Management of Arrhythmogenic Right Ventricular Dysplasia (ARVD)
Beta-blockers and implantable cardioverter-defibrillators (ICDs) are the cornerstones of ARVD management, with strict exercise restriction being essential for all patients to prevent disease progression and sudden cardiac death. 1
Diagnosis and Risk Stratification
Before initiating treatment, proper diagnosis and risk stratification are crucial:
- Cardiac MRI: Essential for patients with suspected ARVD who have ventricular arrhythmias or ECG abnormalities (Class I recommendation) 1
- Signal-averaged ECG: Useful for diagnosis and risk stratification (Class IIa recommendation) 1
- Genetic testing: Recommended for first-degree relatives of patients with ARVD if the proband has a disease-causing mutation (Class I recommendation) 1
- Family screening: Clinical screening of first-degree relatives is recommended (Class I recommendation) 1
Treatment Algorithm
1. Exercise Restriction
- Avoid competitive sports and intensive exercise in all patients with ARVD (Class I recommendation) 1
- Exercise restriction is critical as endurance training exacerbates the ARVD phenotype and increases risk of arrhythmias 1, 2
2. Pharmacological Management
- Beta-blockers: First-line therapy for all patients with ARVD, even those without ventricular arrhythmias (Class I recommendation for those with VA, Class IIa for those without) 1
- Amiodarone: Consider for patients with frequent ventricular arrhythmias not controlled by beta-blockers 1
- Other medications: ACE inhibitors and spironolactone may be considered to limit structural progression 3, 4
3. ICD Implantation
ICD placement is recommended in the following scenarios:
High-risk patients (Class I recommendation):
- Resuscitated sudden cardiac arrest
- Sustained ventricular tachycardia
- Significant ventricular dysfunction (RVEF or LVEF ≤35%) 1
Intermediate-risk patients (Class IIa recommendation):
Primary prevention: Consider in all patients meeting Task Force criteria for definite ARVD, as 39% of primary prevention patients receive appropriate ICD therapy 5
4. Catheter Ablation
- Consider for patients with recurrent symptomatic VT despite beta-blocker therapy (Class IIa recommendation) 1
- Combined endocardial/epicardial approach is beneficial in patients with recurrent VT unresponsive to medical therapy 1
- Ablation should be performed in experienced centers 1
- Note: Ablation reduces arrhythmia frequency but does not eliminate the risk of SCD 1
5. Electrophysiological Study
- May be considered for risk stratification in asymptomatic patients with clinical evidence of ARVD (Class IIb recommendation) 1
- However, electrophysiological testing has not been shown to reliably predict appropriate ICD interventions in primary prevention patients 5
Special Considerations
Disease Progression Monitoring
- Regular follow-up with cardiac imaging to assess ventricular function
- Holter monitoring to evaluate arrhythmia burden
- Reassessment of risk factors periodically
Family Management
- Genetic counseling for all patients and family members 1
- Regular screening of unaffected family members from adolescence 4
Common Pitfalls to Avoid
- Underestimating risk in asymptomatic patients: Even those without symptoms can experience sudden cardiac death as their first manifestation 6
- Relying solely on antiarrhythmic drugs: Neither medications nor catheter ablation provide sufficient protection against SCD 1
- Inadequate exercise restriction: Failure to emphasize exercise restriction can lead to disease progression and increased arrhythmic events 2
- Delayed ICD implantation: Waiting for symptoms may be too late, as 21% of patients receive ICD therapy for life-threatening arrhythmias 5
By following this comprehensive management approach, the risk of sudden cardiac death can be significantly reduced while maintaining quality of life for patients with ARVD.