Which Therapeutic Approach for Patients with Hypertrophic Cardiomyopathy and Ventricular Arrhythmias?

  • M. Di Biase
  • M. V. Pitzalis
  • G. Luzzi
  • C. Forleo
  • C. D. Dicandia
  • S. Favale
  • P. Rizzon
Conference paper


Sudden unexpected cardiac death is a common feature in the natural history of hypertrophic cardiomyopathy (HCM) and in some patients is the first clinical manifestation of the disease (1–4).


Sudden Death Ventricular Tachycardia Ventricular Arrhythmia Sudden Cardiac Death Ventricular Fibrillation 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Frank S, Braunwald E (1968) Idiopathic hypertrophic subaortic stenosis: clinical analysis of 126 patients with an emphasis on the natural history. Circulation 37: 759–788PubMedGoogle Scholar
  2. 2.
    McKenna W, Deanfield J, Farouqui A, England D, Oakley CM, Goodwin JF (1981) Prognosis in hypertrophie cardiomyopathy: role of age and clinical, electrocardiographic and hemodynamic features. Am J Cardiol 47: 532–538PubMedCrossRefGoogle Scholar
  3. 3.
    Maron BJ, Bonow RO, Cannon RO III, Leon MB, Epstein SE (1987) Hypertrophie cardiomyopathy: interrelations of clinical manifestations, pathophysiology and therapy. N Engl J Med 316:780–789, 844–852Google Scholar
  4. 4.
    Maron BJ, Roberts WC, Epstein SE (1982) Sudden death in hypertrophie cardiomyopathy: a profile of 78 patients. Circulation 67: 1388–1394CrossRefGoogle Scholar
  5. 5.
    McKenna WJ, Goodwin JF (1981) The natural history of hypertrophie cardiomyopathy. In: Harvey P (ed) Current problems in cardiology, vol 6. Year Book Medical, Chicago, pp 526Google Scholar
  6. 6.
    Shapiro LM, Zezulka N (1983) Hypertrophie cardiomyopathy: a common disease with a good prognosis: five year experience of a district general hospital. Br Heart J 50: 530–533PubMedCrossRefGoogle Scholar
  7. 7.
    Spirito P, Chiarella F, Carratino L, Zoni-Berisso M, Bellotti P, Vecchio C (1989) Clinical course and prognosis of hypertrophie cardiomyopathy in an outpatient population. N Engl J Med 320: 749–755PubMedCrossRefGoogle Scholar
  8. 8.
    Kofflard MJ, Waldstein DJ, Vos J, Folkert J, Cate T(1993) Prognosis in hypertrophic cardiomyopathy observed in a large clinic population. Am J Cardiol. 72: 939–943Google Scholar
  9. 9.
    Spirito P, Rapezzi C, Autore C, Bruzzi P, Bellone P, Ortolani P, Fragola PV, Chiarella F, Zoni-Berisso M, Branzi A, CannataGoogle Scholar
  10. D, Magnani B, Vecchio C (1994) Prognosis of asymptomatic patients with hypertrophie cadiomyopathy and nonsustained ventricular tachycardia. Circulation 90: 2743–2747Google Scholar
  11. 10.
    Maron BJ, Roberts WC, Edwards JE, McAllister HA Jr, Foley DD, Epstein SE (1978) Sudden death in patients with hypertrophic cardiomyopathy: characterization of 26 patients without functional limitation. Am J Cardiol41: 803–810Google Scholar
  12. 11.
    McKenna WJ, England D, Doi YL, Deanfield JE, Oakley CM, Goodwin JF (1981) Arrhythmia in hypertrophie cardiomyopathy I: influence on prognosis. Br Heart J 46: 168–172PubMedCrossRefGoogle Scholar
  13. 12.
    Favale S, Minafra F, Rizzo U, Di Biase M, Rizzon P (1987) Ventricular fibrillation induced by transesophageal atrial pacing in hypertrophie cardiomyopathy. Eur Heart J 8: 912–916PubMedGoogle Scholar
  14. 13.
    Stafford WJ, Trohman RG, Bilsker M, Zaman L, Castellanos A, Myerburg RJ (1986) Cardiac arrest in an adolescent with atrial fibrillation and hypertrophie cardiomyopathy. J Am Coll Cardiol 7: 701–704PubMedCrossRefGoogle Scholar
  15. 14.
    Krikler DM, Davies MJ, Rowland E, Goodwin JF, Evans RC, Shaw DB (1980) Sudden death in hypertrophie cardiomyopathy: associated accessory atrioventricular pathways. Br Heart J 43: 245–251PubMedCrossRefGoogle Scholar
  16. 15.
    Frenneaux MP, Counihan PJ, Webb D, McKenna WJ (1989) Evidence for an abnormal vasodilator response in hypertrophie cardiomyopathy. J Am Coll Cardiol 13: 117AGoogle Scholar
  17. 16.
    Madariaga I, Carmona JR, Mateas FR, Lezaun R, De Los Ar-cos E (1994) Supraventricular arrhythmia as the cause of sudden death in hypertrophie cardiomyopathy. Eur Heart J 15: 134137Google Scholar
  18. 17.
    Maron BJ, Savage DD, Wolfson JK, Epstein SE (1981) Prognostic significance of 24-hour ambulatory electrocardiographic monitoring in patients with hypertrophie cardiomyopathy: a prospective study. Am J Cardiol 48: 252–257PubMedCrossRefGoogle Scholar
  19. 18.
    Fananapazir L, Epstein SE, Epstein ND (1991) Investigation and clinical significance of arrhythmias in patients with hypertrophie cardiomyopathy. J Cardiovasc Electrophysiol 2: 525–530CrossRefGoogle Scholar
  20. 19.
    Aronson R (1991) Mechanisms of arrhythmias in ventricular hypertrophy. J Cardiovasc Electrophysiol 2: 249–261CrossRefGoogle Scholar
  21. 20.
    Charpentier F, Baudet S, Le Maree H (1991) Triggered activity as a possible mechanism for arrhythmias in ventricular hypertrophy. Pace 14: 1735–1741PubMedCrossRefGoogle Scholar
  22. 21.
    Samuarez RC, Panagos A, De Beider M, Simpson I, Camm AJ, McKenna WJ (1992) Abnormal intraventricular conduction in patients with hypertrophie cardiomyopathy and out of hospital ventricular fibrillation. J Am Coll Cardiol 19: 367A (Abstr)Google Scholar
  23. 22.
    McKenna WJ, Sadoul N, Slade AKB, Samuarez RC (1994) The prognostic significance of nonsustained ventricular tachycardia in hypertrophie cardiomyopathy. Circulation 1994 90: 3115–3117Google Scholar
  24. 23.
    Fananapazir L, Tracy CM, Leon MB, Winkler JB, Cannon III RO, Bonow RO, Maron BJ, Epstein SE (1989) Electrophysiologic abnormalities in hypertrophie cardiomyopathy: a consecutive analysis in 155 patients. Circulation 80: 1259–1268PubMedCrossRefGoogle Scholar
  25. 24.
    Fananapazir L, Epstein S (1991) Value of clectrophysiologic studies in hypertrophie cardiomyopathy treated with amiodarone. Am J Cardiol 67: 175–182PubMedCrossRefGoogle Scholar
  26. 25.
    Samuarez RC, Camm AJ, Panagos A, Gill JS, Stewart JT, De Beider MA, Simpson IA, McKenna WJ (1992) Ventricular fib-Google Scholar
  27. rillation in hypertrophie cardiomyopathy is associated with increased fractionation of paced right ventricular electrograms. Circulation 86:467–474Google Scholar
  28. 26.
    Cripp TR, Peter J, Counihan MB, Frenneaux MP, Ward DE, Camm J, Mckenna WJ (1990) Signal-averaged electrocardiography in hypertrophie cardiomyopathy. J Am Coll Cardiol 15: 956–961CrossRefGoogle Scholar
  29. 27.
    Fananapazir L, Chang AC, Epstein SE, MCAreavey D (1992) Prognostic determinants in hypertrophie cardiomyopathy: prospective evaluation of a therapeutic strategy based on clinical, Holler, hemodynamic and electrophysiological findings. Circulation 86: 730–740Google Scholar
  30. 28.
    Cecchi F, Maron BJ, Epstein SE (1989) Long-term outcome of patients with hypertrophie cardiomyopathy successfully resuscitated after cardiac arrest. J Am Coll Cardiol 13: 1283–1288PubMedCrossRefGoogle Scholar
  31. 29.
    McKenna WJ (1988) The natural history of hypertrophie cardiomyopathy. In: Cardiomyopathies: clinical presentation, differential diagnosis and management. Cardiovasc Clin. 4:135149Google Scholar
  32. 30.
    McKenna WJ, Harris L, Perez G, Kriker DM, Oakley C, Goodwin JK (1981) Arrhythmia in hypertrophie cardiomyopathy II: comparison of amiodarone and verapamil in treatment. Br Heart J 46: 173–178PubMedCrossRefGoogle Scholar
  33. 31.
    Doi YL, McKenna WJ, Chetty S, Oakley CM, Goodwin JF (1980) Prediction of mortality and serious ventricular arrhythmia in hypertrophie cardiomyopathy: an echocardiographic study. Br Heart J 44: 150–157PubMedCrossRefGoogle Scholar
  34. 32.
    Pelliccia F, Cianfocca C, Romeo F (1990) Hypertrophie cardiomyopathy: long term effects of propanolol versus verapamil in preventing sudden death in “low risk” patients. Cardiovasc Drugs Ther 4: 1515–1518PubMedCrossRefGoogle Scholar
  35. 33.
    Sherrid M, Delia E, Dwyer E (1988) Oral disopyramide therapy for obstructive hypertrophie cardiomyopathy. Am J Cardiol 62: 1085–1088PubMedCrossRefGoogle Scholar
  36. 34.
    Blanchard DG, Ross J (1991) Hypertrophie cardiomyopathy: prognosis with medical or surgical therapy. Clin Cardiol 14: 11–19PubMedCrossRefGoogle Scholar
  37. 35.
    Dritsas A, Gilligan D, Nihoyannopoulos P, Oakley CM (1992) Amiodarone reduces QT dispersion in patients with hypertrophie cardiomyopathy. lot J Cardiol 36: 345–349Google Scholar
  38. 36.
    McKenna WJ, Oakley CM, Krikler DM, Goodwin JF (1985) Improved survival with amiodarone in patients with hypertrophie cardiomyopathy and ventricular tachycardia. Br Heart J 53: 412–416PubMedCrossRefGoogle Scholar
  39. 37.
    Fananapazir L, Leon MB, Bonow RO, Tracy CM, Cannon RO, Epstein SE (1991) Sudden death during empiric amiodarone therapy in symptomatic hypertrophie cardiomyopathy. Am J Cardiol 67: 169–174PubMedCrossRefGoogle Scholar
  40. 38.
    Alfonso F, Frenneaux M, McKenna WJ (1989) Clinical sustained monomorphic ventricular tachycardia in hypertrophie cardiomyopathy: association with left ventricular apical aneurysm. Br Heart J 61: 178–181PubMedCrossRefGoogle Scholar
  41. 39.
    Tendera M, Wycisk A, Schneeweiss A, Polonski L, Wodniecki J (1993) Effect of sotalol on arrhythmias and exercise tolerance in patients with hypertrophie cardiomyopathy. Cardiology 82: 335–342PubMedCrossRefGoogle Scholar
  42. 40.
    DeRose J, Banas JS, Winters SL (1994) Current perspectives on sudden cardiac death in hypertrophie cardiomyopathy. Pro-gr Cardiovasc Dis 36: 475–484CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Italia, Milano 1996

Authors and Affiliations

  • M. Di Biase
    • 1
  • M. V. Pitzalis
    • 1
  • G. Luzzi
    • 1
  • C. Forleo
    • 1
  • C. D. Dicandia
    • 1
  • S. Favale
    • 1
  • P. Rizzon
    • 1
  1. 1.Istituto di CardiologiaUniversità degli StudiPoliclinico, BariItaly

Personalised recommendations