Arrhythmogenic Right Ventricular Cardiomyopathy: A Review of Living and Deceased Probands

Abstract

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a potentially life-threatening genetic cardiomyopathy with a spectrum of clinical presentations including sudden cardiac death (SCD). Methods: Clinical and genetic data of 44 probands referred to a cardiac genetics clinic (2007–2017) who met 2010 Task Force Criteria (TFC) for ARVC diagnosis were included. Results: Thirty-three (33)(75%) male, 20 (45%) were referred by the Victorian Institute of Forensic Medicine. Presentation that lead to diagnosis included ARVC-related SCD (n = 19), SCD due to alternate cause of death (n = 1), aborted cardiac arrest (n = 6), stable symptomatic ventricular tachycardia (n = 14), palpitation..