What is the treatment for Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)?

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Last updated: May 23, 2025View editorial policy

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From the Guidelines

Treatment for Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) should prioritize preventing life-threatening arrhythmias and managing heart failure symptoms, with a focus on individualized care based on symptom severity, arrhythmia risk, and extent of cardiac involvement, as recommended by the 2017 AHA/ACC/HRS guideline 1.

Key Recommendations

  • An implantable cardioverter-defibrillator (ICD) is recommended for high-risk patients with ARVC who have experienced cardiac arrest, sustained ventricular tachycardia, or have severe right ventricular dysfunction, as stated in the 2017 AHA/ACC/HRS guideline 1.
  • Beta-blockers, such as metoprolol or bisoprolol, are recommended for patients with ARVC to reduce arrhythmias, as suggested by the 2017 AHA/ACC/HRS guideline 1.
  • Antiarrhythmic medications like amiodarone or sotalol may be prescribed for patients with recurrent ventricular tachycardia, as mentioned in the acc/aha/esc 2006 guidelines 1.
  • Lifestyle modifications, including avoiding competitive sports and strenuous exercise, are crucial to prevent arrhythmias, as recommended by the 2017 AHA/ACC/HRS guideline 1.
  • Family screening is important since ARVC is often genetic, with first-degree relatives requiring cardiac evaluation, as stated in the 2017 AHA/ACC/HRS guideline 1.

Additional Considerations

  • Cardiac MRI is useful for establishing a diagnosis and for risk stratification in patients with suspected ARVC, as recommended by the 2017 AHA/ACC/HRS guideline 1.
  • Catheter ablation with availability of a combined endocardial/epicardial approach can be beneficial for patients with recurrent symptomatic sustained VT, as suggested by the 2017 AHA/ACC/HRS guideline 1.
  • Heart transplantation may be considered for advanced cases with severe heart failure, as mentioned in the acc/aha/esc 2006 guidelines 1.

From the Research

Treatment Options for ARVC

  • The primary goal of treatment for Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is to prevent sudden cardiac death (SCD) and reduce the burden of ventricular tachyarrhythmias 2, 3.
  • Treatment strategies for ARVC include:
    • Antiarrhythmic drug therapy: Antiarrhythmic drugs such as sotalol, amiodarone, and flecainide may be used to suppress ventricular tachycardia (VT) recurrences in patients with ARVC 2, 4, 5.
    • Catheter ablation: Catheter ablation using conventional or electroanatomic mapping techniques may be used to eliminate the targeted arrhythmia substrate, although recurrence rates can be high 2, 6, 5.
    • Implantable cardioverter-defibrillator (ICD) therapy: ICD therapy is the only proven "lifesaving" therapy for preventing SCD in patients with ARVC, although it is associated with significant morbidity due to device-related complications and inappropriate ICD interventions 2, 3, 5.

Efficacy of Treatment Options

  • A study found that flecainide associated with beta-blockers is safe and effective in preventing VA in ARVC patients, with a significant decrease in premature ventricular contraction burden and a low event rate of sustained VA on treatment 4.
  • A systematic review and meta-analysis found that amiodarone significantly reduced VT recurrence and ICD shocks in patients with an ICD, while catheter ablation reduced ICD shocks 5.
  • Sotalol was found to increase VT recurrence and ICD shocks compared to amiodarone, highlighting the importance of careful selection of antiarrhythmic drugs for patients with ARVC 5.

Considerations for Treatment

  • The selection of patients for ICD implantation is a challenging issue in the clinical management of ARVC, and requires careful consideration of individual patient characteristics and risk factors 3.
  • The use of antiarrhythmic drugs and catheter ablation should be tailored to the individual patient, taking into account the presence of underlying heart disease, the severity of symptoms, and the risk of SCD 2, 6, 5.

References

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Research

Safety and efficacy of flecainide associated with beta-blockers in arrhythmogenic right ventricular cardiomyopathy.

Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology, 2022

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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