ACC/AHA TASK FORCE MEMBERS Glenn N. Levine, MD, FACC, FAHA, Chair; Patrick T. O Gara, MD, MACC, FAHA, Chair-Elect; Jonathan L. Halperin, MD, FACC, FAHA, Immediate Past Chair; Nancy M. Albert, PhD, RN, FAHA*; Sana M. Al-Khatib, MD, MHS, FACC, FAHA; Joshua A. Beckman, MD, MS, FAHA; Kim K. Birtcher, PharmD, MS, AACC; Biykem Bozkurt, MD, PhD, FACC, FAHA*; Ralph G. Brindis, MD, MPH, MACC*; Joaquin E. Cigarroa, MD, FACC; Lesley H. Curtis, PhD, FAHA*; Anita Deswal, MD, MPH, FACC, FAHA; Lee A. Fleisher, MD, FACC, FAHA; Federico Gentile, MD, FACC; Samuel S. Gidding, MD, FAHA*; Zachary D. Goldberger, MD, MS, FACC, FAHA; Mark A. Hlatky, MD, FACC; John Ikonomidis, MD, PhD, FAHA; Jos Joglar, MD, FACC, FAHA; Richard J. Kovacs, MD, FACC, FAHA*; Laura Mauri, MD, MSc, FAHA; E. Magnus Ohman, MD, FACC*; Mariann R. Piano, RN, PhD, FAHA, FAAN; Susan J. Pressler, PhD, RN, FAHA*; Barbara Riegel, PhD, RN, FAHA; Frank W. Sellke, MD, FACC, FAHA*; Win-Kuang Shen, MD, FACC, FAHA*; Duminda N. Wijeysundera, MD, PhD PRESIDENTS AND STAFF American College of Cardiology C. Michael Valentine, MD, FACC, President Cathleen C. Gates, Interim Chief Executive Officer and Chief Operating Officer William J. Oetgen, MD, MBA, FACC, Executive Vice Presi- dent, Science, Education, Quality, and Publishing MaryAnne Elma, MPH, Senior Director, Science, Educa- tion, Quality, and Publishing Amelia Scholtz, PhD, Publications Manager, Science, Education, Quality, and Publishing American College of Cardiology/ American Heart Association Katherine A. Sheehan, PhD, Director, Guideline Strategy and Operations Abdul R. Abdullah, MD, Senior Manager, Guideline Science American Heart Association Ivor Benjamin, MD, FAHA, President Nancy Brown, Chief Executive Officer Rose Marie Robertson, MD, FAHA, Chief Science and Medical Officer Gayle R. Whitman, PhD, RN, FAHA, FAAN, Senior Vice President, Office of Science Operations Prashant Nedungadi, PhD, Science and Medicine Advi- sor, Office of Science Operations Jody Hundley, Production and Operations Manager, Sci- entific Publications, Office of Science Operations ARTICLE INFORMATION This document was approved by the American College of Cardiology Clinical Policy Approval Committee in May 2018, the American Heart Association Sci- ence Advisory and Coordinating Committee in June 2018, and the American Heart Association Executive Committee in July 2018. The Comprehensive RWI Data Supplement table is available with this article at https://www.ahajournals.org/doi/suppl/10.1161/CIR.0000000000000603. The online Data Supplement is available with this article at https://www. ahajournals.org/doi/suppl/10.1161/CIR.0000000000000603. This article has been copublished in the Journal of the American College of Cardiology. Copies: This document is available on the websites of the American College of Cardiology (www.acc.org) and the American Heart Association (professional. heart.org). A copy of the document is also available at https://professional.heart. org/statements by selecting the Guidelines & Statements button. To purchase additional reprints, call 843-216-2533 or e-mail kelle.ramsay@wolterskluwer.com. The expert peer review of AHA-commissioned documents (eg, scientific statements, clinical practice guidelines, systematic reviews) is conducted by the AHA Office of Science Operations. For more on AHA statements and guide- lines development, visit https://professional.heart.org/statements. Select the Guidelines & Statements drop-down menu near the top of the webpage, then click Publication Development. Permissions: Multiple copies, modification, alteration, enhancement, and/ or distribution of this document are not permitted without the express permis- sion of the American Heart Association. Instructions for obtaining permission are located at https://www.heart.org/permissions. A link to the Copyright Per- missions Request Form appears in the second paragraph (https://www.heart. org/en/about-us/statements-and-policies/copyright-request-form). * Former Task Force member; current member during the writing effort. CLINICAL STATEMENTS AND GUIDELINES REFERENCES PREAMBLE P-1. Committee on Standards for Developing Trustworthy Clinical Practice Guidelines, Institute of Medicine (US). Clinical Practice Guidelines We Can Trust. Washington, DC: The National Academies Press, 2011. P-2. Committee on Standards for Systematic Reviews of Comparative Effectiveness Research, Institute of Medicine (US). Finding What Works in Health Care: Standards for Systematic Reviews. Washington, DC: The National Academies Press, 2011. P-3. Anderson JL, Heidenreich PA, Barnett PG, et al. ACC/AHA statement on cost/value methodology in clinical practice guidelines and perfor- mance measures: a report of the American College of Cardiology/ American Heart Association Task Force on Performance Measures and Task Force on Practice Guidelines. Circulation. 2014;129:2329 45. P-4. ACCF/AHA Task Force on Practice Guidelines. Methodology Manual and Policies From the ACCF/AHA Task Force on Practice Guidelines. American College of Cardiology and American Heart Association, 2010. Available at: http://assets.cardiosource.com/Methodology_ Manual_for_ACC_AHA_Writing_Committees.pdf and http:// professional.heart.org/idc/groups/ahamah-public/@wcm/@sop/doc- 2017. P-5. Halperin JL, Levine GN, Al-Khatib SM, et al. Further evolution of the ACC/AHA clinical practice guideline recommendation classification system: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2016;133:1426 8. P-6. Jacobs AK, Kushner FG, Ettinger SM, et al. ACCF/AHA clinical prac- tice guideline methodology summit report: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation. 2013;127:268 310. P-7. Jacobs AK, Anderson JL, Halperin JL, et al. The evolution and future of ACC/AHA clinical practice guidelines: a 30-year journey: a report of the American College of Cardiology/American Heart Association Task Force on practice guidelines. Circulation. 2014;130:1208 17. P-8. Arnett DK, Goodman RA, Halperin JL, et al. AHA/ACC/HHS strat- egies to enhance application of clinical practice guidelines in patients with cardiovascular disease and comorbid conditions: from the American Heart Association, American College of Cardiology, and US Department of Health and Human Services. Circulation. 2014;130:1662 7. 1.1. Methodology and Evidence Review S1.1-1. Zaragoza-Macias E, Zaidi A, Dendukuri N, et al. Medical therapy for systemic right ventricles: a systematic review (part 1) for the 2018 AHA/ACC guideline for the management of adults with congen- ital heart disease: a report of the American College of Cardiology/ American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019;139:e000 000. S1.1-2. Oster M, Bhatt A, Zaragoza-Macias E, et al. Interventional therapy versus medical therapy for secundum atrial septal defect: a systematic review (part 2) for the 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019;139:e000 000. 1.4. Scope of the Guideline S1.4-1. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guide- lines 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). Circulation. 2008;118:e714 833. S1.4-2. Nishimura RA, Otto CM, Bonow RO, et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation. 2014;129:e521 643. S1.4-3. Moons P, Bovijn L, Budts W, et al. Temporal trends in survival to adulthood among patients born with congenital heart disease from 1970 to 1992 in Belgium. Circulation. 2010;122:2264 72. S1.4-4. Gilboa SM, Salemi JL, Nembhard WN, et al. Mortality resulting from congenital heart disease among children and adults in the United States, 1999 to 2006 Circulation. 2010;122:2254 63. S1.4-5. van der Bom T, Bouma BJ, Meijboom FJ, et al. The prevalence of adult congenital heart disease, results from a systematic review and evidence based calculation. Am Heart J. 2012;164:568 75. S1.4-6. Hoffman JIE, Kaplan S, Liberthson RR. Prevalence of congenital heart disease. Am Heart J. 2004;147:425 39. S1.4-7. Lien WP, Chen JJ, Chen JH, et al. Frequency of various congenital heart diseases in Chinese adults: analysis of 926 consecutive patients over 13 years of age. Am J Cardiol. 1986;57:840 4. S1.4-8. Sam nek M, Slav k Z, Zborilov , B, et al. Prevalence, treatment, and outcome of heart disease in live-born children: a prospective analysis of 91,823 live-born children. Pediatr Cardiol. 1989;10:205 11. S1.4-9. Mylotte D, Pilote L, Ionescu-Ittu R, et al. Specialized adult congenital heart disease care: the impact of policy on mortality. Circulation. 2014;129:1804 12. S1.4-10. Shen W-K, Sheldon RS, Benditt DG, et al. 2017 ACC/AHA/HRS guideline for the evaluation and management of patients with syn- cope: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2017;136:e60 122. S1.4-11. Page RL, Joglar JA, Caldwell MA, et al. 2015 ACC/AHA/HRS guideline for the management of adult patients with supraventricular tach- ycardia: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2016;133:e506 74. S1.4-12. Callaway CW, Donnino MW, Fink EL, et al. Part 8: post-cardiac arrest care: 2015 American Heart Association guidelines update for car- diopulmonary resuscitation and emergency cardiovascular care. Circulation. 2015;132:S465 82. S1.4-13. Amsterdam EA, Wenger NK, Brindis RG, et al. 2014 AHA/ACC guideline for the management of patients with non-ST-elevation acute coronary syndromes: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation. 2014;130:e344 426. S1.4-14. Fleisher LA, Fleischmann KE, Auerbach AD, et al. 2014 ACC/AHA guideline on perioperative cardiovascular evaluation and manage- ment of patients undergoing noncardiac surgery: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation. 2014;130:e278 333. S1.4-15. January CT, Wann LS, Alpert JS, et al. 2014 AHA/ACC/HRS guide- line for the management of patients with atrial fibrillation: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines and the Heart Rhythm Society. Circulation. 2014;130:e199 267. S1.4-16. Fihn SD, Blankenship JC, Alexander KP, et al. 2014 ACC/AHA/AATS/ PCNA/SCAI/STS focused update of the guideline for the diagnosis and management of patients with stable ischemic heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines, and the American Association for Thoracic Surgery, Preventive Cardiovascular Nurses Association, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation. 2014;130:1749 67. S1.4-17. Fihn SD, Gardin JM, Abrams J, et al. 2012 ACCF/AHA/ACP/AATS/ PCNA/SCAI/STS guideline for the diagnosis and management of patients with stable ischemic heart disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, and the American College of Physicians, American Association for Thoracic Surgery, Preventive Cardiovascular Nurses Association, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation. 2012;126:e354 471. S1.4-18. Goff DC Jr, Lloyd-Jones DM, Bennett G, et al. 2013 ACC/AHA guideline on the assessment of cardiovascular risk: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation. 2014;129:S49 73. S1.4-19. Stone NJ, Robinson JG, Lichtenstein AH, et al. 2013 ACC/AHA guide- line on the treatment of blood cholesterol to reduce atherosclerotic CLINICAL STATEMENTS AND GUIDELINES cardiovascular risk in adults: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation. 2014;129:S1 45. S1.4-20. Jensen MD, Ryan DH, Apovian CM, et al. 2013 AHA/ACC/TOS guideline for the management of overweight and obesity in adults: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines and The Obesity Society. Circulation. 2014;129:S102 38. S1.4-21. Eckel RH, Jakicic JM, Ard JD, et al. 2013 AHA/ACC guideline on lifestyle management to reduce cardiovascular risk: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation. 2014;129:S76 99. S1.4-22. Nishimura RA, Otto CM, Bonow RO, et al. 2017 AHA/ACC focused update of the 2014 AHA/ACC guideline for the man- agement of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2017;135: e1159 95. S1.4-23. Whelton PK, Carey RM, Aronow WS, et al. 2017 ACC/AHA/AAPA/ ABC/ACPM/AGS/APhA/ASH/ASPC/NMA/PCNA guideline for the prevention, detection, evaluation, and management of high blood pressure in adults: a report of the American College of Cardiology/ American Heart Association Task Force on Clinical Practice Guidelines. Hypertension. 2018;71:e13 115. S1.4-24. Svensson LG, Adams DH, Bonow RO, et al. Aortic valve and as- cending aorta guidelines for management and quality measures. Ann Thorac Surg. 2013;95:S1 66. S1.4-25. O Gara PT, Kushner FG, Ascheim DD, et al. 2013 ACCF/AHA guide- line for the management of ST-elevation myocardial infarction: a re- port of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation. 2013;127:e362 425. S1.4-26. Yancy CW, Jessup M, Bozkurt B, et al. 2017 ACC/AHA/HFSA fo- cused update of the 2013 ACCF/AHA guideline for the management of heart failure: a report of the American College of Cardiology/ American Heart Association Task Force on Clinical Practice Guidelines and the Heart Failure Society of America. Circulation. 2017;136:e137 61. S1.4-27. Tracy CM, Epstein AE, Darbar D, et al. 2012 ACCF/AHA/HRS fo- cused update of the 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines and the Heart Rhythm Society. Circulation. 2012;126:1784 800. S1.4-28. Hillis LD, Smith PK, Anderson JL, et al. 2011 ACCF/AHA guideline for coronary artery bypass graft surgery: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation. 2011;124:e652 735. S1.4-29. Levine GN, Bates ER, Blankenship JC, et al. 2011 ACCF/AHA/SCAI guideline for percutaneous coronary intervention: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines and the Society for Cardiovascular Angiography and Interventions. Circulation. 2011;124:e574 651. S1.4-30. Smith SC Jr, Benjamin EJ, Bonow RO, et al. AHA/ACCF sec- ondary prevention and risk reduction therapy for patients with coronary and other atherosclerotic vascular disease: 2011 up- date: a guideline from the American Heart Association and American College of Cardiology Foundation. Circulation. 2011;124: 2458 73. S1.4-31. Mosca L, Benjamin EJ, Berra K, et al. Effectiveness-based guidelines for the prevention of cardiovascular disease in women 2011 up- date: a guideline from the American Heart Association. Circulation. 2011;123:1243 62. S1.4-32. Baumgartner H, Bonhoeffer P, De Groot NMS, et al. ESC guidelines for the management of grown-up congenital heart disease (new ver- sion 2010) Eur Heart J. 2010;31:2915 57. S1.4-33. Hiratzka LF, Bakris GL, Beckman JA, et al. 2010 ACCF/AHA/AATS/ ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation. 2010;121:e266 369. S1.4-34. Silversides CK, Marelli A, Beauchesne L, et al. Canadian Cardiovascular Society 2009 consensus conference on the manage- ment of adults with congenital heart disease: executive summary. Can J Cardiol. 2010;26:143 50. S1.4-35. Habib G, Hoen B, Tornos P, et al. Guidelines on the prevention, di- agnosis, and treatment of infective endocarditis (new version 2009): the Task Force on the Prevention, Diagnosis, and Treatment of Infective Endocarditis of the European Society of Cardiology (ESC) Eur Heart J. 2009;30:2369 413. S1.4-36. Cohen MS, Eidem BW, Cetta F, et al. Multimodality imaging guide- lines of patients with transposition of the great arteries: a report from the American Society of Echocardiography. Developed in col- laboration with the Society for Cardiovascular Magnetic Resonance and the Society of Cardiovascular Computed Tomography. J Am Soc Echocardiogr. 2016;29:571 621. S1.4-37. Lang RM, Badano LP, Mor-Avi V, et al. Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr. 2015;28:1 39.e14. S1.4-38. Khairy P, Van Hare GF, Balaji S, et al. PACES/HRS expert consensus statement on the recognition and management of arrhythmias in adult congenital heart disease. Developed in partnership be- tween the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS) Heart Rhythm. 2014;11:e102 65. S1.4-39. Valente AM, Cook S, Festa P, et al. Multimodality imaging guide- lines for patients with repaired tetralogy of Fallot: a report from the American Society of Echocardiography. Developed in collab- oration with the Society for Cardiovascular Magnetic Resonance and the Society for Pediatric Radiology. J Am Soc Echocardiogr. 2014;27:111 41. S1.4-40. Boodhwani M, Andelfinger G, Leipsic J, et al. Canadian Cardiovascular Society position statement on the management of thoracic aortic disease. Can J Cardiol. 2014;30:577 89. S1.4-41. Longmuir PE, Brothers JA, de Ferranti SD, et al. Promotion of physical activity for children and adults with congenital heart disease: a sci- entific statement from the American Heart Association. Circulation. 2013;127:2147 59. S1.4-42. Marino BS, Lipkin PH, Newburger JW, et al. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation. 2012;126:1143 72. S1.4-43. European Society of Gynecology (ESG), Association for European Paediatric Cardiology (AEPC), German Society for Gender Medicine (DGesGM), et al. ESC guidelines on the management of car- diovascular diseases during pregnancy: the Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC) Eur Heart J. 2011;32: 3147 97. S1.4-44. Sable C, Foster E, Uzark K, et al. Best practices in managing tran- sition to adulthood for adolescents with congenital heart disease: the transition process and medical and psychosocial issues: a sci- entific statement from the American Heart Association. Circulation. 2011;123:1454 85. S1.4-45. McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a re- port of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association. Developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association. Circulation. 2009;119: 2250 94. S1.4-46. Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endo- carditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. 2007;116:1736 54. CLINICAL STATEMENTS AND GUIDELINES 2. BACKGROUND AND PATHOPHYSIOLOGY 2.1. Anatomic and Physiological Terms S2.1-1. Giroud JM, Jacobs JP, Spicer D, et al. Report from the International Society for Nomenclature of Paediatric and Congenital Heart Disease: creation of a visual encyclopedia illustrating the terms and definitions of the international pediatric and congenital cardiac code. World J Pediatr Congenit Heart Surg. 2010;1:300 13. S2.1-2. Bergersen L, Giroud JM, Jacobs JP, et al. Report from The International Society for Nomenclature of Paediatric and Congenital Heart Disease: cardiovascular catheterisation for congenital and paediatric cardiac disease (Part 2 - Nomenclature of complications associated with interventional cardiology) Cardiol Young. 2011;21: 260 5. S2.1-3. Bergersen L, Everett AD, Giroud JM, et al. Report from the International Society for Nomenclature of Paediatric and Congenital Heart Disease: cardiovascular catheterisation for congenital and paediatric cardiac disease (Part 1 - Procedural nomenclature) Cardiol Young. 2011;21:252 9. S2.1-4. Franklin RCG, Jacobs JP, Krogmann ON, et al. Nomenclature for con- genital and paediatric cardiac disease: historical perspectives and The International Pediatric and Congenital Cardiac Code. Cardiol Young. 2008;18 suppl 2:70 80. S2.1-5. Franklin RCG, B land MJ, Krogmann ON. Mapping and coding of nomenclatures for paediatric and congenital heart disease. Cardiol Young. 2006;16:105 6. S2.1-6. International Society for Nomenclature of Paediatric and Congenital Heart Disease. International Paediatric and Congenital Cardiac Code. Available at: http://ipccc.net/. Accessed: May 29, 2018. 2.2. Severity of ACHD S2.2-1. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guide- lines 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) Circulation. 2008;118:e714 833. S2.2-2. Nishimura RA, Otto CM, Bonow RO, et al. 2014 AHA/ACC guideline for the management of patients with valvular heart di- sease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation. 2014;129:e521 643. S2.2-3. Boodhwani M, Andelfinger G, Leipsic J, et al. Canadian Cardiovascular Society position statement on the management of thoracic aortic disease. Can J Cardiol. 2014;30:577 89. S2.2-4. Svensson LG, Adams DH, Bonow RO, et al. Aortic valve and as- cending aorta guidelines for management and quality measures. Ann Thorac Surg. 2013;95:S1 66. S2.2-5. Verheugt CL, Uiterwaal CSPM, van der Velde ET, et al. Mortality in adult congenital heart disease. Eur Heart J. 2010;31:1220 9. S2.2-6. Khairy P, Aboulhosn J, Gurvitz MZ, et al. Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study. Circulation. 2010;122:868 75. S2.2-7. Khairy P, Fernandes SM, Mayer JE Jr, et al. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation. 2008;117:85 92. S2.2-8. Khairy P, Harris L, Landzberg MJ, et al. Implantable cardioverter-defi- brillators in tetralogy of Fallot. Circulation. 2008;117:363 70. S2.2-9. Dimopoulos K, Diller G-P, Koltsida E, et al. Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease. Circulation. 2008;117:2320 8. S2.2-10. Dimopoulos K, Diller G-P, Giannakoulas G, et al. Anemia in adults with congenital heart disease relates to adverse outcome. J Am Coll Cardiol. 2009;54:2093 100. S2.2-11. Dimopoulos K, Diller G-P, Petraco R, et al. Hyponatraemia: a strong predictor of mortality in adults with congenital heart disease. Eur Heart J. 2010;31:595 601. S2.2-12. Diller G-P, Dimopoulos K, Okonko D, et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation. 2005;112:828 35. S2.2-13. Buys R, Cornelissen V, Van De Bruaene A, et al. Measures of exer- cise capacity in adults with congenital heart disease. Int J Cardiol. 2011;153:26 30. S2.2-14. Gratz A, Hess J, Hager A. Self-estimated physical functioning poorly predicts actual exercise capacity in adolescents and adults with con- genital heart disease. Eur Heart J. 2009;30:497 504. S2.2-15. Dimopoulos K, Okonko DO, Diller G-P, et al. Abnormal ventilatory response to exercise in adults with congenital heart disease relates to cyanosis and predicts survival. Circulation. 2006;113:2796 802. S2.2-16. Inuzuka R, Diller G-P, Borgia F, et al. Comprehensive use of cardi- opulmonary exercise testing identifies adults with congenital heart disease at increased mortality risk in the medium term. Circulation. 2012;125:250 9. S2.2-17. M ller J, Hager A, Diller G-P, et al. Peak oxygen uptake, venti- latory efficiency and QRS-duration predict event free survival in patients late after surgical repair of tetralogy of Fallot. Int J Cardiol. 2015;196:158 64. S2.2-18. Kempny A, Dimopoulos K, Uebing A, et al. Reference values for exer- cise limitations among adults with congenital heart disease. Relation to activities of daily life single centre experience and review of pub- lished data. Eur Heart J. 2012;33:1386 96. S2.2-19. New York Heart Association. Dolgin M. Nomenclature and Criteria for Diagnosis of Diseases of the Heart and Great Vessels. 9th ed. Boston, MA: Little, Brown and Co, 1994. S2.2-20. Hoeper MM, Bogaard HJ, Condliffe R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62:D42 50. 2.3. The ACHD AP Classification S2.3-1. Verheugt CL, Uiterwaal CSPM, van der Velde ET, et al. Mortality in adult congenital heart disease. Eur Heart J. 2010;31:1220 9. S2.3-2. Diller G-P, Dimopoulos K, Okonko D, et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation. 2005;112:828 35. S2.3-3. Dimopoulos K, Diller G-P, Koltsida E, et al. Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease. Circulation. 2008;117:2320 8. S2.3-4. Dimopoulos K, Okonko DO, Diller G-P, et al. Abnormal venti- latory response to exercise in adults with congenital heart di- sease relates to cyanosis and predicts survival. Circulation. 2006;113:2796 802. S2.3-5. Hebson CL, McCabe NM, Elder RW, et al. Hemodynamic pheno- type of the failing Fontan in an adult population. Am J Cardiol. 2013;112:1943 7. S2.3-6. Khairy P, Aboulhosn J, Gurvitz MZ, et al. Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study. Circulation. 2010;122:868 75. S2.3-7. Khairy P, Harris L, Landzberg MJ, et al. Sudden death and defibrilla- tors in transposition of the great arteries with intra-atrial baffles: a multicenter study. Circ Arrhythm Electrophysiol. 2008;1:250 7. S2.3-8. Inuzuka R, Diller G-P, Borgia F, et al. Comprehensive use of cardi- opulmonary exercise testing identifies adults with congenital heart disease at increased mortality risk in the medium term. Circulation. 2012;125:250 9. S2.3-9. M ller J, Hager A, Diller G-P, et al. Peak oxygen uptake, venti- latory efficiency and QRS-duration predict event free survival in patients late after surgical repair of tetralogy of Fallot. Int J Cardiol. 2015;196:158 64. S2.3-10. Stefanescu A, Macklin EA, Lin E, et al. Usefulness of the Seattle Heart Failure Model to identify adults with congenital heart disease at high risk of poor outcome. Am J Cardiol. 2014;113:865 70. S2.3-11. Giannakoulas G, Dimopoulos K, Engel R, et al. Burden of coronary artery disease in adults with congenital heart disease and its rela- tion to congenital and traditional heart risk factors. Am J Cardiol. 2009;103:1445 50. S2.3-12. Giannakoulas G, Dimopoulos K, Bolger AP, et al. Usefulness of na- triuretic peptide levels to predict mortality in adults with congenital heart disease. Am J Cardiol. 2010;105:869 73. S2.3-13. Khairy P, Fernandes SM, Mayer JE Jr, et al. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation. 2008;117:85 92. S2.3-14. Khairy P, Harris L, Landzberg MJ, et al. Implantable cardioverter-defi- brillators in tetralogy of Fallot. Circulation. 2008;117:363 70. CLINICAL STATEMENTS AND GUIDELINES S2.3-15. Dimopoulos K, Diller G-P, Giannakoulas G, et al. Anemia in adults with congenital heart disease relates to adverse outcome. J Am Coll Cardiol. 2009;54:2093 100. S2.3-16. Dimopoulos K, Diller G-P, Petraco R, et al. Hyponatraemia: a strong predictor of mortality in adults with congenital heart disease. Eur Heart J. 2010;31:595 601. S2.3-17. Yancy CW, Jessup M, Bozkurt B, et al. 2013 ACCF/AHA guideline for the management of heart failure: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation. 2013;128:e240 327. 3. GENERAL PRINCIPLES 3.1. ACHD Program S3.1-1. Mylotte D, Pilote L, Ionescu-Ittu R, et al. Specialized adult congenital heart disease care: the impact of policy on mortality. Circulation. 2014;129:1804 12. 3.2. Access to Care S3.2-1. Gurvitz M, Valente AM, Broberg C, et al. Prevalence and predic- tors of gaps in care among adult congenital heart disease patients: HEART-ACHD (The Health, Education, and Access Research Trial). J Am Coll Cardiol. 2013;61:2180 4. S3.2-2. Mackie AS, Islam S, Magill-Evans J, et al. Healthcare transition for youth with heart disease: a clinical trial. Heart. 2014;100:1113 8. S3.2-3. Yeung E, Kay J, Roosevelt GE, et al. Lapse of care as a predictor for morbidity in adults with congenital heart disease. Int J Cardiol. 2008;125:62 5. S3.2-4. Wacker A, Kaemmerer H, Hollweck R, et al. Outcome of operated and unoperated adults with congenital cardiac disease lost to fol- low-up for more than five years. Am J Cardiol. 2005;95:776 9. S3.2-5. Mylotte D, Pilote L, Ionescu-Ittu R, et al. Specialized adult congenital heart disease care: the impact of policy on mortality. Circulation. 2014;129:1804 12. 3.3. Delivery of Care S3.3-1. Mylotte D, Pilote L, Ionescu-Ittu R, et al. Specialized adult congenital heart disease care: the impact of policy on mortality. Circulation. 2014;129:1804 12. S3.3-2. Karamlou T, Diggs BS, Person T, et al. National practice patterns for management of adult congenital heart disease: operation by pediatric heart surgeons decreases in-hospital death. Circulation. 2008;118:2345 52. S3.3-3. Hijazi ZM, Ruiz CE, Zahn E, et al. SCAI/AATS/ACC/STS operator and institutional requirements for transcatheter valve repair and replace- ment, part III: pulmonic valve. J Am Coll Cardiol. 2015;65:2556 63. S3.3-4. Ruiz CE, Feldman TE, Hijazi ZM, et al. Interventional fellowship in structural and congenital heart disease for adults. JACC Cardiovasc Interv. 2010;3:e1 5. S3.3-5. Armsby L, Beekman RHr, Benson L, et al. SCAI expert consensus statement for advanced training programs in pediatric and con- genital interventional cardiac catheterization. Catheter Cardiovasc Interv. 2014;84:779 84. S3.3-6. Mascio CE, Pasquali SK, Jacobs JP, et al. Outcomes in adult congen- ital heart surgery: analysis of the Society of Thoracic Surgeons data- base. J Thorac Cardiovasc Surg. 2011;142:1090 7. S3.3-7. H rer J, Vogt M, Wottke M, et al. Evaluation of the Aristotle com- plexity models in adult patients with congenital heart disease. Eur J Cardiothorac Surg. 2013;43:128 34. S3.3-8. van Gameren M, Putman LM, Takkenberg JJM, et al. Risk stratifi- cation for adult congenital heart surgery. Eur J Cardiothorac Surg. 2011;39:490 4. S3.3-9. Kogon B, Oster M. Assessing surgical risk for adults with congenital heart disease: are pediatric scoring systems appropriate? J Thorac Cardiovasc Surg. 2014;147:666 71. S3.3-10. Gajjar TP, Hiremath CS, Desai NB. Surgical closure of sinus venosus atrial septal defect using a single patch transcaval repair technique. J Card Surg. 2011;26:429 34. S3.3-11. Videb k J, Laursen HB, Olsen M, et al. Long-term nationwide fol- low-up study of simple congenital heart disease diagnosed in other- wise healthy children. Circulation. 2016;133:474 83. S3.3-12. Karonis T, Scognamiglio G, Babu-Narayan SV, et al. Clinical course and potential complications of small ventricular septal defects in adulthood: late development of left ventricular dysfunction justifies lifelong care. Int J Cardiol. 2016;208:102 6. S3.3-13. Bonello B, Kilner PJ. Review of the role of cardiovascular magnetic resonance in congenital heart disease, with a focus on right ventricle assessment. Arch Cardiovasc Dis. 2012;105:605 13. S3.3-14. Kilner PJ. Imaging congenital heart disease in adults. Br J Radiol. 2011;84 Spec No 3:S258 68. S3.3-15. Cohen MS, Eidem BW, Cetta F, et al. Multimodality imaging guide- lines of patients with transposition of the great arteries: a report from the American Society of Echocardiography. Developed in col- laboration with the Society for Cardiovascular Magnetic Resonance and the Society of Cardiovascular Computed Tomography. J Am Soc Echocardiogr. 2016;29:571 621. S3.3-16. Valente AM, Cook S, Festa P, et al. Multimodality imaging guide- lines for patients with repaired tetralogy of Fallot: a report from the American Society of Echocardiography. Developed in collab- oration with the Society for Cardiovascular Magnetic Resonance and the Society for Pediatric Radiology. J Am Soc Echocardiogr. 2014;27:111 41. S3.3-17. Ginde S, Bartz PJ, Hill GD, et al. Restrictive lung disease is an inde- pendent predictor of exercise intolerance in the adult with congen- ital heart disease. Congenit Heart Dis. 2013;8:246 54. S3.3-18. Alonso-Gonzalez R, Borgia F, Diller G-P, et al. Abnormal lung func- tion in adults with congenital heart disease: prevalence, relation to cardiac anatomy, and association with survival. Circulation. 2013;127:882 90. S3.3-19. Zach KJ, Ramakrishna H, Chandrasekaran K, et al. Laparoscopic colectomy in an adult with single ventricle physiology: anesthetic implications and management. Ann Card Anaesth. 2015;18: 252 6. S3.3-20. Duffels MGJ, Engelfriet PM, Berger RMF, et al. Pulmonary arterial hy- pertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol. 2007;120:198 204. S3.3-21. Lowe BS, Therrien J, Ionescu-Ittu R, et al. Diagnosis of pulmonary hypertension in the congenital heart disease adult population impact on outcomes. J Am Coll Cardiol. 2011;58:538 46. S3.3-22. Van De Bruaene A, Delcroix M, Pasquet A, et al. The importance of pulmonary artery pressures on late atrial arrhythmia in trans- catheter and surgically closed ASD type secundum. Int J Cardiol. 2011;152:192 5. S3.3-23. Barst RJ, Ivy DD, Foreman AJ, et al. Four- and seven-year outcomes of patients with congenital heart disease-associated pulmonary arterial hypertension (from the REVEAL Registry). Am J Cardiol. 2014;113:147 55. S3.3-24. D'Alto M, Romeo E, Argiento P, et al. Therapy for pulmonary arterial hypertension due to congenital heart disease and Down s syndrome. Int J Cardiol. 2013;164:323 6. S3.3-25. Engelfriet PM, Duffels MGJ, M ller T, et al. Pulmonary arterial hy- pertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart disease. Heart. 2007;93: 682 7. S3.3-26. Manes A, Palazzini M, Leci E, et al. Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups. Eur Heart J. 2014;35:716 24. S3.3-27. O Donnell C, Ruygrok PN, Whyte K, et al. Progressive pulmonary hypertension post atrial septal defect device closure early symp- tomatic improvement may not predict outcome. Heart Lung Circ. 2010;19:713 6. S3.3-28. Van De Bruaene A, Moons P, Belmans A, et al. Predictive model for late atrial arrhythmia after closure of an atrial septal defect. Int J Cardiol. 2013;164:318 22. S3.3-29. D Alto M, Romeo E, Argiento P, et al. Hemodynamics of patients developing pulmonary arterial hypertension after shunt closure. Int J Cardiol. 2013;168:3797 801. S3.3-30. Steele PM, Fuster V, Cohen M, et al. Isolated atrial septal defect with pulmonary vascular obstructive disease long-term follow-up and prediction of outcome after surgical correction. Circulation. 1987;76:1037 42. CLINICAL STATEMENTS AND GUIDELINES 3.4. Evaluation of Suspected and Known CHD S3.4.1-1. Graham TP Jr, Bernard YD, Mellen BG, et al. Long-term outcome in congenitally corrected transposition of the great arteries: a multi- institutional study. J Am Coll Cardiol. 2000;36:255 61. S3.4.1-2. Connelly MS, Liu PP, Williams WG, et al. Congenitally corrected transposition of the great arteries in the adult: functional status and complications. J Am Coll Cardiol. 1996;27:1238 43. S3.4.1-3. Bergin ML, Warnes CA, Tajik AJ, et al. Partial atrioventricular canal defect: long-term follow-up after initial repair in patients > or = 40 years old. J Am Coll Cardiol. 1995;25:1189 94. S3.4.1-4. Borkon AM, Pieroni DR, Varghese PJ, et al. The superior QRS axis in ostium primum ASD: a proposed mechanism. Am Heart J. 1975;90:215 21. S3.4.1-5. Wei W, Zhan X, Xue Y, et al. Features of accessory pathways in adult Ebstein s anomaly. Europace. 2014;16:1619 25. S3.4.1-6. Iturralde P, Nava S, S lica G, et al. Electrocardiographic characteris- tics of patients with Ebstein s anomaly before and after ablation of an accessory atrioventricular pathway. J Cardiovasc Electrophysiol. 2006;17:1332 6. S3.4.1-7. Reich JD, Auld D, Hulse E, et al. The Pediatric Radiofrequency Ablation Registry s experience with Ebstein s anomaly. Pediatric Electrophysiology Society. J Cardiovasc Electrophysiol. 1998;9:1370 7. S3.4.1-8. Liberman L, Pass RH, Hordof AJ, et al. Late onset of heart block after open heart surgery for congenital heart disease. Pediatr Cardiol. 2008;29:56 9. 3.4.2. Ionizing Radiation Principles S3.4.2-1. Glatz AC, Purrington KS, Klinger A, et al. Cumulative exposure to medical radiation for children requiring surgery for congenital heart disease. J Pediatr. 2014;164:789 94.e10. S3.4.2-2. Johnson JN, Hornik CP, Li JS, et al. Cumulative radiation exposure and cancer risk estimation in children with heart disease. Circulation. 2014;130:161 7. S3.4.2-3. Yakoumakis E, Kostopoulou H, Makri T, et al. Estimation of radiation dose and risk to children undergoing cardiac catheterization for the treatment of a congenital heart disease using Monte Carlo simula- tions. Pediatr Radiol. 2013;43:339 46. S3.4.2-4. Andreassi MG, Ait-Ali L, Botto N, et al. Cardiac catheterization and long-term chromosomal damage in children with congenital heart disease. Eur Heart J. 2006;27:2703 8. S3.4.2-5. Brenner DJ, Doll R, Goodhead DT, et al. Cancer risks attributable to low doses of ionizing radiation: assessing what we really know. Proc Natl Acad Sci USA. 2003;100:13761 6. S3.4.2-6. Eisenberg MJ, Afilalo J, Lawler PR, et al. Cancer risk related to low- dose ionizing radiation from cardiac imaging in patients after acute myocardial infarction. CMAJ. 2011;183:430 6. S3.4.3-1. Randolph GR, Hagler DJ, Connolly HM, et al. Intraoperative trans- esophageal echocardiography during surgery for congenital heart defects. J Thorac Cardiovasc Surg. 2002;124:1176 82. 3.4.4. CMR Imaging S3.4.4-1. Puchalski MD, Williams RV, Askovich B, et al. Assessment of right ventricular size and function: echo versus magnetic resonance im- aging. Congenit Heart Dis. 2007;2:27 31. S3.4.4-2. Blalock SE, Banka P, Geva T, et al. Interstudy variability in cardiac magnetic resonance imaging measurements of ventricular volume, mass, and ejection fraction in repaired tetralogy of Fallot: a prospec- tive observational study. J Magn Reson Imaging. 2013;38:829 35. S3.4.4-3. Crean AM, Maredia N, Ballard G, et al. 3D Echo systematically underestimates right ventricular volumes compared to cardiovas- cular magnetic resonance in adult congenital heart disease patients with moderate or severe RV dilatation. J Cardiovasc Magn Reson. 2011;13:78. S3.4.4-4. Bonello B, Kilner PJ. Review of the role of cardiovascular magnetic resonance in congenital heart disease, with a focus on right ventricle assessment. Arch Cardiovasc Dis. 2012;105:605 13. S3.4.4-5. Kilner PJ. Imaging congenital heart disease in adults. Br J Radiol. 2011;84 Spec No. 3:S258 68. S3.4.4-6. Festa P, Ait-Ali L, Cerillo AG, et al. Magnetic resonance imaging is the diagnostic tool of choice in the preoperative evaluation of patients with partial anomalous pulmonary venous return. Int J Cardiovasc Imaging. 2006;22:685 93. S3.4.4-7. van der Linde D, Rossi A, Yap SC, et al. Ascending aortic diameters in congenital aortic stenosis: cardiac magnetic resonance versus trans- thoracic echocardiography. Echocardiography. 2013;30:497 504. S3.4.4-8. Arheden H, Holmqvist C, Thilen U, et al. Left-to-right cardiac shunts: comparison of measurements obtained with MR velocity mapping and with radionuclide angiography. Radiology. 1999;211:453 8. S3.4.4-9. Brenner DJ, Hall EJ. Computed tomography an increasing source of radiation exposure. N Engl J Med. 2007;357:2277 84. S3.4.4-10. Teo KSL, Disney PJ, Dundon BK, et al. Assessment of atrial septal defects in adults comparing cardiovascular magnetic resonance with transoesophageal echocardiography. J Cardiovasc Magn Reson. 2010;12:44. S3.4.4-11. Grewal J, Majdalany D, Syed I, et al. Three-dimensional echocardio- graphic assessment of right ventricular volume and function in adult patients with congenital heart disease: comparison with magnetic resonance imaging. J Am Soc Echocardiogr. 2010;23:127 33. S3.4.4-12. Kilner PJ, Geva T, Kaemmerer H, et al. Recommendations for car- diovascular magnetic resonance in adults with congenital heart di- sease from the respective working groups of the European Society of Cardiology. Eur Heart J. 2010;31:794 805. S3.4.4-13. Kilner PJ. The role of cardiovascular magnetic resonance in adults with congenital heart disease. Prog Cardiovasc Dis. 2011;54:295 304. S3.4.4-14. Boxt LM. Magnetic resonance and computed tomographic eval- uation of congenital heart disease. J Magn Reson Imaging. 2004;19:827 47. S3.4.4-15. van der Zwaan HB, Helbing WA, McGhie JS, et al. Clinical value of real-time three-dimensional echocardiography for right ven- tricular quantification in congenital heart disease: validation with cardiac magnetic resonance imaging. J Am Soc Echocardiogr. 2010;23:134 40. S3.4.4-16. Prasad SK, Soukias N, Hornung T, et al. Role of magnetic reso- nance angiography in the diagnosis of major aortopulmonary col- lateral arteries and partial anomalous pulmonary venous drainage. Circulation. 2004;109:207 14. 3.4.5. Cardiac Computed Tomography S3.4.5-1. Brenner DJ, Hall EJ. Computed tomography an increasing source of radiation exposure. N Engl J Med. 2007;357:2277 84. S3.4.5-2. Ghoshhajra BB, Sidhu MS, El-Sherief A, et al. Adult congenital heart disease imaging with second-generation dual-source computed tomography: initial experiences and findings. Congenit Heart Dis. 2012;7:516 25. 3.4.6. Cardiac Catheterization S3.4.6-1. Moore JW, Vincent RN, Beekman RH 3rd, et al. Procedural results and safety of common interventional procedures in congenital heart disease: initial report from the National Cardiovascular Data Registry. J Am Coll Cardiol. 2014;64:2439 51. S3.4.6-2. Learn CP, Holzer RJ, Daniels CJ, et al. Adverse events rates and risk factors in adults undergoing cardiac catheterization at pedi- atric hospitals results from the C3PO. Catheter Cardiovasc Interv. 2013;81:997 1005. S3.4.6-3. Sutton NJ, Greenberg MA, Menegus MA, et al. Caring for the adult with congenital heart disease in an adult catheterization laboratory by pediatric interventionalists safety and efficacy. Congenit Heart Dis. 2013;8:111 6. S3.4.6-4. Opotowsky AR, Landzberg MJ, Kimmel SE, et al. Percutaneous clo- sure of patent foramen ovale and atrial septal defect in adults: the impact of clinical variables and hospital procedure volume on in- hospital adverse events. Am Heart J. 2009;157:867 74. S3.4.6-5. Reant P, Brunot S, Lafitte S, et al. Predictive value of noninvasive coronary angiography with multidetector computed tomography to detect significant coronary stenosis before valve surgery. Am J Cardiol. 2006;97:1506 10. S3.4.6-6. Gilard M, Cornily J-C, Pennec P-Y, et al. Accuracy of multislice computed tomography in the preoperative assessment of coronary CLINICAL STATEMENTS AND GUIDELINES disease in patients with aortic valve stenosis. J Am Coll Cardiol. 2006;47:2020 4. S3.4.6-7. Manghat NE, Morgan-Hughes GJ, Broadley AJ, et al. 16-Detector row computed tomographic coronary angiography in patients undergoing evaluation for aortic valve replacement: comparison with catheter angiography. Clin Radiol. 2006;61:749 57. S3.4.6-8. Meijboom WB, Mollet NR, Van Mieghem CAG, et al. Pre-operative computed tomography coronary angiography to detect significant coronary artery disease in patients referred for cardiac valve surgery. J Am Coll Cardiol. 2006;48:1658 65. S3.4.6-9. Galas A, Hryniewiecki T, K pka C, et al. May dual-source computed tomography angiography replace invasive coronary angiography in the evaluation of patients referred for valvular disease surgery? Kardiol Pol. 2012;70:877 82. 3.4.7. Exercise Testing S3.4.7-1. Diller G-P, Dimopoulos K, Okonko D, et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation. 2005;112:828 35. S3.4.7-2. Hager A, Hess J. Comparison of health related quality of life with cardiopulmonary exercise testing in adolescents and adults with congenital heart disease. Heart. 2005;91:517 20. S3.4.7-3. Ross RM, Murthy JN, Wollak ID, et al. The six minute walk test ac- curately estimates mean peak oxygen uptake. BMC Pulm Med. 2010;10:31. S3.4.7-4. Gungor H, Fatih Ayik M, Engin C, et al. Transthoracic echocardi- ographic and cardiopulmonary exercise testing parameters in Eisenmenger s syndrome. Association with six-minute walk test dis- tance. Herz. 2014;39:633 7. 3.5. Transition Education S3.5-1. Mackie AS, Islam S, Magill-Evans J, et al. Healthcare transition for youth with heart disease: a clinical trial. Heart. 2014;100:1113 8. S3.5-2. Goossens E, Van Deyk K, Zupancic N, et al. Effectiveness of struc- tured patient education on the knowledge level of adolescents and adults with congenital heart disease. Eur J Cardiovasc Nurs. 2014;13:63 70. S3.5-3. Mackie AS, Rempel GR, Rankin KN, et al. Risk factors for loss to follow-up among children and young adults with congenital heart disease. Cardiol Young. 2012;22:307 15. S3.5-4. Sable C, Foster E, Uzark K, et al. Best practices in managing tran- sition to adulthood for adolescents with congenital heart disease: the transition process and medical and psychosocial issues: a scien- tific statement from the American Heart Association. Circulation. 2011;123:1454 85. S3.5-5. Gurvitz M, Valente AM, Broberg C, et al. Prevalence and predic- tors of gaps in care among adult congenital heart disease patients: HEART-ACHD (The Health, Education, and Access Research Trial). J Am Coll Cardiol. 2013;61:2180 4. S3.5-6. Yeung E, Kay J, Roosevelt GE, et al. Lapse of care as a predictor for morbidity in adults with congenital heart disease. Int J Cardiol. 2008;125:62 5. 3.6. Exercise and Sports S3.6-1. Pemberton VL, McCrindle BW, Barkin S, et al. Report of the National Heart, Lung, and Blood Institute s Working Group on obesity and other cardiovascular risk factors in congenital heart disease. Circulation. 2010;121:1153 9. S3.6-2. Myers J, Kaykha A, George S, et al. Fitness versus physical activity patterns in predicting mortality in men. Am J Med. 2004;117:912 8. S3.6-3. Warburton DER, Nicol CW, Bredin SSD. Health benefits of physical activity: the evidence. CMAJ. 2006;174:801 9. S3.6-4. King AC, Taylor CB, Haskell WL, et al. Influence of regular aerobic exercise on psychological health: a randomized, controlled trial of healthy middle-aged adults. Health Psychol. 1989;8:305 24. S3.6-5. Reybrouck T, Mertens L. Physical performance and physical activity in grown-up congenital heart disease. Eur J Cardiovasc Prev Rehabil. 2005;12:498 502. S3.6-6. Duppen N, Takken T, Hopman MTE, et al. Systematic review of the effects of physical exercise training programmes in children and young adults with congenital heart disease. Int J Cardiol. 2013;168:1779 87. S3.6-7. Dua JS, Cooper AR, Fox KR, et al. Exercise training in adults with congenital heart disease: feasibility and benefits. Int J Cardiol. 2010;138:196 205. S3.6-8. Holloway TM, Chesssex C, Grace SL, et al. A call for adult congen- ital heart disease patient participation in cardiac rehabilitation. Int J Cardiol. 2011;150:345 6. S3.6-9. Sandberg C, Pomeroy J, Thil n U, et al. Habitual physical activity in adults with congenital heart disease compared with age- and sex- matched controls. Can J Cardiol. 2016;32:547 53. S3.6-10. Diller G-P, Dimopoulos K, Okonko D, et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation. 2005;112:828 35. S3.6-11. Kempny A, Dimopoulos K, Uebing A, et al. Reference values for exercise limitations among adults with congenital heart disease. Relation to activities of daily life single centre experience and re- view of published data. Eur Heart J. 2012;33:1386 96. S3.6-12. Westhoff-Bleck M, Schieffer B, Tegtbur U, et al. Aerobic training in adults after atrial switch procedure for transposition of the great arteries improves exercise capacity without impairing systemic right ventricular function. Int J Cardiol. 2013;170:24 9. S3.6-13. Tikkanen AU, Opotowsky AR, Bhatt AB, et al. Physical activity is as- sociated with improved aerobic exercise capacity over time in adults with congenital heart disease. Int J Cardiol. 2013;168:4685 91. S3.6-14. Van Hare GF, Ackerman MJ, Evangelista JA, et al. Eligibility and dis- qualification recommendations for competitive athletes with car- diovascular abnormalities: Task Force 4: congenital heart disease: a scientific statement from the American Heart Association and American College of Cardiology. Circulation. 2015;132:e281 91. S3.6-15. Pelliccia A, Fagard R, Bj rnstad HH, et al. Recommendations for competitive sports participation in athletes with cardiovascular disease: a consensus document from the Study Group of Sports Cardiology of the Working Group of Cardiac Rehabilitation and Exercise Physiology and the Working Group of Myocardial and Pericardial Diseases of the European Society of Cardiology. Eur Heart J. 2005;26:1422 45. S3.6-16. Mitchell JH, Haskell W, Snell P, et al. Task Force 8: classification of sports. J Am Coll Cardiol. 2005;45:1364 7. S3.6-17. Takken T, Giardini A, Reybrouck T, et al. Recommendations for physical activity, recreation sport, and exercise training in pae- diatric patients with congenital heart disease: a report from the Exercise, Basic & Translational Research Section of the European Association of Cardiovascular Prevention and Rehabilitation, the European Congenital Heart and Lung Exercise Group, and the Association for European Paediatric Cardiology. Eur J Prev Cardiol. 2012;19:1034 65. S3.6-18. Cordina R, O Meagher S, Gould H, et al. Skeletal muscle abnormali- ties and exercise capacity in adults with a Fontan circulation. Heart. 2013;99:1530 4. S3.6-19. Cordina RL, O Meagher S, Karmali A, et al. Resistance train- ing improves cardiac output, exercise capacity and tolerance to positive airway pressure in Fontan physiology. Int J Cardiol. 2013;168:780 8. S3.6-20. Longmuir PE, Brothers JA, de Ferranti SD, et al. Promotion of physical activity for children and adults with congenital heart di- sease: a scientific statement from the American Heart Association. Circulation. 2013;127:2147 59. S3.6-21. Buys R, Van De Bruaene A, Budts W, et al. In adults with atrial switch operation for transposition of the great arteries low physical activity relates to reduced exercise capacity and decreased perceived phys- ical functioning. Acta Cardiol. 2012;67:49 57. S3.6-22. Winter MM, van der Bom T, de Vries LCS, et al. Exercise train- ing improves exercise capacity in adult patients with a sys- temic right ventricle: a randomized clinical trial. Eur Heart J. 2012;33:1378 85. 3.7. Mental Health and Neurodevelopmental Issues S3.7-1. Kovacs AH, Saidi AS, Kuhl EA, et al. Depression and anxiety in adult congenital heart disease: predictors and prevalence. Int J Cardiol. 2009;137:158 64. CLINICAL STATEMENTS AND GUIDELINES S3.7-2. Kovacs AH, Sears SF, Saidi AS. Biopsychosocial experiences of adults with congenital heart disease: review of the literature. Am Heart J. 2005;150:193 201. S3.7-3. van Rijen EHM, Utens EMWJ, Roos-Hesselink JW, et al. Psychosocial functioning of the adult with congenital heart disease: a 20 33 years follow-up. Eur Heart J. 2003;24:673 83. S3.7-4. Callus E, Quadri E, Ricci C, et al. Update on psychological func- tioning in adults with congenital heart disease: a systematic review. Expert Rev Cardiovasc Ther. 2013;11:785 91. S3.7-5. Amianto F, Bergui G, Abbate-Daga G, et al. Growing up with a congenital heart disease: neuro-cognitive, psychopathological and quality of life outcomes. Panminerva Med. 2011;53:109 27. S3.7-6. Granberg M, Rydberg A, Fisher AG. Activities in daily living and schoolwork task performance in children with complex congenital heart disease. Acta Paediatr. 2008;97:1270 4. S3.7-7. Karsdorp PA, Everaerd W, Kindt M, et al. Psychological and cogni- tive functioning in children and adolescents with congenital heart disease: a meta-analysis. J Pediatr Psychol. 2007;32:527 41. S3.7-8. van der Rijken REA, Maassen BAM, Walk TLM, et al. Outcome after surgical repair of congenital cardiac malformations at school age. Cardiol Young. 2007;17:64 71. S3.7-9. Kirshbom PM, Flynn TB, Clancy RR, et al. Late neurodevelopmental outcome after repair of total anomalous pulmonary venous connec- tion. J Thorac Cardiovasc Surg. 2005;129:1091 7. S3.7-10. Sananes R, Manlhiot C, Kelly E, et al. Neurodevelopmental out- comes after open heart operations before 3 months of age. Ann Thorac Surg. 2012;93:1577 83. S3.7-11. Schaefer C, von Rhein M, Knirsch W, et al. Neurodevelopmental outcome, psychological adjustment, and quality of life in ado- lescents with congenital heart disease. Dev Med Child Neurol. 2013;55:1143 9. S3.7-12. Gaynor JW, Kim DS, Arrington CB, et al. Validation of association of the apolipoprotein E 2 allele with neurodevelopmental dysfunction after cardiac surgery in neonates and infants. J Thorac Cardiovasc Surg. 2014;148:2560 6. S3.7-13. Marino BS, Lipkin PH, Newburger JW, et al. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation. 2012;126:1143 72. S3.7-14. Cassidy AR, White MT, DeMaso DR, et al. Executive function in chil- dren and adolescents with critical cyanotic congenital heart disease. J Int Neuropsychol Soc. 2015;21:34 49. 3.8. Endocarditis Prevention S3.8-1. Nishimura RA, Otto CM, Bonow RO, et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation. 2014;129:e521 643. S3.8-2. Verheugt CL, Uiterwaal CSPM, van der Velde ET, et al. Turning 18 with congenital heart disease: prediction of infective endocarditis based on a large population. Eur Heart J. 2011;32:1926 34. S3.8-3. Habib G, Hoen B, Tornos P, et al. Guidelines on the prevention, di- agnosis, and treatment of infective endocarditis (new version 2009): the Task Force on the Prevention, Diagnosis, and Treatment of Infective Endocarditis of the European Society of Cardiology (ESC). Eur Heart J. 2009;30:2369 413. S3.8-4. Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. 2007;116:1736 54. S3.8-5. Allen U. Infective endocarditis: updated guidelines. Paediatr Child Health. 2010;15:205 12. 3.9. Concomitant Syndromes S3.9-1. van Engelen K, Topf A, Keavney BD, et al. 22q11.2 Deletion syn- drome is under-recognised in adult patients with tetralogy of Fallot and pulmonary atresia. Heart. 2010;96:621 4. S3.9-2. Fung WLA, Chow EWC, Webb GD, et al. Extracardiac features predicting 22q11.2 deletion syndrome in adult congenital heart di- sease. Int J Cardiol. 2008;131:51 8. S3.9-3. Hartman RJ, Rasmussen SA, Botto LD, et al. The contribution of chromosomal abnormalities to congenital heart defects: a popula- tion-based study. Pediatr Cardiol. 2011;32:1147 57. S3.9-4. Lin AE, Basson CT, Goldmuntz E, et al. Adults with genetic syn- dromes and cardiovascular abnormalities: clinical history and man- agement. Genet Med. 2008;10:469 94. S3.9-5. Goldmuntz E, Clark BJ, Mitchell LE, et al. Frequency of 22q11 deletions in patients with conotruncal defects. J Am Coll Cardiol. 1998;32:492 8. S3.9-6. Richards AA, Garg V. Genetics of congenital heart disease. Curr Cardiol Rev. 2010;6:91 7. S3.9-7. Kitsiou-Tzeli S, Kolialexi A, Fryssira H, et al. Detection of 22q11.2 deletion among 139 patients with Di George/velocardiofacial syn- drome features. In Vivo. 2004;18:603 8. S3.9-8. Bassett AS, Chow EWC, Husted J, et al. Clinical features of 78 adults with 22q11 deletion syndrome. Am J Med Genet A. 2005;138:307 13. S3.9-9. Barisic I, Boban L, Greenlees R, et al. Holt Oram syndrome: a regis- try-based study in Europe. Orphanet J Rare Dis. 2014;9:156. S3.9-10. Pierpont MEM, Magoulas PL, Adi S, et al. Cardio-facio-cutaneous syndrome: clinical features, diagnosis, and management guidelines. Pediatrics. 2014;134:e1149 62. 3.10. Acquired Cardiovascular Disease S3.10-1. Engelfriet P, Boersma E, Oechslin E, et al. The spectrum of adult con- genital heart disease in Europe: morbidity and mortality in a 5 year follow-up period. The Euro Heart Survey on adult congenital heart disease. Eur Heart J. 2005;26:2325 33. S3.10-2. Pillutla P, Shetty KD, Foster E. Mortality associated with adult con- genital heart disease: trends in the US population from 1979 to 2005 Am Heart J. 2009;158:874 9. S3.10-3. Stuart AG. Changing lesion demographics of the adult with con- genital heart disease: an emerging population with complex needs. Future Cardiol. 2012;8:305 13. S3.10-4. Tutarel O, Kempny A, Alonso-Gonzalez R, et al. Congenital heart disease beyond the age of 60: emergence of a new population with high resource utilization, high morbidity, and high mortality. Eur Heart J. 2014;35:725 32. S3.10-5. Foster E, Graham TP Jr, Driscoll DJ, et al. Task force 2: special health care needs of adults with congenital heart disease. J Am Coll Cardiol. 2001;37:1176 83. S3.10-6. Diller G-P, Kempny A, Alonso-Gonzalez R, et al. Survival prospects and circumstances of death in contemporary adult congenital heart disease patients under follow-up at a large tertiary centre. Circulation. 2015;132:2118 25. S3.10-7. Verheugt CL, Uiterwaal CSPM, van der Velde ET, et al. Mortality in adult congenital heart disease. Eur Heart J. 2010;31:1220 9. S3.10-8. Partington SL, Valente AM, Bruyere J, et al. Diagnostic value of TC99M SPECT myocardial perfusion imaging in complex congenital heart disease. J Am Coll Cardiol. 2014; 63. S3.10-9. Longmuir PE, Brothers JA, de Ferranti SD, et al. Promotion of physical activity for children and adults with congenital heart di- sease: a scientific statement from the American Heart Association. Circulation. 2013;127:2147 59. S3.10-10. Zomer AC, Vaartjes I, Uiterwaal CSPM, et al. Social burden and lifestyle in adults with congenital heart disease. Am J Cardiol. 2012;109:1657 63. 3.11. Noncardiac Medical Issues S3.11-1. Wang A, Book WM, McConnell M, et al. Prevalence of hepatitis C infection in adult patients who underwent congenital heart surgery prior to screening in 1992 Am J Cardiol. 2007;100:1307 9. 3.12. Noncardiac Surgery S3.12-1. Ammash NM, Connolly HM, Abel MD, et al. Noncardiac surgery in Eisenmenger syndrome. J Am Coll Cardiol. 1999;33:222 7. CLINICAL STATEMENTS AND GUIDELINES S3.12-2. Maxwell BG, Posner KL, Wong JK, et al. Factors contributing to ad- verse perioperative events in adults with congenital heart disease: a structured analysis of cases from the closed claims project. Congenit Heart Dis. 2015;10:21 9. S3.12-3. Maxwell BG, Wong JK, Lobato RL. Perioperative morbidity and mor- tality after noncardiac surgery in young adults with congenital or early acquired heart disease: a retrospective cohort analysis of the National Surgical Quality Improvement Program database. Am Surg. 2014;80:321 6. S3.12-4. Maxwell BG, Williams GD, Ramamoorthy C. Knowledge and atti- tudes of anesthesia providers about noncardiac surgery in adults with congenital heart disease. Congenit Heart Dis. 2014;9:45 53. S3.12-5. Eagle SS, Daves SM. The adult with Fontan physiology: systematic approach to perioperative management for noncardiac surgery. J Cardiothorac Vasc Anesth. 2011;25:320 34. S3.12-6. Maxwell BG, Wong JK, Kin C, et al. Perioperative outcomes of major noncardiac surgery in adults with congenital heart disease. Anesthesiology. 2013;119:762 9. S3.12-7. Maxwell BG, Wong JK, Sheikh AY, et al. Heart transplantation with or without prior mechanical circulatory support in adults with con- genital heart disease. Eur J Cardiothorac Surg. 2014;45:842 6. S3.12-8. Rabbitts JA, Groenewald CB, Mauermann WJ, et al. Outcomes of general anesthesia for noncardiac surgery in a series of patients with Fontan palliation. Paediatr Anaesth. 2013;23:180 7. S3.12-9. Mylotte D, Quenneville SP, Kotowycz MA, et al. Long-term cost-ef- fectiveness of transcatheter versus surgical closure of secundum atrial septal defect in adults. Int J Cardiol. 2014;172:109 14. S3.12-10. Fleisher LA, Fleischmann KE, Auerbach AD, et al. 2014 ACC/AHA guideline on perioperative cardiovascular evaluation and manage- ment of patients undergoing noncardiac surgery: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation. 2014;130:e278 333. S3.12-11. Christensen RE, Gholami AS, Reynolds PI, et al. Anaesthetic man- agement and outcomes after noncardiac surgery in patients with hypoplastic left heart syndrome: a retrospective review. Eur J Anaesthesiol. 2012;29:425 30. S3.12-12. Warner MA, Lunn RJ, O Leary PW, et al. Outcomes of noncardiac surgical procedures in children and adults with congenital heart disease. Mayo Perioperative Outcomes Group. Mayo Clin Proc. 1998;73:728 34. S3.12-13. Mott AR, Fraser CD Jr, McKenzie ED, et al. Perioperative care of the adult with congenital heart disease in a free-standing tertiary pedi- atric facility. Pediatr Cardiol. 2002;23:624 30. S3.12-14. Maxwell BG, Maxwell TG, Wong JK. Decentralization of care for adults with congenital heart disease in the United States: a geo- graphic analysis of outpatient surgery. PLoS One. 2014;9:e106730. 3.13. Pregnancy, Reproduction, and Sexual Health S3.13.1-1. Balint OH, Siu SC, Mason J, et al. Cardiac outcomes after pregnancy in women with congenital heart disease. Heart. 2010;96:1656 61. S3.13.1-2. Roos-Hesselink JW, Ruys TPE, Stein JI, et al. Outcome of preg- nancy in patients with structural or ischaemic heart disease: results of a registry of the European Society of Cardiology. Eur Heart J. 2013;34:657 65. S3.13.1-3. Ouyang DW, Khairy P, Fernandes SM, et al. Obstetric outcomes in pregnant women with congenital heart disease. Int J Cardiol. 2010;144:195 9. S3.13.1-4. Egidy Assenza G, Cassater D, Landzberg M, et al. The effects of pregnancy on right ventricular remodeling in women with repaired tetralogy of Fallot. Int J Cardiol. 2013;168:1847 52. S3.13.1-5. Chan WS, Anand S, Ginsberg JS. Anticoagulation of pregnant women with mechanical heart valves: a systematic review of the literature. Arch Intern Med. 2000;160:191 6. S3.13.1-6. Cutts BA, Dasgupta D, Hunt BJ. New directions in the diagnosis and treatment of pulmonary embolism in pregnancy. Am J Obstet Gynecol. 2013;208:102 8. S3.13.1-7. Siu SC, Sermer M, Colman JM, et al. Prospective multicenter study of pregnancy outcomes in women with heart disease. Circulation. 2001;104:515 21. S3.13.1-8. Greutmann M, VK K, Brooks R, et al. Pregnancy outcome in women with congenital heart disease and residual haemody- namic lesions of the right ventricular outflow tract. Eur Heart J. 2010;31:1764 70. S3.13.1-9. Gill HK, Splitt M, Sharland GK, et al. Patterns of recurrence of con- genital heart disease: an analysis of 6,640 consecutive pregnancies evaluated by detailed fetal echocardiography. J Am Coll Cardiol. 2003;42:923 9. S3.13.1-10. Ohuchi H, Tanabe Y, Kamiya C, et al. Cardiopulmonary variables during exercise predict pregnancy outcome in women with con- genital heart disease. Circ J. 2013;77:470 6. S3.13.1-11. Lui GK, Silversides CK, Khairy P, et al. Heart rate response during exercise and pregnancy outcome in women with congenital heart disease. Circulation. 2011;123:242 8. S3.13.1-12. Siu SC, Colman JM, Sorensen S, et al. Adverse neonatal and car- diac outcomes are more common in pregnant women with cardiac disease. Circulation. 2002;105:2179 84. S3.13.1-13. Holland BJ, Myers JA, Woods CR Jr. Prenatal diagnosis of critical congenital heart disease reduces risk of death from cardiovascular compromise prior to planned neonatal cardiac surgery: a meta- analysis. Ultrasound Obstet Gynecol. 2015;45:631 8. S3.13.1-14. van Hagen IM, Roos-Hesselink JW, Ruys TPE, et al. Pregnancy in women with a mechanical heart valve: data of the European Society of Cardiology Registry of Pregnancy and Cardiac Disease (ROPAC). Circulation. 2015;132:132 42. S3.13.1-15. Khairy P, Ouyang DW, Fernandes SM, et al. Pregnancy out- comes in women with congenital heart disease. Circulation. 2006;113:517 24. S3.13.1-16. Thorne S, Nelson-Piercy C, MacGregor A, et al. Pregnancy and con- traception in heart disease and pulmonary arterial hypertension. J Fam Plann Reprod Health Care. 2006;32:75 81. S3.13.1-17. Drenthen W, Pieper PG, van der Tuuk K, et al. Fertility, pregnancy and delivery in women after biventricular repair for double outlet right ventricle. Cardiology. 2008;109:105 9. S3.13.1-18. Drenthen W, Pieper PG, Roos-Hesselink JW, et al. Outcome of pregnancy in women with congenital heart disease: a literature review. J Am Coll Cardiol. 2007;49:2303 11. S3.13.1-19. Pedersen LM, Pedersen TAL, Ravn HB, et al. Outcomes of pregnancy in women with tetralogy of Fallot. Cardiol Young. 2008;18:423 9. S3.13.1-20. Vriend JWJ, Drenthen W, Pieper PG, et al. Outcome of preg- nancy in patients after repair of aortic coarctation. Eur Heart J. 2005;26:2173 8. S3.13.1-21. Kovacs AH, Harrison JL, Colman JM, et al. Pregnancy and contra- ception in congenital heart disease: what women are not told. J Am Coll Cardiol. 2008;52:577 8. S3.13.1-22. Beauchesne LM, Connolly HM, Ammash NM, et al. Coarctation of the aorta: outcome of pregnancy. J Am Coll Cardiol. 2001;38:1728 33. S3.13.1-23. Krieger EV, Landzberg MJ, Economy KE, et al. Comparison of risk of hypertensive complications of pregnancy among women with versus without coarctation of the aorta. Am J Cardiol. 2011;107:1529 34. S3.13.1-24. Balci A, Sollie-Szarynska KM, van der Bijl AGL, et al. Prospective validation and assessment of cardiovascular and offspring risk models for pregnant women with congenital heart disease. Heart. 2014;100:1373 81. S3.13.1-25. Nishimura RA, Otto CM, Bonow RO, et al. 