Arrhythmogenic right ventricular dysplasia

Arrhythmogenic right ventricular dysplasia (ARVD) is a new form of cardiomy opathy probably more frequent than commonly reported. It is a rare but impo rtant cause of sudden arrhythmic death in young, otherwise healthy persons, as well as a subtle cause of congestive heart failure. It may lead to temp orary incapacitation with catastrophic consequences. Proper electrocardiogr aphic criteria, echocardiography, nuclear medicine, or magnetic resonance i maging could identify most of these individuals. With the exception of full -thickness histological examination of the right ventricular free wall, con trast ventriculography remains the most definitive standard for a positive diagnosis. The wide clinical spectrum of arrhythmogenic right ventricular c ardiomyopathies/dysplasia appears to be the result of one or possibly two f actors: (a) replacement of most of the right ventricular myocardium by fat and (b) genetic susceptibility to environmental agents (myocarditis). Curre nt treatment modalities include drug therapy, catheter or surgical ablative techniques, and modern treatments of congestive heart failure. Heart trans plant is exceptional. Implantable defibrillators, used alone or in combinat ion with drug therapy, will probably play an increasing role in ARVD and re lated cardiomyopathies.