Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/ARVC) Treatment & Management

Updated: Dec 29, 2020
  • Author: Gyanendra K Sharma, MD, FACC, FASE; Chief Editor: Jose M Dizon, MD  more...
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Treatment

Approach Considerations

The goal of clinical management of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/ARVC) is to reduce mortality, prevent disease progression, improve symptoms and quality of life, limit heart failure symptoms, and improve functional capacity. Options include lifestyle changes, pharmacologic therapy, catheter-based ablation, placement of an implantable cardioverter-defibrillator (ICD), and heart transplantation. [63]

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Prevention of Sudden Cardiac Death

Prevention of sudden cardiac death is a major goal of treatment in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/ARVC).

An implantable cardioverter-defibrillator (ICD) is recommended for secondary prevention of sudden cardiac death in patients with sustained ventricular tachycardia or ventricular fibrillation and for primary prevention in high-risk patients. [64, 65] There is a lack of data from prospective trials to define markers to predict sudden cardiac death. However, some of the clinically relevant high-risk factors include the following:

  • Induction of ventricular tachycardia during electrophysiology testing

  • Nonsustained ventricular tachycardia during noninvasive evaluation/monitoring

  • Male sex

  • Early disease onset (< 5 years)

  • Severe RV dilatation

  • Extensive RV involvement

  • LV involvement

  • Prior cardiac arrest

  • Unexplained syncope

Patients with autosomal-dominant genotypes of ARVD/ARVC associated with a high risk for sudden cardiac death should be considered for ICD therapy. [66]

Most available data from the registry or cohort of patients and families favors ICD for primary prevention, as it was associated with appropriate ICD shocks. In one study, syncope was an important predictor of appropriate ICD shock. However, asymptomatic patients based on family history of sudden death alone may not derive benefit from a prophylactic ICD. [67]

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Suppression of Symptomatic Cardiac Arrhythmias

The goal of therapy is to reduce the frequency and severity of arrhythmias for symptomatic relief. [63]

Antiarrhythmic therapy is recommended as an adjunct therapy to implantable cardioverter-defibrillator (ICD) in patients with frequent appropriate device discharges. Antiarrhythmic therapy may also be considered in addition to catheter ablation without backup ICD in selected patients with recurrent, hemodynamically stable ventricular tachycardia.

Beta-blockers are generally considered the first line of drug therapy. Beta-blocker therapy is recommended in patients with recurrent ventricular tachycardia, appropriate ICD therapy, or inappropriate ICD therapy due to sinus tachycardia, supraventricular tacycardia (SVT), or atrial flutter/fibrillation with high ventricular rate. Beta-blocker therapy should be considered in all patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/ARVC) irrespective of arrhythmias. However, it is not recommended for prophylactic use in healthy gene carriers.

Antiarrhythmic agents include flecainide, propafenone, sotalol, and amiodarone, alone or in combination. Sotalol is the most effective drug for inducible or noninducible ventricular tachycardia. Amiodarone may not be useful because of side effects associated with long-term use in young patients. [68]  

Amiodarone alone or in combination with a beta-blocker is the most effective drug therapy to prevent symptomatic ventricular tachycardia, but it may not be useful to prevent sudden cardiac death. [69]

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Management of Heart Failure

The usual heart failure therapy (angiotensin-converting enzyme inhibitors [ACEIs], angiotensin II receptor blockers [ARBs], beta blockers, and diuretics) should be considered, especially in patients with features of biventricular failure. In patients with asymptomatic right ventricular and or left ventricular dysfunction, heart failure therapy may be considered.

Long-term anticoagulation therapy is indicated in patients with documented intracavitary thrombosis and or venous/systemic thromboembolism. Anticoagulation therapy is not recommended for primary prevention. 

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Radiofrequency Ablation

Radiofrequency ablation has limited utility in treating arrhythmogenic foci in arrhythmogenic right ventricular cardiomyopathy (ARVC) owing to the patchy and progressive nature of the disease. [70] However, it has been used with some success in limited cases as an alternative or adjunct to an implantable cardioverter-defibrillator (ICD) to reduce the burden of ventricular tachycardia. [71, 72]

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Lifestyle Modification

In general, vigorous exercise should be avoided in persons with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/ARVC), as it can trigger arrhythmias. Patients should be advised against participation in competitive athletic activities and/or endurance sports.

Participation in low intensity recreational sports appears acceptable.

Restriction from competitive sports may be considered in ARVD/ARVC family members with a negative phenotype, either healthy gene carriers or with unknown genotype. [63]

It is also best to avoid cardiac stimulants such as caffeine/pseudoephedrine.

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