From the Guidelines
The initial treatment approach for arrhythmogenic right ventricular dysplasia (ARVD) should focus on preventing life-threatening arrhythmias and managing symptoms, with beta-blockers such as metoprolol or nadolol being typically first-line medications to reduce ventricular arrhythmias, as recommended by the 2017 AHA/ACC/HRS guideline 1. The treatment approach for ARVD involves a comprehensive strategy that includes:
- Medications: Beta-blockers are the first-line treatment to reduce ventricular arrhythmias, with a recommended dose of metoprolol (25-100 mg twice daily) or nadolol (40-160 mg daily) 1.
- Implantable cardioverter-defibrillator (ICD) placement: Recommended for patients with sustained ventricular tachycardia, ventricular fibrillation, or those at high risk for sudden cardiac death, as stated in the 2017 AHA/ACC/HRS guideline 1.
- Lifestyle modifications: Restriction from competitive and endurance sports is crucial, as physical exertion can trigger arrhythmias, as recommended by the 2017 AHA/ACC/HRS guideline 1.
- Regular cardiac monitoring: Essential for disease progression assessment, including ECGs, Holter monitors, and imaging, as stated in the 2017 AHA/ACC/HRS guideline 1. This approach targets the underlying pathophysiology of ARVD, which involves progressive fibrofatty replacement of right ventricular myocardium leading to electrical instability and mechanical dysfunction. Key considerations in the treatment of ARVD include:
- Risk stratification: Identifying patients at high risk for sudden cardiac death, such as those with a history of ventricular tachycardia or ventricular fibrillation, as stated in the 2012 ACCF/AHA/HRS focused update 1.
- Genetic counseling: Important for patients with a family history of ARVD, as recommended by the 2017 AHA/ACC/HRS guideline 1.
- Multidisciplinary care: Collaboration between cardiologists, electrophysiologists, and other healthcare professionals is essential for optimal management of ARVD, as stated in the 2017 AHA/ACC/HRS guideline 1.
From the Research
Initial Treatment Approach for Arrhythmogenic Right Ventricular (RV) Dysplasia
The initial treatment approach for arrhythmogenic right ventricular (RV) dysplasia involves a combination of medical therapy, catheter ablation, and implantable cardioverter-defibrillator (ICD) therapy.
- Medical therapy: Antiarrhythmic drugs such as sotalol or amiodarone in combination with beta-blockers may be used to suppress ventricular tachycardia (VT) recurrences in patients with low risk of sudden death 2.
- Catheter ablation: This procedure can be used to eliminate the targeted arrhythmia substrate, but its curative value is limited due to the progressive nature of the disease 2.
- ICD therapy: ICDs are recommended for patients who have experienced syncope, sudden death, or a sustained ventricular arrhythmia, and also for patients with overt evidence of ARVD, particularly if the electrophysiology study is abnormal or there is a family history of sudden death 3.
Treatment Goals
The main goal of treatment is to prevent life-threatening cardiac arrhythmias and sudden death.
- Treatment strategies aim at the suppression or effective termination of recurrent ventricular tachyarrhythmias and prevention of sudden death 2.
- Beta-blockers and angiotensin-converting enzyme inhibitors may be used to treat patients with ARVD/C, and all patients with ARVD/C should be screened for a mutation in the gene for plakophilin-2 3.
Additional Treatment Options
Other treatment options include: