Cardiac Manifestations of Neuromuscular Disease

Neuromuscular diseases are a group of complex multisystem disorders that commonly and prominently affect striated muscle. Cardiac involvement in neuromuscular diseases namely conduction disorders, ventricular arrhythmias and cardiomyopathy with its impact on prognosis, is often dissociated with the peripheral myopathy. Therefore, close surveillance is mandatory in the affected patients. In this context, preventive therapy has been recently recommended in the most common neuromuscular diseases. This chapter focuses on the heart involvement of the muscular dystrophies exhibiting prominent cardiac manifestations, including Myotonic dystrophy type 1, Duchenne (DMD), Becker (BMD), Emery-Dreifuss (EDMD) and Facio-Scapulo-Humeral muscular dystrophies.