What is the definitive management for patients with Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)?

Definitive Management of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)

Beta-blockers are the first-line therapy for all patients with ARVC, even those without ventricular arrhythmias, followed by ICD implantation for high-risk patients and consideration of catheter ablation for recurrent ventricular tachycardia. 1

Risk Stratification and Initial Management

Risk Assessment

• Evaluate for high-risk features:
  • History of sustained ventricular tachycardia (VT) or ventricular fibrillation (VF)
  • Resuscitated cardiac arrest
  • Unexplained syncope
  • Significant ventricular dysfunction (RVEF or LVEF ≤35%)
  • Family history of sudden cardiac death
  • Extensive disease with LV involvement

First-Line Therapy

• Beta-blockers should be initiated in all patients with ARVC, regardless of arrhythmia presence (Class I recommendation) 1
• Strict exercise restriction is essential as exercise can accelerate disease progression and trigger arrhythmias (Class I recommendation) 1
• Regular cardiac screening with ECG, Holter monitoring, and imaging to assess disease progression

Definitive Management Algorithm

For High-Risk Patients (Class I recommendation):

1. ICD implantation for:
   • Documented sustained VT or VF
   • Resuscitated sudden cardiac arrest
   • Significant ventricular dysfunction (RVEF or LVEF ≤35%)
   • Reasonable life expectancy >1 year with good functional status 2

For Intermediate-Risk Patients (Class IIa recommendation):

1. Consider ICD implantation for:
   • Extensive disease including LV involvement
   • Family history of sudden cardiac death
   • Unexplained syncope when VT/VF has not been excluded 2
   • Multiple risk factors 1

For Patients with Recurrent VT Despite Optimal Medical Therapy:

1. Catheter ablation as adjunctive therapy (Class IIa recommendation)
   • Combined endocardial/epicardial approach is beneficial 1
   • Note: Ablation alone does not provide sufficient protection against SCD 1

When ICD Implantation is Not Feasible:

1. Antiarrhythmic medications:
   • Amiodarone or sotalol for treatment of sustained VT or VF (Class IIa recommendation) 2
   • Amiodarone may be considered for patients with frequent ventricular arrhythmias not controlled by beta-blockers 1

ECG Findings and Diagnostic Considerations

• Characteristic ECG findings include:

  • Precordial T-wave inversion (usually V1-V3)
  • QRS duration >110 ms
  • Epsilon waves (low voltage potentials following QRS)
  • Late potentials on signal-averaged ECG in >50% of patients 2
  • Ventricular arrhythmias with LBBB morphology 1

• Diagnostic workup should include:

  • 12-lead ECG and 48-hour ambulatory ECG monitoring
  • Cardiac MRI (particularly valuable for ARVC diagnosis)
  • Genetic testing if a disease-causing mutation is identified in the family 1

Long-Term Management

• Regular follow-up with cardiac imaging and Holter monitoring
• Reassessment of risk factors periodically
• Genetic counseling for all patients and family members 1
• Clinical screening for all first-degree relatives of ARVC patients 1
• Heart transplantation consideration for patients with refractory heart failure 3

Important Caveats

• Relying solely on antiarrhythmic drugs is insufficient to protect against sudden cardiac death 1
• Most rapid VT/VF events occur within the first 2 years after diagnosis (83.3%), with late occurrence being rare 4
• Early recognition and appropriate therapy are crucial, as delays in management can be life-threatening 5
• While ARVC traditionally affects the right ventricle, biventricular involvement is increasingly recognized 6

The management of ARVC requires a systematic approach focusing on prevention of sudden cardiac death, control of arrhythmias, and monitoring of disease progression. Beta-blockers form the cornerstone of therapy, with ICD implantation being the most effective safeguard against arrhythmic sudden death in high-risk patients.