Neuromuscular Disorders and the Role of the Clinical Electrophysiologist

Abstract Cardiac involvement is common and may be the presenting or predominant manifestation in a variety of neuromuscular disorders, most notably the inherited muscle disorders, or muscular dystrophies. Cardiac manifestations of the neuromuscular disorders result from pathological involvement of the myocardium and the cardiac conduction system, with resulting cardiomyopathy or rhythm disturbances including supraventricular arrhythmias, life-threatening ventricular arrhythmias, and sudden cardiac death. Many of these neuromuscular disorders are rare and may be unrecognized by even experienced specialists in internal and cardiovascular medicine. Furthermore, the initial cardiac manifestations in these patients are often asymptomatic. The goal of this investigation is to review the scope of cardiac conduction defects and rhythm disturbances in these disorders and to propose some practical recommendations for arrhythmia monitoring and management of these patients.