Proarrhythmic Manifestations of Neuromuscular Dystrophinopathies

Muscular dystrophy has been an elusive term ever since it was first described in the 19th century. Introduced in 1891 by Wilhelm Heinrich Erb, muscular dystrophy has been classified as part of a larger group of genetically determined, progressive degenerative neuromuscular disorders termed "dystrophinopathies." Cardiac arrhythmias may occur during the neurologic course of the disease. Although descriptions of the dystrophinopathies have been reported in the literature, few articles address the use of antiarrhythmic pharmacotherapy in patients with muscular dystrophy. We discuss the pathophysiology of the most common dystrophinopathies, their proarrhythmic sequelae, and the therapeutic use of antiarrhythmic agents in the clinical setting.