Arrhythmogenic right ventricular cardiomyopathy and sports activity

Extract

This editorial refers to ‘Association of competitive and recreational sport participation with cardiac events in patients with arrhythmogenic right ventricular cardiomyopathy: results from the North American multidisciplinary study of arrhythmogenic right ventricular cardiomyopathy’^†, by A.C. Ruwald et al., on page 1735.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart muscle disease characterized by ventricular electrical instability which may lead to arrhythmic cardiac arrest, mostly in young people and athletes.¹ Molecular genetics studies have provided significant insights towards our understanding of the pathogenesis of ARVC, showing that it is a genetic disorder resulting from defective desmosomal proteins.² The pathological hallmark of the disease is the progressive loss of myocardium with subsequent myocyte death and fibrofatty scar which predisposes to life-threatening ventricular arrhythmias.³ Competitive sports activity increases the risk of sudden cardiac death (SCD) by five-fold in adolescent and young adults with ARVC.⁴ Sports has also been implicated as a factor promoting disease progression and worsening of the arrhythmic substrate (Figure 1). Genetically determined impairment of cell to cell adhesion may lead to myocyte death, especially during mechanical stress that occurs during competitive sports activity.¹ Kirchhof et al.⁵ demonstrated that in heterozygous plakoglobin-deficient mice, endurance training accelerated the development of right ventricular (RV) abnormalities and ventricular arrhythmias. James et al.⁶ confirmed in humans that endurance sports and frequent exercise increase age-related penetrance, risk of ventricular tachyarrhythmias, and occurrence of heart failure in ARVC desmosomal gene carriers.¹