The mystery of arrhythmogenic right ventricular dysplasia/cardiomyopathy: from observation to mechanistic explanation.

Only 24 years have elapsed from the time that the clinical profile of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) was first described.1 Since then, this entity has been found to have a prevalence of about 1 in 5000 persons2 and is well recognized in the United States, Europe, and Asia. The usual clinical presentation of ARVD/C is that of palpitations, nonsustained ventricular tachycardia, and sustained ventricular arrhythmias. Uncommonly, sudden cardiac death may be the first manifestation of the disease. Most patients with this condition experience the onset of these symptoms between the ages of 20 and 40 years, and the disease shows a predisposition to occur in men. A familial incidence was noted in the early description of the disease.