Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/ARVC) 

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

Overview

Background

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/ARVC) is an inherited cardiomyopathy characterized by structural and functional abnormalities in the right ventricle (RV) resulting in ventricular arrhythmias.[1] It is an important cause of sudden cardiac death (SCD) in young adults, accounting for 11% of all cases and 22% of cases among athletes.[2, 3]

ARVD was first described in 1977 and was included in the World Health Organization (WHO) classification of cardiomyopathies in 1996.[4] Since then, there have been significant advances in the understanding of its etiopathogenesis, diagnosis, and management.[5]

Pathophysiology

The structural abnormalities in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/ARVC) result from the fatty infiltration and fibrosis of the RV myocardium. This leads to progressive RV dilatation and dysfunction. The left ventricle (LV) is less commonly involved, and the septum is relatively spared.[6] However, in a cohort of 200 probands, Sen-Chowdhry et al found that LV involvement may even precede the onset of significant RV dysfunction.[7] The prognosis is worse in patients with LV involvement.[8]

The mechanisms for myocardial loss include the following:

  • Apoptosis (programmed cell death)

  • Inflammation, enhanced fibrosis, and loss of function

  • Fatty replacement of myocardium

Etiology

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/ARVC) is an inherited disorder, as it is already present in the fetus. Familial cases account for 30%-90% of cases.[9] In other cases, it may result from an acquired etiology such as viral infection (myocarditis) or unidentified inheritance. It is also likely that patients with a genetic predisposition are more likely to develop myocarditis.

The disease manifests more frequently in active individuals, when mechanical sheer stress can cause cell membrane damage, inflammation, and fibrosis in genetically predisposed RV.

Genetics

ARVD is considered a genetic disorder, as most cases are familial, and there is geographical clustering. The most common pattern of inheritance is autosomal dominance, with a variable penetrance ranging from 20%-35% of family members.[10, 11] People living in the Veneto region of Italy have a higher penetrance. The autosomal-recessive (Naxos disease) pattern of inheritance is localized to the Greek island of Naxos and is associated with palmoplantar keratosis and wooly hair. The genetic mutation occurs on chromosome 17q21, and penetrance is almost 100%.[12, 13]

Genetic abnormalities in ARVD are located on chromosomes 1, 2, 3, 6, 7, 10, 12, and 14. There is no single unique genetic abnormality, posing a challenge in evaluation of patients and families with suspected ARVD. The responsible genes include plakoglobin (JUP), desmoplakin (DSP), plakophilin-2 (PKP2), desmoglein-2 (DSG2), desmocollin-2 (DSC2), and others.[14, 15, 16]  In some cases, mutation in the SCN5A gene may cause dysfunction in the cardiac voltage-gated sodium channel (Nav1.5), resulting in cardiomyopathy.[17]

The Heart Rhythm Society and the European Heart Rhythm Association published a consensus statement on genetic testing for cardiomyopathies.[18]

Epidemiology

Because of the diagnostic challenge, the exact incidence and prevalence of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/ARVC) remains unknown, as clinically silent cases may go unrecognized.[19] It is estimated that it affects 1 in 1000-5000 population and is more common in individuals of Greek and Italian origin.[20]

In a cohort of 100 patients from the United States, the median age at presentation was 26 years, and 51% were males. The median time to diagnosis was one year from the initial presentation, and median survival in the entire cohort was 60 years.[21]

Prognosis

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/ARVC) is an important cause of sudden cardiac death in young adults, accounting for 11% of all cases and 22% of cases among athletes.

The prognosis is worse in patients with left ventricular (LV) involvement.

Pregnancy is mostly well tolerated, but Hodes et al found 13% of pregnancies in women with ARVD/ARVC were complicated by ventricular arrhythmias and 5% by heart failure.[22]

Sustained ventricular arrhythmias are more common in men, and they have significantly abnormal electrocardiograms.[23]

Cardiac magnetic resonance imaging (CMRI) to assess tissue characteristics and a 5-year risk score model has been proposed.[24]  In a study of 140 patients with ARVC, CMRI was normal in 14 patients who did not have any major events. LV-dominant abnormalities were found in 16 patients (12%).[25] Isolated RV involvement was seen in 41% patients and biventricular involvement was seen in 37% patients. LV involvement had a worse prognosis than RV involvement alone. However, the risk score model underestimated the risk in those who had LV involvement.[25]

 

Presentation

History

Patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/ARVC) have a wide range of presentations, ranging from being asymptomatic to biventricular failure and/or sudden cardiac death. In one series from the United States, median age at presentation was 26 years, and median time from symptom onset to diagnosis was one year.

Common symptoms reported in different series[21, 26] include the following:

  • Palpitation (27%-67%)

  • Syncope (26%-32%)

  • Sudden cardiac death (10%-26%)

  • Atypical chest pain (27%)

  • Dyspnea (11%)

Palpitation is the most frequent symptom and is caused by ventricular arrhythmias. Depending on the disease severity, ventricular ectopics may be isolated or may result in nonsustained/sustained ventricular tachycardia, ventricular fibrillation, and sudden cardiac death.

Progressive RV dysfunction results in dyspnea and leg swelling. In more severe cases with left ventricular involvement, patients may present with biventricular congestive heart failure that may mimic dilated cardiomyopathy. Supraventricular arrhythmias, including atrial flutter and fibrillation, may be seen in about 25% of cases.[27]

Exercise can induce ventricular arrhythmias and sudden cardiac death. ARVD accounts for 22% of sudden cardiac death cases among young athletes in northern Italy.[2, 28] In the United States, in a series of 286 cases of sudden cardiac death in athletes, hypertrophic cardiomyopathy was the most common cause, and ARVD was reported in only 4% cases.

Extracardiac manifestations include palmoplantar keratosis and curly hair seen in individuals with autosomal-recessive inheritance.

 

DDx

Diagnostic Considerations

Idiopathic right ventricular tachycardia

Idiopathic right ventricular tachycardia is a benign disorder that may mimic arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/ARVC) owing to the occurrence of exercise-induced left bundle branch block (LBBB) morphology ventricular tachycardia with inferior axis. The heart is structurally normal, and there is no history of ARVD/ARVC or sudden cardiac death in the family.

Electrocardiography (ECG) in RV tachycardia shows upright T waves in V2 -V5, and epsilon waves are absent.[29, 30]

Uhl anomaly

This is a rare disorder characterized by total lack of RV myocardium and results in a very thin-walled RV (parchment RV).[31] In ARVD, the myocardium is not completely absent and is replaced by a variable degree of fibrosis.

Dilated cardiomyopathy

Biventricular dilatation and congestive heart failure may mimic advanced ARVD with LV involvement. Characteristic ECG and cardiac MRI (CMRI) abnormalities in ARVD help to distinguish the two entities.

Sarcoidosis

Patients with pulmonary sarcoidosis may have RV dilatation. Sarcoidosis is more common in women and Black persons. ECG may show a heart block. Chest x-rays show hilar lymphadenopathy. The LV is more often involved, resulting in LV systolic dysfunction. Late gadolinium enhancement may be seen in the interventricular septum in a nonischemic pattern.

Myocarditis

Typically, myocarditis has a more acute presentation, preceded by a prodrome such as fever, chills, myalgia, chest pain. Troponin may be elevated. CMRI reveals edema on T2-weighted images, and late gadolinium enhancement is seen in the subepicardium in a nonischemic distribution.

Brugada syndrome

The ECG in Brugada syndrome reveals right BBB and persistent ST segment elevation in the right precordial leads. However, no abnormalities are found on imaging studies, including echocardiography and late gadolinium enhancement MRI.

Differential Diagnoses

 

Workup

Laboratory Studies

The diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/ARVC) poses a great challenge because of its variable presentation and requires greater attention in at-risk populations. In the past, the diagnosis was made based on postmortem findings or histological confirmation on endomyocardial biopsy. However, endomyocardial biopsy has limited sensitivity owing to patchy involvement of the RV.[32]

In 1994, an International task force first proposed the major and minor diagnostic criteria of ARVD based on family history, arrhythmias, ECG abnormalities, tissue characterization, and structural and functional RV abnormalities.[1] These criteria were highly specific but lacked sensitivity to detect early and familial disease.

In 2010, the task force criteria were revised to include quantitative criteria and abnormalities defined based on normal subject data.[33] The proposed terminology for the diagnosis of ARVD include major or minor criteria from 6 different categories.

The different categories include the following:

  • Global or regional dysfunction and structural alterations on echocardiography, CMRI, and/or RV angiography

  • Tissue characterization of wall as shown on endomyocardial biopsy

  • Repolarization abnormalities on ECG

  • Depolarization/conduction abnormalities on ECG and/or signal-averaged ECG (SAECG)

  • Arrhythmias on Holter monitoring

  • Family history (see below)

A definite diagnosis is defined as the presence of 2 major criteria, 1 major and 2 minor criteria, or 4 minor criteria.

A borderline diagnosis is defined as the presence of 1 major and 1 minor criteria or 3 minor criteria.

A possible diagnosis is defined as the presence of 1 major criterion or 2 minor criteria.

Major criteria include associated with family history include (1) confirmed diagnosis of ARVD based on task force criteria/autopsy or surgery in a first-degree relative (2) or identification of a pathogenic mutation categorized as associated or probably associated with ARVD/C. Minor criteria include (1) history of ARVD (not confirmed based on task force criteria) or premature sudden death (< 35 years) from suspected ARVD in a first-degree relative or (2) confirmed diagnosis of ARVD in a second-degree relative.

All other categories require diagnostic workup, as discussed below.

Electrocardiography

Electrocardiography

Electrocardiographic (ECG) findings may be normal in the latent phase of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/ARVC), but abnormalities (depolarization/repolarization and/or conduction) are seen in most patients upon progression of the disease.[21, 34]  The images below represent ECG abnormalities in ARVD.

Arrhythmogenic Right Ventricular Dysplasia/Cardiom Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/ARVC). Intraventricular conduction abnormality
Arrhythmogenic Right Ventricular Dysplasia/Cardiom Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/ARVC). T-wave abnormalities in the presence of right bundle branch block.
Arrhythmogenic Right Ventricular Dysplasia/Cardiom Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/ARVC). Anteroseptal T-wave inversion.

According to the revised task force criteria, major ECG criteria include the following:

  • Inverted T waves in right precordial leads (V1 -V3) or beyond in individuals older than 14 years in the absence of complete right bundle branch block (RBBB); this feature is seen in 87% of patients with ARVD[35]

  • Epsilon waves (low-amplitude signals between end of QRS complex to the onset of T wave) in leads V1 -V3; these are seen in 33% of patients with ARVD

    Arrhythmogenic Right Ventricular Dysplasia/Cardiom Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/ARVC). Arrhythmogenic right ventricular dysplasia epsilon wave.

Minor ECG criteria include the following:

  • Inverted T waves in leads V1 and V2 in individuals older than 14 years in the absence of complete RBBB or in V4, V5, or V6

  • Terminal activation of QRS =55 ms measured from the nadir of the S wave to the end of the QRS, including R', in V1, V2, or V3, in the absence of complete RBBB

Ventricular ectopics, when present, have a LBBB configuration.

Signal-averaged electrocardiography

Abnormalities on signal-averaged electrocardiogram (SAECG) are common and have excellent sensitivity and specificity but do not reliably predict spontaneous or inducible ventricular tachycardia.[36, 37, 38]  SAECG abnormalities are included as minor criteria and should have at least 1 of 3 parameters in the absence of a QRS duration of 110 ms or more on standard ECG. These parameters include the following:

  • Filtered QRS duration =114 ms

  • Duration of terminal QRS < 40 µV (low-amplitude signal duration) =38 ms

  • Root-mean-square voltage of terminal 40 ms =20 µV

Twenty-Four–Hour Holter Monitoring

Twenty-four-hour Holter monitoring is helpful in detecting arrhythmias (eg, ventricular premature contractions, nonsustained or sustained ventricular tachycardia of left bundle branch block [LBBB] morphology).

Ventricular tachycardia of LBBB morphology with superior axis is a major criterion, while ventricular tachycardia with inferior or indeterminate axis and/or more than 500 ventricular extra systoles per 24 hours are considered minor criteria per the revised task force.

Echocardiography

Echocardiography serves as a screening tool to evaluate patients with suspected arrhythmogenic right ventricular cardiomyopathy (ARVC).[39, 40]

RV dilatation and wall motion abnormalities constitute diagnostic criteria for ARVC. The abnormalities of the RV posterior wall underneath the tricuspid valve are most common. RV outflow dilatation was seen with increasing frequency upon progression of disease severity.[41]  Left ventricular (LV) involvement was seen in 16% of cases in this series. The RV morphologic abnormalities include trabecular derangement (most common), hyperreflective moderator band, and sacculations seen in the probands, but not in controls.[42]

Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/ARVC). Apical four-chamber transthoracic echocardiogram (TTE) showing right ventricular dilatation and dysfunction.
Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/ARVC). Parasternal long-axis view with right ventricular outflow tract dilatation and dysfunction.

Revised task force criteria using two-dimensional echocardiography are discussed below.

Major criteria are defined as regional RV akinesia or dyskinesia or aneurysm and one of the following (end-diastole):

  • Parasternal long-axis view (PLAX) right ventricular outflow tract (RVOT) = 32 mm (= 19 mm/m2 body surface area [BSA] corrected)

  • Parasternal short-axis view (PSAX) RVOT >36 mm (= 21 mm/m2 BSA corrected)

  • Fractional area change = 33%

Minor criteria are defined as regional RV akinesia or dyskinesia and one of the following:

  • PLAX RVOT =29 to < 32 mm (= 16 to < 19 mm/m2 BSA corrected)

  • PSAX RVOT =32 to < 36 mm (= 18 to < 21 mm/m2 BSA corrected)

  • Fractional area change >33 to = 40%

Cardiac Magnetic Resonance Imaging

The right ventricle (RV) is better visualized on cardiac magnetic resonance imaging (CMRI) than on echocardiography, making CMRI the modality of choice for evaluation. CMRI is used to evaluate RV size, function, wall motion abnormalities, intramyocardial fat (using fat suppression sequence), and late gadolinium enhancement to assess areas of fibrosis.[43] RV wall thickening or thinning and prominent trabeculations are also seen in arrhythmogenic right ventricular cardiomyopathy (ARVC).[44, 45, 46, 47, 48]

Arrhythmogenic Right Ventricular Dysplasia/Cardiom Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/ARVC). Axial black blood image of the right ventricle showing fatty infiltration in the free wall of the right ventricle.

Major limitations of CMRI include limited experience of readers due to low prevalence and significant interobserver variability, resulting in high false-positive rates. Fatty infiltration of the RV wall is no longer considered pathognomonic of ARVC, as it may be seen as a normal variant, especially in elderly persons. RV fat infiltration is less reproducible and lacks specificity compared with RV kinetic abnormalities.[49]  In a cohort of 70 patients, RV fatty infiltration was seen in 28.9% patients whereas LV late gadolinium enhancement was found in 35.5% patients.[50] These abnormalities were most often found in patients who met major task force criteria for ARVD/ARVC. [50]

In a tagged MRI study, regional LV dysfunction was seen in 37.5% of segments in definite ARVD and 18.7% in probable ARVD based on task force criteria.[51]

Revised task force criteria for RV abnormalities are discussed below.

Major criteria are defined as regional RV akinesia or dyskinesia or dyschronous RV contraction and one of the following:

  • Ratio of RV end-diastolic volume to BSA in males = 110 mL/m2 or BSA = 100 mL/m2 in females

  • RV ejection fraction (RVEF) = 40%

Minor criteria are defined as regional RV akinesia or dyskinesia or dyschronous RV contraction and one of the following:

  • Ratio of RV end-diastolic volume to BSA = 100 to < 110 mL/m2 in males or BSA = 90 to < 100 mL/m2 in females

  • RVEF >40% to = 45%

Increased native T1 mapping in the LV has been reported in patients with ARVD; however, the RV could not be evaluated because of its thin walls. [52]

 Lack of MRI abnormalities does not exclude ARVD, and the diagnosis should be based on task force criteria. 

Right Ventriculography

Right ventricular (RV) angiography may show RV enlargement, hypertrophic trabeculae, and wall motion abnormalities. However, since this method is invasive, it is rarely used.

RV regional akinesia, dyskinesia, or aneurysm on angiography is a major diagnostic criterion. The transversally arranged hypertrophic trabeculae, separated by deep fissures, were associated with the highest probability of arrhythmogenic RV cardiomyopathy (ARVC).[53]

Other Tests

Exercise stress testing

Exercise-induced ventricular tachycardia of left bundle branch block (LBBB) morphology may be detected with this method.

Electrophysiology studies

Electrophysiology testing can be of value in reproducing ventricular tachycardia. The dysfunctional areas of the right ventricle (RV), as shown on magnetic resonance imaging (MRI) or echocardiography, are characterized by discrete areas of abnormally low-amplitude electrograms.[54]  Abnormal RV electrocardiograms correlate better with endomyocardial biopsy and improve its diagnostic yield.[55]

Genetic tests

ARVC is a genetic disorder, and gene mutation of desmosomes is seen in 40%-50% cases. Clinical application of genetic testing is limited owing to multiple types of gene mutations and variable penetrance and timing of disease expression, requiring long-term follow-up.[56, 57, 58]

Genetic testing is indicated for symptomatic patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/ARVC) and family members of a patient with a positive mutation.

Relatives with more than one genetic variant are at increased risk of developing clinical disease, potentially an important determinant of the phenotypic heterogeneity seen within families with ARVC.[59]  At present, genetic testing is available for seven types of abnormalities (DSC2, DSG2, DSP, JUP, PKP2, RYR2, TMEM43).[60]

Endomyocardial Biopsy

Endomyocardial biopsy is not routinely performed owing to patchy distribution of the abnormalities, resulting in a poor diagnostic yield. Changes are typically seen in the right ventricular (RV) free wall, and the septum is rarely involved. RV free wall biopsy carries a small risk of perforation.

Histological demonstration of fibrofatty infiltration is not pathognomonic of arrhythmogenic RV cardiomyopathy (ARVC), as fatty infiltration may be a normal finding in elderly persons.[49] In a recent study, reduction of plakoglobin signal via immunohistochemical analysis of endomyocardial biopsy sample was found to be highly sensitive and specific for ARVC.[61]

Criteria used for tissue characterization (fibrofatty replacement of the myocardium) of the RV wall are discussed below.

Major criteria include residual myocytes < 60% by morphometric analysis (or < 50% by estimation), with fibrous replacement of the RV free wall myocardium in =1 sample, with or without fatty replacement of tissue.

Minor criteria include residual myocytes 60%-75% by morphometric analysis (or 50%-65% by estimation), with fibrous replacement of the RV free wall myocardium in =1 sample, with or without fatty replacement of tissue.

Anatomy and Histology

The most classic feature of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/ARVC) is fatty infiltration of the RV free wall. This results in variable areas of fibrosis and scarring, causing aneurysm formation or generalized dilatation and loss of function. The RV wall may be thickened or thinned out.

The typical sites of involvement include the RV apex, inflow, and outflow, but the subendocardial layer is not involved. The epicardial and mediomural layers have a variable degree of fibrosis with interspersed strands of cardiomyocytes. In about two thirds of all cases, features of myocarditis are seen. Fatty infiltration of the RV apex and infundibulum is not uncommon and may represent the normal aging process. However, there is no fibrosis, and the risk of sudden death is minimal.

There is variable involvement of the LV, and trabeculae carneae of the apex and septum are hypertrophied.

In a small series of 21 patients diagnosed based on task force criteria, electron microscopy showed various abnormalities in the desmosomes (D) in 75% of patients and positive screening of D protein encoding genes in half of probands.[62] Desmosomes are responsible for cell-to-cell binding and function of the gap junction.

 

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]

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]

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]

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. 

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]

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.