Evidence that pharmacological strategies lack efficacy for the prevention of sudden death in hypertrophic cardiomyopathy

Objective: To determine the efficacy of pharmacological treatment in the prevention of sudden cardiac death in hypertrophic cardiomyopathy (HCM). Design: Clinical outcome was assessed retrospectively in 293 patients with HCM, including 173 who were taking cardioactive medications. Setting: Department of Cardiology, University of Padua, Padua, Italy; a tertiary HCM Centre. Interventions: Medical treatment with β blockers, verapamil, sotalol and amiodarone. Main outcome measure: HCM-related sudden cardiac death. Results: 17 of 173 (10%) patients died suddenly or had aborted cardiac arrest, while being treated continuously with drugs having antiarrhythmic properties, over a period of 62 (56) months. Sudden death occurred in 20% of patients administered amiodarone (6/30), 9% each of patients taking verapamil (4/46) or β blockers (7/76), and 0% of those taking sotalol (0/21). Patients taking cardioactive drugs (n = 173) and those without pharmaceutical therapy (n = 120) did not differ with respect to sudden death mortality. Conclusion: Medical treatment is not absolutely protective against the risk of sudden death in HCM. The present data inferentially support the use of the implantable defibrillator as the primary treatment choice for prevention of sudden death in high-risk patients with HCM.

[1]  C. Semsarian,et al.  Long-term follow-up of implantable cardioverter defibrillator therapy for hypertrophic cardiomyopathy. , 2004, The American journal of cardiology.

[2]  M. Link,et al.  Primary prevention of sudden death as a novel treatment strategy in hypertrophic cardiomyopathy. , 2003, Circulation.

[3]  B. Maron Hypertrophic cardiomyopathy: a systematic review. , 2002, JAMA.

[4]  E. Behr,et al.  Hypertrophic cardiomyopathy: management, risk stratification, and prevention of sudden death , 2002, Heart.

[5]  P. Elliott,et al.  Sudden death in hypertrophic cardiomyopathy: identification of high risk patients. , 2000, Journal of the American College of Cardiology.

[6]  B. Maron,et al.  Epidemiology of hypertrophic cardiomyopathy-related death: revisited in a large non-referral-based patient population. , 2000, Circulation.

[7]  M. Link,et al.  Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. , 2000, The New England journal of medicine.

[8]  T. Riesenfeld,et al.  A cohort study of childhood hypertrophic cardiomyopathy: improved survival following high-dose beta-adrenoceptor antagonist treatment. , 1999, Journal of the American College of Cardiology.

[9]  S. Hohnloser,et al.  Prevention of implantable-defibrillator shocks by treatment with sotalol. d,l-Sotalol Implantable Cardioverter-Defibrillator Study Group. , 1999, The New England journal of medicine.

[10]  F. Triposkiadis,et al.  Hypertrophic cardiomyopathy in Greece: clinical course and outcome. , 1998, Chest.

[11]  B. Maron,et al.  Prognostic value of non-sustained ventricular tachycardia and the potential role of amiodarone treatment in hypertrophic cardiomyopathy: assessment in an unselected non-referral based patient population , 1998, Heart.

[12]  M. Burch,et al.  Hypertrophic cardiomyopathy. , 1994, Archives of disease in childhood.

[13]  C. Oakley,et al.  Improved survival with amiodarone in patients with hypertrophic cardiomyopathy and ventricular tachycardia. , 1985, British heart journal.