Abstract
ARRHYTHMOGENIC RIGHT ventricular dysplasia (ARVD) is a cardiomyopathy,1 which is familial in some patients,2 with hypokinetic areas involving the wall of the right ventricle. The most common pattern of inheritance is autosomal dominant, but penetrance ranges from 20% to 50%.3 Its prevalence is estimated at 1:10,000.4 The risk of ARVD is sudden death caused by arrhythmia, but evolution toward chronic heart failure has been described.5 ARVD is similar to Uhl's anomaly (parchment-thin right ventricular wall) and can be an important cause of ventricular arrhythmias in children and young adults with an apparently normal heart and occasionally in older patients.
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