HYPERTROPHIC CARDIOMYOPATHY - EVALUATION AND TREATMENT OF PATIENTS ATHIGH-RISK FOR SUDDEN-DEATH

Hypertrophic cardiomyopathy (HCM) is a heritable disease characterized by LV hypertrophy with markedly variable clinical, morphological, and genetic manifestations. It is the most common cause of sudden death i n otherwise healthy young individuals. HCM patients often have disabli ng symptoms and are prone to arrhythmias. Frequently, there is associa ted LV systolic and diastolic dysfunction, LV outflow obstruction, and myocardial ischemia. Over the past decade, progress has been made in identifying patients who are at high risk for sudden death, in elucida ting potential mechanisms of sudden death, and in defining therapeutic algorithms that may improve prognosis. It has also been possible to d etermine the genetic defect in some of the patients and to correlate c linical findings with the molecular defects. An exciting development h as been the use of the dual chamber pacemaker as an alternative to car diac surgery to improve symptoms and relieve LV outflow obstruction.