Arrhythmogenic right ventricular dysplasia: an uncommon cause of syncope in a young male

Arrhythmogenic right ventricular dysplasia (ARVD) is a relatively rare disease first described in 1977. It is characterized by replacement of the right ventricle (RV) by fat or fibrous tissue. Patients often present with symptomatic ventricular tachycardia (VT) and the risk of sudden death is approximately 2.5% per year. Physical exam is often unremarkable. To date, the best diagnostic test is the MRI, however, electrocardiographic and echocardiographic data are useful. Treatment can be with beta blockers if a medical approach is desired. Multiple surgical options are available. No long-term studies are available to guide therapy; thus each case should be dealt with on its merits. This article describes the case of a 41-year-old male who had been asymptomatic until he presented with syncope. An extensive work up was undertaken and ARVD was diagnosed by MRI.