Arrhythmogenic right-ventricular cardiomyopathy (ARVC) is a genetically determined heart disease characterized by progressive myocyte death and substitution by fibrofatty tissue. Life-threatening ventricular arrhythmias may occur during the course of the disease and are distinctively triggered by sports activity: for this reason, ARVC is one of the leading causes of sudden death in the athlete. Early identification of affected athletes by preparticipation screening in the pre-symptomatic phase is essential, but differential diagnosis with the athlete's heart may be challenging. Variants with predominant involvement of the left ventricle are difficult to diagnose unless cardiac magnetic resonance is performed. Athletes with overt ARVC or asymptomatic carriers of pathological gene mutations, including those with an implantable cardioverter defibrillator, should refrain from competitive sports, while a moderate-intensity recreational physical activity appears safe.
Keywords: Athlete’s heart; Cardiomyopathy; Preparticipation screening; Sports cardiology; Sudden cardiac death.