Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)

Background

• Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited cardiomyopathy characterized by abnormalities in ventricular structure and function predominantly affecting the right ventricle and associated with risk for ventricular arrhythmias and sudden death.
• The most common causes (reported in > 50%) are autosomal dominant inherited mutations in genes encoding desmosomal proteins.
• Estimated prevalence ranges from 1 per 2,000 persons to 1 per 5,000 persons.

Evaluation

• There is no single gold standard diagnostic test for arrhythmogenic right ventricular dysplasia (ARVD).
• Initial evaluation should include history, physical exam, and noninvasive testing with:
  • electrocardiography (ECG)
  • comprehensive imaging of both ventricles (echocardiography and/or magnetic resonance imaging)
• If initial noninvasive testing is suggestive but not diagnostic, consider invasive testing, such as:
  • electrophysiologic study (EPS) (with or without electroanatomic mapping)
  • endomyocardial biopsy
• Diagnosis is made using International Task Force (ITF) criteria. The diagnosis is:
  • definite if there are 2 major criteria, 1 major and 2 minor criteria, or 4 minor criteria in different categories
  • borderline if there is 1 major criterion and 1 minor criterion, or there are 3 minor criteria from different categories
  • possible if there is 1 major criterion, or there are 2 minor criteria from different categories

Management

• Treatment is directed at preventing sudden cardiac death and reducing symptom burden of ventricular arrhythmias and heart failure.

Activity restrictions

• Patients with definite Arrhythmogenic right ventricular dysplasia (ARVD) should:
  • not participate in competitive and/or endurance sports (Strong recommendation)
  • consider restricting participation in athletic activities other than low-intensity sports (Weak recommendation)

Surgery and procedures

• Offer implantable cardioverter defibrillator (ICD) therapy to high-risk patients (Strong recommendation).
• Consider ICD implantation for patients with any of the following (Weak recommendation):
  • intermediate risk
  • ≥ 1 risk factor for sudden cardiac death
  • ≥ 1 episode of hemodynamically stable sustained ventricular tachycardia
• Do not offer an ICD to low-risk patients (Strong recommendation).
• Catheter ablation may interrupt ventricular tachycardia (VT) but does not prevent sudden cardiac death.
• Offer catheter ablation to patients with incessant VT or frequent appropriate ICD interventions on VT despite maximal medication therapy, including amiodarone (Strong recommendation).
• Consider cardiac transplantation as a final treatment option for patients with untreatable heart failure or uncontrollable VT.

Medications

• Administer antiarrhythmic medications to ARVD patients with (Strong recommendation):
  • ICDs and frequent appropriate device discharges
  • symptomatic ventricular arrhythmias
• Consider administration of antiarrhythmic medications to patients with ARVD as adjunct therapy to catheter ablation without back-up ICD in select patients with recurrent, hemodynamically stable ventricular tachycardia (Weak recommendation).
• Offer beta blockers to patients with ARVD and recurrent VT, appropriate ICD shocks, or inappropriate ICD shock due to sinus tachycardia, supraventricular tachycardia, atrial flutter, or atrial fibrillation with high ventricular rate (Strong recommendation).