Cardiac Complications of Neuromuscular Disorders

Neuromuscular disorders are a diverse group of disorders with variable cardiac involvement necessitating specialized cardiac evaluation. Duchenne muscular dystrophy is most severely affected, and cardiac disease is now the leading cause of death. Advanced imaging technology including echocardiography and cardiac magnetic resonance imaging can lead to early identification of affected myocardium and direct pharmacotherapies to slow the progression of cardiomyopathy. Becker muscular dystrophy is also associated with cardiomyopathy, usually with a later onset and slower course. Carriers of dystrophin mutations can also develop cardiomyopathy and require screening. Several subtypes of limb-girdle muscular dystrophy have similar risks of cardiac disease as Duchenne muscular dystrophy. Some neuromuscular disorders including myotonic dystrophies and Emery-Dreifuss muscular dystrophy are primarily associated with conduction defects and can require pacemaker implantation. And other disorders such as Friedreich ataxia and Pompe disease can be associated with hypertrophic cardiomyopathy. New therapies and interventional strategies can be used to improve the quality and duration of life in neuromuscular patients with cardiac involvement. Based on this diversity, a multidisciplinary approach including a cardiologist is essential to optimize care for the patient with a neuromuscular disorder.