'Unexpected' sudden death avoided by implantable cardioverter-defibrillator in Emery-Dreifuss patient

A female patient just over 20 years of age developed first grade atrioventricular block, and later atrial fibrillation. When she was 41 years old she was diagnosed with Emery-Dreifuss muscular dystrophy (EDMD). A VVIR pacemaker was implanted in 2002, replaced in 2003 with an ICD. Nine months later, during febrile illness, the patient experienced three appropriate ICD discharges. No further ICD interventions occurred. The transient course of arrhythmic activity and the possible influence of triggering factors lessen the role of electrophysiologic study to identify risk of sudden death, and suggest that in patients with EDMD requiring pacemaker implantation, an ICD would be more properly indicated.