Ten key facts about ARVC

Arrhythmogenic right ventricular cardiomyopathy (ARVC, also known as arrhythmogenic right ventricular dysplasia or ARVD) is one of the genetic cardiomyopathies. Here are ten key facts about ARVC that you should be aware of in your cardiology clinic.

Cardiac MRI showing ARVC (4-chamber view)

1. ARVC predominantly affects the right ventricle, causing the replacement of myocardium by fibrofatty tissue. However it can also involve the left ventricle as the disease progresses
2. The population prevalence of ARVC is thought to be somewhere between 1 in 2,500 and 1 in 5,000
3. ARVC has an autosomal dominant pattern of inheritance (with incomplete penetrance and variable expression), and affects males more often than females (ratio 2.7:1)
4. The genetic mutations causing ARVC affect the desmosomal proteins, which connect cells and play a role in signal transduction
5. Common presentations of ARVC include syncope and sudden cardiac death
6. The diagnosis of ARVC depends upon the presence of major and/or minor criteria (see Task Force reference below)
7. ECG criteria include the presence of T wave inversion in leads V1-V3 and epsilon waves
8. Cardiac imaging (e.g. echo, CMR, contrast ventriculography) plays an important role in the diagnosis of ARVC but the diagnosis must not be based solely upon imaging findings, but instead must be based upon Task Force criteria
9. Cardiac biopsy is of limited value in the diagnosis of ARVC due to the segmental nature of the disease
10. Treatment options in ARVC include antiarrhythmic drugs, catheter ablation therapy and/or ICD implantation. Treatment with heart failure drugs may also be indicated

You can read more about ARVC in these key references:

Corrado D, Basso C, Thiene G. Arrhythmogenic right ventricular cardiomyopathy: an update. Heart 2009; 95: 766-773.

Marcus FI, et al. Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: Proposed Modification of the Task Force Criteria. Circulation 2010; 121: 1533-1541.