Arrhythmogenic right ventricular cardiomyopathy: clinical registry, tissue and DNA bank

A multidisciplinary collaborative European study has been designed with the aim to investigate the clinical, pathological and genetic features of arrhythmogenic right ventricular cardiomyopathy (ARVC), which is a progressive, genetically determined disorder of the right ventricular myocardium and a major risk of sudden death particularly in the young.1–3 The disease is reported familial up to 50% with autosomal dominant inheritance while an autosomal recessive form (Naxos disease) associated with cutaneous abnormalities also exists. Nine genetic loci and mutations in three genes have been discovered so far.4–7 Treatment and prevention of ventricular tachyarrhythmias and sudden death include antiarrhythmic drug therapy, catheter ablation and the implantable cardioverter-defibrillator.However, a systematic evaluation of treatment options is not yet available.