.3. Clinical Course (Unrepaired)…e757 6.4. Recommendations for Evaluation of the Unoperated Patient…e757 6.4.1. Clinical Examination…e758 6.4.2. Electrocardiogram…e758 6.4.3. Chest X-Ray…e758 6.4.4. Echocardiography…e758 6.4.5. Magnetic Resonance Imaging/Computed Tomography…e758 6.4.6. Stress Testing…e758 6.4.7. Cardiac Catheterization…e758 6.5. Problems and Pitfalls…e758 6.6. Management Strategies for Left Ventricular Outflow Tract Obstruction and Associated Lesions…e758 6.6.1. Recommendations for Medical Therapy…e758 6.6.2. Catheter and Surgical Intervention…e759 6.6.2.1. Recommendations for Catheter Interventions for Adults With Valvular Aortic Stenosis …e759 6.6.2.2. Recommendations for Aortic Valve Repair/Replacement and Aortic Root Replacement…e760 6.7. Recommendations for Key Issues to Evaluate and Follow-Up…e760 6.7.1. Reproduction…e761 6.7.2. Activity/Exercise…e761 6.8. Isolated Subaortic Stenosis…e761 6.8.1. Definition…e761 6.8.2. Associated Lesions…e761 6.8.3. Clinical Course With/Without Previous Intervention…e761 6.8.4. Clinical Features and Evaluation…e762 6.8.4.1. Clinical Examination…e762 6.8.4.2. Electrocardiogram…e762 6.8.4.3. Chest X-Ray…e762 6.8.4.4. Echocardiography…e762 6.8.5. Diagnostic Cardiac Catheterization…e762 6.8.6. Problems and Pitfalls…e762 6.8.7. Management Strategies…e762 6.8.7.1. Medical Therapy…e762 6.8.7.2. Recommendations for Surgical Intervention…e762 6.8.8. Recommendations for Key Issues to Evaluate and Follow-Up…e763 6.8.9. Special Issues…e763 6.8.9.1. Pregnancy…e763 6.8.9.2. Exercise and Athletics…e763 6.9. Supravalvular Aortic Stenosis…e763 6.9.1. Definition…e763 6.9.2. Associated Lesions…e763 6.9.3. Clinical Course (Unrepaired)…e763 6.10. Recommendations for Evaluation of the Unoperated Patient…e763 6.10.1. Clinical Examination…e764 6.10.2. Electrocardiogram…e764 6.10.3. Chest X-Ray…e764 6.10.4. Imaging…e764 6.10.5. Stress Testing…e764 6.10.6. Myocardial Perfusion Imaging…e764 6.10.7. Cardiac Catheterization…e764 6.11. Management Strategies for Supravalvular Left Ventricular Outflow Tract…e764 6.11.1. Recommendations for Interventional and Surgical Therapy…e764 6.11.2. Recommendations for Key Issues to Evaluate and Follow-Up…e764 6.11.3. Special Issues…e765 6.11.4. Exercise and Athletics…e765 6.11.5. Recommendations for Reproduction…e765 6.12. Aortic Coarctation…e765 6.12.1. Definition…e765 6.12.2. Associated Lesions…e765 6.12.3. Recommendations for Clinical Evaluation and Follow-Up…e765 6.13. Clinical Features and Evaluation of Unrepaired Patients…e765 6.13.1. Electrocardiogram…e765 6.13.2. Chest X-Ray…e766 6.13.3. Echocardiography and Doppler…e766 6.13.4. Stress Testing…e766 6.13.5. Magnetic Resonance Imaging/Magnetic Resonance Angiography or Computed Tomography With 3-Dimensional Reconstruction…e766 6.13.6. Catheterization Hemodynamics/Angiography…e766 6.13.7. Problems and Pitfalls…e766 6.14. Management Strategies for Coarctation of the Aorta…e766 6.14.1. Medical Therapy…e766 6.14.2. Recommendations for Interventional and Surgical Treatment of Coarctation of the Aorta in Adults…e766 6.14.3. Recommendations for Key Issues to Evaluate and Follow-Up…e767 6.14.4. Exercise and Athletics…e767 6.14.5. Reproduction…e767 6.14.6. Endocarditis Prophylaxis…e7677. Right Ventricular Outflow Tract Obstruction…e768 7.1. Definition…e768 7.2. Associated Lesions…e768 7.3. Valvular Pulmonary Stenosis…e768 7.3.1. Definition…e768 7.4. Clinical Course…e769 7.4.1. Unrepaired Patients…e769 7.4.2. Noonan Syndrome Patients With Prior Repair…e769 7.5. Recommendations for Evaluation of the Unoperated Patient…e769 7.5.1. Clinical Examination…e769 7.5.2. Electrocardiogram…e769 7.5.3. Chest X-Ray…e769 7.5.4. Echocardiography…e769 7.5.5. Magnetic Resonance Imaging/Computed Tomography…e769 7.5.6. Cardiac Catheterization…e770 7.5.7. Relationship Between Peak Instantaneous Doppler Echocardiographic Pressure Gradients and Peak-to-Peak Cardiac Catheterization Gradients…e770 7.6. Problems and Pitfalls…e770 7.6.1. Dyspnea…e770 7.6.2. Chest Pain…e770 7.6.3. Enlarging Right Ventricle…e770 7.6.4. Pulmonary Arterial Hypertension…e770 7.6.5. Cyanosis…e770 7.6.6. Systemic Venous Congestion…e770 7.7. Management Strategies…e770 7.7.1. Recommendations for Intervention in Patients With Valvular Pulmonary Stenosis…e771 7.7.2. Percutaneous Balloon Pulmonary Valvotomy…e771 7.7.3. Surgical Pulmonary Valvotomy or Valve Replacement…e771 7.8. Recommendation for Clinical Evaluation and Follow-Up After Intervention…e772 7.8.1. Reproduction…e772 7.8.2. Endocarditis Prophylaxis…e772 7.8.3. Exercise and Athletics…e772 7.9. Right-Sided Heart Obstruction Due to Supravalvular, Branch, and Peripheral Pulmonary Artery Stenosis…e773 7.9.1. Definition and Associated Lesions…e773 7.9.2. Clinical Course…e773 7.10. Clinical Features and Evaluation of the Unrepaired Patient…e773 7.10.1. Electrocardiogram…e773 7.10.2. Chest X-Ray…e773 7.10.3. Echocardiography…e773 7.10.4. Magnetic Resonance Imaging/Computed Tomography…e773 7.10.5. Cardiac Catheterization…e774 7.11. Recommendations for Evaluation of Patients With Supravalvular, Branch, and Peripheral Pulmonary Stenosis…e774 7.11.1. Problems and Pitfalls…e774 7.11.2. Management Strategies…e774 7.11.2.1. Medical Therapy…e774 7.12. Recommendations for Interventional Therapy in the Management of Branch and Peripheral Pulmonary Stenosis…e774 7.12.1. Recommendations for Evaluation and Follow-Up…e774 7.13. Right-Sided Heart Obstruction Due to Stenotic Right Ventricular–Pulmonary Artery Conduits or Bioprosthetic Valves…e775 7.13.1. Definition and Associated Lesions…e775 7.13.2. Recommendation for Evaluation and Follow-Up After Right Ventricular–Pulmonary Artery Conduit or Prosthetic Valve…e775 7.13.3. Clinical Examination…e775 7.13.4. Electrocardiogram…e775 7.13.5. Chest X-Ray…e775 7.13.6. Echocardiography…e775 7.13.7. Magnetic Resonance Imaging/Computed Tomography…e775 7.13.8. Cardiac Catheterization…e775 7.14. Recommendations for Reintervention in Patients With Right Ventricular–Pulmonary Artery Conduit or Bioprosthetic Pulmonary Valve Stenosis…e775 7.14.1. Medical Therapy…e775 7.14.2. Interventional Catheterization…e775 7.14.3. Surgical Intervention…e776 7.14.4. Key Issues to Evaluate and Follow-Up…e776 7.15. Double-Chambered Right Ventricle…e776 7.15.1. Definition and Associated Lesions…e776 7.15.2. Clinical Features and Evaluation of the Unoperated Patient…e776 7.15.3. Clinical Examination…e776 7.15.4. Electrocardiogram…e776 7.15.5. Echocardiography-Doppler Imaging…e776 7.15.6. Magnetic Resonance Imaging…e776 7.15.7. Cardiac Catheterization…e776 7.16. Problems and Pitfalls…e776 7.16.1. Multiple Levels of Right Ventricular Outflow Tract Obstruction…e776 7.17. Management Strategies…e777 7.17.1. Recommendations for Intervention in Patients With Double-Chambered Right Ventricle…e777 7.18. Key Issues to Evaluate and Follow-Up…e7778. Coronary Artery Abnormalities…e777 8.1. Definition and Associated Lesions…e777 8.1.1. General Recommendations for Evaluation and Surgical Intervention…e777 8.2. Recommendations for Coronary Anomalies Associated With Supravalvular Aortic Stenosis…e777 8.2.1. Clinical Course (Unrepaired) …e777 8.2.2. Clinical Features…e778 8.3. Recommendation for Coronary Anomalies Associated With Tetralogy of Fallot…e778 8.3.1. Preintervention Evaluation…e778 8.3.2. Surgical and Catheterization-Based Interventions…e778 8.4. Recommendation for Coronary Anomalies Associated With Dextro-Transposition of the Great Arteries After Arterial Switch Operation…e778 8.4.1. Definition and Associated Lesions…e778 8.4.2. Clinical Course…e778 8.4.3. Clinical Features and Evaluation After Arterial Switch Operation…e778 8.4.4. Surgical and Catheterization-Based Intervention…e778 8.5. Recommendations for Congenital Coronary Anomalies of Ectopic Arterial Origin…e778 8.5.1. Definition, Associated Lesions, and Clinical Course…e779 8.5.2. Clinical Features and Evaluation of the Unoperated Patient…e779 8.5.2.1. Preintervention Evaluation…e779 8.5.3. Management Strategies…e779 8.5.3.1. Surgical and Catheterization-Based Intervention…e779 8.6. Recommendations for Anomalous Left Coronary Artery From the Pulmonary Artery…e779 8.6.1. Definition and Associated Lesions and Clinical Course…e779 8.7. Management Strategies…e780 8.7.1. Surgical Intervention…e780 8.7.2. Surgical and Catheterization-Based Intervention…e780 8.8. Recommendations for Coronary Arteriovenous Fistula…e780 8.8.1. Definition…e780 8.8.2. Clinical Course…e780 8.8.3. Preintervention Evaluation…e780 8.9. Recommendations for Management Strategies…e780 8.9.1. Surgical Intervention…e780 8.9.2. Catheterization-Based Intervention…e781 8.9.3. Preintervention Evaluation After Surgical or Catheterization-Based Repair…e7819. Pulmonary Hypertension/Eisenmenger Physiology…e781 9.1. Definition…e781 9.2. Clinical Course…e781 9.2.1. Dynamic Congenital Heart Disease– Pulmonary Arterial Hypertension…e781 9.2.2. Immediate Postoperative Congenital Heart Disease–Pulmonary Arterial Hypertension…e782 9.2.3. Late Postoperative Congenital Heart Disease–Pulmonary Arterial Hypertension…e782 9.2.4. Normal to Mildly Abnormal Pulmonary Vascular Resistance States…e782 9.2.5. Eisenmenger Physiology…e782 9.3. Problems and Pitfalls…e782 9.4. Recommendations for Evaluation of the Patient With Congenital Heart Disease–Pulmonary Arterial Hypertension…e782 9.4.1. Dynamic Congenital Heart Disease– Pulmonary Arterial Hypertension…e783 9.4.2. Eisenmenger Physiology…e783 9.5. Management Strategies…e783 9.5.1. Recommendations for Medical Therapy of Eisenmenger Physiology…e783 9.6. Key Issues to Evaluate and Follow-Up…e785 9.6.1. Recommendations for Reproduction…e785 9.6.2. Pregnancy…e785 9.6.3. Other Interventions…e785 9.6.4. Recommendations for Follow-Up…e785 9.6.5. Endocarditis Prophylaxis…e78510. Tetralogy of Fallot…e785 10.1 Definition and Associated Lesions…e785 10.2. Clinical Course (Unrepaired)…e786 10.2.1. Presentation as an Unoperated Patient…e786 10.2.2. Postsurgical Presentation…e786 10.3. Clinical Features and Evaluation…e786 10.3.1. Clinical Examination…e786 10.3.2. Electrocardiogram…e786 10.3.3. Chest X-Ray…e786 10.3.4. Initial Surgical Repair…e786 10.4. Recommendations for Evaluation and Follow-Up of the Repaired Patient…e786 10.4.1. Recommendation for Imaging…e787 10.5. Recommendations for Diagnostic and Interventional Catheterization for Adults With Tetralogy of Fallot…e787 10.5.1. Branch Pulmonary Artery Angioplasty…e788 10.5.2. Exercise Testing…e788 10.5.3. Diagnostic Catheterization…e788 10.6. Problems and Pitfalls in the Patient With Prior Repair…e788 10.7. Management Strategy for the Patient With Prior Repair…e788 10.7.1. Medical Therapy…e788 10.8. Recommendations for Surgery for Adults With Previous Repair of Tetralogy of Fallot…e789 10.8.1. Recommendations for Interventional Catheterization…e789 10.9. Key Issues to Evaluate and Follow-Up…e789 10.9.1. Recommendations for Arrhythmias: Pacemaker/Electrophysiology Testing…e789 10.9.2. Reproduction…e791 10.9.3. Exercise…e791 10.9.4. Endocarditis Prophylaxis…e79111. Dextro-Transposition of the Great Arteries…e791 11.1. Definition…e791 11.2. Associated Lesions…e791 11.3. Clinical Course: Unrepaired…e792 11.4. Recommendation for Evaluation of the Operated Patient With Dextro- Transposition of the Great Arteries…e792 11.4.1. Clinical Features and Evaluation of Dextro-Transposition of the Great Arteries After Atrial Baffle Procedure…e792 11.4.2. Clinical Examination…e792 11.4.3. Electrocardiogram…e792 11.4.4. Imaging for Dextro-Transposition of the Great Arteries After Atrial Baffle Procedure…e792 11.4.4.1. Recommendations for Imaging for Dextro-Transposition of the Great Arteries After Atrial Baffle Procedure…e792 11.4.5. Cardiac Catheterization…e793 11.5. Clinical Features and Evaluation of Dextro-Transposition of the Great Arteries After Arterial Switch Operation…e793 11.5.1. Clinical Examination…e793 11.5.2. Electrocardiogram…e793 11.5.3. Chest X-Ray…e793 11.5.4. Recommendations for Imaging for Dextro-Transposition of the Great Arteries After Arterial Switch Operation…e793 11.5.5. Recommendation for Cardiac Catheterization After Arterial Switch Operation…e794 11.6. Clinical Features and Evaluation: Dextro-Transposition of the Great Arteries After Rastelli Operation…e794 11.6.1. Electrocardiogram…e794 11.6.2. Chest X-Ray…e794 11.6.3. Imaging…e794 11.7. Recommendations for Diagnostic Catheterization for Adults With Repaired Dextro-Transposition of the Great Arteries…e794 11.7.1. Problems and Pitfalls…e794 11.8. Management Strategies…e795 11.8.1. Medical Therapy…e795 11.8.2. Recommendations for Interventional Catheterization for Adults With Dextro-Transposition of the Great Arteries…e795 11.8.2.1. Interventional Catheter Options After Atrial Baffle…e795 11.8.2.2. Interventional Catheter Options After Arterial Switch Operation…e795 11.8.2.3. Interventional Catheter Options After Rastelli Repair…e795 11.8.3. Recommendations for Surgical Interventions…e795 11.8.3.1. After Atrial Baffle Procedure (Mustard, Senning) …e795 11.8.3.2. After Arterial Switch Operation…e795 11.8.3.3. After Rastelli Procedure…e796 11.8.3.4. Reoperation After Atrial Baffle Procedure…e796 11.8.3.5. Reoperation After Arterial Switch Operation…e796 11.8.3.6. Reoperation After Rastelli Repair…e797 11.8.3.7. Other Reoperation Options…e797 11.9. Recommendations for Electrophysiology Testing/Pacing Issues in Dextro- Transposition of the Great Arteries…e797 11.10. Key Issues to Evaluate and Follow-Up…e798 11.10.1. Recommendations for Endocarditis Prophylaxis…e798 11.10.2. Recommendation for Reproduction…e798 11.10.3. Activity and Exercise…e79812. Congenitally Corrected Transposition of the Great Arteries…e798 12.1. Definition…e798 12.2. Associated Lesions…e798 12.3. Clinical Course…e799 12.3.1. Presentation in Adulthood: Unoperated…e799 12.4. Clinical Features and Evaluation of the Unoperated Patient…e799 12.4.1. Clinical Examination…e799 12.4.2. Electrocardiogram…e800 12.4.3. Exercise Testing…e800 12.4.4. Chest X-Ray…e800 12.4.5. Two-Dimensional Echocardiography…e800 12.4.6. Magnetic Resonance Imaging…e800 12.4.7. Cardiac Catheterization…e800 12.5. Recommendations for Evaluation and Follow-Up of Patients With Congenitally Corrected Transposition of the Great Arteries…e800 12.6. Key Issues of Unoperated Patients…e801 12.7. Management Strategies…e801 12.8. Interventional Therapy…e801 12.8.1. Recommendations for Catheter Interventions…e801 12.8.2. Initial Surgical Repair…e801 12.8.3. Recommendations for Surgical Intervention…e802 12.8.4. Problems and Pitfalls…e802 12.9. Arrhythmias/Pacemaker/Electrophysiology Testing…e802 12.10. Recommendations for Postoperative Care…e802 12.10.1. Recommendations for Endocarditis Prophylaxis…e802 12.10.2. Recommendation for Reproduction…e803 12.10.3. Activity…e80313. Ebstein’s Anomaly…e803 13.1. Definition…e803 13.2. Clinical Course (Unoperated)…e803 13.2.1. Pediatric Presentation…e803 13.2.2. Initial Adult Presentation…e803 13.3. Clinical Features and Evaluation of the Unoperated Patient…e804 13.4. Recommendation for Evaluation of Patients With Ebstein’s Anomaly…e804 13.4.1. Clinical Examination…e804 13.4.2. Electrocardiogram…e804 13.4.3. Chest X-Ray…e804 13.4.4. Echocardiography…e804 13.4.5. Magnetic Resonance Imaging/Computed Tomography…e804 13.5. Recommendations for Diagnostic Tests…e804 13.5.1. Cardiac Catheterization…e805 13.5.2. Problems and Pitfalls…e805 13.6. Management Strategies…e805 13.6.1. Recommendation for Medical Therapy…e805 13.6.2. Physical Activity…e805 13.7. Recommendation for Catheter Interventions for Adults With Ebstein’s Anomaly…e805 13.7.1. Recommendation for Electrophysiology Testing/Pacing Issues in Ebstein’s Anomaly…e805 13.7.2. Recommendations for Surgical Interventions…e806 13.7.3. Postoperative Findings…e806 13.7.4. Expected Postoperative Course…e806 13.8. Problems and Pitfalls…e806 13.9. Recommendation for Reproduction…e807 13.10. Recommendation for Endocarditis Prophylaxis…e80714. Tricuspid Atresia/Single Ventricle…e807 14.1. Definition…e807 14.2. Clinical Course (Unoperated and Palliated)…e807 14.3. Clinical Features and Evaluation of the Unoperated or Palliated Patient…e807 14.3.1. Presentation…e807 14.3.2. Clinical Examination…e807 14.3.3. Electrocardiogram…e808 14.3.4. Chest X-Ray…e808 14.3.5. Echocardiography…e808 14.3.6. Magnetic Resonance Imaging/Computed Tomography…e808 14.3.7. Recommendation for Catheterization Before Fontan Procedure…e808 14.4. Recommendation for Surgical Options for Patients With Single Ventricle…e808 14.5. Recommendation for Evaluation and Follow-Up After Fontan Procedure…e810 14.6. Clinical Features and Evaluation…e810 14.6.1. Clinical Examination…e810 14.6.2. Electrocardiogram…e810 14.6.3. Chest X-Ray…e810 14.6.4. Recommendation for Imaging…e810 14.7. Recommendation for Diagnostic and Interventional Catheterization After Fontan Procedure…e810 14.7.1. Evaluation of Patients With Protein-Losing Enteropathy…e810 14.7.2. Problems and Pitfalls…e811 14.8. Recommendations for Management Strategies for the Patient With Prior Fontan Repair…e811 14.8.1. Recommendations for Medical Therapy…e811 14.9. Recommendations for Surgery for Adults With Prior Fontan Repair…e811 14.10. Key Issues to Evaluate and Follow-Up…e812 14.10.1. Recommendations for Electrophysiology Testing/Pacing Issues in Single-Ventricle Physiology and After Fontan Procedure…e812 14.10.2. Other Issues to Evaluate and Follow-Up…e813 14.10.3. Recommendations for Endocarditis Prophylaxis…e813 14.10.4. Activity…e814 14.10.5. Recommendations for Reproduction…e814Appendix 1. Author Relationships With Industry and Other Entities…e814Appendix 2. Peer Reviewer Relationships With Industry and Other Entities…e815Appendix 3. Abbreviations List…e816Appendix 4. Definitions of Surgical Procedures for the Management of Adults With CHD…e817References…e820PreambleIt 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
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