The Cardiac Society of Australia and New Zealand Position Statement on the Diagnosis and Management of Arrhythmogenic Right Ventricular Cardiomyopathy (2019 Update)

Abstract

Key Points: 1. Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disease affecting the right, and/or the left ventricle, and commonly presents as an electrical disorder with ventricular arrhythmias and increased risk of sudden cardiac death.

2. ARVC cannot be diagnosed by a single test, and is a clinical diagnosis supported by electrophysiologic, morpho-functional, genetic, and histological data based on the 2010 Task Force 2 criteria.

3. Familial involvement occurs in more than 50%, and referral to a specialist centre with access to genotyping is appropriate in selected cases.

4. Implantable cardioverter-defibrillator (ICD) therapy carries risk, and appropriate thresholds should apply before offering this, particularly in younger individuals.