Jornal Internacional de Pesquisa Cardiovascular

Role of an Implantable Cardioverter Defibrillator in Emery Dreifuss Muscular Dystrophy: A Case Report and a Review of the Literature

Priyank Shah, Nishant Gupta, Sharad Bajaj, Mahesh Bikkina, Fayez Shamoon and Raja?a Kaddaha

Role of an Implantable Cardioverter Defibrillator in Emery Dreifuss Muscular Dystrophy: A Case Report and a Review of the Literature

Emery dreifuss muscular dystrophy (EDMD) is one of the three most common inherited muscular dystrophies characterized by a triad of contractures, muscle weakness, and cardiac involvement (conduction abnormalities and/or cardiomyopathy). It can be inherited as X-linked recessive, autosomal dominant or autosomal recessive pattern. Cardiac involvement is evident in virtually all cases by the end of 4th decade. There is no relationship between the severity of cardiac and skeletal muscle involvement, irrespective of the mode of inheritance and type of mutations.