This article reviews epidemiology, clinical-morphological aspects, and primary and secondary prevention of sudden cardiac death in patients with chronic Chagas' heart disease in the current era. Chagas' disease patients with life-threatening ventricular tachyarrhythmias are at risk of sudden cardiac death. No evidence-based support is available for guiding prophylaxis of sudden cardiac death in patients with this condition. Therefore, measurements for sudden cardiac death prevention in Chagas' disease patients have been derived from data obtained in non-Chagas' disease patients as well as on expertise of physicians dealing with this disorder in areas where Chagas' disease is endemic. For primary prevention, therefore, we suggest that patients with non-sustained VT be referred to programmed ventricular stimulation. Patients with inducible VT will be better treated with Implantable Cardioverter-Defibrillator (ICD) than with amiodarone therapy. For secondary prevention, we suggest that patients with malignant ventricular tachyarrhythmias with hemodynamic instability receive ICD therapy. For patients with life-threatening ventricular arrhythmias and no hemodynamic instability, however, secondary prevention can be accomplished with ICD therapy or catheter ablation in those with a left ventricular ejection fraction (LVEF) of 30% or less, and with electrophysiologic testing-guided drug therapy or empiric treatment with amiodarone in those with a LVEF of 30% or high. (C) 2008 Elsevier Ireland Ltd. All rights reserved.