Sudden death due to ventricular tachycardia during amiodarone therapy in familial hypertrophic cardiomyopathy

The potential modes of sudden death in hypertrophic cardiomyopathy (HC) can be broadly divided into those in which arrhythmia is the primary cause and those in which initial hemodynamic deterioration or ischemia are followed by secondary arrhythmia. Fortuitous electrocardiographic monitoring at the time of cardiac arrest may elucidate the mechanism responsible in individual patients and improve our understanding of sudden death in this condition. We report on 1 patient from a family with HC who died suddenly during amiodarone therapy and who was wearing a Holter monitor at the time of death. Death was due to primary ventricular tachycardia progressing to ventricular fibrillation.