Arrhythmogenic right ventricular cardiomyopathy/dysplasia

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a progressive disease of predominantly right ventricle, characterized by ventricular arrhythmias possible leading to sudden cardiac death. Genetic predisposition was confirmed more than 15 years ago. Autosomal dominant are forms ARVD1-9, Naxos disease (with subtype Carvajal syndrome) is recessive. In ARVC/D forms associated with desmosomal disorders are ventricular arrhythmias caused by the presence of myocardial damage and in forms associated with ryanodine receptor mutation is electrical instability and subsequent myocardial damage caused by calcium cell overload. Main clinical signs are ventricular arrhythmias originated from areas with slow conduction. Progression of ARVC/D is manifested by RV dilatation and LV echocardiographic abnormalities both considered as main risk factors of fatal ventricular arrhythmias and sudden cardiac death. Therapeutic possibilities include antiarrhythmic drugs, catheter ablation and implantation of cardioverter-defibrillator, in severe right or both ventricle involvement even heart failure treatment (Tab. 1, Ref. 56).