Ventricular Arrhythmias and Sudden Cardiac Death in Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disease characterized by myocardial hypertrophy and disarray caused by mutations in genes coding for cardiac sarcomeres. HCM is the most common inherited heart disease with a prevalence of 0.2%. Patients with HCM are at an increased risk of potentially lethal ventricular arrhythmias. Prevention of sudden cardiac death is best accomplished by implantation of a cardioverter-defibrillator (ICD) in patients who are at substantial risk of sudden cardiac death due to ventricular arrhythmias. Selection of patients in whom ICD implantation is appropriate is guided by the presence or absence of specific clinical risk factors.