Treatment Options for ARVD
The cornerstone of ARVD treatment is ICD implantation for patients meeting Task Force criteria, particularly those with sustained VT, cardiac arrest, or unexplained syncope, combined with lifestyle modification (complete cessation of competitive athletics) and beta-blocker therapy to prevent sudden cardiac death and slow disease progression. 1
Primary Prevention of Sudden Cardiac Death
ICD Implantation - High-Risk Features
ICD placement is reasonable for primary prevention in ARVD patients with the following high-risk features: 1
- Prior cardiac arrest or resuscitated sudden cardiac death - strongest indication 1
- Unexplained syncope (presumed arrhythmic) 1
- Sustained ventricular tachycardia causing hemodynamic compromise 1
- Extensive right ventricular disease with severe RV dilation 1
- Left ventricular involvement (occurs in >50% of cases) 1, 2
- Inducible VT on electrophysiological testing 1
- Nonsustained VT on ambulatory monitoring 1
- Male gender (independent risk factor) 1
- Young age at presentation (<35 years) 1
- Family history of sudden cardiac death in affected relatives 1, 2
- High-risk genotypes (particularly chromosome 1q42-43 locus) 1
Important Caveats About ICD Therapy
The decision for primary prevention ICD remains challenging because predictive markers of sudden cardiac death have not been validated in large prospective studies. 1 The appropriate ICD intervention rate is 9.5% per year, but inappropriate shocks occur at 3.7% per year with device-related complications in 20% of patients. 3 Despite lack of randomized trial data, observational studies show sudden death is rare after ICD implantation while appropriate shocks are common, supporting aggressive ICD use in high-risk patients. 1
Lifestyle Modifications
Complete avoidance of competitive sports and intensive exercise is mandatory for all patients with clinically diagnosed ARVD, regardless of symptoms. 2, 4 This is a Class I recommendation despite being based solely on observational data, as exercise accelerates disease progression and triggers arrhythmias. 2
Pharmacological Therapy
Beta-Blockers (First-Line)
Beta-blockers are recommended as first-line antiarrhythmic therapy for all ARVD patients with ventricular arrhythmias. 2, 4 They should be initiated even in asymptomatic patients with clinical evidence of ARVD to prevent arrhythmias and potentially slow structural progression. 2, 4
Amiodarone (Second-Line)
Amiodarone is superior to beta-blockers and sotalol for preventing ventricular arrhythmias in ARVD based on observational data. 2 It should be considered for patients with persistent symptomatic arrhythmias despite beta-blocker therapy or for reducing ICD shock frequency. 4, 5
ACE Inhibitors/ARBs
ACE inhibitors should be initiated to limit structural disease progression and prevent ventricular dysfunction. 4 This is particularly important given that biventricular failure requiring transplantation can develop. 2
Critical Limitation
The impact of medical therapy on mortality has not been established in ARVD. 1 Antiarrhythmic drugs reduce arrhythmia burden and symptoms but do not prevent sudden cardiac death. 6
Catheter Ablation
Catheter ablation is recommended for patients with recurrent symptomatic sustained VT despite optimal medical therapy, primarily to reduce arrhythmia frequency and prevent ICD shocks. 2
Technical Approach
- Combined endocardial/epicardial approach is preferred when beta-blockers fail or are not tolerated 2
- Epicardial ablation increases success rates compared to endocardial-only approach 2
- EP testing is used to reproduce clinical VT and guide ablation 1
Important Limitation
Elimination of clinical tachycardias by ablation improves symptoms but may not be sufficient to prevent sudden cardiac death. 1 Therefore, ablation is adjunctive therapy, not a substitute for ICD in high-risk patients. 1
Advanced Surgical Options
For Medically Refractory Patients
Total electrical RV disconnection has proved successful in medically refractory patients with normal LV function, but carries risk of postoperative right heart failure. 1
End-Stage Disease
Heart transplantation and ventricular assist devices are options for patients with biventricular failure. 1 Annual heart transplant rate is 0.9% in ARVD patients with ICDs. 3
Family Screening and Genetic Testing
All first-degree relatives should undergo clinical screening including 12-lead ECG, echocardiography, and genetic testing if the proband has an identified mutation. 2 Genetic counseling is recommended for diagnosis and gene-specific targeted family screening. 2 Cardiac MRI is the most useful imaging modality for establishing diagnosis and risk stratification in suspected cases. 2
Management of Asymptomatic Gene Carriers
ICD is NOT routinely recommended for asymptomatic genetic carriers without clinical manifestations. 2 However, these individuals should avoid competitive athletics and undergo regular surveillance from adolescence. 2, 4
Risk Stratification Tools
Signal-averaged ECG and cardiac MRI can be useful for diagnosis and risk stratification. 2 Electrophysiological study may be considered for risk stratification in asymptomatic patients with clinical evidence of ARVD, though its role remains poorly defined. 2 EP testing has limited positive predictive value (55%) for recurrent VT in patients presenting with nonsustained VT. 1
Common Pitfalls to Avoid
- Do not withhold ICD in probands meeting Task Force criteria - sudden death is rare with ICD while appropriate shocks are common 1
- Do not allow patients to continue competitive athletics even if asymptomatic - this accelerates disease progression 2
- Do not rely solely on ablation for sudden death prevention - it reduces symptoms but not mortality 1
- Do not assume isolated RV disease - left ventricular involvement occurs in >50% of cases and increases risk 2
- Do not use EP testing to exclude high-risk patients from ICD - negative EP study does not reliably predict low risk 1