Abstract

Idiopathic dilated cardiomyopathy (DCM) accounts for about 10,000 deaths per year in western countries. Of these deaths, 8% to 51% occur suddenly, with more than half of the events due to a ventricular arrhythmia. Improvement in diagnostic techniques and therapeutic strategies, together with changes in secular trends, have likely contributed to the reported trend toward improved survival in recent years. Identification of DCM patients at higher risk of sudden death is difficult. Poor left ventricular function is the strongest predictor of all-cause death, whereas a history of sustained unstable ventricular arrhythmia or cardiac arrest identifies patients at high risk of sudden death. Recent data suggest that a history of syncope, regardless of inducibility at programmed electrical stimulation, may be a risk factor of sudden death. Despite the absence of controlled studies, use of implantable cardioverter defibrillator therapy for primary prevention can be considered in patients with unexplained syncope as well as subgroups of DCM patients awaiting transplantation. In patients who survive a cardiac arrest or an unstable ventricular tachycardia, use of implantable cardioverter defibrillator therapy is associated with improved survival during follow-up and should be considered as a first-line therapy.

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