A 10-year follow-up of a patient affected by myotonic dystrophy type 1 with implantable cardioverter-defibrillator implanted for secondary prevention

Cardiac involvement in myotonic dystrophy type 1 (DM1) is frequent with an increased incidence of conduction disturbances and sudden cardiac death when compared with the general population. We described the case of a young man in whom the diagnosis of DM1 was made 8 years after the occurrence of cardiac arrest (CA) owing to ventricular fibrillation and ICD was implanted 4 years later (12 years after CA). During the subsequent 10 years, despite the deterioration of neuromuscular conditions, no appropriate or inappropriate shocks occurred.