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.


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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.


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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.


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.


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.


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.


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.


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.


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.


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.


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.


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


Key Words: AHA Scientific Statements acute coronary syndrome ambulatory ECG monitoring antiarrhythmic drug therapy arrhythmogenic cardiomyopathy athletes cardiac electrophysiology cardiac resynchronization therapy cardiomyopathy catheter ablation congenital heart disease CT imaging ECG echocardiography electrophysiological testing genetic arrhythmias guidelines heart failure imaging implantable cardioverter- defibrillator implantable and external cardioverter devices medication- induced arrhythmias MR imaging myocardial infarction premature ventricular beats resuscitation sarcoidosis specific pathology (eg, congenital heart disease, myocarditis, renal failure) stable coronary artery disease sudden cardiac arrest sudden cardiac death torsades de pointes ventricular fibrillation ventricular tachycardia WRITING COMMITTEE MEMBERS Sana M. Al-Khatib, MD, MHS, FACC, FAHA, FHRS, Chair William G. Stevenson, MD, FACC, FAHA, FHRS, Vice Chair* Michael J. Ackerman, MD, PhD* William J. Bryant, JD, LLM David J. Callans, MD, FACC, FHRS* Anne B. Curtis, MD, FACC, FAHA, FHRS* Barbara J. Deal, MD, FACC, FAHA Timm Dickfeld, MD, PhD, FHRS* Michael E. Field, MD, FACC, FAHA, FHRS Gregg C. Fonarow, MD, FACC, FAHA, FHFSA* Anne M. Gillis, MD, FHRS* Christopher B. Granger, MD, FACC, FAHA* Stephen C. Hammill, MD, FACC, FHRS Mark A. Hlatky, MD, FACC, FAHA Jos A. Joglar, MD, FACC, FAHA, FHRS G. Neal Kay, MD Daniel D. Matlock, MD, MPH Robert J. Myerburg, MD, FACC Richard L. Page, MD, FACC, FAHA, FHRS 2017 by the American College of Cardiology Foundation, the American Heart Association, Inc., and the Heart Rhythm Society. AHA/ACC/HRS GUIDELINE 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society Circulation *Writing committee members are required to recuse themselves from voting on sections to which their specific rela- tionships with industry may apply; see Appendix 1 for detailed information. ACC/AHA Representative. HRS Rep- resentative.


AHA/ACC/HRS GUIDELINE 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society Circulation *Writing committee members are required to recuse themselves from voting on sections to which their specific rela- tionships with industry may apply; see Appendix 1 for detailed information. ACC/AHA Representative. HRS Rep- resentative. ACC/AHA Task Force on Performance Measures Liaison/HFSA Representative. ACC/AHA Task Force on Clinical Practice Guidelines Liaison. https://www.ahajournals.org/journal/circ ACC/AHA Task Force Members, see page e354 The American Heart Association requests that this document be cited as follows: Al-Khatib SM, Stevenson WG, Ackerman MJ, Bryant WJ, Callans DJ, Curtis AB, Deal BJ, Dickfeld T, Field ME, Fonarow GC, Gillis AM, Granger CB, Hammill SC, Hlatky MA, Joglar JA, Kay GN, Matlock DD, Myerburg RJ, Page RL. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2018;138:e272 e391. doi: 10.1161/CIR.0000000000000549. Developed in Collaboration With the Heart Failure Society of America CLINICAL STATEMENTS AND GUIDELINES TABLE OF CONTENTS Preamble e274 1. Introduction e275 1.1. Methodology and Evidence Review e275 1.2. Organization of the Writing Committee e276 1.3. Document Review and Approval e276 1.4. Scope of the Guideline e277 1.5. Abbreviations e279 2. Epidemiology e279 2.1. General Concepts e279 2.1.1. Premature Ventricular Complexes and Nonsustained VT e279 2.1.2. VT and VF During ACS e281 2.1.3. Sustained VT and VF Not Associated With ACS e281 2.2. Sudden Cardiac Death e281 2.2.1. Incidence of SCD e281 2.2.2. Population Subgroups and Risk Prediction e282 3. Mechanisms of VA e283 3.1.


Population Subgroups and Risk Prediction e282 3. Mechanisms of VA e283 3.1. Cellular Mechanisms and Substrates e283 3.2. Automaticity e283 3.3. Triggered Activity e283 3.4. Reentry e284 4. General Evaluation of Patients With Documented or Suspected VA e284 4.1. History and Physical Examination e284 4.2. Noninvasive Evaluation e286 4.2.1. 12-lead ECG and Exercise Testing e286 4.2.2. Ambulatory Electrocardiography e286 4.2.3. Implanted Cardiac Monitors e287 4.2.4. Noninvasive Cardiac Imaging e287 4.2.5. Biomarkers e288 4.2.6. Genetic Considerations in Arrhythmia Syndromes e288 4.3. Invasive Testing e289 4.3.1. Invasive Cardiac Imaging: Cardiac Catheterization or CT Angiography e289 4.3.2. Electrophysiological Study for VA e289 5. Therapies for Treatment or Prevention of VA e290 5.1. Medication Therapy e290 5.1.1. Medications With Prominent Sodium Channel Blockade e290 5.1.2. Beta Blockers e293 5.1.3. Amiodarone and Sotalol e293 5.1.4. Calcium Channel Blockers e294 5.1.5. Nonantiarrhythmic Medications and Therapies e294 5.2. Preventing SCD With HF Medications e295 5.3. Defibrillators for Treatment of VA and SCD e295 5.4. Catheter Ablation e295 5.4.1. General Considerations e295 5.4.2. VA in Patients With No Apparent Structural Heart Disease e296 5.4.3. Scar-Related VT e296 5.5. Surgery and Revascularization Procedures in Patients With Ischemic Heart Disease e296 5.5.1. Surgery for Arrhythmia Management e297 5.6. Autonomic Modulation e297 6. Acute Management of Specific VA e297 7. Ongoing Management of VA and SCD Risk Related to Specific Disease States e301 7.1. Ischemic Heart Disease e301 7.1.1. Secondary Prevention of SCD in Patients With Ischemic Heart Disease e301 7.1.2. Primary Prevention of SCD in Patients With Ischemic Heart Disease e304 7.1.3. Treatment and Prevention of Recurrent VA in Patients With Ischemic Heart Disease e306 7.2. Nonischemic Cardiomyopathy e308 7.2.1. Secondary Prevention of SCD in Patients With NICM e308 7.2.2. Primary Prevention of SCD in Patients With NICM e309 7.2.3. Treatment of Recurrent VA in Patients With NICM e310 7.3.


Treatment of Recurrent VA in Patients With NICM e310 7.3. Arrhythmogenic Right Ventricular Cardiomyopathy e312 7.4. Hypertrophic Cardiomyopathy e315 7.5. Myocarditis e318 7.6. Cardiac Sarcoidosis e318 7.6.1. Other Infiltrative Cardiomyopathies e320 7.7. Heart Failure e321 7.7.1. HF With Reduced Ejection Fraction e321 7.7.2. HF With Preserved Ejection Fraction e321 7.7.3. Left Ventricular Assist Device e322 7.7.4. ICD Use After Heart Transplantation e322 7.8. Neuromuscular Disorders e322 7.9. Cardiac Channelopathies e324 7.9.1. Specific Cardiac Channelopathy Syndromes e325 8. VA in the Structurally Normal Heart e334 8.1. Outflow Tract and Atrioventricular Annular VA e335 8.2. Papillary Muscle VA e335 8.3. Interfascicular Reentrant VT (Belhassen Tachycardia) e335 8.4. Idiopathic Polymorphic VT/VF e336 9. PVC-Induced Cardiomyopathy e337 10. VA and SCD Related to Specific Populations e338 10.1. Athletes e338 10.2. Pregnancy e338 10.3. Older Patients With Comorbidities e339 10.4. Chronic Kidney Disease e340 10.5. Valvular Heart Disease e340 10.6. Sex-Related Differences in the Risk of SCD e340 10.7. Medication-Induced Arrhythmias e341 10.8. Adult Congenital Heart Disease e342 11. Defibrillators Other than Transvenous ICDs e347 11.1. Subcutaneous Implantable Cardioverter-Defibrillator e347 11.2. Wearable Cardioverter-Defibrillator e348 11.3. Automated External Defibrillator e349 12. Special Considerations for Catheter Ablation e349 13. Postmortem Evaluation of SCD e350 14. Terminal Care e351 15. Shared Decision-Making e351 CLINICAL STATEMENTS AND GUIDELINES 16. Cost and Value Considerations e352 17. Quality of Life e353 18. Evidence Gaps and Future Research Needs e354 Appendix 1: Author Relationships With Industry and Other Entities (Relevant) e387 Appendix 2: Reviewer Relationships With Industry and Other Entities (Comprehensive) e389 PREAMBLE Since 1980, the American College of Cardiology (ACC) and American Heart Association (AHA) have translated scientific evidence into clinical practice guidelines with recommendations to improve cardiovascular health. These guidelines, which are based on systematic meth- ods to evaluate and classify evidence, provide a corner- stone for quality cardiovascular care.


These guidelines, which are based on systematic meth- ods to evaluate and classify evidence, provide a corner- stone for quality cardiovascular care. The ACC and AHA sponsor the development and publication of guidelines without commercial support, and members of each or- ganization volunteer their time to the writing and review efforts. Guidelines are official policy of the ACC and AHA. Intended Use Practice guidelines provide recommendations applica- ble to patients with or at risk of developing cardiovas- cular disease. The focus is on medical practice in the United States, but guidelines developed in collabora- tion with other organizations may have a global impact. Although guidelines may be used to inform regulatory or payer decisions, their intent is to improve patients quality of care and align with patients interests. Guide- lines are intended to define practices meeting the needs of patients in most, but not all, circumstances and should not replace clinical judgment. Clinical Implementation Guideline-recommended management is effective only when followed by healthcare providers and patients. Adherence to recommendations can be enhanced by shared decision-making between healthcare provid- ers and patients, with patient engagement in selecting interventions based on individual values, preferences, and associated conditions and comorbidities. Methodology and Modernization The ACC/AHA Task Force on Clinical Practice Guidelines (Task Force) continuously reviews, updates, and modi- fies guideline methodology on the basis of published standards from organizations including the Institute of and on the basis of internal reevaluation. Similarly, the presentation and delivery of guidelines are reevaluated and modified on the basis of evolving tech- nologies and other factors to facilitate optimal dissemi- nation of information at the point of care to healthcare professionals. Toward this goal, this guideline heralds the introduction of an evolved format of presenting guideline recommenda- tions and associated text called the modular knowledge chunk format.


Toward this goal, this guideline heralds the introduction of an evolved format of presenting guideline recommenda- tions and associated text called the modular knowledge chunk format. Each modular chunk includes a table of related recommendations, a brief synopsis, recommenda- tion-specific supportive text and, when appropriate, flow diagrams or additional tables. References are provided within the modular chunk itself to facilitate quick review. This format also will facilitate seamless updating of guide- lines with focused updates as new evidence is published, and content tagging for rapid electronic retrieval of related recommendations on a topic of interest. This evolved for- mat was instituted when this guideline was near comple- tion; therefore, the current document represents a transi- tional formatting that best suits the text as written. Future guidelines will fully implement this format, including provisions for limiting the amount of text in a guideline. Recognizing the importance of cost value consider- ations in certain guidelines, when appropriate and fea- sible, an analysis of the value of a medication, device, or intervention may be performed in accordance with the To ensure that guideline recommendations remain current, new data are reviewed on an ongoing basis, with full guideline revisions commissioned in approxi- mately 6-year cycles. Publication of new, potentially practice-changing study results that are relevant to an existing or new medication, device, or management strategy will prompt evaluation by the Task Force, in consultation with the relevant guideline writing com- mittee, to determine whether a focused update should be commissioned. For additional information and poli- cies regarding guideline development, we encourage readers to consult the ACC/AHA guideline methodol- and other methodology articles.P-5 P-8 Selection of Writing Committee Members The Task Force strives to avoid bias by selecting experts from a broad array of backgrounds.


For additional information and poli- cies regarding guideline development, we encourage readers to consult the ACC/AHA guideline methodol- and other methodology articles.P-5 P-8 Selection of Writing Committee Members The Task Force strives to avoid bias by selecting experts from a broad array of backgrounds. Writing commit- tee members represent different geographic regions, sexes, ethnicities, races, intellectual perspectives/biases, and scopes of clinical practice. The Task Force may also invite organizations and professional societies with re- lated interests and expertise to participate as partners, collaborators, or endorsers. Relationships With Industry and Other Entities The ACC and AHA have rigorous policies and methods to ensure that guidelines are developed without bias or improper influence. The complete relationships with industry and other entities (RWI) policy can be found CLINICAL STATEMENTS AND GUIDELINES online. Appendix 1 of the current document lists writing committee members relevant RWI. For the purposes of full transparency, writing committee members compre- hensive disclosure information is available online, as is the comprehensive disclosure information for the Task Force. Evidence Review and Evidence Review Committees When developing recommendations, the writing com- mittee uses evidence-based methodologies that are based on all available data.P-4 P-7 Literature searches focus on randomized controlled trials (RCTs) but also include registries, nonrandomized comparative and descriptive studies, case series, cohort studies, systematic reviews, and expert opinion. Only key references are cited. An independent evidence review committee (ERC) is commissioned when there are 1 questions deemed of utmost clinical importance that merit formal systematic review. This systematic review will strive to determine which patients are most likely to benefit from a test, med- ication, device, or treatment strategy and to what degree.


This systematic review will strive to determine which patients are most likely to benefit from a test, med- ication, device, or treatment strategy and to what degree. Criteria for commissioning an ERC and formal systematic review include: a) the absence of a current authoritative systematic review; b) the feasibility of defining the ben- efit and risk in a time frame consistent with the writing of a guideline; c) the relevance to a substantial number of patients; and d) the likelihood that the findings can be translated into actionable recommendations. ERC members may include methodologists, epidemiologists, healthcare providers, and biostatisticians. When a formal systematic review has been commissioned, the recom- mendations developed by the writing committee on the basis of the systematic review are marked with SR . Guideline-Directed Management and Therapy The term guideline-directed management and therapy (GDMT) encompasses clinical evaluation, diagnostic test- ing, and pharmacological and procedural treatments. For these and all recommended medication treatment regi- mens, the reader should confirm the dosage by review- ing product insert material and evaluate the treatment regimen for contraindications and interactions. The rec- ommendations are limited to medications, devices, and treatments approved for clinical use in the United States. Class of Recommendation and Level of Evidence The Class of Recommendation (COR) indicates the strength of the recommendation, encompassing the esti- mated magnitude and certainty of benefit in proportion to risk. The Level of Evidence (LOE) rates the quality of scientific evidence that supports the intervention on the basis of the type, quantity, and consistency of data from clinical trials and other sources (Table 1).P-4,P-6,P-8 Glenn N. Levine, MD, FACC, FAHA Chair, ACC/AHA Task Force on Clinical Practice Guidelines 1.1. Methodology and Evidence Review The recommendations listed in this clinical practice guide- line are, whenever possible, evidence-based.


Methodology and Evidence Review The recommendations listed in this clinical practice guide- line are, whenever possible, evidence-based. An initial extensive evidence review, which included literature de- rived from research involving human subjects, published in English, and indexed in MEDLINE (through PubMed), EMBASE, the Cochrane Library, the Agency for Health- care Research and Quality, and other selected databas- es relevant to this guideline, was conducted from April 2016 to September 2016. Key search words included, but were not limited, to the following: sudden cardiac death, ventricular tachycardia, ventricular fibrillation, pre- mature ventricular contractions, implantable cardioverter- defibrillator, subcutaneous implantable cardioverter-defi- brillator, wearable cardioverter-defibrillator, and catheter ablation. Additional relevant studies published through March 2017, during the guideline writing process, were also considered by the writing committee, and added to the evidence tables when appropriate. The final evidence tables are included in the Online Data Supplement and summarize the evidence used by the writing committee to formulate recommendations. Additionally, the writing committee reviewed documents related to ventricular arrhythmias (VA) and sudden cardiac death (SCD) previ- ously published by the ACC, AHA, and the Heart Rhythm Society (HRS). References selected and published in this document are representative and not all-inclusive. As noted in the Preamble, an independent ERC was commissioned to perform a formal systematic review of 2 important clinical questions for which clear literature and prior guideline consensus were felt to be lacking or limited (Table 2). The results of the ERC review were considered by the writing committee for incorporation into this guide- line. Concurrent with this process, writing committee members evaluated other published data relevant to the guideline. The findings of the ERC and the writing com- mittee members were formally presented and discussed, then guideline recommendations were developed.


The findings of the ERC and the writing com- mittee members were formally presented and discussed, then guideline recommendations were developed. The Systematic Review for the 2017 AHA/ACC/HRS Guide- line for Management of Patients With Ventricular Ar- rhythmias and the Prevention of Sudden Cardiac Death is published in conjunction with this guideline.S1.4-1 The ACC and AHA have acknowledged the impor- tance of value in health care and have called for even- tual development of a Level of Value for clinical practice Available cost-effectiveness data were determined to be sufficient to support 2 specific CLINICAL STATEMENTS AND GUIDELINES recommendations in this guideline (see Sections 7.1.1 and 7.1.2). As a result, a Level of Value was assigned to those 2 recommendations on the basis of the ACC/ AHA Statement on Cost/Value Methodology in Clini- cal Practice Guidelines and Performance Measures, as shown in Table 3.S1.4-2 Available quality of life (QoL) data were deemed to be insufficient to support specific rec- ommendations in this guideline. 1.2. Organization of the Writing Committee The writing committee consisted of cardiac electro- physiologists (including those specialized in pediatrics), general adult and pediatric cardiologists (including those specialized in critical care and acute coronary syndromes [ACS], genetic cardiology, heart failure, and cost-effectiveness analyses), a geriatrician with exper- tise in terminal care and shared decision-making, and a lay representative, in addition to representatives from the ACC, AHA, HRS, and the Heart Failure Society of America (HFSA). 1.3. Document Review and Approval This document was reviewed by 2 official reviewers nominated by the ACC, AHA, and HRS; 1 official lay re- Table 1.


Document Review and Approval This document was reviewed by 2 official reviewers nominated by the ACC, AHA, and HRS; 1 official lay re- Table 1. Applying Class of Recommendation and Level of Evidence to Clinical Strategies, Interventions, Treatments, or Diagnostic Testing in Patient Care* (Updated August 2015) CLINICAL STATEMENTS AND GUIDELINES viewer nominated by the AHA; 1 organizational review- er nominated by the HFSA; and 28 individual content reviewers. Reviewers RWI information was distributed to the writing committee and is published in this docu- ment (Appendix 2). This document was approved for publication by the governing bodies of the ACC, the AHA, and the HRS; and endorsed by the HFSA. 1.4. Scope of the Guideline The purpose of this AHA/ACC/HRS document is to pro- vide a contemporary guideline for the management of adults who have VA or who are at risk for SCD, including diseases and syndromes associated with a risk of SCD from VA. This guideline supersedes the ACC/AHA/ ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden It also supersedes some sections of the ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities, S1.4-5 specifi- cally those sections on indications for the implantable cardioverter-defibrillator (ICD); and, it updates the SCD prevention recommendations in the 2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hyper- Some recommendations from the earlier guidelines have been updated as war- ranted by new evidence or a better understanding of existing evidence, and irrelevant or overlapping recom- mendations were deleted or modified. In the current guideline, sudden cardiac arrest (SCA) is defined as the sudden cessation of cardiac activity so that the victim becomes unresponsive, with no normal breathing and no signs of circulation. S1.4-7 If corrective measures are not taken rapidly, this condition progresses to SCD.


S1.4-7 If corrective measures are not taken rapidly, this condition progresses to SCD. Cardiac arrest is used to signify an event that can be reversed, usually by cardiopulmonary resuscitation (CPR), administration of medications and/or defibrillation or cardioversion. SCA and SCD can result from causes other than VA, such as bradyarrhythmias, electrome- chanical dissociation, pulmonary embolism, intracranial hemorrhage, and aortic dissection; however, the scope of this document includes only SCA and SCD due to VA. This guideline includes indications for ICDs for the treatment of VA and prevention of SCD, but it does not delve into details on individual device selection and programming, including considerations relevant to car- diac resynchronization therapy (CRT), bradycardia pac- ing, and hemodynamic monitoring. These important aspects of ICD management have been covered in an HRS expert consensus statement.S1.4-8 An AHA science advisory discusses the use of wearable cardioverter- The findings of that document were reviewed; however, recommendations on this topic were developed independently of that document. This guideline includes indications for catheter ablation of VA, but does not provide recommendations on specific techniques or ablation technologies, which were be- yond the scope of this document. Recommendations for interventional therapies, in- cluding ablation and the implantation of devices, apply only if these therapies can be implemented by quali- fied clinicians, such that outcomes consistent with pub- lished literature are a reasonable expectation. The writ- ing committee agreed that a high degree of expertise was particularly important for performance of catheter ablation of VA, and this point is further emphasized in relevant sections. In addition, all recommendations re- lated to ICDs require that meaningful survival of >1 year is expected; meaningful survival means that a patient has a reasonable quality of life and functional status.


In addition, all recommendations re- lated to ICDs require that meaningful survival of >1 year is expected; meaningful survival means that a patient has a reasonable quality of life and functional status. Although this document is aimed at the adult popu- lation ( 18 years of age) and offers no specific recom- mendations for pediatric patients, some of the litera- ture on pediatric patients was examined. In some cases, the data from pediatric patients beyond infancy helped to inform this guideline. The writing committee recognized the importance of shared decision-making and patient-centered care and, when possible, it endeavored to formulate recommenda- Table 2. Systematic Review Questions on SCD Prevention Question Number Question Section Number 1 For asymptomatic patients with Brugada syndrome, what is the association between an abnormal programmed ventricular stimulation study and SCD and other arrhythmia endpoints? 2 What is the impact of ICD implantation for primary prevention in older patients and patients with significant comorbidities? ICD indicates implantable cardioverter-defibrillator; and SCD, sudden cardiac death. Table 3.


ICD indicates implantable cardioverter-defibrillator; and SCD, sudden cardiac death. Table 3. Proposed Integration of Level of Value Into Clinical Practice Guideline Recommendations* Level of Value High value: Better outcomes at lower cost or ICER <$50000 per QALY gained Intermediate value: $50000 to <$150000 per QALY gained Low value: $150000 per QALY gained Uncertain value: Value examined but data are insufficient to draw a conclusion because of no studies, low-quality studies, conflicting studies, or prior studies that are no longer relevant Not assessed: Value not assessed by the writing committee Proposed abbreviations for each value recommendation: Level of Value: H to indicate high value; I, intermediate value; L, low value; U, uncertain value; and NA, value not assessed *Dollar amounts used in this table are based on US GDP data from 2012 and were obtained from WHO-CHOICE Cost-Effectiveness Thresholds.S1.4-3 GDP indicates gross domestic product; ICER, incremental cost-effectiveness ratio; QALY, quality-adjusted life-years; and WHO-CHOICE, World Health Organization Choosing Interventions that are Cost-Effective. Reproduced from Anderson, et al.S1.4-2 CLINICAL STATEMENTS AND GUIDELINES tions relevant to these important concepts. The importance of a shared decision-making process in which the patient, family, and clinicians discuss risks and benefits of diag- nostic and treatment options and consider the patients personal preferences is emphasized (see Section 15). In developing this guideline, the writing committee reviewed previously published guidelines and related statements. Table 4 contains a list of guidelines and statements deemed pertinent to this writing effort and is intended for use as a resource, obviating repetition of existing guideline recommendations. During final production review of the guidelines, several recommendations were refined to better reflect the data and current recommended medical practice. These refinements were reviewed and approved by the writing committee, the Task Force, and ACC, AHA, and HRS organizational leadership.


These refinements were reviewed and approved by the writing committee, the Task Force, and ACC, AHA, and HRS organizational leadership. These recommendations were: Section 7.1.1., recommendation 1 Section 7.1.3., recommendation 2 Section 7.2.1., recommendation 1 Section 7.9.1.4., recommendation 2 Section 10.8., recommendation 6 Readers should refer to these sections for the up- dated text. Table 4.


These recommendations were: Section 7.1.1., recommendation 1 Section 7.1.3., recommendation 2 Section 7.2.1., recommendation 1 Section 7.9.1.4., recommendation 2 Section 10.8., recommendation 6 Readers should refer to these sections for the up- dated text. Table 4. Associated Guidelines and Statements Title Organization Publication Year (Reference) Guidelines Syncope ACC/AHA/HRS 2017S1.4 10 Heart failure ACCF/AHA 2017S1.4 11 and Valvular heart disease AHA/ACC 2017S1.4 14 Supraventricular tachycardia ACC/AHA/HRS 2015S1.4 16 Ventricular arrhythmias and the prevention of sudden cardiac death ESC 2015S1.4 17 Guidelines for cardiopulmonary resuscitation and emergency cardiovascular care AHA 2015S1.4 18 Atrial fibrillation AHA/ACC/HRS 2014S1.4 19 Non ST-elevation acute coronary syndromes AHA/ACC 2014S1.4 20 Assessment of cardiovascular risk ACC/AHA 2013S1.4 21 ST-elevation myocardial infarction ACCF/AHA 2013S1.4 22 Acute myocardial infarction in patients presenting with ST-segment elevation ESC 2012S1.4 23 Device-based therapies for cardiac rhythm abnormalities ACCF/AHA/HRS 2012S1.4 24 Coronary artery bypass graft surgery ACCF/AHA 2011S1.4 25 Hypertrophic cardiomyopathy ACCF/AHA 2011S1.4 6 Percutaneous coronary intervention ACCF/AHA/SCAI 2011S1.4 26 Secondary prevention and risk reduction therapy for patients with coronary and other atherosclerotic vascular disease Scientific Statements Wearable cardioverter-defibrillator therapy for the prevention of sudden cardiac death Optimal implantable cardioverter defibrillator programming and testing HRS/EHRA/APHRS/SOLAECE 2016S1.4 8 Treatment of cardiac arrest: current status and future directions: strategies to improve cardiac arrest survival Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities Ventricular arrhythmias EHRA/HRS/APHRS 2014S1.4 30 Arrhythmias in adult congenital heart disease PACES/HRS 2014S1.4 31 Implantable cardioverter-defibrillator therapy in patients who are not included or not well represented in clinical trials Cardiac sarcoidosis HRS 2014S1.4 33 Inherited primary arrhythmia syndromes HRS/EHRA/APHRS 2013S1.4 34 ACC indicates American College of Cardiology; ACCF, American College of Cardiology Foundation; AHA, American Heart Association; APHRS, Asia Pacific Heart Rhythm Society; EHRA, European Heart Rhythm Association; ESC, European Society of Cardiology; HRS, Heart Rhythm Society; PACES, Pediatric and Congenital Electrophysiology Society; SCAI, Society for Cardiovascular Angiography and Interventions; and, SOLAECE, Sociedad Latinoamericana de Estimulacion Cardiaca y Electrofisiologia.


Associated Guidelines and Statements Title Organization Publication Year (Reference) Guidelines Syncope ACC/AHA/HRS 2017S1.4 10 Heart failure ACCF/AHA 2017S1.4 11 and Valvular heart disease AHA/ACC 2017S1.4 14 Supraventricular tachycardia ACC/AHA/HRS 2015S1.4 16 Ventricular arrhythmias and the prevention of sudden cardiac death ESC 2015S1.4 17 Guidelines for cardiopulmonary resuscitation and emergency cardiovascular care AHA 2015S1.4 18 Atrial fibrillation AHA/ACC/HRS 2014S1.4 19 Non ST-elevation acute coronary syndromes AHA/ACC 2014S1.4 20 Assessment of cardiovascular risk ACC/AHA 2013S1.4 21 ST-elevation myocardial infarction ACCF/AHA 2013S1.4 22 Acute myocardial infarction in patients presenting with ST-segment elevation ESC 2012S1.4 23 Device-based therapies for cardiac rhythm abnormalities ACCF/AHA/HRS 2012S1.4 24 Coronary artery bypass graft surgery ACCF/AHA 2011S1.4 25 Hypertrophic cardiomyopathy ACCF/AHA 2011S1.4 6 Percutaneous coronary intervention ACCF/AHA/SCAI 2011S1.4 26 Secondary prevention and risk reduction therapy for patients with coronary and other atherosclerotic vascular disease Scientific Statements Wearable cardioverter-defibrillator therapy for the prevention of sudden cardiac death Optimal implantable cardioverter defibrillator programming and testing HRS/EHRA/APHRS/SOLAECE 2016S1.4 8 Treatment of cardiac arrest: current status and future directions: strategies to improve cardiac arrest survival Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities Ventricular arrhythmias EHRA/HRS/APHRS 2014S1.4 30 Arrhythmias in adult congenital heart disease PACES/HRS 2014S1.4 31 Implantable cardioverter-defibrillator therapy in patients who are not included or not well represented in clinical trials Cardiac sarcoidosis HRS 2014S1.4 33 Inherited primary arrhythmia syndromes HRS/EHRA/APHRS 2013S1.4 34 ACC indicates American College of Cardiology; ACCF, American College of Cardiology Foundation; AHA, American Heart Association; APHRS, Asia Pacific Heart Rhythm Society; EHRA, European Heart Rhythm Association; ESC, European Society of Cardiology; HRS, Heart Rhythm Society; PACES, Pediatric and Congenital Electrophysiology Society; SCAI, Society for Cardiovascular Angiography and Interventions; and, SOLAECE, Sociedad Latinoamericana de Estimulacion Cardiaca y Electrofisiologia. CLINICAL STATEMENTS AND GUIDELINES Abbreviation Meaning/Phrase ACS acute coronary syndromes AED automated external defibrillator AMI acute myocardial infarction BNP B-type natriuretic peptide CABG coronary artery bypass graft CKD chronic kidney disease CPR cardiopulmonary resuscitation CRT cardiac resynchronization therapy CT computed tomography ECG electrocardiogram ERC evidence review committee ESRD end-stage renal disease GDMT guideline-directed management and therapy HCM hypertrophic cardiomyopathy HF heart failure HFpEF heart failure with preserved ejection fraction HFrEF heart failure with reduced ejection fraction ICD implantable cardioverter-defibrillator LV left ventricular LVAD left ventricular assist device LVEF left ventricular ejection fraction MI myocardial infarction NICM nonischemic cardiomyopathy NSVT nonsustained ventricular tachycardia PET positron emission tomography PCI percutaneous coronary intervention PVC premature ventricular complex QoL quality of life RCT randomized controlled trial RV right ventricular RVOT right ventricular outflow tract SCA sudden cardiac arrest SCD sudden cardiac death SVT supraventricular tachycardia TOF tetralogy of Fallot VA ventricular arrhythmia VT ventricular tachycardia 2.1.


CLINICAL STATEMENTS AND GUIDELINES Abbreviation Meaning/Phrase ACS acute coronary syndromes AED automated external defibrillator AMI acute myocardial infarction BNP B-type natriuretic peptide CABG coronary artery bypass graft CKD chronic kidney disease CPR cardiopulmonary resuscitation CRT cardiac resynchronization therapy CT computed tomography ECG electrocardiogram ERC evidence review committee ESRD end-stage renal disease GDMT guideline-directed management and therapy HCM hypertrophic cardiomyopathy HF heart failure HFpEF heart failure with preserved ejection fraction HFrEF heart failure with reduced ejection fraction ICD implantable cardioverter-defibrillator LV left ventricular LVAD left ventricular assist device LVEF left ventricular ejection fraction MI myocardial infarction NICM nonischemic cardiomyopathy NSVT nonsustained ventricular tachycardia PET positron emission tomography PCI percutaneous coronary intervention PVC premature ventricular complex QoL quality of life RCT randomized controlled trial RV right ventricular RVOT right ventricular outflow tract SCA sudden cardiac arrest SCD sudden cardiac death SVT supraventricular tachycardia TOF tetralogy of Fallot VA ventricular arrhythmia VT ventricular tachycardia 2.1. General Concepts VA include a spectrum that ranges from premature ventricular complex (PVC) to ventricular fibrillation (VF), with a clinical presentation that ranges from a total lack of symptoms to cardiac arrest. Most life-threatening VA are associated with ischemic heart disease, particularly in older patients.S2.2.2-1 The risks of VA and SCD vary in specific populations with different underlying cardiac conditions, and with specific family history and genetic variants, and this variation has important implications for studying and applying therapies. 2.1.1. Premature Ventricular Complexes and Nonsustained VT PVCs are common and increase in frequency with age.


Premature Ventricular Complexes and Nonsustained VT PVCs are common and increase in frequency with age. Although PVCs were found in a healthy military population in only 0.6% of those <20 years of age and 2.7% of those >50 years of ageS2.2.2-5 ECGs, longer term monitoring shows PVCs in about 50% of all people with or without heart disease.S2.2.2-6 The presence of PVCs on 2 minutes of monitoring of middle-aged patients in the ARIC (Atherosclerosis Risk In Communities) study was associated with in- creased risk of both ischemic heart disease events and mortality, with or without prevalent ischemic heart In the general population, frequent PVCs, which are defined as the presence of at least 1 PVC on a 12-lead ECG or >30 PVCs per hour, are associated with increased cardiovascular risk and in- In a study from Taiwan of pa- tients without sustained VT or structural heart disease who had 24-hour Holter monitoring for clinical evalu- ation, multifocal PVCs were associated with increased risk of death and nonfatal cardiovascular adverse In the same population, nonsustained ventricular tachycardia (NSVT) was independently as- sociated with increased risk of death and other cardio- vascular adverse outcomes, including stroke.S2.2.2-11 An association of PVCs with increased risk of stroke was also seen in the ARIC population.S2.2.2-8 Because some studies have shown an association of PVCs with adverse outcomes, the detection of PVCs, particularly if multifocal and frequent, is generally con- sideredariskfactorforadversecardiovascularoutcomes, and such patients are generally evaluated to ensure they do not have underlying conditions (eg, ischemic heart disease, left ventricular [LV] dysfunction) that warrant further treatment to reduce risk.


Although PVCs were found in a healthy military population in only 0.6% of those <20 years of age and 2.7% of those >50 years of ageS2.2.2-5 ECGs, longer term monitoring shows PVCs in about 50% of all people with or without heart disease.S2.2.2-6 The presence of PVCs on 2 minutes of monitoring of middle-aged patients in the ARIC (Atherosclerosis Risk In Communities) study was associated with in- creased risk of both ischemic heart disease events and mortality, with or without prevalent ischemic heart In the general population, frequent PVCs, which are defined as the presence of at least 1 PVC on a 12-lead ECG or >30 PVCs per hour, are associated with increased cardiovascular risk and in- In a study from Taiwan of pa- tients without sustained VT or structural heart disease who had 24-hour Holter monitoring for clinical evalu- ation, multifocal PVCs were associated with increased risk of death and nonfatal cardiovascular adverse In the same population, nonsustained ventricular tachycardia (NSVT) was independently as- sociated with increased risk of death and other cardio- vascular adverse outcomes, including stroke.S2.2.2-11 An association of PVCs with increased risk of stroke was also seen in the ARIC population.S2.2.2-8 Because some studies have shown an association of PVCs with adverse outcomes, the detection of PVCs, particularly if multifocal and frequent, is generally con- sideredariskfactorforadversecardiovascularoutcomes, and such patients are generally evaluated to ensure they do not have underlying conditions (eg, ischemic heart disease, left ventricular [LV] dysfunction) that warrant further treatment to reduce risk. PVC and NSVT in pa- tients with cardiovascular disease are common and have been associated with adverse outcomes.S2.2.2-12,S2.2.2-13 In CAST (Cardiac Arrhythmia Suppression Trials), treat- ment of patients with post-myocardial infarction (MI) who took antiarrhythmic medications (eg, flecainide, encainide, moricizine) increased the risk of death de- spite suppression of VA.S2.2.2-14,S2.2.2-15 Treatment of PVCs with antiarrhythmic medications has not been shown to reduce mortality and, in the post-MI population, treatment with class I sodium channel blocking medi- cations (eg, quinidine, flecainide) increases the risk of Likewise, in patients with a reduced LVEF class I, sodium channel blocking medications and d-sotalol increase the risk of death.S2.2.2-16,S2.2.2-17 Beta blockers, nondihydropyridines calcium channel block- CLINICAL STATEMENTS AND GUIDELINES Table 5.


PVC and NSVT in pa- tients with cardiovascular disease are common and have been associated with adverse outcomes.S2.2.2-12,S2.2.2-13 In CAST (Cardiac Arrhythmia Suppression Trials), treat- ment of patients with post-myocardial infarction (MI) who took antiarrhythmic medications (eg, flecainide, encainide, moricizine) increased the risk of death de- spite suppression of VA.S2.2.2-14,S2.2.2-15 Treatment of PVCs with antiarrhythmic medications has not been shown to reduce mortality and, in the post-MI population, treatment with class I sodium channel blocking medi- cations (eg, quinidine, flecainide) increases the risk of Likewise, in patients with a reduced LVEF class I, sodium channel blocking medications and d-sotalol increase the risk of death.S2.2.2-16,S2.2.2-17 Beta blockers, nondihydropyridines calcium channel block- CLINICAL STATEMENTS AND GUIDELINES Table 5. Table of Definitions of Commonly Used Terms in this Document Term Definition or Description Cardiac arrhythmia of 3 consecutive complexes originating in the ventricles at a rate >100 bpm (cycle length: <600 ms). Types of VT: Sustained: VT >30 s or requiring termination due to hemodynamic compromise in <30 s. Nonsustained/unsustained: 3 beats, terminating spontaneously. Monomorphic: Stable single QRS morphology from beat to beat. Polymorphic: Changing or multiform QRS morphology from beat to beat. Bidirectional: VT with a beat-to-beat alternation in the QRS frontal plane axis, often seen in the setting of digitalis toxicity or catecholaminergic polymorphic VT Monomorphic VT Polymorphic VT Bidirectional VT Torsades de pointesS2.2.2-2 Torsades de pointes is polymorphic VT that occurs in the setting of a long QT interval and is characterized by a waxing and waning QRS amplitude. It often has a long-short initiating sequence with a long coupling interval to the first VT beat and may present with salvos of NSVT.


It often has a long-short initiating sequence with a long coupling interval to the first VT beat and may present with salvos of NSVT. The twisting of the points, although characteristic, may not always be seen, especially if the episode is nonsustained or if only a limited number of leads are available. Torsades de pointes can result from bradycardia including high-grade AV block that leads to a long-short sequence initiating torsades de pointes. A regular VA 300 bpm (cycle length: 200 ms) with a sinusoidal, monomorphic appearance; no isoelectric interval between successive QRS complexes. Rapid, grossly irregular electrical activity with marked variability in electrocardiographic waveform, ventricular rate usually >300 bpm (cycle length: <200 ms). Sudden cardiac arrestS2.2.2-2 SCA is the sudden cessation of cardiac activity such that the victim becomes unresponsive, with either persisting gasping respirations or absence of any respiratory movements, and no signs of circulation as manifest by the absence of a perceptible pulse. An arrest is presumed to be of cardiac etiology unless it is known or likely to have been caused by trauma, drowning, respiratory failure or asphyxia, electrocution, drug overdose, or any other noncardiac cause. Sudden cardiac deathS2.2.2-2 Sudden and unexpected death occurring within an hour of the onset of symptoms, or occurring in patients found dead within 24 h of being asymptomatic and presumably due to a cardiac arrhythmia or hemodynamic catastrophe. VT/VF storm (electrical storm or arrhythmic storm) refers to a state of cardiac electrical instability that is defined by 3 episodes of sustained VT, VF, or appropriate shocks from an ICD within 24 h. Primary prevention ICDS2.2.2-2 ICD placement with the intention of preventing SCD in a patient who has not had sustained VT or SCA but who is at an increased risk for these events.


VT/VF storm (electrical storm or arrhythmic storm) refers to a state of cardiac electrical instability that is defined by 3 episodes of sustained VT, VF, or appropriate shocks from an ICD within 24 h. Primary prevention ICDS2.2.2-2 ICD placement with the intention of preventing SCD in a patient who has not had sustained VT or SCA but who is at an increased risk for these events. Secondary prevention ICDS2.2.2-2 ICD placement in a patient with prior SCA, sustained VT, or syncope caused by VA. Structural heart disease* This term encompasses IHD, all types of cardiomyopathy, valvular heart disease, and adult congenital heart disease. Arrhythmogenic disease due to a genetic abnormality that results in dysfunction of a cardiac ion channel (eg, long QT syndrome, catecholaminergic polymorphic VT). *The definition of this term may differ across publications. Refer to the entry for the definition used in this document. AV indicates atrioventricular; ICD, implantable cardioverter-defibrillator; IHD, ischemic heart disease; NSVT, nonsustained ventricular tachycardia; SCA, sudden cardiac arrest; SCD, sudden cardiac death; VA, ventricular arrhythmia; VF, ventricular fibrillation; and VT, ventricular tachycardia. CLINICAL STATEMENTS AND GUIDELINES ers, and some antiarrhythmic medications may relieve symptoms of palpitations.S2.2.2-18 PVCs that occur during an exercise test are associ- ated with a higher risk of death.S2.2.2-19 In 1 study, PVCs that occur during recovery are a stronger predictor of death than PVCs occurring only during exercise.S2.2.2-20 However, PVCs are common in trained athletes who have palpitations, in whom there does not appear to be increased risk of death based on studies of small num- bers of athletes, at least in those without other cardio- Complex PVCs may not represent a benign finding in endurance athletes.


CLINICAL STATEMENTS AND GUIDELINES ers, and some antiarrhythmic medications may relieve symptoms of palpitations.S2.2.2-18 PVCs that occur during an exercise test are associ- ated with a higher risk of death.S2.2.2-19 In 1 study, PVCs that occur during recovery are a stronger predictor of death than PVCs occurring only during exercise.S2.2.2-20 However, PVCs are common in trained athletes who have palpitations, in whom there does not appear to be increased risk of death based on studies of small num- bers of athletes, at least in those without other cardio- Complex PVCs may not represent a benign finding in endurance athletes. An electrophysiological study may be needed to as- sess patients arrhythmogenic risk.S2.2.2-22 Very frequent PVCs, >10000 to 20000 a day, can be associated with depressed LV function in some patients that is revers- ible with control of the PVCs, and has been referred to as PVC-induced cardiomyopathy.S2.2.2-23,S2.2.2-24 (See also Section 8.5. PVC-Induced Cardiomyopathy.) Very rarely, idiopathic PVCs from the outflow tract may trig- ger malignant VA in patients without structural heart 2.1.2. VT and VF During ACS Approximately half of patients with out-of-hospital cardiac arrest with the first rhythm identified as VF and who survive to hospital admission have evidence of acute MI (AMI).S2.2.2-27 Of all out-of-hospital cardiac arrests, >50% will have significant coronary artery le- sions on acute coronary angiography.S2.2.2-27 Of patients hospitalized with AMI, 5% to 10% have VF or sus- tained VT prior to hospital presentation, and another 5% will have VF or sustained VT after hospital arrival, most within 48 hours of admission.


VT and VF During ACS Approximately half of patients with out-of-hospital cardiac arrest with the first rhythm identified as VF and who survive to hospital admission have evidence of acute MI (AMI).S2.2.2-27 Of all out-of-hospital cardiac arrests, >50% will have significant coronary artery le- sions on acute coronary angiography.S2.2.2-27 Of patients hospitalized with AMI, 5% to 10% have VF or sus- tained VT prior to hospital presentation, and another 5% will have VF or sustained VT after hospital arrival, most within 48 hours of admission. A study of patients with non ST-elevation ACS who underwent cardiac catheterization within 48 hours found VT/VF in 7.6% of patients, with 60% of those events within 48 hours Accelerated idioventricular rhythm is a common arrhythmia in patients with acute MI, including patients with ST-segment elevation MI un- dergoing primary percutaneous coronary intervention (PCI). Accelerated idioventricular rhythm is more closely related to the extent of infarction than to reperfusion Sustained VA that occurs in the setting of an ACS is more often polymorphic VT or VF than monomorphic VT. Risk factors for VT/VF include prior history of hy- pertension, prior MI, ST-segment changes at presenta- tion, and chronic obstructive pulmonary disease.S2.2.2-30 A nationwide Danish study found that 11.6% of pa- tients with ST-segment elevation MI who underwent PCI had VF prior to the PCI, and that VF was associ- ated with alcohol consumption, preinfarction angina, anterior infarct location, and complete coronary occlu- sion at the time of coronary angiography.S2.2.2-31 In a select group of patients undergoing primary PCI in a clinical trial, 5.7% developed sustained VT or VF, with two thirds of these events occurring prior to the end of the catheterization, and 90% within 48 hours from the procedure.


Accelerated idioventricular rhythm is more closely related to the extent of infarction than to reperfusion Sustained VA that occurs in the setting of an ACS is more often polymorphic VT or VF than monomorphic VT. Risk factors for VT/VF include prior history of hy- pertension, prior MI, ST-segment changes at presenta- tion, and chronic obstructive pulmonary disease.S2.2.2-30 A nationwide Danish study found that 11.6% of pa- tients with ST-segment elevation MI who underwent PCI had VF prior to the PCI, and that VF was associ- ated with alcohol consumption, preinfarction angina, anterior infarct location, and complete coronary occlu- sion at the time of coronary angiography.S2.2.2-31 In a select group of patients undergoing primary PCI in a clinical trial, 5.7% developed sustained VT or VF, with two thirds of these events occurring prior to the end of the catheterization, and 90% within 48 hours from the procedure. VT or VF after primary PCI was associ- ated with lower blood pressure, higher heart rate, poor coronary flow at the end of the procedure, and incom- plete resolution of ST elevation.S2.2.2-32 Importantly, and in contrast to some earlier studies, VT or VF at any time was associated with a substantially higher risk of death within 90 days. Late VT or VF (after 48 hours of hos- pital presentation) was associated with a higher risk of death than early VT or VF (within 48 hours of hospital 2.1.3. Sustained VT and VF Not Associated With ACS Patients with structural heart disease are at an in- creased risk for sustained VT and VF.


Sustained VT and VF Not Associated With ACS Patients with structural heart disease are at an in- creased risk for sustained VT and VF. Sustained VT that is not associated with an ACS is often monomorphic as it is usually due to scar-related reentry, but it may degenerate to VF.S2.2.2-34 The risk and predictors of VT in patients with structural heart disease depend on the type, severity, and duration of structural heart disease, increasing with the severity of ventricular dysfunc- tion and the presence of symptomatic HF. Monomor- phic VT occurring in the absence of structural heart disease is commonly referred to as idiopathic VT and is often due to an automatic focus in a characteris- tic location, giving rise to typical electrocardiographic appearances. Polymorphic VT and VF occurring in the absence of structural heart disease are rare and may be due to a cardiac channelopathy,S2.2.2-35,S2.2.2-36 med- ication-induced long QT syndrome,S2.2.2-36 or they may 2.2. Sudden Cardiac Death 2.2.1. Incidence of SCD SCA and its most common consequence, SCD, constitute major public health problems, account- ing for approximately 50% of all cardiovascular with at least 25% being first symp- tomatic cardiac events.S2.2.2-1,S2.2.2-40,S2.2.2-41 In addition, analyses of the magnitude of SCD are limited, in part because of the broad range of estimates of the risk based on different epidemiological methods.S2.2.2-42 During the past 20 to 30 years, SCD accounted for in the United States, with a range of <170 000 to >450 000, depending on epidemiological methods, data sources, and inclusion criteria.S2.2.2-41,S2.2.2-43 The lowest of these extremes came from national extrapo- lation of data from specific local programs, while the highest rates included noncardiac causes of sudden death such as pulmonary embolism or intracranial bleeding.


Incidence of SCD SCA and its most common consequence, SCD, constitute major public health problems, account- ing for approximately 50% of all cardiovascular with at least 25% being first symp- tomatic cardiac events.S2.2.2-1,S2.2.2-40,S2.2.2-41 In addition, analyses of the magnitude of SCD are limited, in part because of the broad range of estimates of the risk based on different epidemiological methods.S2.2.2-42 During the past 20 to 30 years, SCD accounted for in the United States, with a range of <170 000 to >450 000, depending on epidemiological methods, data sources, and inclusion criteria.S2.2.2-41,S2.2.2-43 The lowest of these extremes came from national extrapo- lation of data from specific local programs, while the highest rates included noncardiac causes of sudden death such as pulmonary embolism or intracranial bleeding. The mid-range numbers were largely based CLINICAL STATEMENTS AND GUIDELINES on death certificate studies that required a code inclu- sive of ischemic heart disease. The 2017 update of cardiovascular statistics from the AHA estimated the total annual burden of out- of-hospital cardiac arrest at 356500.S2.2.2-44 An ad- ditional 209000 in-hospital cardiac arrests occur Among the out-of-hospital cardiac arrest group, approximately 357000 events trigger emergency rescue response, with 97% occurring in adults >18 years of age. The survival statistics for out-of-hospital cardiac ar- rest remain disappointing, with an estimated 10% overall survival rate.S2.2.2-44 Among the subgroup of 70% of out-of-hospital cardiac arrests that occur in the home, survival is 6%.


The survival statistics for out-of-hospital cardiac ar- rest remain disappointing, with an estimated 10% overall survival rate.S2.2.2-44 Among the subgroup of 70% of out-of-hospital cardiac arrests that occur in the home, survival is 6%. The best reported outcomes are from locations with highly developed and publicly vis- ible emergency rescue response, along with the com- bination of public location of cardiac arrest, bystander witnesses willing to provide CPR, first responders arriv- ing quickly, shockable rhythm at initial contact, avail- ability of automated external defibrillators (AEDs), and possibly a benefit from telecommunication-directed Survival to hospital discharge after in- hospital cardiac arrests is estimated to be 24%.S2.2.2-48 In all settings, survival statistics appear to be better when rhythms recorded by responders are shockable (VF, pulseless VT), compared with pulseless electrical activ- ity or asystole.S2.2.2-49 Although the apparent increase in the incidence of pulseless electrical activity or asystole could be due to the later arrival of medical care, the decrease in the incidence of shockable rhythm has also been attributed, in part, to improvements in diagnosis and treatment of structural heart disease.S2.2.2-40 Figure 1A. SCD incidence and total events.S2.2.2-1 EF indicates ejection fraction; and SCD, sudden cardiac death. Figure 1B. SCD and clinical subsets.S2.2.2-1 SCD indicates sudden cardiac death. CLINICAL STATEMENTS AND GUIDELINES 2.2.2. Population Subgroups and Risk Prediction Risk prediction for SCA and SCD is complex. Risk analy- sis is divided into 2 general categories: population risk prediction and individual risk prediction.S2.2.2-41,S2.2.2-50 Conventional epidemiological markers provide insight into probabilities for the development of ischemic heart disease within a general class of subjects, but ade- quately tested and validated profiles for SCA risk strati- fication of individuals in the general population do not presently exist.


Risk analy- sis is divided into 2 general categories: population risk prediction and individual risk prediction.S2.2.2-41,S2.2.2-50 Conventional epidemiological markers provide insight into probabilities for the development of ischemic heart disease within a general class of subjects, but ade- quately tested and validated profiles for SCA risk strati- fication of individuals in the general population do not presently exist. The challenge of defining SCA risk in individuals derives from a population model character- ized by large numbers of events diluted into a very large denominator (Figure 1). The overall population can be subgrouped into categories based on integration of age, presence and extent of disease, and identification of small, high-risk subgroups within the large denomi- nator general population. Increasing age is a strong predictor of risk for SCA, but it is not linear. Risk in the general population, over time, beginning at 35 years of age has been estimated at 1 per 1000 population per year, increasing from a risk <1000 at the younger end of that spectrum to a higher risk in the elderly.S2.2.2-41 However, an analysis of lifetime risk of SCD, derived from the Framingham data, suggested that the incidence of SCD decreases in later years, especially in people >75 years of age.S2.2.2-51 The data also suggested that SCD is uniformly more common in men than in women at all age groups.


Risk in the general population, over time, beginning at 35 years of age has been estimated at 1 per 1000 population per year, increasing from a risk <1000 at the younger end of that spectrum to a higher risk in the elderly.S2.2.2-41 However, an analysis of lifetime risk of SCD, derived from the Framingham data, suggested that the incidence of SCD decreases in later years, especially in people >75 years of age.S2.2.2-51 The data also suggested that SCD is uniformly more common in men than in women at all age groups. In contrast, the population of children, adolescents, and young adults has an overall annual risk of 1 per 100000, and there is somewhat a higher risk of SCD at the younger end of that age range.S2.2.2-41 An age- associated transition range, from the mid-20s to 35 to 40 years of age, is characterized by a steep increase in risk from that of the adolescent group to the middle- aged group, corresponding to the emergence of isch- emic heart disease.