Implantable Cardioverter Defibrillator Use in Males with Duchenne Muscular Dystrophy and Severe Left Ventricular Dysfunction

Abstract

Purpose Duchenne muscular dystrophy (DMD) is characterized by myocardial fibrosis and left ventricular (LV) dysfunction. Implantable cardioverter defibrillator (ICD) use has not been characterized in this population but is generally considered for patients with severe LV dysfunction receiving goal directed medical therapy (GDMT), including a beta blocker plus an angiotensin converting enzyme inhibitor. We evaluated utilization and efficacy of ICD use in patients with DMD. Methods Retrospective cohort study of DMD patients followed at 17 centers across the US and Canada from January 2, 2005-January 1, 2018. ICD use and its effect on survival was evaluated in those patients with severe LV dysfunction (shortening fraction (SF) Results Severe LV dysfunction was present in 57/436 (13.1%) patients, of which 11 (19.3%) died during the study period, as compared to 17/379 (4.5%) who died without severe LV dysfunction (p Conclusion ICD use may be associated with improved survival and minimal complications in DMD cardiomyopathy with severe LV dysfunction. However, inconsistent utilization of GDMT may be a significant confounder.Future studies are needed to define optimal ICD use in patients with DMD related cardiomyopathy.