[Arrhythmogenic right ventricular cardiomyopathy. Etiology, diagnosis and therapy].

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by regional atrophy of right ventricular myocardium and subsequent replacement by fatty and fibrous tissue. The disease manifests in young adulthood with a predominance of males. Hallmarks of ARVC are ventricular tachyarrhythmias of left bundle branch block pattern which frequently occur during exercise. However, sudden death may also be the first manifestation of the disease. Characteristic findings are repolarization abnormalities and QRS prolongation in the right precordial leads of the surface ECG and regional abnormalities of right ventricular structure and wall motion. Left ventricular involvement may occur in later stages of the disease but rarely leads to progressive biventricular heart failure. Therapeutic efforts are mainly directed to the treatment of ventricular tachyarrhythmias and the prevention of sudden death. A tailored treatment strategy including antiarrhythmic drug therapy, catheter ablation and implantation of cardioverter-defibrillators may be used to improve the long-term prognosis of patients with ARVC.