Diagnosis of arrhythmogenic right ventricular cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an infrequently diagnosed condition with a high incidence of sudden cardiac death. While the only option for cure is orthotopic cardiac transplantation, the use of an implantable cardiac defibrillator can be life saving. Accordingly, the prompt recognition of ARVC is crucial. Fortunately, a definitive diagnosis of ARVC can often be made by a combination of the clinical history and electrocardiogram alone, as illustrated by the present case. In our patient, who presented with multiple syncopal episodes and in whom ventricular tachycardia was observed shortly after her admission to the emergency department, a lengthy and expensive workup was initiated, which ultimately may have delayed implantation of an implantable cardiac defibrillator. Clinicians should be made aware of the clinical guidelines for dealing with this potentially dangerous condition so that appropriate therapy can be promptly initiated.