arrhythmogenic right ventricular dysplasia (ARVD)

Last edited 08/2022 and last reviewed 08/2022

Arrhythmogenic right ventricular dysplasia is a rare cause of dilated cardiomyopathy in which there is gradual replacement of the myocardium of the right ventricle by fibroadipose tissue. The left ventricle is normal or nearly normal.

It is most common in young men and may be familial

Summary features (1,2):

• prevalence - ranges from 1:1000 to 1:5000 individuals, with male predominance among probands (2)
• predominant inheritance - AD with variable penetrance and variable clinical expression
• diagnostic features - ECG abnormalities in leads V1-3. Fibrofatty replacement of RV myocardium sometimes identifiable on magnetic resonance imaging. 30% have LV involvement (1)

Click here for an example ECG and further information

Presentation may be with ventricular tachycardia of right ventricular origin, or in some cases it may be asymptomatic and the first presentation is with sudden death

• syncopes occur in about 10-20% and represent a sign of poor prognosis (3). In most cases the reason for syncopes are recurrent sustained or non-sustained ventricular arrhythmias (3)
• patients with SCD (sudden cardiac death)/VF (ventricular fibrillation) at first presentation are typically younger (median age 23 years [range 13-57]) (2)

The condition is progressive and results in:

• eventual right ventricular dilatation
• right ventricular failure
• may be also left ventricular failure

Management:

• the use of an implantable cardioverter defibrillator should be considered in patients with this condition (4)
• symptomatic arrhythmia sometimes requires treatment with beta blockers or anti-arrhythmic agents such as sotalol or amiodarone. As the disease progresses some patients may require management of cardiac failure (5)

Notes:

• there seems to be an overlap between arrhythmogenic right ventricular dysplasia and Brugada syndrome (3) - study data revealed a number of patients wih arrhythmmogenic right ventricular dysplasia and pharmacologically provocable Brugada-type I coved ST-segment elevation and right bundle branch block in right precordial leads (3)

Reference:

1. British Heart Foundation (2009).Factfile 5/09, Inherited cardiovascular disease.
2. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Developed by the task force for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death of the European Society of Cardiology (ESC) Endorsed by the Association for European Paediatric and Congenital Cardiology (AEPC), European Heart Journal, 2022;, ehac262, https://doi.org/10.1093/eurheartj/ehac262
3. Peters S et al. Mechanisms of syncopes in arrhythmogenic right ventricular dysplasia - cardiomyopathy beyond monomorphic ventricular tachycardia. Int Journ of Cardiol 2006; 106 (1):52-54
4. NICE (September 2000). Implantable cardioverter defibrillators for arrhythmias.
5. Pulse (May 28th 2005): 54-61.