Implantable Defibrillators and Prevention of Sudden Death in Hypertrophic Cardiomyopathy


  • Dr. Maron received research support from Medtronic Inc.

  • Manuscript received 20 December 2007; Revised manuscript received 29 January 2008; Accepted for publication 30 January 2008.

  • Editor: Stephen C. Hammill, M.D.

Address for correspondence: Barry J. Maron, M.D., Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, 920 E. 28th Street, Suite 620, Minneapolis, MN 55407, USA. Fax: 612-863-3875; E-mail:


Hypertrophic cardiomyopathy (HCM) is the most common cause of sudden cardiac death in young people, including trained athletes. The implantable cardioverter-defibrillator (ICD), although initially designed as a treatment for older patients with coronary artery disease, has more recently proved to be a safe and effective therapeutic intervention in young patients with HCM, both for primary or secondary prevention of sudden death. The largest such report of >500 patients showed that the ICD intervened appropriately to abort ventricular tachycardia/fibrillation (VT/VF) in 20% of patients over an average follow-up period of only 3.7 years, at a rate of about 4% per year in those patients implanted prophylactically, and often with considerable delays of up to 10 years. Extensive experience with high-risk HCM patients showed that appropriate device discharges for VT/VF occur with similar frequency in patients with 1, 2, or ≥3 noninvasive risk markers. Despite the extreme morphology characteristic of HCM, often with massive degrees of left ventricular (LV) hypertrophy and/or LV outflow tract obstruction, ICDs performed in a highly effective fashion, with failure to convert life-threatening arrhythmias extraordinarily rare. In conclusion, in a large high-risk HCM cohort, ICD interventions for life-threatening ventricular tachyarrhythmias were frequent and highly effective in restoring normal rhythm. An important proportion of ICD discharges occurred in primary prevention patients with only one risk factor. Therefore, a single marker of high risk may represent sufficient evidence to justify the recommendation for a prophylactic ICD in selected patients with HCM.