Arrhythmogenic right ventricular cardiomyopathy: diagnosis and risk stratification

Arrhythmogenic right ventricular cardiomyopathy (ARVCM) is a genetically determined disease characterized by the progressive replacement of cardiomyocytes by fibrofatty tissue, predominantly in the right ventricle. It leads to electrical instability and is therefore a major cause of sudden cardiac death (SCD) in apparently healthy young individuals, particularly in athletes. The diagnosis of ARVCM can be challenging and is based on a set of major and minor criteria which include structural, functional, histological, imaging, electrocardiographic and anamnestic parameters. ARVCM can be diagnosed, when two major criteria, or one major and two minor criteria, or four minor criteria from different categories are present. An implantable cardioverter defibrillator (ICD) should be used in patients who were resuscitated from SCD or who present with sustained ventricular tachycardia or otherwise unexplained syncope. The role of ICD therapy in primary prevention of SCD is a matter of ongoing debate and has to be decided on an individualized basis. Due to the familial accumulation of the disease, the screening of relatives is important. For the symptomatic treatment of arrhythmias, beta-blockers, sotalol, amiodarone and catheter ablation can be used. Arrhythmias in patients with ARVCM often occur in conjunction with physical exercise. Patients with ARVCM should therefore abstain from competitive sports or leisure-time activities where any possible loss of consciousness poses an increased hazard (scuba diving, hang gliding, parachuting, etc.).