Exercise May Trigger Clinical Manifestations of ARVC in Mutation Carriers

Mark S. Link, MD

In an analysis of data from an ARVC registry, phenotypic expression was strongly associated with endurance athletic activities.

Sudden cardiac death in competitive athletes is generally attributable to underlying heart disease. In patients with arrhythmogenic right ventricular cardiomyopathy (ARVC), rigorous exercise is believed to give rise to malignant arrhythmias. However, recent studies in mice suggest that strenuous activity not only triggers life-threatening arrhythmias in individuals with clinical ARVC, but also increases the risk for disease development in unaffected individuals with ARVC-associated desmosomal mutations. To investigate further, researchers used data from a registry of ARVC patients and their families to evaluate the influence of strenuous exercise on the development of clinical ARVC in humans.

Of 87 ARVC-mutation–positive individuals (age range at baseline, 11–88 years), 64% were characterized as endurance athletes based on predefined criteria for exercise intensity and duration. Only endurance athletes had first ventricular arrhythmias during follow-up (mean, 8.4 years). Compared with nonparticipants in endurance athletics, endurance athletes developed symptoms at a younger age (30 vs. 40 years) and were significantly more likely to meet 2010 Task Force Criteria for ARVC (82% vs. 35%) and to have clinical ventricular arrhythmias. Furthermore, exercise reduction significantly decreased the risk for malignant arrhythmias in individuals who exercised the most.

Citation(s):

James CA et al. Exercise increases age-related penetrance and arrhythmic risk in arrhythmogenic right ventricular dysplasia/cardiomyopathy associated desmosomal mutation carriers. J Am Coll Cardiol 2013 Jul 17; [e-pub ahead of print].