Arrhythmogenic right ventricular cardiomyopathy - Issues in sports medicine

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a major cause of sudden cardiac death and ventricular tachyarrhythmias in young, apparently healthy individuals and athletes. Myocardial atrophy with subsequent fibrofatty replacement predominantly affects right ventricular myocardium and results in global and regional dysfunction as well as areas of slow conduction and dispersion of refractoriness which are prerequisites for reentrant ventricular tachyarrhythmias. Clinical and molecular genetics have recently identified mutations in genes encoding for desmosomal cell adhesion proteins. These may predispose for rupture of mechanical cell contacts, triggered by stretch of the right ventricular free wall during exercise and sports activity. This may explain the relatively high prevalence of ARVC among athletes, the dominant manifestation in the right ventricle and the frequent provocation of arrhythmias during exercise. Therefore, ARVC has become important for sports eligibility, preparticipation screening and follow-up evaluation of athletes. Patients affected with ARVC should be excluded from competitive sports and vigorous training. To provide optimal treatment, a detailed diagnostic evaluation and risk stratification is mandatory. Tailored treatment strategies aim at the suppression or effective termination of recurrent ventricular tachyarrhythmias and prevention of sudden death by antiarrhythmic drug therapy, catheter ablation, or implantation of a cardioverter defibrillator (ICD).