Abstract
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease of the cardiac muscle characterized by right ventricular dysfunction (global or regional) with or without left ventricular (LV) involvement in the presence of histological evidence of the disease and/or electrocardiographic abnormalities according to published criteria. ARVC has age-dependent penetrance and is mainly characterized by episodes of ventricular arrhythmia that can progress to sudden cardiac death (SCD). The estimated prevalence ranges from 1:2,000 to 1:5,000, with a predominance in the Caucasian population and in people who practice strenuous exercise or competitive sports. Despite its low prevalence, ARVC represents approximately 5–20% of CSM cases in young people.
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