Overcoming challenges in the management of arrhythmogenic right ventricular cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy (ARVC) appears in most patients to be an inherited disease characterized by fibrofatty replacement of myocytes extending from the epicardium to the endocardium in the right ventricle. The disease process results in life‑threatening ventricular arrhythmias and ventricular dysfunction. In the absence of a gold‑standard diagnostic test and despite the progress in imaging techniques, ARVC is often misdiagnosed and earlier detection of the disease is challenging. Preprocedural identification and localization of the substrate can be determined from the analysis of surface electrocardiography and cardiac magnetic resonance imaging. Typically, perivalvular arrhythmogenic substrate, defined by electroanatomic mapping, is present and can be isolated to the epicardium. Ablation targets are further identified with activation, entrainment, and local electrogram abnormalities based on detailed electroanatomic mapping. Extensive combined endo / epicardial ablation performed in experienced centers is frequently required to prevent ventricular tachycardia (VT). Catheter ablation significantly reduces recurrences of VT, appropriate implantable cardioverter‑defibrillator shocks, and the use of antiarrhythmic drugs and cardiac transplant as a management strategy for refractory arrhythmias is rarely required. Progression of the disease is poorly understood and may require a distinct triggering mechanism. Biventricular involvement is more common than previously recognized. However, left ventricular involvement leading to significant terminal heart failure is fortunately uncommon and left ventricular tachycardias are also infrequent. Many questions remain regarding prevention and management of coexisting tricuspid valve regurgitation, atrial arrhythmias, and intracardiac thrombosis. Although data on genotype‑phenotype correlations is growing, long‑term follow‑up studies of families with ARVC are still lacking. Ongoing research will contribute to better understanding of this pathological condition