Herz Kardiovaskuläre Erkrankungen

, Volume 34, Issue 4, pp 305–314

Hypertrophic cardiomyopathy as a cause of sudden death

  • Hubert Seggewiss
  • Christoph Blank
  • Barbara Pfeiffer
  • Angelos Rigopoulos
Article
  • 171 Downloads

Abstract

Hypertrophic cardiomyopathy (HCM) is the main cause of sudden unexpected death in the young and especially in young athletes with an incidence up to 2.3 per 100,000 athletes and year. Risk stratification models including patient history (syncope, familial risk of sudden death) and findings in noninvasive tests (nonsustained ventricular tachycardia, abnormal blood pressure response during exercise, maximum left ventricular wall thickness ≥ 30 mm) have been developed in order to estimate the risk of individual patients. Echocardiographic parameters are helpful in distinguishing HCM from athlete’s heart. Definitive diagnosis of HCM implicates disqualification from competitive sports resulting in a significant reduction of sudden cardiac death due to HCM during sports competition. This positive development should lead to a widespread preparticipation screening of athletes including historical, clinical, and electrocardiographic examination. At least in borderline findings and symptomatic athletes, an additional echocardiogram should be performed in order to minimize or better exclude the risk of sudden cardiac death.

Key Words:

Hypertrophic cardiomyopathy Young athlete Sudden death Athlete’s heart Preparticipation screening Prevention 

Hypertrophe Kardiomyopathie als Ursache für den plötzlichen Herztod

Zusammenfassung

Die hypertrophe Kardiomyopathie (HCM) ist die häufigste Todesursache junger Menschen und insbesondere Athleten mit einer jährlichen Inzidenz bis 2,3 pro 100 000 Athleten. Durch Risikostratifizierungsmodelle unter Einschluss anamnestischer Angaben (Synkope, familiär auftretende plötzliche Todesfälle) und nichtinvasiver Parameter (nichtanhaltende ventrikuläre Tachykardien, pathologische Belastungsblutdruckreaktion, maximale linksventrikuläre Wanddicke ≥ 30 mm) kann das individuelle Patientenrisiko abgeschätzt werden. Die Differentialdiagnose zum Sportherz ist in der Regel durch echokardiographische und weitere nichtinvasive Parameter möglich. Die gesicherte Diagnose einer HCM führt zum Ausschluss von wettkampfsportlichen Aktivitäten, wodurch das Auftreten plötzlicher Todesfälle bei sportlicher Aktivität gesenkt werden kann. Hieraus ergibt sich als logische Konsequenz die Notwendigkeit flächendeckender Vorsorgeuntersuchungen inklusive Anamnese, körperlicher Untersuchung und Ruhe-EKG. Zumindest in Grenzfällen sollte eine Echokardiographie durchgeführt werden, um das Auftreten eines plötzlichen Herztodes möglichst sicher verhindern zu können.

Schlüsselwörter:

Hypertrophe Kardiomyopathie Plötzlicher Tod Junger Athlet Sportherz Voruntersuchung Prävention 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Maron BJ. Hypertrophic cardiomyopathy: a systematic review. JAMA 2002;287:1308–20.PubMedCrossRefGoogle Scholar
  2. 2.
    Elliott P, McKenna WJ. Hypertrophic cardiomyopathy. Lancet 2004;363:1881–91.PubMedCrossRefGoogle Scholar
  3. 3.
    Teare D. Asymmetrical hypertrophy of the heart in young adults. Br Heart J 1958;20:1–8.PubMedCrossRefGoogle Scholar
  4. 4.
    Braunwald E, Lambrew CT, Rockoff SD, et al. Idiopathic hypertrophic subaortic stenosis. I. A description of the disease based upon an analysis of 64 patients. Circulation 1964;30:Suppl 4:3–119.PubMedGoogle Scholar
  5. 5.
    McKenna W, Deanfield J, Faruqui A, et al. Prognosis in hypertrophic cardiomyopathy: role of age and clinical, electrocardiographic and hemodynamic features. Am J Cardiol 1981;47:532–8.PubMedCrossRefGoogle Scholar
  6. 6.
    Maron BJ, Spirito P. Impact of patient selection biases on the perception of hypertrophic cardiomyopathy and its natural history. Am J Cardiol 1993;72:970–2.PubMedCrossRefGoogle Scholar
  7. 7.
    Myerburg RJ, Kessler KM, Bassett AL, et al. A biological approach to sudden cardiac death: structure, function and cause. Am J Cardiol 1989;63:1512–6.PubMedCrossRefGoogle Scholar
  8. 8.
    Maron BJ, Shen WK, Link MS, et al. Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. N Engl J Med 2000;342:365–73.PubMedCrossRefGoogle Scholar
  9. 9.
    Adabag AS, Maron BJ. Implications of arrhythmias and prevention of sudden death in hypertrophic cardiomyopathy. Ann Noninvasive Electrocardiol 2007;12:171–80.PubMedCrossRefGoogle Scholar
  10. 10.
    Lim PO, Morris-Thurgood JA, Frenneaux MP. Vascular mechanisms of sudden death in hypertrophic cardiomyopathy, including blood pressure responses to exercise. Cardiol Rev 2002;10:15–23.PubMedCrossRefGoogle Scholar
  11. 11.
    Nicod P, Polikar R, Peterson KL. Hypertrophic cardiomyopathy and sudden death. N Engl J Med 1988;318:1255–7.PubMedGoogle Scholar
  12. 12.
    Maron BJ, Roberts WC, Epstein SE. Sudden death in hypertrophic cardiomyopathy: a profile of 78 patients. Circulation 1982;65:1388–94.PubMedGoogle Scholar
  13. 13.
    Sadoul N, Prasad K, Elliott PM, et al. Prospective prognostic assessment of blood pressure response during exercise in patients with hypertrophic cardiomyopathy. Circulation 1997;96:2987–91.PubMedGoogle Scholar
  14. 14.
    Prasad K, Williams L, Campbell R, et al. Episodic syncope in hypertrophic cardiomyopathy: evidence for inappropriate vasodilation. Heart 2008;94:1312–7.PubMedCrossRefGoogle Scholar
  15. 15.
    Mark AL, Kioschos JM, Abboud FM, et al. Abnormal vascular responses to exercise in patients with aortic stenosis. J Clin Invest 1973;52:1138–46.PubMedCrossRefGoogle Scholar
  16. 16.
    Corrado D, Basso C, Rizzoli G, et al. Does sports activity enhance the risk of sudden death in adolescents and young adults? J Am Coll Cardiol 2003;42:1959–63.PubMedCrossRefGoogle Scholar
  17. 17.
    Maron BJ, Shirani J, Poliac LC, et al. Sudden death in young competitive athletes. Clinical, demographic, and pathological profiles. JAMA 1996;276:199–204.PubMedCrossRefGoogle Scholar
  18. 18.
    Maron BJ. Sudden death in young athletes. N Engl J Med 2003;349:1064–75.PubMedCrossRefGoogle Scholar
  19. 19.
    Sack S. [Sports death — an internal medicine problem?] Herz 2004;29:414–9.PubMedCrossRefGoogle Scholar
  20. 20.
    Maron BJ, Epstein SE, Roberts WC. Causes of sudden death in competitive athletes. J Am Coll Cardiol 1986;7:204–14.PubMedGoogle Scholar
  21. 21.
    Maron BJ, Roberts WC, McAllister HA, et al. Sudden death in young athletes. Circulation 1980;62:218–29.PubMedGoogle Scholar
  22. 22.
    Corrado D, Basso C, Schiavon M, et al. Screening for hypertrophic cardiomyopathy in young athletes. N Engl J Med 1998;339:364–9.PubMedCrossRefGoogle Scholar
  23. 23.
    Maron BJ, Gohman TE, Aeppli D. Prevalence of sudden cardiac death during competitive sports activities in Minnesota high school athletes. J Am Coll Cardiol 1998;32:1881–4.PubMedCrossRefGoogle Scholar
  24. 24.
    Maron BJ, Doerer JJ, Haas TS, et al. Sudden deaths in young competitive athletes: analysis of 1866 deaths in the United States, 1980–2006. Circulation 2009;119:1085–92.PubMedCrossRefGoogle Scholar
  25. 25.
    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: 974–80.PubMedCrossRefGoogle Scholar
  26. 26.
    Maron BJ, Pelliccia A. The heart of trained athletes: cardiac remodeling and the risks of sports, including sudden death. Circulation 2006;114:1633–44.PubMedCrossRefGoogle Scholar
  27. 27.
    Maron BJ, Isner JM, McKenna WJ. 26th Bethesda conference: recommendations for determining eligibility for competition in athletes with cardiovascular abnormalities. Task Force 3: hypertrophic cardiomyopathy, myocarditis and other myopericardial diseases and mitral valve prolapse. J Am Coll Cardiol 1994;24:880–5.PubMedCrossRefGoogle Scholar
  28. 28.
    Pelliccia A, Zipes DP, Maron BJ. 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 2008;52:1990–6.PubMedCrossRefGoogle Scholar
  29. 29.
    Maron BJ, Thompson PD, Puffer JC, et al. 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 1996;94:850–6.PubMedGoogle Scholar
  30. 30.
    Glover DW, Maron BJ. Profile of preparticipation cardiovascular screening for high school athletes. JAMA 1998;279: 1817–9.PubMedCrossRefGoogle Scholar
  31. 31.
    Pfister GC, Puffer JC, Maron BJ. Preparticipation cardiovascular screening for US collegiate student-athletes. JAMA 2000;283:1597–9.PubMedCrossRefGoogle Scholar
  32. 32.
    Corrado D, Pelliccia A, Bjornstad HH, et al. 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 2005;26:516–24.PubMedCrossRefGoogle Scholar
  33. 33.
    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;92:785–9.PubMedGoogle Scholar
  34. 34.
    Pelliccia A, Di Paolo FM, Corrado D, et al. Evidence for efficacy of the Italian national pre-participation screening programme for identification of hypertrophic cardiomyopathy in competitive athletes. Eur Heart J 2006;27: 2196–200.PubMedCrossRefGoogle Scholar
  35. 35.
    Corrado D, Basso C, Pavei A, et al. Trends in sudden cardiovascular death in young competitive athletes after implementation of a preparticipation screening program. JAMA 2006;296:1593–601.PubMedCrossRefGoogle Scholar
  36. 36.
    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;115:1643–55.PubMedCrossRefGoogle Scholar
  37. 37.
    Basavarajaiah S, Wilson M, Whyte G, et al. Prevalence of hypertrophic cardiomyopathy in highly trained athletes: relevance to pre-participation screening. J Am Coll Cardiol 2008;51:1033–9.PubMedCrossRefGoogle Scholar
  38. 38.
    Faber L, van Buuren F. Athlete screening for occult cardiac disease: no risk, no fun? J Am Coll Cardiol 2008;51:1040–1.PubMedCrossRefGoogle Scholar
  39. 39.
    Rowland TW. Evaluating cardiac symptoms in the athlete: is it safe to play? Clin J Sport Med 2005;15:417–20.PubMedCrossRefGoogle Scholar
  40. 40.
    Maron BJ. Structural features of the athlete heart as defined by echocardiography. J Am Coll Cardiol 1986;7:190–203.PubMedGoogle Scholar
  41. 41.
    Maron BJ, Pelliccia A, Spirito P. Cardiac disease in young trained athletes. Insights into methods for distinguishing athlete’s heart from structural heart disease, with particular emphasis on hypertrophic cardiomyopathy. Circulation 1995;91:1596–601.PubMedGoogle Scholar
  42. 42.
    Pelliccia A, Maron BJ, Spataro A, et al. The upper limit of physiologic cardiac hypertrophy in highly trained elite athletes. N Engl J Med 1991;324:295–301.PubMedGoogle Scholar
  43. 43.
    Pelliccia A, Corrado D, Bjornstad HH, et al. Recommendations for participation in competitive sport and leisure-time physical activity in individuals with cardiomyopathies, myocarditis and pericarditis. Eur J Cardiovasc Prev Rehabil 2006;13:876–85.PubMedCrossRefGoogle Scholar
  44. 44.
    Sharma S, Elliott PM, Whyte G, et al. Utility of metabolic exercise testing in distinguishing hypertrophic cardiomyopathy from physiologic left ventricular hypertrophy in athletes. J Am Coll Cardiol 2000;36:864–70.PubMedCrossRefGoogle Scholar
  45. 45.
    Anastasakis A, Kotsiopoulou C, Rigopoulos A, et al. Similarities in the profile of cardiopulmonary exercise testing between patients with hypertrophic cardiomyopathy and strength athletes. Heart 2005;91:1477–8.PubMedCrossRefGoogle Scholar
  46. 46.
    Ehsani AA, Hagberg JM, Hickson RC. Rapid changes in left ventricular dimensions and mass in response to physical conditioning and deconditioning. Am J Cardiol 1978;42:52–6.PubMedCrossRefGoogle Scholar
  47. 47.
    D’Andrea A, D’Andrea L, Caso P, et al. The usefulness of Doppler myocardial imaging in the study of the athlete’s heart and in the differential diagnosis between physiological and pathological ventricular hypertrophy. Echocardiography 2006;23:149–57.PubMedCrossRefGoogle Scholar
  48. 48.
    Scheffold T, Binner P, Erdmann J, et al. [Hypertrophic cardiomyopathy.] Herz 2005;30:550–7.PubMedCrossRefGoogle Scholar
  49. 49.
    Moon JC, McKenna WJ, McCrohon JA, et al. Toward clinical risk assessment in hypertrophic cardiomyopathy with gadolinium cardiovascular magnetic resonance. J Am Coll Cardiol 2003;41:1561–7.PubMedCrossRefGoogle Scholar
  50. 50.
    Vohringer M, Mahrholdt H, Yilmaz A, et al. Significance of late gadolinium enhancement in cardiovascular magnetic resonance imaging (CMR). Herz 2007;32:129–37.PubMedCrossRefGoogle Scholar
  51. 51.
    Elliott PM, Poloniecki J, Dickie S, et al. Sudden death in hypertrophic cardiomyopathy: identification of high risk patients. J Am Coll Cardiol 2000;36:2212–8.PubMedCrossRefGoogle Scholar
  52. 52.
    Maron BJ, Spirito P, Shen WK, et al. Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy. JAMA 2007;298:405–12.PubMedCrossRefGoogle Scholar
  53. 53.
    Zipes DP, Camm AJ, Borggrefe M, et al. ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death — executive summary: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death) developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. Eur Heart J 2006;27: 2099–140.PubMedCrossRefGoogle Scholar
  54. 54.
    Monserrat L, Elliott PM, Gimeno JR, et al. Non-sustained ventricular tachycardia in hypertrophic cardiomyopathy: an independent marker of sudden death risk in young patients. J Am Coll Cardiol 2003;42:873–9.PubMedCrossRefGoogle Scholar
  55. 55.
    Elliott P, Spirito P. Prevention of hypertrophic cardiomyopathy-related deaths: theory and practice. Heart 2008;94: 1269–75.PubMedCrossRefGoogle Scholar
  56. 56.
    Drezner JA, Courson RW, Roberts WO, et al. Inter-association task force recommendations on emergency preparedness and management of sudden cardiac arrest in high school and college athletic programs: a consensus statement. Heart Rhythm 2007;4:549–65.PubMedCrossRefGoogle Scholar
  57. 57.
    Mitchell JH, Haskell W, Snell P, et al. Task Force 8: classification of sports. J Am Coll Cardiol 2005;45:1364–7.PubMedCrossRefGoogle Scholar
  58. 58.
    Olivotto I, Maron BJ, Cecchi F. Clinical significance of atrial fibrillation in hypertrophic cardiomyopathy. Curr Cardiol Rep 2001;3:141–6.PubMedCrossRefGoogle Scholar
  59. 59.
    Maron MS, Olivotto I, Betocchi S, et al. Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy. N Engl J Med 2003;348:295–303.PubMedCrossRefGoogle Scholar
  60. 60.
    Elliott PM, Gimeno JR, Tome MT, et al. Left ventricular outflow tract obstruction and sudden death risk in patients with hypertrophic cardiomyopathy. Eur Heart J 2006;27: 1933–41.PubMedCrossRefGoogle Scholar

Copyright information

© Urban & Vogel, Muenchen 2009

Authors and Affiliations

  • Hubert Seggewiss
    • 1
    • 3
  • Christoph Blank
    • 1
  • Barbara Pfeiffer
    • 1
  • Angelos Rigopoulos
    • 2
  1. 1.Medical Department 1Leopoldina HospitalSchweinfurtGermany
  2. 2.2nd Department of CardiologyUniversity of Athens Medical SchoolAthensGreece
  3. 3.Medizinische Klinik 1Leopoldina-KrankenhausSchweinfurtGermany

Personalised recommendations