Arrhythmogenic right ventricular dysplasia / cardiomyopathy (ARVD/C) is a rare familial disorder that may cause ventricular tachycardia and sudden cardiac death in young, apparently healthy individuals. The clinical hallmark of the disease is ventricular arrhythmias, arising predominantly from the right ventricle. The pathological hallmark of the disease is fibrofatty replacement of right ventricular myocardium.
ARVD/C is caused by mutations in genes that encode desmosomal proteins. These proteins are involved with cell-to-cell adhesion. This important observation helps explain why ARVD/C is more common in athletes, and the delayed onset of the disease.
Source: Johns Hopkins
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