Cardiac arrhythmias as presenting symptoms in patients with limb-girdle muscular dystrophy

Clinically manifest muscular dystrophy is often accompanied by functional and anatomic derangements in the myocardium which often have prognostic significance. We describe two young patients who had unrecognized limb-girdle muscular dystrophy who presented with cardiac arrhythmia. One developed dilated cardiomyopathy complicated by ventricular tachyarrhythmia. The other patient had atrial paralysis requiring permanent pacing. It is important to consider the possibility of underlying muscular dystrophy in patients who present with cardiac arrhythmia without an obvious cause.