Risk of sudden cardiac death in athletes
- Antonio Pelliccia, MD
Antonio Pelliccia, MD
- Chief of Cardiology
- Institute of Sport Medicine and Science, Rome, Italy
- Mark S Link, MD
Mark S Link, MD
- Section Editor — Cardiac Arrhythmias
- Professor of Medicine
- Tufts Medical School
- Section Editors
- Peter J Zimetbaum, MD
Peter J Zimetbaum, MD
- Section Editor — Cardiac Arrhythmias
- Professor of Medicine
- Harvard Medical School
- Scott Manaker, MD, PhD
Scott Manaker, MD, PhD
- Section Editor — Critical Care
- Professor of Medicine
- University of Pennsylvania School of Medicine
Sudden cardiac death (SCD) associated with athletic activity is a rare but devastating event. Victims are usually young and apparently healthy, but many have underlying cardiovascular disease that is not diagnosed until after the event. As a result, there is great interest in detecting such abnormalities early, and then defining appropriate activity restrictions for affected individuals to minimize the risk of SCD.
The majority of SCD events in athletes are due to malignant arrhythmias, usually sustained ventricular tachycardia (VT) degenerating into ventricular fibrillation (VF), or primary VF itself. In the small number of susceptible individuals with certain underlying cardiac disorders, athletics can increase the likelihood of these events in two ways: prolonged physical training induces changes in cardiac structure (eg, chamber dilation, myocyte death, and fibrosis) that create arrhythmic substrate; and, the immediate physiologic demands of intense athletics can trigger malignant arrhythmias and SCD.
The potential for SCD associated with athletic activity generates two questions:
●How should individuals be evaluated prior to initiating athletic activity?
●What restrictions should be placed upon individuals with known cardiovascular disease?
- Thompson PD, Franklin BA, Balady GJ, et al. Exercise and acute cardiovascular events placing the risks into perspective: a scientific statement from the American Heart Association Council on Nutrition, Physical Activity, and Metabolism and the Council on Clinical Cardiology. Circulation 2007; 115:2358.
- Maron BJ, Chaitman BR, Ackerman MJ, et al. Recommendations for physical activity and recreational sports participation for young patients with genetic cardiovascular diseases. Circulation 2004; 109:2807.
- Lampert R, Olshansky B, Heidbuchel H, et al. Safety of sports for athletes with implantable cardioverter-defibrillators: results of a prospective, multinational registry. Circulation 2013; 127:2021.
- Maron BJ, Shirani J, Poliac LC, et al. Sudden death in young competitive athletes. Clinical, demographic, and pathological profiles. JAMA 1996; 276:199.
- Van Camp SP, Bloor CM, Mueller FO, et al. Nontraumatic sports death in high school and college athletes. Med Sci Sports Exerc 1995; 27:641.
- Maron BJ, Poliac LC, Roberts WO. Risk for sudden cardiac death associated with marathon running. J Am Coll Cardiol 1996; 28:428.
- Phillips M, Robinowitz M, Higgins JR, et al. Sudden cardiac death in Air Force recruits. A 20-year review. JAMA 1986; 256:2696.
- Mitchell JH, Haskell W, Snell P, Van Camp SP. Task Force 8: classification of sports. J Am Coll Cardiol 2005; 45:1364.
- Belonje A, Nangrahary M, de Swart H, Umans V. Major adverse cardiac events during endurance sports. Am J Cardiol 2007; 99:849.
- Hillis WS, McIntyre PD, Maclean J, et al. ABC of sports medicine. Sudden death in sport. BMJ 1994; 309:657.
- Thompson PD, Funk EJ, Carleton RA, Sturner WQ. Incidence of death during jogging in Rhode Island from 1975 through 1980. JAMA 1982; 247:2535.
- Vuori I. The cardiovascular risks of physical activity. Acta Med Scand Suppl 1986; 711:205.
- Gibbons LW, Cooper KH, Meyer BM, Ellison RC. The acute cardiac risk of strenuous exercise. JAMA 1980; 244:1799.
- Corrado D, Basso C, Schiavon M, Thiene G. Screening for hypertrophic cardiomyopathy in young athletes. N Engl J Med 1998; 339:364.
- Maron BJ, Thompson PD, Ackerman MJ, et al. Recommendations and considerations related to preparticipation screening for cardiovascular abnormalities in competitive athletes: 2007 update: a scientific statement from the American Heart Association Council on Nutrition, Physical Activity, and Metabolism: endorsed by the American College of Cardiology Foundation. Circulation 2007; 115:1643.
- Roberts WO, Stovitz SD. Incidence of sudden cardiac death in Minnesota high school athletes 1993-2012 screened with a standardized pre-participation evaluation. J Am Coll Cardiol 2013; 62:1298.
- Harmon KG, Asif IM, Klossner D, Drezner JA. Incidence of sudden cardiac death in National Collegiate Athletic Association athletes. Circulation 2011; 123:1594.
- Eckart RE, Shry EA, Burke AP, et al. Sudden death in young adults: an autopsy-based series of a population undergoing active surveillance. J Am Coll Cardiol 2011; 58:1254.
- 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.
- Waller BF, Roberts WC. Sudden death while running in conditioned runners aged 40 years or over. Am J Cardiol 1980; 45:1292.
- Virmani R, Robinowitz M, McAllister HA Jr. Nontraumatic death in joggers. A series of 30 patients at autopsy. Am J Med 1982; 72:874.
- Hausmann R, Hammer S, Betz P. Performance enhancing drugs (doping agents) and sudden death--a case report and review of the literature. Int J Legal Med 1998; 111:261.
- Kennedy MC, Lawrence C. Anabolic steroid abuse and cardiac death. Med J Aust 1993; 158:346.
- Maron BJ, Gardin JM, Flack JM, et al. Prevalence of hypertrophic cardiomyopathy in a general population of young adults. Echocardiographic analysis of 4111 subjects in the CARDIA Study. Coronary Artery Risk Development in (Young) Adults. Circulation 1995; 92:785.
- Maron BJ, Mathenge R, Casey SA, et al. Clinical profile of hypertrophic cardiomyopathy identified de novo in rural communities. J Am Coll Cardiol 1999; 33:1590.
- Maron BJ, Carney KP, Lever HM, et al. Relationship of race to sudden cardiac death in competitive athletes with hypertrophic cardiomyopathy. J Am Coll Cardiol 2003; 41:974.
- Elliott PM, Poloniecki J, Dickie S, et al. Sudden death in hypertrophic cardiomyopathy: identification of high risk patients. J Am Coll Cardiol 2000; 36:2212.
- Gersh BJ, Maron BJ, Bonow RO, et al. 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation 2011; 124:e783.
- Maron BJ, Ackerman MJ, Nishimura RA, et al. Task Force 4: HCM and other cardiomyopathies, mitral valve prolapse, myocarditis, and Marfan syndrome. J Am Coll Cardiol 2005; 45:1340.
- Pelliccia A, Fagard R, Bjørnstad HH, et al. Recommendations for competitive sports participation in athletes with cardiovascular disease: a consensus document from the Study Group of Sports Cardiology of the Working Group of Cardiac Rehabilitation and Exercise Physiology and the Working Group of Myocardial and Pericardial Diseases of the European Society of Cardiology. Eur Heart J 2005; 26:1422.
- Heidbüchel H, Corrado D, Biffi A, et al. Recommendations for participation in leisure-time physical activity and competitive sports of patients with arrhythmias and potentially arrhythmogenic conditions. Part II: ventricular arrhythmias, channelopathies and implantable defibrillators. Eur J Cardiovasc Prev Rehabil 2006; 13:676.
- Taylor AJ, Rogan KM, Virmani R. Sudden cardiac death associated with isolated congenital coronary artery anomalies. J Am Coll Cardiol 1992; 20:640.
- Liberthson RR. Sudden death from cardiac causes in children and young adults. N Engl J Med 1996; 334:1039.
- Liberthson RR, Dinsmore RE, Fallon JT. Aberrant coronary artery origin from the aorta. Report of 18 patients, review of literature and delineation of natural history and management. Circulation 1979; 59:748.
- Taylor AJ, Byers JP, Cheitlin MD, Virmani R. Anomalous right or left coronary artery from the contralateral coronary sinus: "high-risk" abnormalities in the initial coronary artery course and heterogeneous clinical outcomes. Am Heart J 1997; 133:428.
- Basso C, Maron BJ, Corrado D, Thiene G. 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.
- Graham TP Jr, Driscoll DJ, Gersony WM, et al. Task Force 2: congenital heart disease. J Am Coll Cardiol 2005; 45:1326.
- Thompson PD, Balady GJ, Chaitman BR, et al. Task Force 6: coronary artery disease. J Am Coll Cardiol 2005; 45:1348.
- Thiene G, Nava A, Corrado D, et al. Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med 1988; 318:129.
- Blomström-Lundqvist C, Sabel KG, Olsson SB. A long term follow up of 15 patients with arrhythmogenic right ventricular dysplasia. Br Heart J 1987; 58:477.
- Douglas PS, O'Toole ML, Hiller WD, Reichek N. Different effects of prolonged exercise on the right and left ventricles. J Am Coll Cardiol 1990; 15:64.
- Nava A, Canciani B, Buja G, et al. Electrovectorcardiographic study of negative T waves on precordial leads in arrhythmogenic right ventricular dysplasia: relationship with right ventricular volumes. J Electrocardiol 1988; 21:239.
- Nava A, Bauce B, Basso C, et al. Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy. J Am Coll Cardiol 2000; 36:2226.
- Ruwald AC, Marcus F, Estes NA 3rd, et al. Association of competitive and recreational sport participation with cardiac events in patients with arrhythmogenic right ventricular cardiomyopathy: results from the North American multidisciplinary study of arrhythmogenic right ventricular cardiomyopathy. Eur Heart J 2015; 36:1735.
- James CA, Bhonsale A, Tichnell C, et al. Exercise increases age-related penetrance and arrhythmic risk in arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated desmosomal mutation carriers. J Am Coll Cardiol 2013; 62:1290.
- Kligfield P, Levy D, Devereux RB, Savage DD. Arrhythmias and sudden death in mitral valve prolapse. Am Heart J 1987; 113:1298.
- Kligfield P, Devereaux RB. Is the patient with mitral valve prolapse at high risk for sudden death identifiable?. In: Dilemmas in clinical cardiology, Cheitlin MD (Ed), FA Davis, Philadelphia 1990. p.143.
- Schwartz PJ, Priori SG, Spazzolini C, et al. Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias. Circulation 2001; 103:89.
- Takenaka K, Ai T, Shimizu W, et al. Exercise stress test amplifies genotype-phenotype correlation in the LQT1 and LQT2 forms of the long-QT syndrome. Circulation 2003; 107:838.
- Zipes DP, Ackerman MJ, Estes NA 3rd, et al. Task Force 7: arrhythmias. J Am Coll Cardiol 2005; 45:1354.
- Johnson JN, Ackerman MJ. Return to play? Athletes with congenital long QT syndrome. Br J Sports Med 2013; 47:28.
- Antzelevitch C, Brugada P, Borggrefe M, et al. Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation 2005; 111:659.
- Matsuo K, Kurita T, Inagaki M, et al. The circadian pattern of the development of ventricular fibrillation in patients with Brugada syndrome. Eur Heart J 1999; 20:465.
- Corrado D, Basso C, Buja G, et al. Right bundle branch block, right precordial st-segment elevation, and sudden death in young people. Circulation 2001; 103:710.
- Priori SG, Napolitano C, Memmi M, et al. Clinical and molecular characterization of patients with catecholaminergic polymorphic ventricular tachycardia. Circulation 2002; 106:69.
- Choi G, Kopplin LJ, Tester DJ, et al. Spectrum and frequency of cardiac channel defects in swimming-triggered arrhythmia syndromes. Circulation 2004; 110:2119.
- Thompson PD, Stern MP, Williams P, et al. Death during jogging or running. A study of 18 cases. JAMA 1979; 242:1265.
- BALANCE OF RISKS AND BENEFITS
- RECREATIONAL ATHLETES
- COMPETITIVE ATHLETES
- Incidence of sudden death
- Etiology of sudden death
- - Structural heart disease
- Young athletes
- Athletes aged 35 years or older
- - Absence of structural heart disease
- Structural abnormalities associated with SCD
- - Hypertrophic cardiomyopathy
- - Congenital coronary artery abnormalities
- - Arrhythmogenic (right) ventricular cardiomyopathy
- - Marfan syndrome
- - Myocarditis
- - Mitral valve prolapse
- - Congenital heart diseases
- Inherited arrhythmia syndromes
- - Congenital long QT syndrome
- - Brugada syndrome
- - Catecholaminergic polymorphic VT
- Coronary heart disease
- - Risk assessment
- Competitive athletes
- Recreational athletes
- SUMMARY AND RECOMMENDATIONS