"Petrified" right ventricle in long-standing naxos arrhythmogenic right ventricular cardiomyopathy.

A 19-year-old asymptomatic man was evaluated because of his sister’s sudden death due to Naxos disease, a recessive form of arrhythmogenic right ventricular (RV) cardiomyopathy associated with palmoplantar keratoderma and woolly hair. It is caused by a plakoglobin mutation. Twelve-lead ECG showed inverted T waves in V1 through V6, incomplete right bundle branch block, and epsilon waves (Figure 1). Frequent ventricular extrasystoles were recorded on a 24-hour ECG. On echocardiography and angiography, the right ventricle was dilated and diffusely hypokinetic, but the …