WHICH PATIENTS WITH HYPERTROPHIC CARDIOMYOPATHY SHOULD RECEIVE

Hypertrophic cardiomyopathy is a genetic and heterogeneous disease with diverse clinical course, but with a low incidence of sudden car-diac death in the general population. Nonetheless sudden cardiac death accounts for about 50 % of mortality in hypertrophic cardiomyopathy, and ventricular tachyarrhythmais are the principal mechanism of this catastrophic event. Therefore, the identification of high-risk patients who are the potential candidates for implantable car-dioverter-defibrillator (ICD) therapy is the first step in the management of patients with hyper-trophic cardiomyopathy. The factors that best identify such patients include survived cardiac arrest or sustained ventricular tachycardia, a family history of sudden cardiac death due to hypertrophic cardiomyopathy, left ventricular hypertrophy ≥ 30 mm, syncope, symptomatic non-sustained ventricular tachycardia, and exer-cise-induced hypotension in patients ≤ 40 years. The decision to implant an ICD in patients with hypertrophic cardiomyopathy depends on the symptoms and level of risk. ICD therapy is indicated in patients for secondary prevention after sustained ventricular tachycardia or ven-tricular fibrillation. The ICD implantation is recommended for primary prevention of sud-den cardiac death in patients with two or more risk factors in whom annual rates of sudden car-diac death are 3-6 % or more. The presence of extreme left ventricular hypertrophy, or a recur-rent syncope on exertion should be also consid-ered for ICD implantation in individual young patients. At present, other single risk factors, because of its imprecision in risk stratification, cannot justify prophylactic therapy with ICD