Guidelines

Nature and Nurture in Arrhythmogenic Right Ventricular Cardiomyopathy – A Clinical Perspective

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a rare heritable cardiomyopathy characterised by fibro-fatty replacement of the myocardium, which predisposes patients to frequent lifethreatening ventricular arrhythmias and slowly progressive ventricular dysfunction.1,2 Structural involvement of the right ventricle (RV) generally predominates,3,4 although left dominant forms of ARVD/C are increasingly well-recognised.5 Patients typically present in their second to fifth decade with symptoms associated with ventricular a

Arrhythmogenic Right Ventricular Cardiomyopathy – Antiarrhythmic Therapy

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterised by progressive replacement of the ventricular myocardium by fibrofatty tissue.1 Patients with the disease are predisposed to ventricular arrhythmias, heart failure and sudden cardiac death.

Pathophysiology