Skip to main content

Arrhythmias and Arrhythmia Management in Hypertrophic Cardiomyopathy

  • Chapter
  • First Online:
Electrical Diseases of the Heart

Abstract

Cardiomyopathies are diseases of the myocardium associated with cardiac dysfunction. According to the World Health Organization (WHO) classification, cardiomyopathies are classified either as primary or secondary cardiomyopathies. Based on morphological and functional criteria, heritable cardiomyopathies are classified into four primary categories including hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). More recently, left ventricular non-compaction (LVNC) syndrome has been recognized as a separate cardiomyopathy. Secondary cardiomyopathies include for example ischemic and hypertensive cardiomyopathy. This chapter focuses solely on HCM, the most common heritable cardiovascular disease (estimated prevalence 1:500) and the most common cause of sudden cardiac death (SCD) in young adolescents and athletes. This chapter discusses HCM’s clinical and genetic substrate, genetic testing and screening, and has particular focus on its arrhythmogenic features and the role and indication of the implantable cardioverter defibrillator (ICD) and prevention of SCD.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 109.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 139.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 199.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Yetman AT, McCrindle BW. Management of pediatric hypertrophic cardiomyopathy. Curr Opin Cardiol. 2005;20:80–3.

    Article  PubMed  Google Scholar 

  2. Poliac LC, Barron ME, Maron BJ. Hypertrophic cardiomyopathy. Anesthesiology. 2006;104:183–92.

    Article  PubMed  Google Scholar 

  3. Maron BJ, Seidman JG, Seidman CE. Proposal for contemporary screening strategies in families with hypertrophic cardiomyopathy. J Am Coll Cardiol. 2004;44:2125–32.

    Article  PubMed  Google Scholar 

  4. Franz WM, Muller OJ, Katus HA. Cardiomyopathies: from genetics to the prospect of treatment. Lancet. 2001;358:1627–37.

    Article  PubMed  CAS  Google Scholar 

  5. Ellsworth EG, Ackerman MJ. The changing face of sudden cardiac death in the young. Heart Rhythm. 2005;2:1283–5.

    Article  PubMed  Google Scholar 

  6. Puranik R, Chow CK, Duflou JA, Kilborn MJ, McGuire MA. Sudden death in the young. Heart Rhythm. 2005;2:1277–82.

    Article  PubMed  Google Scholar 

  7. Maron BJ, Doerer JJ, Haas TS, Tierney DM, Mueller FO. Sudden deaths in young competitive athletes: analysis of 1866 deaths in the united states, 1980–2006. Circulation. 2009;119:1085–92.

    Article  PubMed  Google Scholar 

  8. Corrado D, Basso C, Thiene G. Sudden cardiac death in young people with apparently normal heart. Cardiovasc Res. 2001;50:399–408.

    Article  PubMed  CAS  Google Scholar 

  9. Roberts R, Sigwart U. Current concepts of the pathogenesis and treatment of hypertrophic cardiomyopathy. Circulation. 2005;112:293–6.

    Article  PubMed  Google Scholar 

  10. Klein GJ, Krahn AD, Skanes AC, Yee R, Gula LJ. Primary prophylaxis of sudden death in hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, and dilated cardiomyopathy. J Cardiovasc Electrophysiol. 2005;16(Suppl 1):S28–34.

    Article  PubMed  Google Scholar 

  11. Elliott PM, Gimeno Blanes JR, Mahon NG, Poloniecki JD, McKenna WJ. Relation between severtiy of left-ventricular hypertrophy and prognosis in patients with hypertrophic cardiomyopathy. Lancet. 2001;357:420–4.

    Article  PubMed  CAS  Google Scholar 

  12. Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE, et al. American College of Cardiology/European Society of Cardiology Clinical Expert Consensus Document on Hypertrophic Cardiomyopathy. A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for practice guidelines. J Am Coll Cardiol. 2003;42:1687–713.

    Article  PubMed  Google Scholar 

  13. Debl K, Djavidani B, Buchner S, Lipke C, Nitz W, Feuerbach S, et al. Delayed hyperenhancement: frequent finding in magnetic resonance imaging of left ventricular hypertrophy due to aortic stenosis and hypertrophic cardiomyopathy. Heart. 2006;92(10):1447–51.

    Article  PubMed  CAS  Google Scholar 

  14. Moon JC, McKenna WJ, McCrohon JA, Elliott PM, Smith GC, Pennell DJ. Toward clinical risk assessment in hypertrophic cardiomyopathy with gadolinium cardiovascular magnetic resonance. J Am Coll Cardiol. 2003;41:1561–7.

    Article  PubMed  Google Scholar 

  15. Binder J, Ommen SR, Gersh BJ, Van Driest SL, Tajik AJ, Nishimura RA, et al. Echocardiography-guided genetic testing in hypertrophic ­cardiomyopathy: septal morphological features predict the presence of myofilament mutations. Mayo Clin Proc. 2006;81:459–67.

    Article  PubMed  Google Scholar 

  16. Lever HM, Karam RF, Currie PJ, Healy BP. Hypertrophic cardiomyopathy in the elderly. Distinctions from the young based on cardiac shape. Circulation. 1989;79:580–9.

    Article  PubMed  CAS  Google Scholar 

  17. Maron BJ. Hypertrophic cardiomyopathy: a systematic review. JAMA. 2002;287:1308–20.

    Article  PubMed  Google Scholar 

  18. Maron BJ, Carney KP, Lever HM, Lewis JF, Barac I, Casey SA, et al. Relationship of race to sudden cardiac death in competitive athletes with hypertrophic cardiomyopathy. J Am Coll Cardiol. 2003;41:974–80.

    Article  PubMed  Google Scholar 

  19. Spirito P, Maron BJ, Bonow RO, Epstein SE. Occurrence and significance of progressive left ventricular wall thinning and relative cavity dilatation in hypertrophic cardiomyopathy. Am J Cardiol. 1987;60:123–9.

    Article  PubMed  CAS  Google Scholar 

  20. Boriani G, Maron BJ, Shen WK, Spirito P. Prevention of sudden death in hypertrophic cardiomyopathy: but which defibrillator for which patient? Circulation. 2004;110:e438–42.

    Article  PubMed  Google Scholar 

  21. Wolf CM, Moskowitz IP, Arno S, Branco DM, Semsarian C, Bernstein SA, et al. Somatic events modify hypertrophic cardiomyopathy pathology and link hypertrophy to arrhythmia. Proc Natl Acad Sci USA. 2005;102:18123–8.

    Article  PubMed  CAS  Google Scholar 

  22. Tomaselli GF, Marban E. Electrophysiological remodeling in hypertrophy and heart failure. Cardiovasc Res. 1999;42:270–83.

    Article  PubMed  CAS  Google Scholar 

  23. Antzelevitch C. Molecular genetics of arrhythmias and cardiovascular conditions associated with arrhythmias. J Cardiovasc Electrophysiol. 2003;14:1259–72.

    Article  PubMed  Google Scholar 

  24. Olivotto I, Cecchi F, Casey SA, Dolara A, Traverse JH, Maron BJ. Impact of atrial fibrillation on the clinical course of hypertrophic cardiomyopathy. Circulation. 2001;104:2517–24.

    Article  PubMed  CAS  Google Scholar 

  25. Spirito P, Autore C. Management of hypertrophic cardiomyopathy. BMJ. 2006;332:1251–5.

    Article  PubMed  Google Scholar 

  26. Attari M, Dhala A. Role of invasive and noninvasive testing in risk stratification of sudden cardiac death in children and young adults: an electrophysiologic perspective. Pediatr Clin North Am. 2004;51:1355–78.

    Article  PubMed  Google Scholar 

  27. Arad M, Maron BJ, Gorham JM, Johnson WH Jr, Saul JP, Perez-Atayde AR, et al. Glycogen storage diseases presenting as hypertrophic cardiomyopathy. N Engl J Med. 2005;352(4):362–72.

    Google Scholar 

  28. Adabag AS, Casey SA, Kuskowski MA, Zenovich AG, Maron BJ. Spectrum and prognostic significance of arrhythmias on ambulatory holter electrocardiogram in hypertrophic cardiomyopathy. J Am Coll Cardiol. 2005;45:697–704.

    Article  PubMed  Google Scholar 

  29. Van Driest SL, Vasile VC, Ommen SR, Will ML, Gersh BJ, Nishimura RA, et al. Myosin binding protein c mutations and compound heterozygosity in hypertrophic cardiomyopathy. J Am Coll Cardiol. 2004;44:1903–10.

    Article  PubMed  Google Scholar 

  30. Van Driest SL, Ommen SR, Tajik AJ, Gersh BJ, Ackerman MJ. Yield of genetic testing in hypertrophic cardiomyopathy. Mayo Clin Proc. 2005;80:739–44.

    PubMed  Google Scholar 

  31. Van Driest SL, Ommen SR, Tajik AJ, Gersh BJ, Ackerman MJ. Sarcomeric genotyping in hypertrophic cardiomyopathy. Mayo Clin Proc. 2005;80:463–9.

    Article  PubMed  Google Scholar 

  32. Ackerman MJ. Genetic testing for risk stratification in hypertrophic cardiomyopathy and long QT syndrome: fact or fiction? Curr Opin Cardiol. 2005;20:175–81.

    Article  PubMed  Google Scholar 

  33. Seidman JG, Seidman C. The genetic basis for cardiomyopathy: from mutation identification to mechanistic paradigms. Cell. 2001;104:557–67.

    Article  PubMed  CAS  Google Scholar 

  34. Maron BJ, Tajik AJ, Ruttenberg HD, Graham TP, Atwood GF, Victorica BE, et al. Hypertrophic ­cardiomyopathy in infants: clinical features and natural history. Circulation. 1982;65:7–17.

    Article  PubMed  CAS  Google Scholar 

  35. Olivotto I, Girolami F, Ackerman MJ, Nistri S, Bos JM, Zachara E, et al. Myofilament protein gene mutation screening and outcome of patients with hypertrophic cardiomyopathy. Mayo Clin Proc. 2008;83:630–8.

    PubMed  CAS  Google Scholar 

  36. Arad M, Maron BJ, Gorham JM, Johnson Jr WH, Saul JP, Perez-Atayde AR, et al. Glycogen storage diseases presenting as hypertrophic cardiomyopathy. N Engl J Med. 2005;352:362–72.

    Article  PubMed  CAS  Google Scholar 

  37. Daw EW, Chen SN, Czernuszewicz G, Lombardi R, Lu Y, Ma J, et al. Genome-wide mapping of modifier chromosomal loci for human hypertrophic cardiomyopathy. Hum Mol Genet. 2007;16:2463–71.

    Article  PubMed  CAS  Google Scholar 

  38. Lind JM, Chiu C, Ingles J, Yeates L, Humphries SE, Heather AK, et al. Sex hormone receptor gene variation associated with phenotype in male hypertrophic cardiomyopathy patients. J Mol Cell Cardiol. 2008;45:217–22.

    Article  PubMed  CAS  Google Scholar 

  39. Perkins MJ, Van Driest SL, Ellsworth EG, Will ML, Gersh BJ, Ommen SR, et al. Gene-specific modifying effects of pro-LVH polymorphisms involving the renin-angiotensin-aldosterone system among 389 unrelated patients with hypertrophic cardiomyopathy. Eur Heart J. 2005;26:2457–62.

    Article  PubMed  CAS  Google Scholar 

  40. Force T, Bonow RO, Houser SR, Solaro RJ, Hershberger RE, Adhikari B, et al. Research priorities in hypertrophic cardiomyopathy: report of a Working Group of the National Heart, Lung, and Blood Institute. Circulation. 2010;122:1130–3.

    Article  PubMed  Google Scholar 

  41. Keren A, Syrris P, McKenna WJ. Hypertrophic cardiomyopathy: the genetic determinants of clinical disease expression. Nature clinical practice. Cardiovasc Med. 2008;5:158–68.

    CAS  Google Scholar 

  42. Olivotto I, Cecchi F, Poggesi C, Yacoub MH. Developmental origins of hypertrophic cardiomyopathy phenotypes: a unifying hypothesis. Nature reviews. Cardiology. 2009;6:317–21.

    PubMed  CAS  Google Scholar 

  43. Thierfelder L, Watkins H, MacRae C, Lamas R, McKenna W, Vosberg HP, et al. Alpha-tropomyosin and cardiac troponin t mutations cause familial hypertrophic cardiomyopathy: a disease of the sarcomere. Cell. 1994;77:701–12.

    Article  PubMed  Google Scholar 

  44. Tyska MJ, Hayes E, Giewat M, Seidman CE, Seidman JG, Warshaw DM. Single-molecule mechanics of R403Q cardiac myosin isolated from the mouse model of familial hypertrophic cardiomyopathy. Circ Res. 2000;86:737–44.

    Article  PubMed  CAS  Google Scholar 

  45. Debold EP, Schmitt JP, Patlak JB, Beck SE, Moore JR, Seidman JG, et al. Hypertrophic and dilated cardiomyopathy mutations differentially affect the molecular force generation of mouse alpha-cardiac myosin in the laser trap assay. Am J Physiol Heart Circ Physiol. 2007;293:H284–91.

    Article  PubMed  CAS  Google Scholar 

  46. Fatkin D, McConnell BK, Mudd JO, Semsarian C, Moskowitz IG, Schoen FJ, et al. An abnormal Ca(2+) response in mutant sarcomere protein-mediated familial hypertrophic cardiomyopathy. J Clin Invest. 2000;106:1351–9.

    Article  PubMed  CAS  Google Scholar 

  47. Frey N, Luedde M, Katus HA. Mechanisms of ­disease: hypertrophic cardiomyopathy. Nat Rev Cardiol. 2011;9:91–100.

    Article  PubMed  Google Scholar 

  48. Van Driest SV, Ackerman MJ, Ommen SR, Shakur R, Will ML, Nishimura RA, et al. Prevalence and severity of “benign” mutations in the beta myosin heavy chain, cardiac troponin-T, and alpha tropomyosin genes in hypertrophic cardiomyopathy. Circulation. 2002;106:3085–90.

    Article  PubMed  Google Scholar 

  49. Ackerman MJ, Van Driest SV, Ommen SR, Will ML, Nishimura RA, Tajik AJ, et al. Prevalence and age-dependence of malignant mutations in the beta-myosin heavy chain and troponin T gene in hypertrophic cardiomyopathy: a comprehensive outpatient perspective. J Am Coll Cardiol. 2002;39:2042–8.

    Article  PubMed  CAS  Google Scholar 

  50. Olivotto I, Maron BJ, Montereggi A, Mazzuoli F, Dolara A, Cecchi F. Prognostic value of systemic blood pressure response during exercise in a community-based patient population with hypertrophic cardiomyopathy. J Am Coll Cardiol. 1999;33:2044–51.

    Article  PubMed  CAS  Google Scholar 

  51. Spirito P, Bellone P, Harris KM, Bernabo P, Bruzzi P, Maron BJ. Magnitude of left ventricular hypertrophy and risk of sudden death in hypertrophic cardiomyopathy. N Engl J Med. 2000;342:1778–85.

    Article  PubMed  CAS  Google Scholar 

  52. Maron BJ. Hypertrophic cardiomyopathy in childhood. Pediatr Clin North Am. 2004;51:1305–46.

    Article  PubMed  Google Scholar 

  53. Sorajja P, Nishimura RA, Ommen SR, Ackerman MJ, Tajik AJ, Gersh BJ. Use of echocardiography in patients with hypertrophic cardiomyopathy: clinical implications of massive hypertrophy. J Am Soc Echocardiogr. 2006;19:788–95.

    Article  PubMed  Google Scholar 

  54. Maron BJ. The electrocardiogram as a diagnostic tool for hypertrophic cardiomyopathy: revisited. Ann Noninvasive Electrocardiol. 2001;6:277–9.

    Article  PubMed  CAS  Google Scholar 

  55. Christiaans I, van Engelen K, van Langen IM, Birnie E, Bonsel GJ, Elliott PM, et al. Risk stratification for sudden cardiac death in hypertrophic cardiomyopathy: systematic review of clinical risk markers. Europace. 2010;12:313–21.

    Article  PubMed  Google Scholar 

  56. Spirito P, Seidman CE, McKenna WJ, Maron BJ. The management of hypertrophic cardiomyopathy. N Engl J Med. 1997;336:775–85.

    Article  PubMed  CAS  Google Scholar 

  57. Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE, et al. American College of Cardiology/European Society of Cardiology Clinical Expert Consensus Document on Hypertrophic Cardiomyopathy. A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for practice guidelines. J Am Coll Cardiol. 2003;42:1687.

    Article  PubMed  Google Scholar 

  58. Adabag AS, Maron BJ, Appelbaum E, Harrigan CJ, Buros JL, Gibson CM, et al. Occurrence and frequency of arrhythmias in hypertrophic cardiomyopathy in relation to delayed enhancement on cardiovascular magnetic resonance. J Am Coll Cardiol. 2008;51:1369–74.

    Article  PubMed  Google Scholar 

  59. Bruder O, Wagner A, Jensen CJ, Schneider S, Ong P, Kispert EM, et al. Myocardial scar visualized by cardiovascular magnetic resonance imaging predicts major adverse events in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol. 2010;56:875–87.

    Article  PubMed  Google Scholar 

  60. Iles L, Pfluger H, Lefkovits L, Butler MJ, Kistler PM, Kaye DM, et al. Myocardial fibrosis predicts appropriate device therapy in patients with implantable cardioverter-defibrillators for primary prevention of sudden cardiac death. J Am Coll Cardiol. 2011;57:821–8.

    Article  PubMed  Google Scholar 

  61. Leonardi S, Raineri C, De Ferrari GM, Ghio S, Scelsi L, Pasotti M, et al. Usefulness of cardiac magnetic resonance in assessing the risk of ventricular arrhythmias and sudden death in patients with hypertrophic cardiomyopathy. Eur Heart J. 2009;30:2003–10.

    Article  PubMed  Google Scholar 

  62. Konno T, Shimizu M, Ino H, Fujino N, Hayashi K, Uchiyama K, et al. Phenotypic differences between electrocardiographic and echocardiographic determination of hypertrophic cardiomyopathy in genetically affected subjects. J Intern Med. 2005;258:216–24.

    Article  PubMed  CAS  Google Scholar 

  63. Konno T, Shimizu M, Ino H, Fujino N, Hayashi K, Uchiyama K, et al. Differences in diagnostic value of four electrocardiographic voltage criteria for hypertrophic cardiomyopathy in a genotyped population. Am J Cardiol. 2005;96:1308–12.

    Article  PubMed  Google Scholar 

  64. Maron BJ, Spirito P, Wesley Y, Arce J. Development and progression of left ventricular hypertrophy in children with hypertrophic cardiomyopathy. N Engl J Med. 1986;315:610–4.

    Article  PubMed  CAS  Google Scholar 

  65. Ozdemir O, Soylu M, Demir AD, Topaloglu S, Alyan O, Turhan H, et al. P-wave durations as a predictor for atrial fibrillation development in patients with hypertrophic cardiomyopathy. Int J Cardiol. 2004;94:163–6.

    Article  PubMed  Google Scholar 

  66. Franz WM, Müller OJ, Katus HA. Cardiomyopathies: from genetics to the prospect of treatment. Lancet. 2001;358:1627–37.

    Article  PubMed  CAS  Google Scholar 

  67. Frey N, Franz WM, Gloeckner K, Degenhardt M, Muller M, Muller O, et al. Transgenic rat hearts expressing a human cardiac troponin T deletion reveal diastolic dysfunction and ventricular arrhythmias. Cardiovasc Res. 2000;47:254–64.

    Article  PubMed  CAS  Google Scholar 

  68. Watkins H, McKenna WJ, Thierfelder L, Suk HJ, Anan R, O’Donoghue A, et al. Mutations in the genes for cardiac troponin t and alpha-tropomyosin in hypertrophic cardiomyopathy. N Engl J Med. 1995;332:1058–65.

    Article  PubMed  CAS  Google Scholar 

  69. Watkins H, McKenna WJ. The prognostic impact of septal myectomy in obstructive hypertrophic cardiomyopathy. J Am Coll Cardiol. 2005;46:477–9.

    Article  PubMed  Google Scholar 

  70. Maron MS, Olivotto I, Betocchi S, Casey SA, Lesser JR, Losi MA, et al. Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy. N Engl J Med. 2003;348:295–303.

    Article  PubMed  Google Scholar 

  71. Montgomery JV, Harris KM, Casey SA, Zenovich AG, Maron BJ. Relation of electrocardiographic patterns to phenotypic expression and clinical outcome in hypertrophic cardiomyopathy. Am J Cardiol. 2005;96:270–5.

    Article  PubMed  Google Scholar 

  72. Jouven X, Hagege A, Charron P, Carrier L, Dubourg O, Langlard JM, et al. Relation between qt duration and maximal wall thickness in familial hypertrophic cardiomyopathy. Heart. 2002;88:153–7.

    Article  PubMed  CAS  Google Scholar 

  73. Martin AB, Garson Jr A, Perry JC. Prolonged QT interval in hypertrophic and dilated cardiomyopathy in children. Am Heart J. 1994;127:64–70.

    Article  PubMed  CAS  Google Scholar 

  74. Uchiyama K, Hayashi K, Fujino N, Konno T, Sakamoto Y, Sakata K, et al. Impact of QT variables on clinical outcome of genotyped hypertrophic cardiomyopathy. Ann Noninvasive Electrocardiol. 2009;14:65–71.

    Article  PubMed  Google Scholar 

  75. Johnson JN, Grifoni C, Bos JM, Saber-Ayad M, Ommen SR, Nistri S, et al. Prevalence and clinical correlates of QT prolongation in patients with hypertrophic cardiomyopathy. Eur Heart J. 2011;32:1114–20.

    Article  PubMed  Google Scholar 

  76. Ostman-Smith I, Wettrell G, Keeton B, Riesenfeld T, Holmgren D, Ergander U. Echocardiographic and electrocardiographic identification of those children with hypertrophic cardiomyopathy who should be considered at high-risk of dying suddenly. Cardiol Young. 2005;15:632–42.

    Article  PubMed  Google Scholar 

  77. D’Andrea A, Caso P, Severino S, Scotto di Uccio F, Vigorito F, Ascione L, et al. Association between intraventricular myocardial systolic dyssynchrony and ventricular arrhythmias in patients with hypertrophic cardiomyopathy. Echocardiography. 2005;22:571–8.

    Article  PubMed  Google Scholar 

  78. Elliott P, McKenna WJ. Hypertrophic cardiomyopathy. Lancet. 2004;363:1881–91.

    Article  PubMed  CAS  Google Scholar 

  79. Maron BJ. Contemporary considerations for risk stratification, sudden death and prevention in hypertrophic cardiomyopathy. Heart. 2003;89:977–8.

    Article  PubMed  CAS  Google Scholar 

  80. Nishimura RA, Holmes Jr DR. Clinical practice. Hypertrophic obstructive cardiomyopathy. N Engl J Med. 2004;350:1320–7.

    Article  PubMed  CAS  Google Scholar 

  81. Maron BJ, Dearani JA, Ommen SR, Maron MS, Schaff HV, Gersh BJ, et al. The case for surgery in obstructive hypertrophic cardiomyopathy. J Am Coll Cardiol. 2004;44:2044–53.

    Article  PubMed  Google Scholar 

  82. Ommen SR, Maron BJ, Olivotto I, Maron MS, Cecchi F, Betocchi S, et al. Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy. J Am Coll Cardiol. 2005;46:470–6.

    Article  PubMed  Google Scholar 

  83. Maron BJ, Towbin JA, Thiene G, Antzelevitch C, Corrado D, Arnett D, et al. Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation. 2006;113:1807–16.

    Article  PubMed  Google Scholar 

  84. Maron BJ, Nishimura RA, McKenna WJ, Rakowski H, Josephson ME, Kieval RS. Assessment of ­permanent dual-chamber pacing as a treatment for drug-refractory symptomatic patients with obstructive hypertrophic cardiomyopathy. A randomized, double-blind, crossover study (M-Pathy). Circulation. 1999;99:2927–33.

    Article  PubMed  CAS  Google Scholar 

  85. Chen MS, McCarthy PM, Lever HM, Smedira NG, Lytle BL. Effectiveness of atrial fibrillation surgery in patients with hypertrophic cardiomyopathy. Am J Cardiol. 2004;93:373–5.

    Article  PubMed  Google Scholar 

  86. Maron BJ, Spirito P, Shen WK, Haas TS, Formisano F, Link MS, et al. Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy. JAMA. 2007;298:405–12.

    Article  PubMed  CAS  Google Scholar 

  87. Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, et al. 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: executive summary: a report of the American College of Cardiology Foundation/American Heart Association Task Force on practice guidelines. Circulation. 2011;124:2761–96.

    Article  PubMed  Google Scholar 

  88. DiMarco JP. Implantable cardioverter-defibrillators. N Engl J Med. 2003;349:1836–47.

    Article  PubMed  CAS  Google Scholar 

  89. Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, 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:e783–831.

    Article  PubMed  Google Scholar 

  90. Maron BJ, Estes NAM, Maron MS, Almquist AK, Link MS, Udelson JE. Primary prevention of sudden death as a novel treatment strategy in hypertrophic cardiomyopathy. Circulation. 2003;107:2872–5.

    Article  PubMed  Google Scholar 

  91. Ackerman MJ, Priori SG, Willems S, Berul C, Brugada R, Calkins H, et al. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies this document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA). Heart Rhythm. 2011;8:1308–39.

    Article  PubMed  Google Scholar 

  92. 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.

    Article  PubMed  CAS  Google Scholar 

  93. Maron BJ, Chaitman BR, Ackerman MJ, BayesdeLuna A, Corrado D, Crosson JE, et al. Recommendations for physical activity and recreational sports participation for young patients with genetic cardiovascular diseases. Circulation. 2004;109:2807–16.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Michael J. Ackerman MD, PhD, FACC .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2013 Springer-Verlag London

About this chapter

Cite this chapter

Bos, J.M., Ommen, S.R., Ackerman, M.J. (2013). Arrhythmias and Arrhythmia Management in Hypertrophic Cardiomyopathy. In: Gussak, I., Antzelevitch, C. (eds) Electrical Diseases of the Heart. Springer, London. https://doi.org/10.1007/978-1-4471-4978-1_2

Download citation

  • DOI: https://doi.org/10.1007/978-1-4471-4978-1_2

  • Published:

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-4977-4

  • Online ISBN: 978-1-4471-4978-1

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics