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Arrhythmogenic Right Ventricular Cardiomyopathy: Toward a Modern Clinical and Genomic Understanding.

Since its first description in the 1700s by the pope’s physician, a professor of anatomy in Rome,1 the condition known to some as arrhythmogenic right ventricular cardiomyopathy (ARVC) has resisted attempts at categorization. In 1982, when the first major case series was published in Circulation , Marcus et al2 described patients with recurrent ventricular tachycardia, RV enlargement, and partially or totally absent RV myocardium replaced instead by fibrofatty tissue. Thought to be a congenital developmental defect of the RV musculature, the condition was classified as a dysplasia. Although this thinking was later revised, the initial observation is memorialized in the hybrid name arrythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), still in wide use. In the intervening years, new information about this progressive, hereditary disease has repeatedly forced us to redraw the lines between categories that, for mental clarity, we like to think of as distinct: cardiomyopathy versus arrhythmia syndrome; hereditary versus lifestyle-induced. In this issue of Circulation: Cardiovascular Genetics , 2 leading groups in ARVC research, from Johns Hopkins University and from an interuniversity consortium in The Netherlands, have joined their patient registries to help better define the natural history of this still-perplexing disease. The result is the largest, most comprehensive long-term outcomes study yet—involving 439 ARVC index patients who were followed for a median of 7 years, and 562 of their family members.3 The results, in line with previous observations, are a valuable contribution.

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The hallmark of this disease is ventricular arrhythmias, which predispose patients to syncope and sudden cardiac death—often during exercise. The first signs of ARVC typically do not appear until adolescence or early adulthood.1,4,5 Disease expression is highly variable, both between and within families, and penetrance is reduced: estimated at perhaps 60% during a lifetime.6 …