Arrhythmogenic Right Ventricular Cardiomyopathy: Management and Outcomes

Posted on Jan 26, 2025 in Agriculture

Arrhythmogenic Right Ventricular Cardiomyopathy: Treatment and Prognosis

Author:
William J McKenna, MD
Section Editor:
Hugh Calkins, MD
Deputy Editor:
Brian C Downey, MD, FACC

Contributor Disclosures

All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Oct 2016. | This topic last updated: Nov 17, 2016.

INTRODUCTION — Arrhythmogenic right ventricular cardiomyopathy (ARVC), also called arrhythmogenic right ventricular dysplasia (ARVD), is an underrecognized clinical entity manifested by ventricular arrhythmias and a specific ventricular pathology [1-3]. It is characterized macroscopically by a fatty appearance of the right ventricular (RV) free wall. The fibrofatty replacement of the RV myocardium initially produces typical regional wall motion abnormalities that later become global, producing RV dilation. The tissue replacement can also involve areas of the left ventricle (LV) with relative sparing of the septum [4].

The prevalence in the general population is estimated to be approximately 1:1000 [1]. ARVC appears to be relatively common in young adults in northern Italy [2,5], accounting for approximately 11 percent of the cases of sudden cardiac death (SCD) overall and 22 percent in athletes [5].

In contrast, ARVC has been rarely diagnosed in the United States, but it appears to be identified with increasing frequency. This may reflect a difference in genetic prevalence, but is more likely due to under recognition of disease. In a series of 100 ARVC patients from a single referral center in the United States, clinical profiles and event rates were similar to those reported in Europe [6].

The treatment and prognosis of ARVC will be reviewed here. The genetics, pathogenesis, clinical manifestations, diagnostic criteria, and evaluation are discussed separately. (See “Arrhythmogenic right ventricular cardiomyopathy: Pathogenesis and genetics” and “Clinical manifestations and diagnosis of arrhythmogenic right ventricular cardiomyopathy”.)

Treatment

General Therapeutic Approach

The optimal strategies for preventing sudden cardiac death (SCD) and the indications for implantable cardioverter-defibrillator (ICD) therapy in patients with ARVC are the subject of international consensus documents [7-9]. Although some studies suggest that patients with hemodynamically tolerated arrhythmias do well when treated with antiarrhythmic drugs, guidelines recommend ICD implantation for secondary prevention of SCD in patients with sustained ventricular tachycardia (VT) or ventricular fibrillation (VF) and for primary prevention in selected high-risk patients [8,9]. Antiarrhythmic drugs may be required in patients with asymptomatic or symptomatic arrhythmia or as an adjunct to an ICD. (See ‘Treatment of arrhythmia’ below and ‘Antiarrhythmic drugs’ below.)

Although the prevention of SCD is the major goal of therapy in patients with ARVC, there are additional issues that should be considered [1]:

The 2006 American College of Cardiology/American Heart Association/European Society of Cardiology (ACC/AHA/ESC) guidelines for the management of ventricular arrhythmias and the prevention of SCD included recommendations for the management of patients with ARVC, which have been supplemented by an international task force statement on treatment of ARVC [8,9]. (See ‘Prevention of arrhythmia and disease progression’ below and ‘Treatment of arrhythmia’below.)

Prevention of Arrhythmia and Disease Progression

Activity Restriction

Because of the risk of disease progression and the association between exercise and the induction of ventricular tachyarrhythmias, which has been documented in both animal and human studies, patients with ARVC should not participate in competitive sports [10-15]. Any activity, competitive or not, that causes symptoms of palpitation, presyncope, or syncope should be avoided. Specific activity recommendations are detailed separately. (See “Risk of sudden cardiac death in athletes”, section on ‘Arrhythmogenic (right) ventricular cardiomyopathy’.)

Role of Beta-Blocking Agents

Accumulating evidence in both humans and animal models indicates that exercise is associated with disease development and arrhythmic risk in ARVC [10,12,14,15]. Although no robust clinical trial evidence exists that attenuating sympathetic stimulation will be beneficial in preventing disease progression and arrhythmia, there is compelling logic and some clinical experience for such a benefit [16]. Given the favorable risk-benefit ratio of beta blockers, the 2015 Task Force Consensus Statement recommended that therapy should be considered in all ARVC patients irrespective of arrhythmia (class IIa). The prophylactic use of beta-blockers in healthy gene carriers, however, is not recommended (class III) until there is proven efficacy in either disease or arrhythmia progression.

Treatment of Arrhythmia

ICD Indications

Among patients with ARVC, ICD implantation is appropriate for the secondary prevention of SCD in those who have experienced a sustained ventricular arrhythmia. ICD implantation is also considered appropriate for the primary prevention of SCD in selected patients considered to be at high risk. Due to the relatively low prevalence of ARVC and the lack of randomized controlled trials, precise indications for ICD implantation for primary prevention of SCD have not been developed, and the guideline-based recommendations of professional societies are primarily based on expert consensus [9]. (See ‘High-risk groups’ below.)

In selected circumstances, genotyping may help to select those patients and families at highest risk of SCD [7,17]. Patients with multiple mutations and patients with Naxos disease (characterized by palmoplantar keratosis and woolly hair), other recessive forms of ARVC, and patients from Newfoundland with the TMEM43 mutation are at an increased risk of SCD and may be candidates for implantation of an ICD for primary prevention [17,18]. In general, however, neither family history nor other specific desmosomal variants help inform decisions regarding ICD implantation. (See “Arrhythmogenic right ventricular cardiomyopathy: Pathogenesis and genetics”, section on ‘Genetics’.)

The 2015 international task force consensus statement on treatment of ARVC made the following recommendations (that supersede earlier guidelines) with regard to the use of an ICD in patients with ARVC [8,9,19]

  • ICD therapy is recommended in ARVC patients who have experienced ≥1 episodes of hemodynamically unstable, sustained VT or VF (class I).
  • ICD therapy is recommended in ARVC patients with severe systolic dysfunction of the RV, LV, or both, irrespective of arrhythmias (class I).
  • ICD therapy should be considered in ARVC patients who have experienced ≥1 episodes of hemodynamically stable, sustained VT (class IIa).
  • ICD therapy should be considered in patients who have “major” risk factors such as unexplained syncope, moderate ventricular dysfunction, or NSVT (class IIa).
  • ICD therapy may be considered in patients with “minor” risk factors after a careful discussion of the long-term risks and benefits of ICD implantation (class IIb).
  • ICD therapy is not recommended in asymptomatic ARVC patients with no risk factors or healthy gene carriers (class III).

ICD Efficacy

Randomized trials have proven the efficacy of ICD implantation in preventing SCD in other clinical settings. The impact of ICD implantation in ARVC has been evaluated in several observational series and a case-control study [6,17,20-23]. In a 2013 meta-analysis of 610 patients from 18 cohorts with ICDs for either primary or secondary prevention (mean age 40 years, 42 percent women, mean follow-up 3.8 years), the annualized rate of appropriate ICD therapies was 9.5 percent [24].

Some examples of larger studies examining the outcomes of ICD use for primary or secondary prevention of SCD include:

  • A multicenter series evaluated outcomes in 132 patients who received an ICD for indications including history of cardiac arrest (10 percent), sustained VT (62 percent), syncope (16 percent), and other risk factors (12 percent) [21]. At a mean follow-up of 39 months, appropriate device interventions occurred in 48 percent and inappropriate interventions occurred in 16 percent. The survival rate was 96 percent and the survival rate free from VF or ventricular flutter was 72 percent.
  • A single-center series from Germany followed 60 patients with ARVC who received an ICD for a mean of 80 months [22]. Indications for an ICD included a history of resuscitated cardiac arrest (33 percent), documented sustained VT (57 percent), syncope (3 percent), and a family history of cardiac arrest and inducible VT on electrophysiologic study (7 percent). Survival at one, five and 10 years was 100, 94, and 76 percent, respectively. Death was due to SCD (two), intractable HF (two), and noncardiac causes (four). Appropriate device therapy (cardioversion or antitachycardia pacing) at one and five years occurred in 51 and 74 percent, respectively.

Other examples of studies examining the outcomes of ICD use only for primary prevention of SCD are illustrated below:

  • A multicenter series included 105 patients with ARVC who received an ICD for primary prevention based on risk factors such as syncope, nonsustained VT, familial sudden death, and inducibility at programmed ventricular stimulation [20]. During 58 months follow-up, 24 percent had an appropriate ICD intervention and 16 percent had shocks for VF or ventricular flutter. History of syncope was a significant predictor of appropriate ICD interventions (HR 2.9, 95% CI, 1.8 to 4.7) and shocks for VF/ventricular flutter (HR 3.2, 95% CI, 1.4 to 5.6). None of 27 asymptomatic patients who received an ICD solely because of family history of sudden death had appropriate ICD therapy. Programmed ventricular stimulation had low-predictive accuracy for ICD therapy as discussed below. (See ‘Electrophysiologic testing’ below.)
  • Finally, in a retrospective case-control study of 367 subjects from 11 families with ARVC5 (ie, the Newfoundland TMEM43 mutation), 197 patients were identified as at risk based upon clinical history, DNA haplotyping, and/or pedigree position [17]. Among these at risk individuals, 48 had an ICD implanted (13 for secondary prevention). These patients were compared to an age-matched control group of 58 other high-risk patients. The five-year mortality for males in the control group was 28 percent, compared to 0 percent in the patients treated with an ICD.

Antitachycardia pacing (ATP) appears highly efficacious in terminating ventricular tachyarrhythmias in the ARVC population. In a prospective cohort study of 108 patients with ARVC and an ICD followed for an average of 3.3 years, 450 episodes of sustained monomorphic VT were treated with ATP, with successful termination of the VT in 412 episodes (92 percent) [9,23].

In summary, the above data and additional smaller reports demonstrate that malignant arrhythmias are common in patients with ARVC, and that ICD therapy (both ATP and shocks) is effective for both primary and secondary prevention of SCD [6,21,22,25,26].

ICD Complications

ICD-related complications including pocket hematoma, lead-related problems, pericardial effusion and infection are discussed separately. The survival benefit is obtained at the expense of significant complications during what is often long-term follow-up with lead/device-related complications of 3.7 percent per year, and inappropriate ICD therapies of 4.4 percent per year [9].(See “Implantable cardioverter-defibrillators: Complications”.)

There are two concerns related to ICD use that are unique to patients with ARVC and may account for the higher rate of complications [1]:

  • Thin areas of the RV myocardium can be perforated during placement of the RV leads.
  • The fibrofatty changes in the RV may interfere with adequate lead positioning and sensing of arrhythmias.

Many ARVC patients in whom ICDs are contemplated are young, and therefore will probably require multiple ICD and possibly lead replacements. Thus, their lifetime risks of procedural and device related complications are greater than many patients treated with ICDs for other indications. A single-chamber system is therefore recommended to minimize long-term lead related complications [9]. In the multicenter series of ICD placement in 132 patients cited above, five required placement of an additional septal lead due to loss of ventricular sensing and/or pacing, and four had an increase in defibrillation threshold requiring additional patch electrodes, high-energy devices, and, in one case, cardiac transplantation [21].

In a 2013 meta-analysis of 610 patients from 18 cohorts with ICDs for either primary or secondary prevention (mean age 40 years, 42 percent women, mean follow-up 3.8 years), the annualized rate of inappropriate ICD therapies was 3.7 percent [24].

Subcutaneous ICD systems, which do not require transvenous insertion of a lead into the right ventricle, are an emerging option for some patients, and intuitively seem very promising for patients with ARVC. (See “Subcutaneous implantable cardioverter defibrillators”.)

Electrical Storm

As with patients who have an ICD implanted for other indications, recurrent ventricular arrhythmias and repeated device therapy, while life-saving, can be debilitating and significantly reduce the patient’s quality of life. In one series of 42 patients with ARVC who received an ICD, five developed repetitive episodes of VT with recurrent ICD discharges (“electrical storm”) [27]. (See “Electrical storm and incessant ventricular tachycardia”.)

For patients who develop electrical storm, pharmacologic therapy can be given to reduce the frequency of ICD discharges. Sotalol may be the most effective agent for reducing VT recurrence in such patients. Of the five patients with electrical storm in the study described above, all were controlled with medication and none required radiofrequency ablation or cardiac transplantation to prevent arrhythmia recurrence [27]. (See ‘Antiarrhythmic drugs’ below and “Pharmacologic therapy in survivors of sudden cardiac arrest”, section on ‘Treatment of breakthrough arrhythmias’.)

Antiarrhythmic Drugs

Antiarrhythmic drugs can reduce the frequency and suppress inducibility of sustained and nonsustained ventricular arrhythmias in patients with ARVC [16]. However, antiarrhythmic drugs have not been shown to reduce the risk of SCD in this disease.

Based upon the established superiority of the ICD to antiarrhythmic drugs in other forms of heart disease that are associated with life-threatening ventricular arrhythmias, antiarrhythmic drugs are not considered an equivalent alternative to an ICD in ARVC patients considered to be at high risk of SCD. (See “Secondary prevention of sudden cardiac death in heart failure and cardiomyopathy” and “Primary prevention of sudden cardiac death in heart failure and cardiomyopathy”.)

A study of 81 ARVC patients compared the efficacy of several antiarrhythmic drugs for the purpose of suppressing inducibility of ventricular arrhythmias at electrophysiology study or reducing the incidence of ventricular arrhythmias on ambulatory monitoring [16]. Sotalol was more effective than class I drugs, beta blockers, calcium channel blockers and amiodarone (table 1).

The recommendations for use of antiarrhythmic drugs for treatment of arrhythmias are [9]:

  • Antiarrhythmic drugs are recommended as adjunct therapy in ARVC patients with frequent appropriate ICD discharges (class I).
  • Antiarrhythmic drugs should be considered to improve symptoms in patients with frequent premature ventricular beats and/or nonsustained VT (class IIb).
  • Antiarrhythmic drugs are not recommended in asymptomatic ARVC patients without documented ventricular arrhythmia or in healthy gene carriers (class III).

The available evidence suggests that amiodarone (loading dose 400 to 600 mg daily for three weeks, maintenance 200 to 400 mg daily) alone or in combination with a beta-blocker are most effective for the prevention of symptomatic ventricular arrhythmia. (See “Secondary prevention of sudden cardiac death in heart failure and cardiomyopathy”, section on ‘Antiarrhythmic drugs’.)

Radiofrequency Ablation

Radiofrequency ablation (RFA) is NOT adequate as a primary or sole therapy for the treatment of ventricular arrhythmias in patients with ARVC. RFA can successfully treat some of the arrhythmogenic foci in ARVC, but due to the patchy and progressive nature of this disease, RFA is not a definitive therapy [28-31].

Several electrophysiologic mapping techniques can be used to ablate ventricular arrhythmias, including entrainment mapping, voltage mapping, substrate mapping and electroanatomic mapping. These methods are discussed in detail separately [28-33]. (See “Invasive cardiac electrophysiology studies”.)

Overall, one-year non-recurrence rates are approximately 75 percent and appear even higher when combined endo- and epicardial mapping techniques are applied

[33]. Longer-term success rates, however, are significantly lower, and this is reflected by current guidelines, which limit the recommendation for catheter ablation of VT to those with incessant VT or frequent ICD interventions despite maximal pharmacological therapy, or those unable or unwilling to take pharmacological therapy. Importantly, catheter ablation is not recommended as an alternative to ICD for sudden death prevention [9].

Surgery for ventricular arrhythmias — Several innovative surgical therapies have been attempted, including RV disarticulation, RV cardiomyoplasty, beating heart cryoablation, and left cardiac sympathetic denervation [34-37]. The availability and success of the ICD, however, has largely eliminated surgical therapy in patients who have VT refractory to antiarrhythmic drugs. (See “Sustained monomorphic ventricular tachycardia in patients with a prior myocardial infarction: Treatment and prognosis”, section on ‘Surgical therapy’.)

Cardiac transplantation — On rare occasions, patients with ARVC who develop heart failure symptoms or ventricular arrhythmias that are progressive and debilitating despite the optimal use of medications, ICDs, and other adjunctive therapies may be candidates for cardiac transplantation. Eighteen patients with ARVC followed at a single center from 1995 to 2009 underwent successful cardiac transplantation (11 males, average age 40 years at time of transplant). At initial presentation, they were younger, and LV dysfunction was common. Their clinical course was prolonged (average duration of ARVC symptoms 17.6 years prior to transplant) [38]. Thirteen of the 18 underwent transplantation because of predominantly heart failure symptoms, while five had a transplant primarily for VT. Both short-term and long-term survival following transplantation were excellent (94 and 88 percent survival at one and six years, respectively). Cardiac transplantation rates are much higher in those referral centers in which ablation and ICD use are most prevalent. The potential negative long-term effects of these therapies, however, have not been systematically evaluated. (See “Indications and contraindications for cardiac transplantation in adults”.)

PROGNOSIS — There are two prognostic issues in patients with ARVC:

●What is the natural history of asymptomatic patients who are detected because of familial disease?

●What is the course of patients with VT?

While patients with ARVC do have a risk of developing heart failure and ventricular arrhythmias, the majority of patients appear to do relatively well. In a 2013 meta-analysis of 610 patients from 18 cohorts with ICDs for either primary or secondary prevention (mean age 40 years, 42 percent women, mean follow-up 3.8 years), the annualized rates of cardiac death, noncardiac death, and heart transplantation were 0.9 percent, 0.8 percent, and 0.9 percent, respectively [24].

Course in asymptomatic patients — Studies of familial disease have revealed that some subjects from an affected family who are initially free of disease develop ARVC later in life. In a review of 37 families, 9.6 percent of initially unaffected subjects developed structural signs of disease on echocardiography during a mean follow-up of 8.5 years; almost 50 percent had symptomatic ventricular arrhythmias [11]. Progression from mild to moderate disease occurred in 5 percent of patients, while progression from moderate to severe disease occurred in 8 percent; only two patients developed LV clinically-evident involvement. ARVC exhibits age-related penetrance from the second to the seventh decades [39].

Course in patients with VT — The prognosis of ARVC patients who experience VT is uncertain. Patients with mild disease and nonsustained VT appear to have a relatively low risk of arrhythmic death [11,40,41]. In the above report of 37 families from Italy, only 1 of 49 patients with ventricular arrhythmia who were treated with antiarrhythmic drugs died during a mean follow-up of 8.5 years [11]. The majority of these patients were treated for nonsustained arrhythmias. The patient who died had discontinued amiodarone 20 days before his death. No other patient died, and the overall mortality rate was 0.08 percent per year.

The prognosis of ARVC suggested by these reports is considerably better than the outcome with sustained VT of LV origin seen in patients with structural heart disease. The lower mortality in ARVC probably reflects better hemodynamic tolerance of VT because of maintained LV function, and a lesser likelihood of degeneration to ventricular fibrillation.

In contrast, in another series of 102 patients with ARVC and VT, 21 died of cardiovascular causes during a mean follow-up of eight years [42]. The reasons for the worse prognosis in this analysis are not clear, although it was carried out at a tertiary referral center and may have been biased toward the inclusion of higher-risk patients. The use of antiarrhythmic drugs was not reported.

Electrophysiologic testing — Electrophysiological studies (EPS) are of diagnostic value in differentiating VT in ARVC from idiopathic RVOT tachycardia and may provide useful information for optimization of device therapy in high-risk patients who require an ICD. There are conflicting data, however, from referral centers regarding the utility of EP testing for prognostic evaluation to determine which patients with ARVC are at high risk and require an ICD. The guidelines for EPS in relation to prognosis are summarized as follows [9]:

●Programmed ventricular stimulation may be considered for arrhythmic risk stratification in asymptomatic ARVC patients (class IIb).

●Endocardial voltage mapping may be considered in the prognostic evaluation of ARVC patients (class IIb). (See ‘Course in patients with VT’ above.)

The importance of inducible VT during invasive electrophysiologic (EP) testing as a predictor of future VT or ICD therapy is controversial because results differ from referral centers:

●In the multicenter cohort of 132 patients with ARVC who received an ICD, in whom preimplantation EP testing was performed in 111, 98 had an inducible sustained ventricular tachyarrhythmia [21]. The positive and negative predictive values of EP testing for subsequent appropriate device therapy were only 49 and 54 percent, respectively. Similarly, in a separate cohort of 67 patients with ARVC who underwent EP testing with programmed ventricular stimulation, the positive and negative predictive values for subsequent appropriate device therapy were 35 and 70 percent, respectively [20].

●In a single-center cohort of 84 patients with ARVC who underwent ICD implantation for primary prevention and were followed for an average of nearly five years, patients who received an appropriate ICD therapy (n = 40) were significantly more likely to have had inducible VT during EP testing (76 versus 37 percent among non-inducible patients) [43]. Inducibility during EP testing was a significant predictor of future ICD therapy on multivariable analysis (hazard ratio 4.5; 95% CI 1.4-15).

Inducible VT, while not necessarily predictive of future VT events, may be important in identifying patients at higher risk of overall cardiac morbidity and mortality. Among a cohort of 62 patients who fulfilled the 2010 criteria for ARVC and underwent invasive EP testing, sustained monomorphic VT was inducible in 34 patients (55 percent) [44]. Over 10 years of follow-up, patients with ARVC and inducible sustained monomorphic VT in this cohort were significantly more likely to experience one or more adverse outcomes (cardiac death, heart transplantation, sudden cardiac death, ventricular fibrillation, or VT with associated hemodynamic compromise/syncope) compared to those with ARVC and no inducible VT (65 versus 29 percent, adjusted hazard ratio 2.3, 95% CI 1.1-5.0). Prior to considering invasive EP testing in all patients with ARVC, we would like to see these data replicated in other populations.

High-risk groups — The following are groups of patients with ARVC at increased risk for SCD or, in those with an ICD, appropriate device intervention:

●Younger patients (decreased risk with increasing age; in one study, OR 0.77 for each additional five years of age) [21].

●Patients with syncope [20].

●Patients with a previous history of cardiac arrest or of VT with hemodynamic compromise [21].

●Patients with two or more disease causing mutations [39].

●Patients with LV involvement [21,45]. The long-term outcome is worse in patients with LV involvement, being associated with a higher incidence of arrhythmia and clinical heart failure (HF) [46]. Such patients with advanced disease may be difficult to distinguish from those with dilated cardiomyopathy.

●Patients with ARVC5, a fully penetrant sex-influenced disorder caused by a missense mutation in the TMEM43 gene [17,47].

●Patients with Naxos disease. In one series of 26 patients followed for 10 years, 62 percent had structural progression of right ventricular abnormalities, and 27 percent developed HF due to LV involvement [48]. Twelve patients (46 percent) developed symptomatic arrhythmias and the annual cardiac and sudden death mortality were 3 and 2.3 percent, respectively. (See “Arrhythmogenic right ventricular cardiomyopathy: Pathogenesis and genetics”, section on ‘Autosomal recessive disease and Naxos disease’.)

Patients with an increase in QRS dispersion (maximum measured QRS duration minus minimum measured QRS duration ≥40 msec) and patients with T wave inversion or significant depolarization changes in three or more ECG leads are at increased arrhythmic risk, but these electrocardiographic features are unlikely to be independent markers of sudden death [49,50]. In contrast, clinical signs of RV failure and left ventricular dysfunction are independent predictors of cardiovascular death (death due to heart failure and sudden death) [42].

SUMMARY AND RECOMMENDATIONS — The following recommendations apply to the management of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). They are in general agreement with those of the 2006 ACC/AHA/ESC guidelines for the management of ventricular arrhythmias and sudden cardiac death, the 2008 American College of Cardiology/American Heart Association/Heart Rhythm Society (ACC/AHA/HRS) guidelines for device-based therapy of cardiac rhythm abnormalities, the 2012 ACCF/AHA/HRS focused update of the 2008 guidelines for ICD therapy, and the 2015 International Task Force consensus statement on treatment of ARVC/D [8,9,19,51].

●Patients with ARVC should be restricted from competitive athletics and high-intensity recreational activities (figure 1) (Grade 1B). Moderate-intensity recreational activities should be approached with caution. (See ‘Activity restriction’ above.)

●Given the compelling logic that attenuation of sympathetic stimulation will reduce arrhythmic risk, and given the favorable risk-benefit ratio, beta-blockers are recommended in all patients with ARVC. (See ‘Role of beta-blocking agents’ above.)

●We recommend ICD implantation for the secondary prevention of SCD in patients with ARVC who experience sustained VT or VF. (Grade 1B) (See ‘Treatment of arrhythmia’ above.)

●We suggest ICD implantation for the primary prevention of SCD in patients with ARVC and any of the following high-risk features: extensive RV disease; LV involvement; or unexplained syncope consistent with a tachyarrhythmia (Grade 2C). (See ‘Treatment of arrhythmia’ above.)

●For patients with ARVC and sustained VT or VF who are not candidates for or do not want an ICD, we suggest antiarrhythmic therapy for the purpose of secondary prevention of SC (Grade 2C). (See ‘Antiarrhythmic drugs’ above.)

●For patients with ARVC and an ICD who experience frequent ventricular arrhythmias and ICD shocks, we recommend adjunctive antiarrhythmic therapy for the purpose of reducing risk and relieving the discomfort and anxiety associated with frequent ICD therapies (Grade 1B). (See ‘Antiarrhythmic drugs’above.)

●For patients with ARVC and an ICD who experience frequent ventricular arrhythmias and ICD shocks despite a trial of antiarrhythmic therapy, we suggest RFA (Grade 2C). (See ‘Radiofrequency ablation’ above.)