Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic disorder of the myocardium that can lead to ventricular arrhythmia and sudden cardiac death. The condition has been identified as a significant cause of arrhythmic death among young people and athletes, therefore, early recognition of the disease by emergency clinicians is critical to prevent subsequent death. The diagnosis of ARVC can be very challenging and requires a systematic approach.

This publication reviews the pathophysiology, classification, clinical presentations, and appropriate approach to diagnosis and management of ARVC.