Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: An Updated Review of Diagnosis and Management.

ABSTRACT

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a condition caused by the replacement of the normal right ventricular myocardium with fibrofatty tissue. ARVC/D can present with a variety of clinical conditions including right ventricular dysfunction, ventricular tachyarrhythmias, sudden cardiac arrest, and sudden cardiac death (SCD). Since the first report of ARVC/D in 1982, many advances have been made in the diagnosis, genetic findings for pathology, and treatment. The 2010 International Task Force diagnostic criteria distinguish between major and minor criteria and focus on gross structural changes, microscopic changes, repolarization defects, conduction defects, arrhythmias, and family history. Implantable cardiac defibrillators and catheter ablation of the endocardium and epicardium with electromagnetic mapping have emerged as successful tools in the treatment and prevention of ventricular tachyarrhythmias and SCD. This review discusses the pathophysiology, genetics, diagnosis, and treatment advances in ARVC/D.