What are the treatment options for Arrhythmogenic Right Ventricular Dysplasia (ARVD)?

From the Guidelines

Treatment for Arrhythmogenic Right Ventricular Dysplasia (ARVD) should prioritize implantable cardioverter-defibrillators (ICDs) for patients at high risk of sudden cardiac death, as recommended by the 2015 ESC guidelines 1. The management of ARVD involves a comprehensive approach to prevent life-threatening arrhythmias and manage heart failure symptoms.

• The cornerstone of ARVD management includes ICDs for patients at high risk of sudden cardiac death, particularly those with extensive disease, left ventricular involvement, or a family history of sudden cardiac death 1.
• Beta-blockers, such as metoprolol or sotalol, are typically prescribed to reduce arrhythmias, with amiodarone or sotalol considered for treatment of sustained ventricular tachycardia (VT) or ventricular fibrillation (VF) when ICD implantation is not feasible 1.
• Ablation can be useful as adjunctive therapy in managing patients with ARVD and recurrent VT, despite optimal anti-arrhythmic drug therapy 1.
• Physical activity restrictions, including avoidance of competitive sports, are crucial to prevent disease progression and arrhythmia triggering, as recommended by the 2015 ESC guidelines 1.
• Family screening is recommended, as ARVD is often genetic, and heart transplantation may be considered in advanced cases with severe heart failure. Key considerations in ARVD management include:
• Prevention of sudden cardiac death through ICD implantation and medical therapy
• Management of heart failure symptoms using ACE inhibitors or angiotensin receptor blockers
• Restriction of physical activity to prevent disease progression and arrhythmia triggering
• Family screening and genetic counseling due to the genetic nature of ARVD.

From the Research

ARVD Treatment Overview

• ARVD treatment is directed toward prevention of sudden cardiac death, prevention or delay of disease progression, and adjustment to life with a lifelong chronic genetic disease 2.
• The first step for treatment is securing an accurate diagnosis through careful application of the diagnostic Task Force criteria 2.

Treatment Options

• Implantable cardioverter defibrillator (ICD) placement is generally recommended in all probands who meet Task Force criteria, especially if they have a history of sustained ventricular tachycardia (VT), arrhythmogenic syncope, or frequent ventricular ectopy and/or nonsustained VT 2, 3.
• Anti-arrhythmic medications, such as beta-blockers and antiarrhythmic drugs, may be used to decrease the frequency of ventricular tachycardia and associated implantable cardioverter defibrillator therapies 2, 4.
• Catheter ablation, including endocardial and epicardial approaches, may be used to eliminate frequent VT episodes and ICD shocks 4, 5.
• Radiofrequency ablation has been shown to be feasible, safe, and effective in treating ARVC-related VT, particularly when combined with scar dechanneling or homogenization of the scar 5.

Lifestyle Modifications

• Patients with ARVD are advised to dramatically reduce exercise and discontinue competitive athletics 2, 3.
• Affected individuals are recommended to limit their activity to activities such as walking and golf 3.

Medical Therapy

• Beta-blockers and angiotensin-converting enzyme inhibitors may be used to limit structural progression and prevent ventricular arrhythmias 2, 3.
• Medical therapy for ARVC-related VT is suboptimal, and catheter ablation may be considered a potentially effective strategy for eliminating frequent VT episodes and ICD shocks 5.