Inherited cardiac muscle disorders: Arrhythmogenic right ventricular cardiomyopathy

Abstract

Arrhythmogenic Cardiomyopathy (AC) is characterized by cardiomyocyte death with fibro-fatty repair, clinically manifesting with palpitations, syncope or cardiac arrest usually in adolescence or young adulthood. AC represents one of the major causes of sudden death in the young and athletes. The estimated prevalence of AC in the general population ranges from 1:2000 to 1:5000. AC affects more frequently males than females (up to 3:1), despite a similar prevalence of carrier status, and due to age-related penetrance becomes clinically overt most often in the second-fourth decade of life.