[Cardiac involvement in the course of Emery-Dreifuss muscular dystrophy].

Emery-Dreifuss muscular dystrophy (EDMD) is a rare hereditary X-linked disease characterized by progressive muscle weakness, cardiac conduction defects and arrhythmias. Sudden cardiac death common in patients suffering from EDMD has been attributed to advanced atrio-ventricular conduction disorders and ventricular tachyarrhythmias. We present a case of 20-year-old female patient with EDMD, with bradycardia, atrio-ventricular block, non sustained tachyarrhythmia recorded on 24-hour ambulatory ECG, complained of palpitations and presyncope and managed by pacemaker insertion.