Chapter 21 - Arrhythmogenic Right Ventricular Cardiomyopathy

Abstract

Arrhythmogenic right ventricular cardiomyopathy is a disorder of myocyte cell-to-cell adhesion related to desmosomal proteins, with the most common mutation being in plakophilin. Ventricular arrhythmias commonly precede structural abnormalities, which predominantly affect the subtricuspid and basal anterior wall of the right ventricle. Clinical disease in the child generally begins after age 10, and a strategy of evaluation and management for the child is needed. The goals of management for the child are to prevent or slow progression of clinical disease. Beta blockade may be of benefit. An implantable cardioverter defibrillator may be considered based on an individualized assessment of risk of sudden cardiac death, but data for primary prevention in a pediatric population are lacking. Of utmost importance is to restrict exercise, particularly for those who already manifest abnormalities.