Voltage and activa- tion mapping: how the recording technique affects the outcome of catheter ablation procedures in patients with congenital heart disease. Circulation. 2003;108:2099 106. S4.4.2-39. Cohen MI, Vetter VL, Wernovsky G, et al. Epicardial pacemaker im- plantation and follow-up in patients with a single ventricle after the Fontan operation. J Thorac Cardiovasc Surg. 2001;121:804 11. S4.4.2-40. Gentles TL, Gauvreau K, Mayer JE Jr, et al. Functional outcome after the Fontan operation: factors influencing late morbidity. J Thorac Cardiovasc Surg. 1997;114:392 403. S4.4.2-41. Cohen MI, Wernovsky G, Vetter VL, et al. Sinus node func- tion after a systematically staged Fontan procedure. Circulation. 1998;98:II352 8. S4.4.2-42. Barber BJ, Burch GH, Tripple D, et al. Resolution of plastic bron- chitis with atrial pacing in a patient with Fontan physiology. Pediatr Cardiol. 2004;25:73 6. S4.4.2-43. Nielsen JC, Pedersen AK, Mortensen PT, et al. Programming a fixed long atrioventricular delay is not effective in preventing ven- tricular pacing in patients with sick sinus syndrome. Europace. 1999;1:113 20. S4.4.2-44. Abrams DJ, Earley MJ, Sporton SC, et al. Comparison of noncon- tact and electroanatomic mapping to identify scar and arrhythmia late after the Fontan procedure. Circulation. 2007;115:1738 46. S4.4.2-45. Rosenthal E, Qureshi SA, Crick JC. Successful long-term ventricular pacing via the coronary sinus after the Fontan operation. Pacing Clin Electrophysiol. 1995;18:2103 5. S4.4.2-46. Blackburn ME, Gibbs JL. Ventricular pacing from the coronary sinus of a patient with a Fontan circulation. Br Heart J. 1993;70:578 9. S4.4.2-47. Walsh EP, Cecchin F. Arrhythmias in adult patients with congenital heart disease. Circulation. 2007;115:534 5. S4.4.2-48. Piran S, Veldtman G, Siu S, et al. Heart failure and ventricular dysfunction in patients with single or systemic right ventricles. Circulation. 2002;105:1189 94. S4.4.2-49. Wells R, Khairy P, Harris L, et al. Dofetilide for atrial arrhythmias in congenital heart disease: a multicenter study. Pacing Clin Electrophysiol. 2009;32:1313 8. S4.4.2-50. Thorne SA, Barnes I, Cullinan P, et al. Amiodarone-associated thy- roid dysfunction: risk factors in adults with congenital heart di- sease. Circulation. 1999;100:149 54. S4.4.2-51. Garg R, Powell AJ, Sena L, et al. Effects of metallic implants on magnetic resonance imaging evaluation of Fontan palliation. Am J Cardiol. 2005;95:688 91. S4.4.2-52. Prabhu SP, Mahmood S, Sena L, et al. MDCT evaluation of pul- monary embolism in children and young adults following a lateral tunnel Fontan procedure: optimizing contrast-enhancement tech- niques. Pediatr Radiol. 2009;39:938 44. S4.4.2-53. Asrani SK, Warnes CA, Kamath PS. Hepatocellular carcinoma after the Fontan procedure. N Engl J Med. 2013;368:1756 7. S4.4.2-54. Ono M, Boethig D, Goerler H, et al. Clinical outcome of patients 20 years after Fontan operation effect of fenestration on late mor- bidity. Eur J Cardiothorac Surg. 2006;30:923 9. S4.4.2-55. Atz AM, Travison TG, McCrindle BW, et al. Late status of Fontan patients with persistent surgical fenestration. J Am Coll Cardiol. 2011;57:2437 43. S4.4.2-56. Poterucha JT, Johnson JN, Taggart NW, et al. Embolization of veno- venous collaterals after the Fontan operation is associated with decreased survival. Congenit Heart Dis. 2015;10:E230 6. S4.4.2-57. Mets JM, Bergersen L, Mayer JE Jr, et al. Outcomes of stent implan- tation for obstruction of intracardiac lateral tunnel Fontan path- ways. Circ Cardiovasc Interv. 2013;6:92 100. S4.4.2-58. Ohuchi H, Yasuda K, Miyazaki A, et al. Haemodynamic charac- teristics before and after the onset of protein losing enteropathy in patients after the Fontan operation. Eur J Cardiothorac Surg. 2013;43:e49 57. S4.4.2-59. Downing TE, McDonnell A, Zhu X, et al. Cumulative medical ra- diation exposure throughout staged palliation of single ventricle congenital heart disease. Pediatr Cardiol. 2015;36:190 5. S4.4.2-60. Mahnke CB, Boyle GJ, Janosky JE, et al. Anticoagulation and inci- dence of late cerebrovascular accidents following the Fontan pro- cedure. Pediatr Cardiol. 2005;26:56 61. S4.4.2-61. Triedman JK, Alexander ME, Love BA, et al. Influence of patient factors and ablative technologies on outcomes of radiofrequency ablation of intra-atrial re-entrant tachycardia in patients with con- genital heart disease. J Am Coll Cardiol. 2002;39:1827 35. S4.4.2-62. Deal BJ, Mavroudis C, Backer CL, et al. Comparison of ana- tomic isthmus block with the modified right atrial maze proce- dure for late atrial tachycardia in Fontan patients. Circulation. 2002;106:575 9. S4.4.2-63. Rychik J, Veldtman G, Rand E, et al. The precarious state of the liver after a Fontan operation: summary of a multidisciplinary sympo- sium. Pediatr Cardiol. 2012;33:1001 12. S4.4.2-64. Monagle P, Cochrane A, Roberts R, et al. A multicenter, random- ized trial comparing heparin/warfarin and acetylsalicylic acid as pri- mary thromboprophylaxis for 2 years after the Fontan procedure in children. J Am Coll Cardiol. 2011;58:645 51. S4.4.2-65. McCrindle BW, Manlhiot C, Cochrane A, et al. Factors associ- ated with thrombotic complications after the Fontan procedure: a secondary analysis of a multicenter, randomized trial of primary thromboprophylaxis for 2 years after the Fontan procedure. J Am Coll Cardiol. 2013;61:346 53. 4.4.6. Severe PAH and Eisenmenger Syndrome 4.4.6.1. Severe PAH S4.4.6.1-1. 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. CLINICAL STATEMENTS AND GUIDELINES S4.4.6.1-2. 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. S4.4.6.1-3. Duffels MGJ, Engelfriet PM, Berger RMF, et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic per- spective from a Dutch registry. Int J Cardiol. 2007;120:198 204. S4.4.6.1-4. Engelfriet PM, Duffels MGJ, M ller T, et al. Pulmonary arte- rial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart disease. Heart. 2007;93:682 7. S4.4.6.1-5. Lowe BS, Therrien J, Ionescu-Ittu R, et al. Diagnosis of pulmonary hypertension in the congenital heart disease adult population im- pact on outcomes. J Am Coll Cardiol. 2011;58:538 46. S4.4.6.1-6. Manes A, Palazzini M, Leci E, et al. Current era survival of patients with pulmonary arterial hypertension associated with congen- ital heart disease: a comparison between clinical subgroups. Eur Heart J. 2014;35:716 24. S4.4.6.1-7. Gabriels C, De Meester P, Pasquet A, et al. A different view on predictors of pulmonary hypertension in secundum atrial septal defect. Int J Cardiol. 2014;176:833 40. S4.4.6.1-8. 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. S4.4.6.1-9. 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. S4.4.6.1-10. 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. S4.4.6.1-11. Berner M, Beghetti M, Spahr-Schopfer I, et al. Inhaled nitric oxide to test the vasodilator capacity of the pulmonary vascular bed in children with long-standing pulmonary hypertension and congen- ital heart disease. Am J Cardiol. 1996;77:532 5. S4.4.6.1-12. Balint OH, Samman A, Haberer K, et al. Outcomes in patients with pulmonary hypertension undergoing percutaneous atrial septal defect closure. Heart. 2008;94:1189 93. S4.4.6.1-13. Gorenflo M, Gu H, Xu Z. Peri-operative pulmonary hypertension in paediatric patients: current strategies in children with congen- ital heart disease. Cardiology. 2010;116:10 7. S4.4.6.1-14. Huang J-B, Liu Y-L, Yu C-T, et al. Lung biopsy findings in previously inoperable patients with severe pulmonary hypertension associ- ated with congenital heart disease. Int J Cardiol. 2011;151:76 83. S4.4.6.1-15. Neutze JM, Ishikawa T, Clarkson PM, et al. Assessment and fol- low-up of patients with ventricular septal defect and elevated pul- monary vascular resistance. Am J Cardiol. 1989;63:327 31. S4.4.6.1-16. 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. S4.4.6.1-17. Yong G, Khairy P, De Guise P, et al. Pulmonary arterial hyper- tension in patients with transcatheter closure of secundum atrial septal defects: a longitudinal study. Circ Cardiovasc Interv. 2009;2:455 62. S4.4.6.1-18. Balzer DT, Kort HW, Day RW, et al. Inhaled nitric oxide as a preop- erative test (INOP Test I): the INOP Test Study Group. Circulation. 2002;106:I76 81. S4.4.6.1-19. Opotowsky AR, Ojeda J, Rogers F, et al. A simple echocardio- graphic prediction rule for hemodynamics in pulmonary hyper- tension. Circ Cardiovasc Imaging. 2012;5:765 75. S4.4.6.1-20. Opotowsky AR, Clair M, Afilalo J, et al. A simple echocardio- graphic method to estimate pulmonary vascular resistance. Am J Cardiol. 2013;112:873 82. S4.4.6.1-21. Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62:D34 41. 4.4.6.2. Eisenmenger Syndrome S4.4.6.2-1. Dimopoulos K, Inuzuka R, Goletto S, et al. Improved survival among patients with Eisenmenger syndrome receiving ad- vanced therapy for pulmonary arterial hypertension. Circulation. 2010;121:20 5. S4.4.6.2-2. Gatzoulis MA, Beghetti M, Gali N, et al. Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open-label extension study. Int J Cardiol. 2008;127:27 32. S4.4.6.2-3. Gali N, Beghetti M, Gatzoulis MA, et al. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, dou- ble-blind, randomized, placebo-controlled study. Circulation. 2006;114:48 54. S4.4.6.2-4. Diller G-P, Alonso-Gonzalez R, Dimopoulos K, et al. Disease targeting therapies in patients with Eisenmenger syndrome: re- sponse to treatment and long-term efficiency. Int J Cardiol. 2013;167:840 7. S4.4.6.2-5. D Alto M, Romeo E, Argiento P, et al. Therapy for pulmonary ar- terial hypertension due to congenital heart disease and Down s syndrome. Int J Cardiol. 2013;164:323 6. S4.4.6.2-6. Iversen K, Jensen AS, Jensen TV, et al. Combination therapy with bosentan and sildenafil in Eisenmenger syndrome: a ran- domized, placebo-controlled, double-blinded trial. Eur Heart J. 2010;31:1124 31. S4.4.6.2-7. Baptista R, Castro G, da Silva AM, et al. Long-term effect of bosentan in pulmonary hypertension associated with complex congenital heart disease. Rev Port Cardiol. 2013;32:123 9. S4.4.6.2-8. Crepaz R, Romeo C, Montanaro D, et al. Long-term results of treatment with bosentan in adult Eisenmenger s syndrome patients with Down s syndrome related to congenital heart di- sease. BMC Cardiovasc Disord. 2013;13:74. S4.4.6.2-9. Duffels MGJ, Vis JC, van Loon RLE, et al. Down patients with Eisenmenger syndrome: is bosentan treatment an option? Int J Cardiol. 2009;134:378 83. S4.4.6.2-10. Serino G, Guazzi M, Micheletti A, et al. Effect of bosentan on exercise capacity and clinical worsening in patients with dual Down and Eisenmenger syndrome. Clin Med Insights Cardiol. 2013;7:29 34. S4.4.6.2-11. Zhang Z-N, Jiang X, Zhang R, et al. Oral sildenafil treatment for Eisenmenger syndrome: a prospective, open-label, multicentre study. Heart. 2011;97:1876 81. S4.4.6.2-12. Garg N, Tripathy N, Sinha N. Comparative efficacy of sildenafil in Eisenmenger s syndrome secondary to atrial septal defect versus ventricular septal defect: a cardiac catheterisation follow-up study. Cardiol Young. 2011;21:631 8. S4.4.6.2-13. Tay ELW, Papaphylactou M, Diller G-P, et al. Quality of life and functional capacity can be improved in patients with Eisenmenger syndrome with oral sildenafil therapy. Int J Cardiol. 2011;149:372 6. S4.4.6.2-14. Bharani A, Patel A, Saraf J, et al. Efficacy and safety of PDE-5 in- hibitor tadalafil in pulmonary arterial hypertension. Indian Heart J. 2007;59:323 8. S4.4.6.2-15. Mukhopadhyay S, Sharma M, Ramakrishnan S, et al. Phosphodiesterase-5 inhibitor in Eisenmenger syndrome: a pre- liminary observational study. Circulation. 2006;114:1807 10. S4.4.6.2-16. Singh TP, Rohit M, Grover A, et al. A randomized, placebo-con- trolled, double-blind, crossover study to evaluate the efficacy of oral sildenafil therapy in severe pulmonary artery hypertension. Am Heart J. 2006;151:851.e1 851.e5. S4.4.6.2-17. Gali N, Hoeper MM, Humbert M, et al. Guidelines for the diag- nosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Eur Heart J. 2009;30:2493 537. S4.4.6.2-18. D Alto M, Romeo E, Argiento P, et al. Bosentan-sildenafil associa- tion in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology. Int J Cardiol. 2012;155:378 82. 4.4.7. Coronary Anomalies S4.4.7-1. Eckart RE, Shry EA, Burke AP, et al. Sudden death in young adults: an autopsy-based series of a population undergoing active sur- veillance. J Am Coll Cardiol. 2011;58:1254 61. S4.4.7-2. Krasuski RA, Magyar D, Hart S, et al. Long-term outcome and im- pact of surgery on adults with coronary arteries originating from the opposite coronary cusp. Circulation. 2011;123:154 62. S4.4.7-3. Eckart RE, Scoville SL, Campbell CL, et al. Sudden death in young adults: a 25-year review of autopsies in military recruits. Ann Intern Med. 2004;141:829 34. CLINICAL STATEMENTS AND GUIDELINES S4.4.7-4. Frescura C, Basso C, Thiene G, et al. Anomalous origin of coronary arteries and risk of sudden death: a study based on an autopsy pop- ulation of congenital heart disease. Hum Pathol. 1998;29:689 95. S4.4.7-5. Basso C, Maron BJ, Corrado D, et al. Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes. J Am Coll Cardiol. 2000;35:1493 501. S4.4.7-6. Kaushal S, Backer CL, Popescu AR, et al. Intramural coronary length correlates with symptoms in patients with anomalous aortic origin of the coronary artery. Ann Thorac Surg. 2011;92:986 91. S4.4.7-7. Sharma V, Burkhart HM, Dearani JA, et al. Surgical unroofing of anomalous aortic origin of a coronary artery: a single-center expe- rience. Ann Thorac Surg. 2014;98:941 5. S4.4.7-8. Frommelt PC, Sheridan DC, Berger S, et al. Ten-year experience with surgical unroofing of anomalous aortic origin of a coronary artery from the opposite sinus with an interarterial course. J Thorac Cardiovasc Surg. 2011;142:1046 51. S4.4.7-9. Angelini P, Uribe C, Monge J, et al. Origin of the right coronary ar- tery from the opposite sinus of Valsalva in adults: characterization by intravascular ultrasonography at baseline and after stent angio- plasty. Catheter Cardiovasc Interv. 2015;86:199 208. S4.4.7-10. Opolski MP, Pregowski J, Kruk M, et al. Prevalence and character- istics of coronary anomalies originating from the opposite sinus of Valsalva in 8,522 patients referred for coronary computed tomog- raphy angiography. Am J Cardiol. 2013;111:1361 7. S4.4.7-11. Lee H-J, Hong YJ, Kim HY, et al. Anomalous origin of the right coro- nary artery from the left coronary sinus with an interarterial course: subtypes and clinical importance. Radiology. 2012;262:101 8. S4.4.7-12. Brothers J, Gaynor JW, Paridon S, et al. Anomalous aortic origin of a coronary artery with an interarterial course: understanding cur- rent management strategies in children and young adults. Pediatr Cardiol. 2009;30:911 21. S4.4.7-13. Poynter JA, Williams WG, McIntyre S, et al. Anomalous aortic origin of a coronary artery: a report from the Congenital Heart Surgeons Society Registry. World J Pediatr Congenit Heart Surg. 2014;5:22 30. S4.4.7-14. Brothers JA, Gaynor JW, Jacobs JP, et al. The Congenital Heart Surgeons Society Registry of Anomalous Aortic Origin of a Coronary Artery: an update. Cardiol Young. 2015;25:1567 71. S4.4.7-15. Maron BJ, Doerer JJ, Haas TS, et al. Sudden deaths in young com- petitive athletes: analysis of 1866 deaths in the United States, 1980 2006 Circulation. 2009;119:1085 92. S4.4.7-16. Davies JE, Burkhart HM, Dearani JA, et al. Surgical management of anomalous aortic origin of a coronary artery. Ann Thorac Surg. 2009;88:844 7. S4.4.7-17. Kragel AH, Roberts WC. Anomalous origin of either the right or left main coronary artery from the aorta with subsequent cours- ing between aorta and pulmonary trunk: analysis of 32 necropsy cases. Am J Cardiol. 1988;62:771 7. S4.4.7-18. Donaldson RM, Raphael M, Radley-Smith R, et al. Angiographic identification of primary coronary anomalies causing impaired my- ocardial perfusion. Cathet Cardiovasc Diagn. 1983;9:237 49. S4.4.7-19. Brothers JA, McBride MG, Seliem MA, et al. Evaluation of myocar- dial ischemia after surgical repair of anomalous aortic origin of a coronary artery in a series of pediatric patients. J Am Coll Cardiol. 2007;50:2078 82. S4.4.7-20. Mainwaring RD, Reddy VM, Reinhartz O, et al. Anomalous aortic origin of a coronary artery: medium-term results after surgical re- pair in 50 patients. Ann Thorac Surg. 2011;92:691 7. S4.4.7-21. Mumtaz MA, Lorber RE, Arruda J, et al. Surgery for anom- alous aortic origin of the coronary artery. Ann Thorac Surg. 2011;91:811 4. 4.4.7.1. Anomalous Coronary Artery Evaluation S4.4.7.1-1. Basso C, Maron BJ, Corrado D, et al. Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes. J Am Coll Cardiol. 2000;35:1493 501. S4.4.7.1-2. Davies JE, Burkhart HM, Dearani JA, et al. Surgical management of anomalous aortic origin of a coronary artery. Ann Thorac Surg. 2009;88:844 7. S4.4.7.1-3. Sharma V, Burkhart HM, Dearani JA, et al. Surgical unroofing of anomalous aortic origin of a coronary artery: a single-center expe- rience. Ann Thorac Surg. 2014;98:941 5. S4.4.7.1-4. Eckart RE, Shry EA, Burke AP, et al. Sudden death in young adults: an autopsy-based series of a population undergoing active sur- veillance. J Am Coll Cardiol. 2011;58:1254 61. S4.4.7.1-5. Angelini P, Uribe C, Monge J, et al. Origin of the right coronary artery from the opposite sinus of Valsalva in adults: characteriza- tion by intravascular ultrasonography at baseline and after stent angioplasty. Catheter Cardiovasc Interv. 2015;86:199 208. S4.4.7.1-6. Opolski MP, Pregowski J, Kruk M, et al. Prevalence and character- istics of coronary anomalies originating from the opposite sinus of Valsalva in 8,522 patients referred for coronary computed tomog- raphy angiography. Am J Cardiol. 2013;111:1361 7. S4.4.7.1-7. Lee H-J, Hong YJ, Kim HY, et al. Anomalous origin of the right coronary artery from the left coronary sinus with an inter- arterial course: subtypes and clinical importance. Radiology. 2012;262:101 8. S4.4.7.1-8. Kaushal S, Backer CL, Popescu AR, et al. Intramural coronary length correlates with symptoms in patients with anomalous aortic origin of the coronary artery. Ann Thorac Surg. 2011;92:986 91. S4.4.7.1-9. Frescura C, Basso C, Thiene G, et al. Anomalous origin of cor- onary arteries and risk of sudden death: a study based on an autopsy population of congenital heart disease. Hum Pathol. 1998;29:689 95. 4.4.7.2. Anomalous Aortic Origin of Coronary Artery S4.4.7.2-1. Basso C, Maron BJ, Corrado D, et al. Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes. J Am Coll Cardiol. 2000;35:1493 501. S4.4.7.2-2. Davies JE, Burkhart HM, Dearani JA, et al. Surgical management of anomalous aortic origin of a coronary artery. Ann Thorac Surg. 2009;88:844 7. S4.4.7.2-3. Sharma V, Burkhart HM, Dearani JA, et al. Surgical unroofing of anomalous aortic origin of a coronary artery: a single-center expe- rience. Ann Thorac Surg. 2014;98:941 5. S4.4.7.2-4. Frommelt PC, Sheridan DC, Berger S, et al. Ten-year experience with surgical unroofing of anomalous aortic origin of a coro- nary artery from the opposite sinus with an interarterial course. J Thorac Cardiovasc Surg. 2011;142:1046 51. S4.4.7.2-5. Gulati R, Reddy VM, Culbertson C, et al. Surgical management of coronary artery arising from the wrong coronary sinus, using standard and novel approaches. J Thorac Cardiovasc Surg. 2007;134:1171 8. S4.4.7.2-6. Mainwaring RD, Reddy VM, Reinhartz O, et al. Anomalous aortic origin of a coronary artery: medium-term results after surgical re- pair in 50 patients. Ann Thorac Surg. 2011;92:691 7. S4.4.7.2-7. Brothers JA, McBride MG, Seliem MA, et al. Evaluation of myocar- dial ischemia after surgical repair of anomalous aortic origin of a coronary artery in a series of pediatric patients. J Am Coll Cardiol. 2007;50:2078 82. S4.4.7.2-8. Donaldson RM, Raphael M, Radley-Smith R, et al. Angiographic identification of primary coronary anomalies causing impaired myocardial perfusion. Cathet Cardiovasc Diagn. 1983;9:237 49. S4.4.7.2-9. Kragel AH, Roberts WC. Anomalous origin of either the right or left main coronary artery from the aorta with subsequent cours- ing between aorta and pulmonary trunk: analysis of 32 necropsy cases. Am J Cardiol. 1988;62:771 7. S4.4.7.2-10. Davis JA, Cecchin F, Jones TK, et al. Major coronary artery anoma- lies in a pediatric population: incidence and clinical importance. J Am Coll Cardiol. 2001;37:593 7. S4.4.7.2-11. Eckart RE, Shry EA, Burke AP, et al. Sudden death in young adults: an autopsy-based series of a population undergoing active sur- veillance. J Am Coll Cardiol. 2011;58:1254 61. S4.4.7.2-12. Maron BJ, Doerer JJ, Haas TS, et al. Sudden deaths in young com- petitive athletes: analysis of 1866 deaths in the United States, 1980 2006 Circulation. 2009;119:1085 92. S4.4.7.2-13. Eckart RE, Scoville SL, Campbell CL, et al. Sudden death in young adults: a 25-year review of autopsies in military recruits. Ann Intern Med. 2004;141:829 34. S4.4.7.2-14. Opolski MP, Pregowski J, Kruk M, et al. Prevalence and character- istics of coronary anomalies originating from the opposite sinus of Valsalva in 8,522 patients referred for coronary computed tomog- raphy angiography. Am J Cardiol. 2013;111:1361 7. CLINICAL STATEMENTS AND GUIDELINES 4.4.7.3. Anomalous Coronary Artery Arising From the PA S4.4.7.3-1. Cochrane AD, Coleman DM, Davis AM, et al. Excellent long-term functional outcome after an operation for anomalous left coro- nary artery from the pulmonary artery. J Thorac Cardiovasc Surg. 1999;117:332 42. S4.4.7.3-2. Paridon SM, Farooki ZQ, Kuhns LR, et al. Exercise performance after repair of anomalous origin of the left coronary artery from the pulmonary artery. Circulation. 1990;81:1287 92. S4.4.7.3-3. Lange R, Vogt M, H rer J, et al. Long-term results of repair of anomalous origin of the left coronary artery from the pulmonary artery. Ann Thorac Surg. 2007;83:1463 71. S4.4.7.3-4. el-Said GM, Ruzyllo W, Williams RL, et al. Early and late result of saphenous vein graft for anomalous origin of left coronary artery from pulmonary artery. Circulation. 1973;48:III2 6. S4.4.7.3-5. Wilson CL, Dlabal PW, McGuire SA. Surgical treatment of anoma- lous left coronary artery from pulmonary artery: follow-up in teen- agers and adults. Am Heart J. 1979;98:440 6. S4.4.7.3-6. Yau JM, Singh R, Halpern EJ, et al. Anomalous origin of the left coronary artery from the pulmonary artery in adults: a comprehen- sive review of 151 adult cases and a new diagnosis in a 53-year-old woman. Clin Cardiol. 2011;34:204 10. S4.4.7.3-7. Rajbanshi BG, Burkhart HM, Schaff HV, et al. Surgical strategies for anomalous origin of coronary artery from pulmonary artery in adults. J Thorac Cardiovasc Surg. 2014;148:220 4. 4.4.8. Coronary Artery Fistula S4.4.8-1. Vavuranakis M, Bush CA, Boudoulas H. Coronary artery fistulas in adults: incidence, angiographic characteristics, natural history. Cathet Cardiovasc Diagn. 1995;35:116 20. S4.4.8-2. Yildiz A, Okcun B, Peker T, et al. Prevalence of coronary artery anomalies in 12,457 adult patients who underwent coronary an- giography. Clin Cardiol. 2010;33:E60 4. S4.4.8-3. Said SM, Burkhart HM, Schaff HV, et al. Late outcome of repair of congenital coronary artery fistulas a word of caution. J Thorac Cardiovasc Surg. 2013;145:455 60. 5. EVIDENCE GAPS AND FUTURE DIRECTIONS S5-1. Gurvitz M, Burns KM, Brindis R, et al. Emerging research direc- tions in adult congenital heart disease: a report from an NHLBI/ ACHA Working Group. J Am Coll Cardiol. 2016;67:1956 64. CLINICAL STATEMENTS AND GUIDELINES Appendix 1. Author Relationships With Industry and Other Entities (Relevant) 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease* (February 2018) Committee Member Employment Consultant Speakers Bureau Ownership/ Partnership/ Principal Personal Research Institutional, Organizational, or Other Financial Benefit Expert Witness Voting Recusals by Section Karen K. Stout, Chair University of Washington Director, Adult Congenital Heart Disease Program, Professor, Internal Medicine and Pediatrics None None None None None None None Curt J. Daniels, Vice Chair The Ohio State University Heart Center and Nationwide Children s Hospital Director, Adult Congenital Heart Disease and Pulmonary Hypertension Program, Professor, Internal Medicine and Pediatrics None None None None Jamil A. Aboulhosn UCLA Adult Congenital Heart Disease Center Director Actelion GE Medical Edward Lifesciences Medtronic None None Gore United Therapeutics Actelion Medtronic St. Jude Edward Lifesciences Biykem Bozkurt Baylor College of Medicine Professor of Medicine None None None None Novartis None None Craig S. Broberg Oregon Health and Science University Associate Professor of Medicine None None None None Jack M. Colman University of Toronto Professor of Medicine and Obstetrics & Gynecology, Toronto Congenital Cardiac Centre for Adults and Pregnancy and Heart Disease Program; University Health Network and Mount Sinai Hospital Senior Attending Cardiologist None None None None None None None Stephen R. Crumb Nationwide Children s Hospital Nurse Practitioner and Coordinator, COACH and Pulmonary Hypertension Programs None None None None None None None Joseph A. Dearani Mayo Clinic Professor of Surgery and Chair, Division of Cardiovascular Surgery None None None None Sorin (LivaNova) Cormatrix None None Stephanie Fuller University of Pennsylvania Perelman School of Medicine Associate Professor of Clinical Surgery None None None None None None None Michelle Gurvitz Harvard Medical School; Brigham and Women s Hospital Instructor of Pediatrics, Assistant Professor None None None None None None None Paul Khairy Montreal Heart Institute Adult Congenital Center Director; Professor of Medicine None None None None Boehringer Ingelheim St. Jude Medical Medtronic Actelion (Continued) CLINICAL STATEMENTS AND GUIDELINES Michael J. Landzberg Boston Children s Hospital Director, Adult Congenital Heart Service; Harvard Medical School Associate Professor of Medicine None None None None Gilead Actelion Arwa Saidi University of Florida College of Medicine Professor, Congenital Heart Center None None None None Anne Marie Valente Boston Children s Hospital, Brigham and Women s Hospital Outpatient Director, Boston Adult Congenital Heart Disease and Pulmonary Hypertension Service; Harvard Medical School Associate Professor of Medicine and Pediatrics None None None None None None None George F. Van Hare Washington University School of Medicine Director, Pediatric Cardiology; St. Louis Children s, Washington University Heart Center Co- Director None None None None None None None This table represents the relationships of committee members with industry and other entities that were determined to be relevant to this document. These relationships were reviewed and updated in conjunction with all meetings and/or conference calls of the writing committee during the document development process. The table does not necessarily reflect relationships with industry at the time of publication. A person is deemed to have a significant interest in a business if the interest represents ownership of >5% of the voting stock or share of the business entity, or ownership of >$5000 of the fair market value of the business entity; or if funds received by the person from the business entity exceed 5% of the person s gross income for the previous year. Relationships that exist with no financial benefit are also included for the purpose of transparency. Relationships in this table are modest unless otherwise noted. According to the ACC/AHA, a person has a relevant relationship IF: a) the relationship or interest relates to the same or similar subject matter, intellectual property or asset, topic, or issue addressed in the document; or b) the company/entity (with whom the relationship exists) makes a drug, drug class, or device addressed in the document or makes a competing drug or device addressed in the document; or c) the person or a member of the person s household, has a reasonable potential for financial, professional or other personal gain or loss as a result of the issues/content addressed in the document. *The ACHD Guideline began in March 2014. Over the initial years of the CMS Open Payment System, understandably, there have been issues related to accurate reporting of food and beverage payments. For this reason, the ACC and AHA have not considered these minor charges relevant relationships with industry. Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry and other entities may apply. Section numbers pertain to those in the full-text guideline. No financial benefit. Significant relationship. CMS reported payments to Dr. Dearani in 2016 related to research for the Sorin Group and Cormatrix; however, he disagrees with this report. The sections authored by Dr. Dearani have been reviewed, and it was affirmed that there was no implication of any influence of industry. ACC indicates American College of Cardiology; AHA, American Heart Association; CMS, Centers for Medicare & Medicaid Services; COACH, Columbus Ohio Adult Congenital Heart; and UCLA, University of California, Los Angeles. Appendix 1. Continued Committee Member Employment Consultant Speakers Bureau Ownership/ Partnership/ Principal Personal Research Institutional, Organizational, or Other Financial Benefit Expert Witness Voting Recusals by Section CLINICAL STATEMENTS AND GUIDELINES Appendix 2. Reviewer Relationships With Industry and Other Entities (Comprehensive) 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease (February 2018) Reviewer Representation Employment Consultant Speakers Bureau Ownership/ Partnership/ Principal Personal Research Institutional, Organizational, or Other Financial Benefit Expert Witness Samuel J. Asirvatham Official Reviewer AHA Mayo Clinic Professor of Medicine and Pediatrics Abiomed AtriCure Biosense Webster Biotronik Boston Scientific* Medtronic Sanofi-aventis St. Jude Medical None None None None None Wendy M. Book Official Reviewer AHA Emory University Professor of Medicine and Director of Emory Adult Congenital Heart Center, Department of Medicine None None None None Actelion Defendant, congenital heart disease, Samuel S. Gidding Official Reviewer ACC/ AHA Task Force on Clinical Practice Guidelines Nemours Cardiac Center DuPont Hospital for Children Chief, Division of Pediatric Cardiology None None None None None None Yuli Y. Kim Official Reviewer AHA University of Pennsylvania Assistant Professor of Medicine; Children s Hospital of Philadelphia Medical Director, Philadelphia Adult Congenital Heart Center None None None None None None Geetha Raghuveer Official Reviewer ACC Board of Governors Children s Mercy Hospital Pediatric Cardiologist; University of Missouri, Kansas City School of Medicine Professor of Pediatrics None None None None None None Carole A. Warnes Official Reviewer ACC Board of Trustees Mayo Clinic Professor of Medicine None None None None None None Peter J. Bartz Organizational Reviewer ASE Children s Hospital of Wisconsin Associate Professor, Medical College of Wisconsin None None None None None None Mitchell I. Cohen Organizational Reviewer HRS Inova Fairfax Children s Hospital Co- Director of the Heart Center and Chief, Pediatric Cardiology None None None None None None Marshall L. Jacobs Organizational Reviewer AATS Johns Hopkins School of Medicine Professor of Surgery and Director, Pediatric Heart Surgery Outcomes Research None None None None None None Larry A. Latson Organizational Reviewer SCAI Joe DiMaggio Children s Hospital Heart Institute Medical Director, Pediatric and Congenital Interventional Cardiology Gore Medical None None None St. Jude Medical None Constantine Mavroudis Organizational Reviewer STS Florida Hospital for Children Medical Director, Pediatric and Congenital Heart Center None None None None None None Doff B. McElhinney Organizational Reviewer SCAI Stanford University Professor, Cardiothoracic Surgery and of Pediatrics Medtronic* None None None None None Erwin N. Oechslin Organizational Reviewer ISACHD University of Toronto Professor of Medicine; Peter Munk Cardiac Centre Director, Adult Congenital Heart Disease Program Actelion None None None None None John K. Triedman Organizational Reviewer HRS Boston Children s Hospital Senior Associate in Cardiology; Harvard Medical School Professor of Pediatrics Biosense Webster None None None None None Sana M. Al-Khatib Content Reviewer ACC/ AHA Task Force on Clinical Practice Guidelines Duke University Medical Center Professor of Medicine None None None None None None Naser Ammash Content Reviewer Mayo Clinic Professor of Medicine None None None None None None Helmut Baumgartner Content Reviewer University of Muenster Professor of Cardiology and Adult Congenital Heart Disease; University Hospital Muenster Director, Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine None None None None None None (Continued) CLINICAL STATEMENTS AND GUIDELINES James C. Blankenship Content Reviewer ACC Interventional Section Leadership Council Geisinger Medical Center Staff Physician and Director, Cardiac Catheterization Laboratory None None None None Abbott Vascular Boston Scientific GlaxoSmithKline Takeda Pharmaceutical None Ralph G. Brindis Content Reviewer ACC/ AHA Task Force on Clinical Practice Guidelines Northern California Kaiser Permanente Senior Advisor, Cardiovascular Disease; University of California, San Francisco Clinical Professor of Medicine None None None None None None Robert M. Campbell Content Reviewer Emory University School of Medicine, Sibley Heart Center Cardiology Professor of Pediatrics None None None None None None Lesley H. Curtis Content Reviewer ACC/ AHA Task Force on Clinical Practice Guidelines Duke University School of Medicine Professor of Medicine None None None None Boston Scientific* GE Healthcare* GlaxoSmithKline* Medtronic* Novartis* None Kristi K. Fitzgerald Content Reviewer Nemours Cardiac Center DuPont Hospital for Children Genetic Counselor, Division of Pediatric Cardiology None None None None None None Lee A. Fleisher Content Reviewer ACC/ AHA Task Force on Clinical Practice Guidelines University of Pennsylvania Health System Department of Anesthesiology and Critical Care Robert Dunning Dripps Professor of Anesthesiology None None None None None None Federico Gentile Content Reviewer ACC/ AHA Task Force on Clinical Practice Guidelines Centro Cardiologico Gentile Director, Cardiovascular Disease None None None None None None Louise Harris Content Reviewer Toronto General Hospital Professor of Medicine St. Jude Medical None None None None None Mark A. Hlatky Content Reviewer ACC/ AHA Task Force on Clinical Practice Guidelines Stanford University School of Medicine Professor of Health Research and Policy, and of Cardiovascular Medicine None None None None None None Craig T. January Content Reviewer University of Wisconsin School of Medicine and Public Health Professor, Division of Cardiovascular Medicine None None None None None None Jos A. Joglar Content Reviewer ACC/ AHA Task Force on Clinical Practice Guidelines UT Southwestern Medical Center Professor of Internal Medicine None None None None None None Thomas K. Jones Content Reviewer Seattle Children s Hospital Professor of Pediatrics and Director, Cardiac Catheterization Laboratories Gore Medical* Medtronic* None None None Gore Medical* Medtronic* St. Jude Medical* None Brian E. Kogon Content Reviewer Emory University School of Medicine Associate Professor, Surgery and Surgical Director of Emory Adult Congenital Heart Center and Chief of Pediatric Cardiac Surgery None None None None None None Gautam Kumar Content Reviewer ACC Interventional Section Leadership Council Emory University School of Medicine, Division of Cardiology Associate Professor of Medicine Abiomed None None None OrbusNeich Medical None Eric V. Krieger Content Reviewer University of Washington Associate Professor of Medicine and Associate Director, Adult Congenital Heart Service Actelion None None None None None Glenn N. Levine Content Reviewer ACC/ AHA Task Force on Clinical Practice Guidelines Baylor College of Medicine Professor of Medicine; Michael E. DeBakey VA Medical Center Director, Cardiac Care Unit None None None None None None C. Huie Lin Content Reviewer SCAI Houston Methodist DeBakey Heart & Vascular Center Cardiologist Gore Medical ACI Clinical (DSMB) Abiomed None St. Jude Medical None None Massimo Mancone Content Reviewer ACC AIG Sapienza University of Rome Cardiology Consultant None None None None None None Appendix 2. Continued Reviewer Representation Employment Consultant Speakers Bureau Ownership/ Partnership/ Principal Personal Research Institutional, Organizational, or Other Financial Benefit Expert Witness (Continued) CLINICAL STATEMENTS AND GUIDELINES Ariane Marelli Content Reviewer McGill University Health Center Professor of Medicine and Director, MAUDE Unit None None None None None None Koichiro Niwa Content Reviewer St. Luke s International Hospital Director, Department of Cardiology None None None None None None Matthew Oster Content Reviewer Emory University School of Medicine Associate Professor; Children s Healthcare of Atlanta Director, Children s Cardiac Outcomes Research Program at Sibley Heart Center None None None None None None Catherine M. Otto Content Reviewer University of Washington School of Medicine Professor of Medicine, Division of Cardiology and Director, Heart Valve Clinic None None None None None None Richard L. Page Content Reviewer University of Wisconsin School of Medicine and Public Health Chair, Department of Medicine None None None None None None James Perry Content Reviewer Rady Children s Hospital; University of California, San Diego Professor of Pediatrics, Affiliate Professor of Bioengineering, and Director, Electrophysiology and Adult Congenital Heart Programs None None None None None None Susan J. Pressler Content Reviewer ACC/ AHA Task Force on Clinical Practice Guidelines Indiana University School of Nursing Professor and Director, Center for Enhancing Quality of Life in Chronic Illness None None None None Pfizer None Candice K. Silversides Content Reviewer University of Toronto Associate Professor None None None None None None Duminda N. Wijeysundera Content Reviewer ACC/ AHA Task Force on Clinical Practice Guidelines University of Toronto Assistant Professor, Department of Anesthesia and Institute of Health Policy Management and Evaluation None None None None None None Ali N. Zaidi Content Reviewer Montefiore Einstein Center for Heart and Vascular Care Director, Montefiore Adult Congenital Heart Disease Program; Albert Einstein College of Medicine Associate Professor, Internal Medicine and Pediatrics None None None None None None Elisa Zaragoza- Macias Content Reviewer PeaceHealth North Cascade Cardiology Cardiologist None None None None None None This table represents the relationships of reviewers with industry and other entities that were disclosed at the time of peer review and determined to be relevant to this document. It does not necessarily reflect relationships with industry at the time of publication. A person is deemed to have a significant interest in a business if the interest represents ownership of >5% of the voting stock or share of the business entity, or ownership of >$5000 of the fair market value of the business entity; or if funds received by the person from the business entity exceed 5% of the person s gross income for the previous year. A relationship is considered to be modest if it is less than significant under the preceding definition. Relationships that exist with no financial benefit are also included for the purpose of transparency. Relationships in this table are modest unless otherwise noted. Names are listed in alphabetical order within each category of review. Please refer to http://www.acc.org/guidelines/about-guidelines-and-clinical- documents/relationships-with-industry-policy for definitions of disclosure categories or additional information about the ACC/AHA Disclosure Policy for Writing Committees. **Significant relationship. No financial benefit. AATS indicates American Association for Thoracic Surgery; ACC, American College of Cardiology; AHA, American Heart Association; AIG, Assembly of International Governors; ASE, American Society of Echocardiography; DSMB, Data Safety Monitoring Board; HRS, Heart Rhythm Society; ISACHD, International Society for Adult Congenital Heart Disease; MAUDE, McGill Adult Unit for Congenital Heart Disease Excellence; SCAI, Society for Cardiovascular Angiography and Interventions; and STS, Society of Thoracic Surgeons; and UT, University of Texas. Appendix 2. Continued Reviewer Representation Employment Consultant Speakers Bureau Ownership/ Partnership/ Principal Personal Research Institutional, Organizational, or Other Financial Benefit Expert Witness