Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by progressive fibrofatty replacement of the myocardium, providing the substrate for ventricular reentrant tachycardia, typical for the overt phase of ARVC. Importantly, life-threatening ventricular arrhythmias may also occur in the concealed phase of the disease. The reported rates for sustained ventricular arrhythmias and all-cause mortality in ARVC differ substantially across cohorts. Survival free from sustained ventricular arrhythmias in patients with ARVC-related pathogenic mutations is approximately 66% and 42% at age 40 and 60 years, respectively, in tertiary referral centres. Participation in competitive sports has been associated with an increased propensity of ventricular arrhythmias/death and should therefore be avoided. Beta-blocker therapy may prevent exercise-induced ventricular arrhythmias and should be considered in all patients with ARVC. In patients with unexplained syncope and other recognized risk factors, the implantation of an implantable cardioverter defibrillator (ICD) for primary prevention may be considered. Decisions should be made carefully, weighing the risk of sudden cardiac death, the lifelong risk of complications, and the impact of an ICD on lifestyle and psychological health. ICDs are associated with significant adverse event rates, in particular in young patients who may carry an ICD for several decades.
Exercise-induced ventricular arrhythmias and risk of sudden cardiac death in patients with hypertrophic cardiomyopathy
