Arrhythmogenic Right Ventricular Cardiomyopathy: Diagnosis, Risk Stratification, and Treatment

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic heart disease with a prevalence of 1 : 2000 to 1 : 5000. It is characterized by the progressive replacement of heart muscle tissue by fatty and connective tissue. It is associated with a high risk of sudden cardiac death. This review presents the current state of knowledge regarding the diagnostic assessment, risk stratification, and treatment of ARVC. Methods: This review is based on pertinent publications retrieved by a search in PubMed using the keywords "ARVC" and "arrhythmogenic right ventricular dysplasia". Guidelines, clinical registry studies, meta-analyses, and randomized controlled trials were evaluated. Results: The diagnosis is established with the aid of (long-term) ECG, echocardiography, magnetic resonance imaging, and genetic tests. The ARVC risk calculator is used to assess the risk of ventricular arrhythmia. Patients' participation in sports is restricted. Beta-blockers are recommended for patients with extrasystoles or ventricular tachycardia (grade I recommendation). If beta-blockers alone have an insufficient effect, amiodarone, flecainide or sotalol can be added (grade IIa). For patients with recurrent ventricular tachyarrhythmia, catheter ablation is an option (grade IIa). While there is a clear recommendation (grade I) for defibrillator implantation for patients who have survived sudden cardiac death, the ARVC risk calculator should be used for decisionmaking in patients for whom primary prophylactic implantation is considered (recommendation grade IIa). Conclusion: ARVC is associated with an increased risk of sudden cardiac death. Risk assessment remains challenging in the absence of randomized controlled trials, particularly with regard to the primary prophylactic implantation of a defibrillator.