Abstract
Sudden cardiac death in young competitive athletes is tragic and usually due to unsuspected cardiovascular disease. Screening programs for athletes remain debatable, and restriction of athletes from sports can have physical, emotional, and legal ramifications. In this article, we review the epidemiology of the more common inherited arrhythmias and congenital heart diseases that are of concern in a newly diagnosed athlete. A comparison of the current American Heart Association/American College of Cardiology and European Society guidelines, which are primarily based on expert opinion due to lack of randomized studies, is then undertaken. Furthermore, certain legal repercussions associated with both qualifying and restricting athletes from competitive sports are discussed. Lastly, we urge physicians to keep in mind that disqualifying an athlete from competitive sports does not mean restriction of all activities, and even patients with inherited arrhythmias and congenital heart disease can participate in low to moderate activity complementary with a healthy lifestyle.
Similar content being viewed by others
References
Americans with Disabilities Act, 42 USC 1210 et seq (1990)
Gathers v Loyola-Marymount No. C7985027. LA sup CT April 20, 1990
Lillard v State of Oregon No. BC2941. LA Sup Ct. January 1993
Rehabilitation Act 29 USC 504,794 (1973)
Alcalai R, Seidman JG, Seidman CE (2008) Genetic basis of hypertrophic cardiomyopathy: from bench to the clinics. J Cardiovasc Electrophysiol 19:104–110
Amato MC, Moffa PJ, Werner KE, Ramires JA (2001) Treatment decision in asymptomatic aortic valve stenosis: role of exercise testing. Heart 86:381–386
Bardy GH, Peter RH (1983) Arteriosclerotic heart disease following correction of tetralogy of Fallot. Chest 83:279–280
Basso C, Corrado D, Marcus FI, Nava A, Thiene G (2009) Arrhythmogenic right ventricular cardiomyopathy. Lancet 373:1289–1300
Basso C, Maron BJ, Corrado D, Thiene G (2000) 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 35:1493–1501
Best TM (2004) The preparticipation evaluation: an opportunity for change and consensus. Clin J Sport Med 14:107–108
Brouwer RM, Erasmus ME, Ebels T, Eijgelaar A (1994) Influence of age on survival, late hypertension, and recoarctation in elective aortic coarctation repair. Including long-term results after elective aortic coarctation repair with a follow-up from 25 to 44 years. J Thorac Cardiovasc Surg 108:525–531
Bundy DG, Feudtner C (2004) Preparticipation physical evaluations for high school athletes: time for a new game plan. Ambul Pediatr 4:260–263
Chugh SS, Senashova O, Watts A, Tran PT, Zhou Z, Gong Q et al (2004) Postmortem molecular screening in unexplained sudden death. J Am Coll Cardiol 43:1625–1629
Clarkson PM, Nicholson MR, Barratt-Boyes BG, Neutze JM, Whitlock RM (1983) Results after repair of coarctation of the aorta beyond infancy: a 10 to 28 year follow-up with particular reference to late systemic hypertension. Am J Cardiol 51:1481–1488
Cohen M, Fuster V, Steele PM, Driscoll D, McGoon DC (1989) Coarctation of the aorta. Long-term follow-up and prediction of outcome after surgical correction. Circulation 80:840–845
Corrado D, Basso C, Poletti A, Angelini A, Valente M, Thiene G (1994) Sudden death in the young. Is acute coronary thrombosis the major precipitating factor? Circulation 90:2315–2323
Corrado D, Basso C, Rizzoli G, Schiavon M, Thiene G (2003) Does sports activity enhance the risk of sudden death in adolescents and young adults? J Am Coll Cardiol 42:1959–1963
Corrado D, Pelliccia A, Bjornstad HH, Vanhees L, Biffi A, Borjesson M et al (2005) Cardiovascular pre-participation screening of young competitive athletes for prevention of sudden death: proposal for a common European protocol. Consensus statement of the study group of sport 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 26:516–524
Coutu M, Poirier NC, Dore A, Carrier M, Perrault LP (2004) Late myocardial revascularization in patients with tetralogy of Fallot. Ann Thorac Surg 77:1454–1455
Cusimano RJ, Guest C (1996) Coronary artery disease following repair of tetralogy of Fallot: implications and management. Can J Cardiol 12:172–174
Das P, Rimington H, Chambers J (2005) Exercise testing to stratify risk in aortic stenosis. Eur Heart J 26:1309–1313
de Divitiis M, Pilla C, Kattenhorn M, Zadinello M, Donald A, Leeson P et al (2001) Vascular dysfunction after repair of coarctation of the aorta: impact of early surgery. Circulation 104:I165–I170
Doolan A, Langlois N, Semsarian C (2004) Causes of sudden cardiac death in young Australians. Med J Aust 180:110–112
Doyle EF, Arumugham P, Lara E, Rutkowski MR, Kiely B (1974) Sudden death in young patients with congenital aortic stenosis. Pediatrics 53:481–489
Drezner JA (2005) Practical guidelines for automated external defibrillators in the athletic setting. Clin J Sport Med 15:367–369
Drezner JA (2009) Preparing for sudden cardiac arrest: the essential role of automated external defibrillators in athletic medicine: a critical review. Br J Sports Med 43:702–707
Estes NA 3rd, Link MS, Cannom D, Naccarelli GV, Prystowsky EN, Maron BJ et al (2001) Report of the NASPE policy conference on arrhythmias and the athlete. J Cardiovasc Electrophysiol 12:1208–1219
Frescura C, Basso C, Thiene G, Corrado D, Pennelli T, Angelini A et al (1998) Anomalous origin of coronary arteries and risk of sudden death: a study based on an autopsy population of congenital heart disease. Hum Pathol 29:689–695
Goldenberg I, Moss AJ (2008) Long QT syndrome. J Am Coll Cardiol 51:2291–2300
Gori F, Basso C, Thiene G (2000) Myocardial infarction in a patient with hypertrophic cardiomyopathy. N Engl J Med 342:593–594
Guerin P, Jimenez M, Vallot M, Thambo JB, Gosse P (2005) Arterial rigidity of patients operated successfully for coarctation of the aorta without residual hypertension [in French]. Arch Mal Coeur Vaiss 98:557–560
Harmon KG, Asif IM, Klossner D, Drezner JA (2011) Incidence of sudden cardiac death in national collegiate athletic association athletes. Circulation 123:1594–1600
Huikuri HV, Castellanos A, Myerburg RJ (2001) Sudden death due to cardiac arrhythmias. N Engl J Med 345:1473–1482
Hulot JS, Jouven X, Empana JP, Frank R, Fontaine G (2004) Natural history and risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circulation 110:1879–1884
Kass RS, Moss AJ (2003) Long QT syndrome: novel insights into the mechanisms of cardiac arrhythmias. J Clin Invest 112:810–815
Maron BJ (2003) Sudden death in young athletes. N Engl J Med 349:1064–1075
Maron BJ, Ackerman MJ, Nishimura RA, Pyeritz RE, Towbin JA, Udelson JE (2005) Task Force 4: HCM and other cardiomyopathies, mitral valve prolapse, myocarditis, and Marfan syndrome. J Am Coll Cardiol 45:1340–1345
Maron BJ, Doerer JJ, Haas TS, Tierney DM, Mueller FO (2009) Sudden deaths in young competitive athletes: analysis of 1866 deaths in the United States, 1980–2006. Circulation 119:1085–1092
Maron BJ, Gardin JM, Flack JM, Gidding SS, Kurosaki TT, Bild DE (1995) 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 92:785–789
Maron BJ, Thompson PD, Ackerman MJ, Balady G, Berger S, Cohen D et al (2007) 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 115:1455–1643
Maron BJ, Thompson PD, Puffer JC, McGrew CA, Strong WB, Douglas PS et al (1996) Cardiovascular preparticipation screening of competitive athletes. A statement for health professionals from the sudden death committee (clinical cardiology) and congenital cardiac defects committee (cardiovascular disease in the young). American Heart Association. Circulation 94:850–856
Maron BJ, Zipes DP (2005) Introduction: eligibility recommendations for competitive athletes with cardiovascular abnormalities-general considerations. J Am Coll Cardiol 45:1318–1321
Massin MM, Hovels-Gurich H, Seghaye MC (2007) Atherosclerosis lifestyle risk factors in children with congenital heart disease. Eur J Cardiovasc Prev Rehabil 14:349–351
Mehran-Pour M, Hirschfeld S, Francioli M, Liebman J (1978) Left ventricular myocardial infarction in tetralogy of Fallot. Am J Dis Child 132:1217–1218
Mitten MJ, Maron BJ, Zipes DP (2005) Task Force 12: Legal aspects of the 36th Bethesda Conference recommendations. J Am Coll Cardiol 45:1373–1375
Moons P, Van Deyk K, Dedroog D, Troost E, Budts W (2006) Prevalence of cardiovascular risk factors in adults with congenital heart disease. Eur J Cardiovasc Prev Rehabil 13:612–616
Moss AJ, Zareba W, Hall WJ, Schwartz PJ, Crampton RS, Benhorin J et al (2000) Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation 101:616–623
Nademanee K, Veerakul G, Nimmannit S, Chaowakul V, Bhuripanyo K, Likittanasombat K et al (1997) Arrhythmogenic marker for the sudden unexplained death syndrome in Thai men. Circulation 96:2595–2600
Nollert G, Fischlein T, Bouterwek S, Bohmer C, Dewald O, Kreuzer E et al (1997) Long-term results of total repair of tetralogy of Fallot in adulthood: 35 years follow-up in 104 patients corrected at the age of 18 or older. Thorac Cardiovasc Surg 45:178–181
Pappone C, Santinelli V (2005) Should catheter ablation be performed in asymptomatic patients with Wolff-Parkinson-White syndrome? Catheter ablation should be performed in asymptomatic patients with Wolff-Parkinson-White syndrome. Circulation 112:2207–2215 discussion 2216
Paterick TE, Paterick TJ, Fletcher GF, Maron BJ (2005) Medical and legal issues in the cardiovascular evaluation of competitive athletes. JAMA 294:3011–3018
Pelliccia A, Fagard R, Bjornstad HH, Anastassakis A, Arbustini E, Assanelli D et al (2005) 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 26:1422–1445
Pelliccia A, Zipes DP, Maron BJ (2008) Bethesda conference #36 and the European society of cardiology consensus recommendations revisited a comparison of U.S. and European criteria for eligibility and disqualification of competitive athletes with cardiovascular abnormalities. J Am Coll Cardiol 52:1990–1996
Pilichou K, Nava A, Basso C, Beffagna G, Bauce B, Lorenzon A et al (2006) Mutations in desmoglein-2 gene are associated with arrhythmogenic right ventricular cardiomyopathy. Circulation 113:1171–1179
Priori SG, Aliot E, Blomstrom-Lundqvist C, Bossaert L, Breithardt G, Brugada P et al (2003) Update of the guidelines on sudden cardiac death of the European society of cardiology. Eur Heart J 24:13–15
Puranik R, Chow CK, Duflou JA, Kilborn MJ, McGuire MA (2005) Sudden death in the young. Heart Rhythm 2:1277–1282
Rampazzo A, Nava A, Malacrida S, Beffagna G, Bauce B, Rossi V et al (2002) Mutation in human desmoplakin domain binding to plakoglobin causes a dominant form of arrhythmogenic right ventricular cardiomyopathy. Am J Hum Genet 71:1200–1206
Roguin A, Bomma CS, Nasir K, Tandri H, Tichnell C, James C et al (2004) Implantable cardioverter-defibrillators in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Am Coll Cardiol 43:1843–1852
Schwartz PJ, Priori SG, Spazzolini C, Moss AJ, Vincent GM, Napolitano C et al (2001) Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias. Circulation 103:89–95
Schwartz PJ, Stramba-Badiale M, Crotti L, Pedrazzini M, Besana A, Bosi G et al (2009) Prevalence of the congenital long-QT syndrome. Circulation 120:1761–1767
Tester DJ, Spoon DB, Valdivia HH, Makielski JC, Ackerman MJ (2004) Targeted mutational analysis of the RyR2-encoded cardiac ryanodine receptor in sudden unexplained death: a molecular autopsy of 49 medical examiner/coroner’s cases. Mayo Clin Proc 79:1380–1384
Thiene G, Carturan E, Corrado D, Basso C (2010) Prevention of sudden cardiac death in the young and in athletes: Dream or reality? Cardiovasc Pathol 19:207–217
Thiene G, Ho SY (1986) Aortic root pathology and sudden death in youth: Review of anatomical varieties. Appl Pathol 4:237–245
Toro-Salazar OH, Steinberger J, Thomas W, Rocchini AP, Carpenter B, Moller JH (2002) Long-term follow-up of patients after coarctation of the aorta repair. Am J Cardiol 89:541–547
Vogt M, Kuhn A, Baumgartner D, Baumgartner C, Busch R, Kostolny M et al (2005) Impaired elastic properties of the ascending aorta in newborns before and early after successful coarctation repair: proof of a systemic vascular disease of the prestenotic arteries? Circulation 111:3269–3273
Vriend JW, de Groot E, Bouma BJ, Hrudova J, Kastelein JJ, Tijssen JG et al (2005) Carotid intima-media thickness in post-coarctectomy patients with exercise induced hypertension. Heart 91:962–963
Wichter T, Paul M, Wollmann C, Acil T, Gerdes P, Ashraf O et al (2004) Implantable cardioverter/defibrillator therapy in arrhythmogenic right ventricular cardiomyopathy: Single-center experience of long-term follow-up and complications in 60 patients. Circulation 109:1503–1508
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Vaseghi, M., Ackerman, M.J. & Mandapati, R. Restricting Sports for Athletes With Heart Disease: Are We Saving Lives, Avoiding Lawsuits, or Just Promoting Obesity and Sedentary Living?. Pediatr Cardiol 33, 407–416 (2012). https://doi.org/10.1007/s00246-012-0170-6
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00246-012-0170-6