Skip to main content
SpringerLink
Log in

Molecular and Electrophysiological Mechanisms of Arrhythmias in Hypertrophic Cardiomyopathy

  • Chapter
Cardiac Arrhythmias, Pacing & Electrophysiology

Part of the book series: Developments in Cardiovascular Medicine ((DICM,volume 201))

  • 181 Accesses

Abstract

The entity of idiopathic cardiac hypertrophy has been recognised for over a century1,2 and that of autosomal dominant familial cardiac hypertrophy for over 40 years3,4. While symptoms are often attributable to the direct haemodynamic consequences of massive hypertrophy, sudden death due to arrhythmias is, in fact, the common mode of death in such patients5,6. Moreover, it is now increasingly clear that while patients with massive hypertrophy are at high risk for sudden death, this catastrophic event can also occur in patients with familial hypertrophic cardiomyopathy (HCM) and near-normal ventricular thickness. Thus, management of the patient with HCM may necessitate a consideration of antiarrhythmic therapy separate from therapy directed at improving symptoms.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 169.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 219.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 219.99
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

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Hallopeau L. Retrecissement ventriculo-aortique. Gaz Med Paris 1869; 24: 683.

    Google Scholar 

  2. Liouville H. Retreciseement cardiaque sous-aortique. Gaz Med Paris 1869; 24: 161.

    Google Scholar 

  3. Morrow AG, Braunwald E. Functional aortic stenosis: a malformation characterized by resistance to left ventricular outflow without anatomic obstruction. Circulation 1959; 20: 181.

    Article  PubMed  CAS  Google Scholar 

  4. Teare RD. Asymmetrical hypertrophy of the heart in young adults. Br Heart J 1959; 20: 1–8.

    Article  Google Scholar 

  5. Slade AK, Camm AJ. Risk assessment and prevention of sudden cardiac death in hypertrophic cardiomyopathy. Arch Mal Coeur Vaiss 1996; 89 ( Spec. No. 1 ): 37–49.

    PubMed  Google Scholar 

  6. 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 

  7. Nicod P, Polikar R, Peterson KL. Hypertrophic cardiomyopathy and sudden death. N Engl J Med 1988; 318: 1255–7.

    Article  PubMed  CAS  Google Scholar 

  8. Dilsizian V, Bonow RO, Epstein SE, Fananapazir L. Myocardial ischemia detected by thallium scintigraphy is frequently related to cardiac arrest and syncope in young patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 1993; 22: 796–804.

    Article  PubMed  CAS  Google Scholar 

  9. Stafford WJ, Trohman RG, Bilsker M, Zaman L, Castellanos A, Myerburg, RI. Cardiac arrest in an adolescent with atrial fibrillation and hypertrophic cardiomyopathy. J Am Coll Cardiol 1986; 7: 701–4.

    Article  PubMed  CAS  Google Scholar 

  10. Dritsas A, Sbarouni E, Gilligan D, Nihoyannopoulos P, Oakley CM. QT-interval abnormalities in hypertrophic cardiomyopathy. Clin Cardiol 1992; 15: 739–42.

    Article  PubMed  CAS  Google Scholar 

  11. Sousa MJ, Aranha J, Cotrim C et al. The QTc interval and its dispersion in hypertrophic myocardiopathy–its relation to complex ventricular arrhythmias and the effect of anti-arrhythmia agents. Rev Portuguesa Cardiol 1995; 14: 461–7.

    CAS  Google Scholar 

  12. Hii JT, Wyse DG, Gillis AM, Duff HJ, Solylo MA, Mitchell LB. Precordial QT interval dispersion as a marker of torsade de pointes. Disparate effects of class Ia antiarrhythmic drugs and amiodarone. Circulation 1992; 86: 1376–82.

    Article  PubMed  CAS  Google Scholar 

  13. Dritsas A, Gilligan D, Nihoyannopoulos P, Oakley CM. Amiodarone reduces QT dispersion in patients with hypertrophic cardiomyopathy. Int J Cardiol 1992; 36: 345–9.

    Article  PubMed  CAS  Google Scholar 

  14. Kulakowski P, Counihan PJ, Camm AJ, McKenna WJ. The value of time and frequency domain, and spectral temporal mapping analysis of the signal-averaged electrocardiogram in identification of patients with hypertrophic cardiomyopathy at increased risk of sudden death. Eur Heart J 1993; 14: 941–50.

    Article  PubMed  CAS  Google Scholar 

  15. Saumarez RC, Camm AJ, Panagos A et al. Ventricular fibrillation in hypertrophic cardiomyopathy is associated with increased fractionation of paced right ventricular electrograms. Circulation 1992; 86: 467–74.

    Article  PubMed  CAS  Google Scholar 

  16. Sepp R, Severs NJ, Gourdie RG. Altered patterns of cardiac intercellular junction distribution in hypertrophic cardiomyopathy. Heart 1996; 76: 412–17.

    Article  PubMed  CAS  Google Scholar 

  17. Peters NS. New insights into myocardial arrhythmogenesis: distribution of gap-junctional coupling in normal, ischaemic and hypertrophied human hearts. Clin Sci 1996; 90: 447–52.

    PubMed  CAS  Google Scholar 

  18. Spach MS, Dolber PC, Heidlage JF. Interaction of inhomogeneities of repolarization with anisotropic propagation in dog atria. Circ Res 1989; 65: 1612–31.

    Article  PubMed  CAS  Google Scholar 

  19. Spach MS. Anisotropic structural complexities in the genesis of reentrant arrhythmias. Circulation 1991; 84: 1447–50.

    Article  PubMed  CAS  Google Scholar 

  20. Aronson RS. Mechanisms of arrhythmias in ventricular hypertrophy. J Cardiovasc Electrophysiol 1991; 2: 249–61.

    Article  Google Scholar 

  21. Hart G. Cellular electrophysiology in cardiac hypertrophy and failure. Cardiovasc Res 1994; 28: 933–46.

    Article  PubMed  CAS  Google Scholar 

  22. Tomaselli GF, Beuckelmann DJ, Calkins HG et al. Sudden cardiac death in heart failure. The role of abnormal repolarization. Circulation 1994; 90: 2534–9.

    Article  PubMed  CAS  Google Scholar 

  23. Beuckelmann DJ, Erdmann E: Ca2+-currents and intracellular [Ca2+]i-transients in single ventricular myocytes isolated from terminally failing human myocardium. Basic Res Cardiol 1992; 87 (Suppl. 1): 235–43.

    PubMed  Google Scholar 

  24. Studer R, Reinecke H, Bilger J et al. Gene expression of the cardiac Na+-Ca2+ exchanger in end-stage human heart failure. Circ Res 1994; 75: 443–53.

    Article  PubMed  CAS  Google Scholar 

  25. Flesch M, Schwinger RH, Schiffer F et al. Evidence for functional relevance of an enhanced expression of the Na+-Ca2+ exchanger in failing human myocardium. Circulation 1996; 94: 992–1002.

    Article  PubMed  CAS  Google Scholar 

  26. Samson RA, Lee HC. Delayed afterdepolarizations and triggered arrhythmias in hypertrophic cardiomyopathic hearts. J Lab Clin Med 1994; 124: 242–8.

    PubMed  CAS  Google Scholar 

  27. Näbauer M, Beuckelmann DJ, Erdmann E. Characteristics of transient outward current in human ventricular myocytes from patients with terminal heart failure. Circ Res 1993; 73: 386–94.

    Article  PubMed  Google Scholar 

  28. Beuckelmann DJ, Näbauer M, Erdmann E. Alterations of K+ currents in isolated human ventricular myocytes from patients with terminal heart failure. Circ Res 1993; 73: 379–85.

    Article  PubMed  CAS  Google Scholar 

  29. Kääb S, Nuss HB, Chiamvimonvat N, O’Rourke B et al. Ionic mechanism of action potential prolongation in ventricular myocytes from dogs with pacing-induced heart failure. Circ Res 1996; 78: 262–73.

    Article  PubMed  Google Scholar 

  30. Kaab S, Duc J, Ashen D et al. Quantitative analysis of K channel mRNA expression in normal and failing human ventricle reveals the molecular identity of Itol. Circulation 1996; 94: I–592.

    Google Scholar 

  31. Boyden PA, Tilley LP, Albala A, Liu SK, Fenoglio JJ, Jr, Wit AL. Mechanisms for atrial arrhythmias associated with cardiomyopathy: a study of feline hearts with primary myocardial disease. Circulation 1984; 69: 1036–47.

    Article  PubMed  CAS  Google Scholar 

  32. Boluyt MO, O’Neill L, Meredith AL et al. Alterations in cardiac gene expression during the transition from stable hypertrophy to heart failure. Marked upregulation of genes encoding extracellular matrix components. Circ Res 1994; 75: 23–32.

    Article  PubMed  CAS  Google Scholar 

  33. Watkins H, Rosenzweig A, Hwang DS et al. Characteristics and prognostic implications of myosin missense mutations in familial hypertrophic cardiomyopathy. N Engl J Med 1992; 326: 1108–14.

    Article  PubMed  CAS  Google Scholar 

  34. Marian AJ, Mares A, Jr, Kelly DP et al. Sudden cardiac death in hypertrophic cardiomyopathy. Variability in phenotypic expression of beta-myosin heavy chain mutations. Eur Heart J 1995; 16: 368–76.

    PubMed  CAS  Google Scholar 

  35. Fananapazir L, Epstein ND. Genotype-phenotype correlations in hypertrophie cardiomyopathy. Insights provided by comparisons of kindreds with distinct and identical beta-myosin heavy chain gene mutations. Circulation 1994; 89: 22–32.

    Article  PubMed  CAS  Google Scholar 

  36. Moolman JC, Corfield VA, Posen B et al. Sudden death due to Troponin T mutations. J Am Coll Cardiol 1997; 29: 549–55.

    Article  PubMed  CAS  Google Scholar 

  37. Geisterfer-Lowrance AA, Christe M, Conner DA et al. A mouse model of familial hypertrophic cardiomyopathy. Science 1996; 272: 731–4.

    Article  PubMed  CAS  Google Scholar 

  38. Berul CI, Christe ME, Aronovitz MJ, Seidman CE, Seidman JG, Mendelsohn ME. Electrophysiological abnormalities and arrhythmias in aMHC mutant familial hypertrophic cardiomyopathy mice. J Clin Invest 1997; 99: 570–6.

    Article  PubMed  CAS  Google Scholar 

Download references

Authors
  1. Dan M. Roden
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Medical School, University of Crete, Heraklion, Crete, Greece

    Panos E. Vardas (Professor of Cardiology) (Professor of Cardiology)

Rights and permissions

Reprints and permissions

Copyright information

© 1998 Springer Science+Business Media Dordrecht

About this chapter

Cite this chapter

Roden, D.M. (1998). Molecular and Electrophysiological Mechanisms of Arrhythmias in Hypertrophic Cardiomyopathy. In: Vardas, P.E. (eds) Cardiac Arrhythmias, Pacing & Electrophysiology. Developments in Cardiovascular Medicine, vol 201. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-5254-9_10

Download citation

  • DOI: https://doi.org/10.1007/978-94-011-5254-9_10

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-010-6210-7

  • Online ISBN: 978-94-011-5254-9

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation