Arrhythmogenic Right Ventricular Dysplasia (ARVD / ARVC)
Overview
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/ARVC) is an inherited myocardial disease characterised by progressive fibro‑fatty replacement of the right ventricular (RV) free wall.
This creates areas of slow, fractionated conduction that predispose to ventricular ectopy, sustained ventricular tachycardia (VT), sudden cardiac death (SCD) and, with disease progression, right‑sided or biventricular heart failure.
Emergency clinicians must recognise acute presentations, treat life‑threatening arrhythmias according to advanced life support (ALS) guidance, and arrange urgent specialist escalation and appropriate documentation (including driving advice and family‑screening pathways).
Epidemiology and genetics
- Prevalence approximately 1:5,000.
- Usually autosomal dominant with variable penetrance; family history of SCD is common.
- Second leading cause of SCD in young people after hypertrophic cardiomyopathy; implicated in up to ~10% of SCD in adults under 65 years.
Pathophysiology
Mutations, commonly in desmosomal proteins, lead to myocyte loss and fibrofatty replacement of RV myocardium. This produces an arrhythmogenic substrate with delayed, fractionated conduction and re‑entry circuits. VT typically shows a left bundle branch block (LBBB) morphology, reflecting RV origin (often RV outflow tract).