Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC), also known as Arrhythmogenic Right Ventricular Dysplasia (ARVD), is an inherited heart muscle disorder characterized by the progressive replacement of right ventricular myocardium with fibro-fatty tissue. This structural remodeling creates an electrically unstable substrate prone to life-threatening ventricular arrhythmias, making ARVC one of the leading causes of sudden cardiac death (SCD) in young people and athletes. Estimated to affect approximately 1 in 2,000 to 5,000 individuals, the condition is caused predominantly by mutations in desmosomal proteins that anchor cardiomyocytes together. Early recognition, genetic testing, exercise restriction, and appropriate use of implantable defibrillators are central to management and survival.

Table of Contents

  1. Overview
  2. Genetics and Pathophysiology
  3. Clinical Presentation
  4. Electrocardiographic Findings
  5. Diagnostic Criteria (2010 Task Force)
  6. Treatment
  7. Risk Stratification and Prevention
  8. Prognosis
  9. Recent Research and Advances
  10. Key Research Papers
  11. Connections
  12. Featured Videos

Overview

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a primary myocardial disease that predominantly affects the right ventricle, though biventricular and left-dominant forms are increasingly recognized. The fundamental pathological process is the gradual replacement of normal heart muscle — cardiomyocytes — with fibrous and adipose tissue. This fibro-fatty infiltration disrupts the organized electrical conduction pathways of the heart, creating zones of slow conduction and reentry circuits that sustain potentially fatal ventricular arrhythmias.

The disease was formally described in 1982 by Marcus and colleagues, who identified its hallmark features of right ventricular structural abnormalities combined with ventricular tachycardia of left bundle branch block (LBBB) morphology. The term "dysplasia" in the older name ARVD reflects the earlier (and now largely abandoned) hypothesis that fibro-fatty replacement represented a developmental defect. Current understanding recognizes the condition as a progressive, genetically determined cardiomyopathy driven by desmosomal dysfunction.

The fibro-fatty replacement in ARVC tends to concentrate in three anatomical regions collectively called the triangle of dysplasia: the right ventricular outflow tract (RVOT), the right ventricular apex, and the subtricuspid (inferior/diaphragmatic) region. These areas are preferential sites for arrhythmia origin and structural wall motion abnormalities detectable by imaging.

The prevalence is estimated at 1 in 2,000 to 1 in 5,000 in the general population, though this is likely an underestimate given the highly variable disease penetrance (approximately 30–50% of mutation carriers show clinical manifestations) and the fact that sudden death may be the first presentation. ARVC accounts for up to 17% of sudden cardiac deaths in young people under age 35 in some populations, and for an even higher proportion in athletes, particularly in regions of Italy where the condition has been extensively studied.

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Genetics and Pathophysiology

ARVC is a genetic disease with autosomal dominant inheritance in the vast majority of cases, although rare autosomal recessive syndromes exist. The molecular basis centers on mutations affecting the cardiac desmosome — the protein complex that mechanically couples adjacent cardiomyocytes and anchors the cytoskeleton at cell-cell junctions.

The five major desmosomal proteins implicated in ARVC are:

Non-desmosomal genes implicated include TMEM43 (a transmembrane protein associated with aggressive ARVC in Newfoundland populations), LMNA, SCN5A, PLN (phospholamban), CDH2, and RYR2. Variants in non-desmosomal genes account for a smaller fraction of cases but can present with overlapping phenotypes including dilated cardiomyopathy.

The pathophysiological cascade from desmosomal mutation to fibro-fatty cardiomyopathy is not completely understood, but two major mechanisms are proposed. The mechanical hypothesis holds that impaired cell-cell adhesion leads to cardiomyocyte detachment and death under the mechanical stress of repeated cardiac contraction, with subsequent replacement by fibrofatty scar. The signaling hypothesis proposes that disruption of the desmosome causes mislocalization of junctional plakoglobin (gamma-catenin) to the nucleus, where it competitively inhibits Wnt/beta-catenin signaling, promoting adipogenesis of cardiac progenitor cells. Both mechanisms likely operate in tandem.

Exercise plays a critical accelerating role: endurance training markedly increases right ventricular wall stress and accelerates fibrofatty replacement, explaining why ARVC manifests earlier and more severely in competitive athletes compared to sedentary mutation carriers.

Naxos disease is a rare autosomal recessive syndrome caused by homozygous mutations in JUP, presenting as a triad of ARVC, palmoplantar keratoderma (thickening of palm and sole skin), and woolly hair. It was first described on the Greek island of Naxos.

Carvajal syndrome is caused by recessive mutations in DSP and presents with a similar triad but with predominant left ventricular involvement rather than classical right-dominant ARVC, along with dilated cardiomyopathy features.

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Clinical Presentation

ARVC typically presents in the second through fourth decades of life, though the age of onset is variable and influenced by genotype, sex, and physical activity level. Men are more commonly and more severely affected than women, even among carriers of the same mutation. The disease is rarely manifest in children under 12 years of age.

Clinical presentations exist on a spectrum from asymptomatic electrocardiogram (ECG) or imaging abnormalities discovered incidentally, to symptomatic arrhythmias, to sudden cardiac arrest as the first manifestation. The classic clinical scenario is a young athlete presenting with palpitations, presyncope, or syncope during or immediately after exercise. The exertional triggering pattern distinguishes ARVC from many other channelopathies such as Long QT syndrome, which is more commonly triggered by emotion or sudden auditory stimuli.

Four clinical phases are recognized:

  1. Concealed phase: Structural and electrical abnormalities are absent or subtle; sudden death can still occur, particularly with intense exertion, making this phase particularly dangerous for competitive athletes who are unaware of their diagnosis.
  2. Overt electrical phase: Symptomatic ventricular arrhythmias (palpitations, presyncope, syncope) emerge; typical LBBB-morphology ventricular tachycardia (VT) indicates origin in the right ventricle. Structural abnormalities are detectable on imaging in most patients at this stage.
  3. Structural right ventricular (RV) disease: Progressive fibro-fatty replacement produces right ventricular dilation and global dysfunction. Symptoms of right heart failure may appear — exercise intolerance, leg edema, elevated jugular venous pressure.
  4. Biventricular failure: In a subset (approximately 10–15%) the disease extends to the left ventricle, producing a picture closely resembling dilated cardiomyopathy with biventricular systolic dysfunction and advanced heart failure requiring transplantation.

Family history of unexplained sudden cardiac death in a young family member, or a family member with documented ARVC or desmosomal mutation, raises clinical suspicion substantially. Because disease penetrance is 30–50%, family screening programmes test the first-degree relatives of an index case even when they have no symptoms.

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Electrocardiographic Findings

The 12-lead electrocardiogram is the single most accessible diagnostic tool in ARVC and shows a characteristic constellation of repolarization and depolarization abnormalities reflecting fibro-fatty replacement of right ventricular myocardium.

Epsilon wave: The most specific electrocardiogram finding in ARVC is the epsilon wave — a small, distinct positive deflection that appears in the terminal portion of the QRS complex in leads V1–V3, representing delayed activation of islands of surviving myocardium within the fibrosed right ventricle. It is best seen with signal-averaged electrocardiogram but is visible on standard 12-lead electrocardiogram in approximately 30% of affected patients. The epsilon wave is considered pathognomonic of ARVC when unequivocally present.

T-wave inversions in V1–V3: Inverted T-waves across the right precordial leads (V1 through at least V3) in the absence of right bundle branch block are a major electrocardiogram criterion for ARVC in patients over 14 years of age. This repolarization abnormality reflects the altered electrical properties of fibro-fatty-replaced myocardium and is present in over 80% of symptomatic patients. T-wave inversions that extend to V4, V5, or V6 suggest more extensive disease or left ventricular involvement (particularly DSP mutations).

Right bundle branch block (RBBB): Complete or incomplete RBBB may develop as fibro-fatty infiltration disrupts the right bundle branch conduction axis. When RBBB is present and T-waves are inverted in V1–V3, this is attributed to the RBBB itself and does not count as an independent criterion.

Prolonged S-wave upstroke (terminal activation delay): A terminal activation delay defined as ≥55 ms measured from the nadir of the S-wave to the end of all depolarization in V1, V2, or V3 (in the absence of complete RBBB) is a minor electrocardiogram criterion.

Ventricular tachycardia morphology: VT in ARVC characteristically shows LBBB morphology because it originates in the right ventricle. The most common form arises from the RVOT and has an inferior axis (positive QRS in leads II, III, and aVF), reflecting superior-to-inferior activation. VT arising from the subtricuspid region tends to have a superior axis. Incessant monomorphic VT, multiple VT morphologies (indicating multiple scar-based reentry circuits), and non-sustained runs are all well-recognized presentations.

Signal-averaged ECG (SAECG): SAECG can detect late potentials — low-amplitude, high-frequency signals at the end of the QRS representing slow conduction through scarred myocardium. They are judged from three measurements (filtered QRS duration, the duration of the low-amplitude terminal signal under 40 µV, and the root-mean-square voltage of the terminal 40 ms), and an abnormal signal-averaged ECG (late potentials) counts as a minor Task Force criterion. The abnormal values are filtered QRS duration >114 ms, duration of terminal QRS <40 µV signal >38 ms, and root mean square voltage of terminal 40 ms <20 µV.

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Diagnostic Criteria (2010 Task Force)

The diagnosis of ARVC is formalized through the revised 2010 Task Force Criteria (TFC), which replaced the original 1994 criteria with more precise quantitative thresholds to improve both sensitivity and specificity. The revised criteria are organized into six categories, each with major and minor criteria. A definitive diagnosis requires 2 major criteria, or 1 major + 2 minor criteria, or 4 minor criteria from different categories. A borderline diagnosis is made with 1 major + 1 minor, or 3 minor from different categories. Possible ARVC is 1 major or 2 minor criteria.

Category I: Structural and Functional Abnormalities by Echocardiography, Cardiac Magnetic Resonance Imaging, or Angiography

Major: Regional RV akinesia, dyskinesia, or aneurysm plus one of the following — RV outflow tract parasternal long-axis (PLAX) dimension ≥32 mm (PLAX/body surface area (BSA) ≥19 mm/m²), parasternal short-axis (PSAX) dimension ≥36 mm (PSAX/BSA ≥21 mm/m²), or fractional area change ≤33%.
Minor: Less severe wall motion abnormality with borderline dimensions.

Category II: Tissue Characterization of Walls (Endomyocardial Biopsy)

Major: Residual myocytes <60% by morphometric analysis with fibrous replacement on ≥1 sample (with or without fatty replacement of RV free wall on endomyocardial biopsy).
Minor: Residual myocytes 60–75% with fibrous replacement.

Category III: Repolarization Abnormalities (Electrocardiogram)

Major: Inverted T-waves in V1–V3 in patients >14 years, without RBBB.
Minor: Inverted T-waves in V1–V2 in patients >14 years without RBBB, or in V4–V6; inverted T-waves in V1–V4 with complete RBBB.

Category IV: Depolarization and Conduction Abnormalities (Electrocardiogram)

Major: Epsilon wave in V1–V3.
Minor: abnormal signal-averaged ECG (late potentials) without complete RBBB; QRS ≥110 ms in V1–V3 without complete RBBB; terminal activation delay ≥55 ms in V1, V2, or V3 without RBBB.

Category V: Arrhythmias

Major: Non-sustained or sustained VT with LBBB morphology and superior axis (negative or indeterminate QRS in leads II, III, aVF).
Minor: Non-sustained or sustained VT with RVOT morphology (LBBB, inferior axis); more than 500 premature ventricular complexes (PVCs) in 24 hours on Holter monitoring.

Category VI: Family History

Major: ARVC confirmed in first-degree relative meeting current TFC; ARVC confirmed at autopsy or surgery in first-degree relative; identification of a pathogenic mutation categorized as disease-causing in the patient.
Minor: ARVC history in first-degree relative where it is not possible to determine whether family member meets current TFC; premature sudden death (<35 years) due to suspected ARVC in first-degree relative; ARVC confirmed in second-degree relative.

Diagnosis has moved on since the 2010 criteria. In 2020 the "Padua criteria" were proposed for both right- and left-sided disease, and a 2023 European Task Force report (published 2024) refined them under the wider name arrhythmogenic cardiomyopathy, covering right-sided, both-sided (biventricular) and left-sided forms. The new criteria add cardiac magnetic resonance scar findings (late gadolinium enhancement), and a "ring-like" pattern of scar is now a recognized hallmark of the left-sided form; they also add electrocardiogram and arrhythmia criteria of left ventricular origin (Corrado 2024).

Cardiac magnetic resonance imaging (MRI) plays an especially important role in modern ARVC evaluation. It can detect RV free wall motion abnormalities and RV dilation with high precision, and late gadolinium enhancement (LGE) identifies fibrous replacement — a finding that has prognostic implications beyond the structural TFC criteria. Fat infiltration on magnetic resonance imaging (high signal on T1-weighted sequences) is a recognized feature, though it requires careful interpretation because physiological epicardial fat is common in the normal RV.

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Treatment

Management of ARVC is individualized based on the patient's arrhythmia burden, risk of sudden cardiac death, degree of structural heart disease, and degree of functional impairment. No treatment has been shown to prevent the underlying fibro-fatty progression, so current therapies target arrhythmia suppression and SCD prevention.

Exercise Restriction

Exercise restriction is arguably the single most impactful intervention available for ARVC. The 2015 International Task Force consensus statement recommends that patients with a definite diagnosis of ARVC not take part in competitive and/or endurance sports (Class I), regardless of symptoms; the consensus notes that competitive sport has been shown to raise the risk of sudden death five-fold in adolescents and young adults with ARVC. Because intense exercise accelerates fibro-fatty replacement and markedly increases arrhythmia risk — including in the concealed phase — the same statement says restriction from competitive sports may be considered for family members without signs of disease, both healthy gene carriers (Class IIa) and those of unknown genotype (Class IIb). It makes a possible exception for recreational low-intensity sports (Class IIa), such as walking or light yoga.

Implantable Cardioverter-Defibrillator (ICD)

ICD implantation is the most effective therapy for preventing sudden death. Under the 2015 International Task Force consensus statement, Class I (recommended) indications are:

An ICD "should be considered" (Class IIa) after one or more episodes of hemodynamically stable sustained VT, and in patients with "major" risk factors such as unexplained syncope, moderate ventricular dysfunction or non-sustained ventricular tachycardia (NSVT).

ICD therapy carries specific complications in ARVC including inappropriate shocks (from T-wave oversensing due to RV electrode displacement as the RV dilates and deforms), lead failure from chronic RV structural changes, and the psychological burden on young patients. Subcutaneous ICDs (S-ICD) are an option for patients without pacing or resynchronization needs, avoiding intravascular leads. Wearable cardioverter-defibrillators (WCD) may bridge patients to ICD implantation or cover acute high-risk periods.

Antiarrhythmic Drug Therapy

Sotalol is the most commonly used antiarrhythmic agent for ARVC, combining beta-blockade with class III potassium channel blockade. It reduces VT frequency and is used in combination with ICD therapy to decrease shock burden. The 2015 consensus statement describes amiodarone, alone or with beta-blockers, as the most effective drug for preventing symptomatic ventricular arrhythmias, with a relatively low proarrhythmic risk even in patients with ventricular dysfunction, although its ability to prevent sudden death is unproved; its cumulative organ toxicity (thyroid, pulmonary, hepatic) limits long-term use. The statement recommends antiarrhythmic drugs as an add-on to the ICD in patients with frequent appropriate device shocks (Class I). Beta-blockers alone (metoprolol, bisoprolol) provide modest VT suppression but are insufficient as monotherapy for sustained VT. Class IC agents (flecainide, propafenone) are generally avoided in structurally abnormal hearts due to proarrhythmic risk.

Catheter Ablation

Catheter ablation is used for VT refractory to or intolerant of antiarrhythmic drugs, or as an adjunct to ICD therapy in patients with frequent appropriate shocks ("VT storm"). Because the critical circuits in ARVC are located in the epicardium (on the outer surface of the RV free wall) as well as the endocardium, combined endocardial-epicardial mapping and ablation through subxiphoid pericardial access achieves substantially better outcomes than endocardial-only ablation. Electroanatomic mapping delineates low-voltage scar borders to guide substrate ablation. Recurrence after ablation is common (30–50% at 3 years) as the underlying disease continues to progress.

Heart Failure Therapy and Transplantation

Patients with biventricular dysfunction receive standard heart failure pharmacotherapy (angiotensin-converting enzyme inhibitors (ACE inhibitors)/angiotensin receptor blockers (ARBs), beta-blockers, diuretics). In advanced end-stage disease with refractory heart failure or intractable arrhythmias unresponsive to all other therapies, cardiac transplantation provides definitive cure — both the structural cardiomyopathy and the arrhythmia substrate are eliminated.

In 2023 the European Society of Cardiology published its first guideline covering all the cardiomyopathies together, including arrhythmogenic right ventricular cardiomyopathy (Arbelo 2023).

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Risk Stratification and Prevention

Identifying which ARVC patients are at highest risk of sudden cardiac death is a critical clinical challenge. Unlike hypertrophic cardiomyopathy, where validated scoring tools exist (HCM Risk-SCD), risk stratification in ARVC remains incompletely standardized. Multiple clinical variables have been identified as predictors of adverse events:

Family screening is a cornerstone of ARVC prevention. Screening of first-degree relatives (parents, siblings, children) of a confirmed case usually includes:

  1. 12-lead electrocardiogram
  2. 24-hour Holter monitoring
  3. Echocardiography
  4. Signal-averaged electrocardiogram
  5. Genetic testing for the family's specific mutation (if identified)
  6. Cardiac magnetic resonance imaging (if any findings are abnormal or equivocal)
  7. Exercise stress testing in selected cases

Relatives found to carry a pathogenic mutation but who do not yet meet diagnostic criteria are usually followed with clinical checks every 1–3 years, as penetrance is incomplete and disease can manifest over time; the 2015 consensus says restriction from competitive sports may be considered for them (Class IIa).

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Prognosis

The prognosis of ARVC is highly variable, ranging from a benign course with minimal symptoms across decades to sudden death at a young age. Disease penetrance of approximately 30–50% means that many gene carriers never develop clinically significant manifestations. However, once the condition is clinically manifest, its trajectory depends heavily on the arrhythmia burden, degree of structural involvement, and adherence to exercise restriction and therapy.

In ICD recipients, the annual rate of appropriate ICD shock (evidence of a treated life-threatening arrhythmia) ranges from approximately 9–17% per year in high-risk populations, underscoring the ongoing arrhythmia burden even in treated patients. Long-term follow-up studies suggest that overall mortality with modern ICD therapy is relatively low (around 1–2% per year), but quality of life can be impaired by frequent ICD interventions, antiarrhythmic drug side effects, and psychological burden.

Heart failure as a cause of death or transplantation is less common than arrhythmic death but affects approximately 10–20% of patients followed over decades, particularly those with biventricular involvement. The 10-year transplant-free survival in unselected ARVC populations is approximately 70–80% in contemporary cohorts managed with ICD.

Genotype has prognostic implications. PKP2 mutations are generally associated with classical right-dominant ARVC and intermediate penetrance. DSP mutations carry a higher risk of LV involvement, severe ventricular dysfunction, and early heart failure. TMEM43 mutations (especially the p.S358L variant in Newfoundland founder populations) carry very high rates of SCD and heart failure, with male carriers having near-universal lethal events before age 50 without ICD therapy.

Exercise restriction remains the most modifiable prognostic factor in the long term. Registry data consistently demonstrate that patients who continue competitive sports have substantially worse outcomes than those who comply with activity restriction, even when controlling for baseline disease severity.

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Recent Research and Advances

ARVC research has advanced substantially over the past decade, spanning molecular mechanisms, improved imaging, novel risk stratification tools, and emerging therapies.

Desmosome biology and Wnt signaling: The discovery that desmosomal disruption suppresses Wnt/beta-catenin signaling — promoting adipogenesis of cardiac progenitor cells — has opened therapeutic avenues. Preclinical models using GSK-3 inhibitors and other Wnt activators have shown reduction in fibro-fatty replacement, though none are yet in clinical trials for ARVC specifically.

Improved magnetic resonance imaging protocols: Advances in cardiac magnetic resonance imaging including phase-sensitive inversion recovery for late gadolinium enhancement and 4D flow sequences have improved both sensitivity and specificity for early ARVC detection. Strain imaging by feature tracking magnetic resonance imaging can detect subtle RV wall motion abnormalities meeting Task Force criteria even before overt dysfunction. LGE extent on magnetic resonance imaging has emerged as an independent predictor of VT recurrence after catheter ablation.

Risk stratification scores: The 2019 ARVC risk calculator (Cadrin-Tourigny 2019; online at arvcrisk.com) was built from 528 patients with a definite diagnosis and no previous sustained ventricular arrhythmia, enrolled from five registries in North America and Europe. Over a median of about 4.8 years, 146 of them (27.7%) had a sustained ventricular arrhythmia (sudden death, aborted sudden death, sustained VT or an appropriate ICD shock). The model estimates each person's yearly risk from age, sex, fainting of cardiac cause (cardiac syncope) in the previous 6 months, non-sustained VT, the number of premature ventricular complexes in 24 hours, the number of electrocardiogram leads with inverted T waves, and right ventricular ejection fraction. Compared with the consensus ICD algorithm, the model would have meant 20.6% fewer ICD implants while protecting the same proportion of patients. This tool is increasingly used to guide ICD implantation decisions in intermediate-risk patients.

Precision catheter ablation: Combined endocardial-epicardial ablation guided by high-density mapping with 3D electroanatomic mapping systems has improved acute success and reduced recurrence rates compared to historical endocardial-only ablation. Ablation of low-voltage scar borders rather than only VT isthmuses (substrate ablation) appears more durable. Centers reporting combined approaches document VT-free survival of 60–75% at 3 years.

Left-dominant ARVC: Recognition of left-dominant and biventricular ARVC variants — where the LV is primarily or equally affected — has expanded the diagnostic concept. These cases, often linked to DSP mutations, may mimic myocarditis or dilated cardiomyopathy and require magnetic resonance imaging and genetic testing for correct diagnosis.

Exercise studies and mechanisms: Longitudinal exercise studies in mouse models and in human athletes have confirmed that endurance exercise accelerates desmosomal damage and fibro-fatty replacement in PKP2 heterozygotes. These findings reinforce the clinical recommendation for activity restriction and have generated interest in whether supervised low-intensity exercise can be individualized rather than universally prohibited.

Gene therapy and RNA approaches: Early-phase work in cardiomyocyte culture and animal models has explored exon-skipping strategies for certain PKP2 variants and antisense oligonucleotide approaches to upregulate residual PKP2 expression in haploinsufficiency. The main approach now is to restore plakophilin-2 with adeno-associated virus (AAV) gene therapy, which a 2026 review reports "demonstrated promising results" in animal and human stem-cell-derived heart-cell models (Geerlings 2026); it is not yet proven in people, and the review notes that the models still disagree in places.

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Key Research Papers

  1. Marcus FI, Fontaine GH, Guiraudon G, et al. Right ventricular dysplasia: a report of 24 adult cases. Circulation. 1982;65(2):384–398. — PubMed PMID: 7053899
  2. Marcus FI, McKenna WJ, Sherrill D, et al. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force Criteria. Eur Heart J. 2010;31(7):806–814. — PubMed PMID: 20172912
  3. Corrado D, Basso C, Thiene G, et al. Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study. J Am Coll Cardiol. 1997;30(6):1512–1520. — PubMed PMID: 9362410
  4. Dalal D, Molin LH, Piccini J, et al. Clinical features of arrhythmogenic right ventricular dysplasia/cardiomyopathy associated with mutations in plakophilin-2. Circulation. 2006;113(13):1641–1649. — PubMed PMID: 16549640
  5. Gerull B, Heuser A, Wichter T, et al. Mutations in the desmosomal protein plakophilin-2 are common in arrhythmogenic right ventricular cardiomyopathy. Nat Genet. 2004;36(11):1162–1164. — PubMed PMID: 15489853
  6. Kirchhof P, Fabritz L, Zwiener M, et al. Age- and training-dependent development of arrhythmogenic right ventricular cardiomyopathy in heterozygous plakoglobin-deficient mice. Circulation. 2006;114(17):1799–1806. — PubMed PMID: 17030684
  7. James CA, Bhonsale A, Tichnell C, et al. Exercise increases age-related penetrance and arrhythmic risk in arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated desmosomal mutation carriers. J Am Coll Cardiol. 2013;62(14):1290–1297. — PubMed PMID: 23871885
  8. Bhonsale A, James CA, Tichnell C, et al. Incidence and predictors of implantable cardioverter-defibrillator therapy in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy undergoing implantable cardioverter-defibrillator implantation for primary prevention. J Am Coll Cardiol. 2011;58(14):1485–1496. — PubMed PMID: 21939834
  9. Corrado D, Wichter T, Link MS, et al. Treatment of arrhythmogenic right ventricular cardiomyopathy/dysplasia: an international task force consensus statement. Circulation. 2015;132(5):441–453. — PubMed PMID: 26216213
  10. Berruezo A, Fernandez-Armenta J, Mont L, et al. Combined endocardial and epicardial catheter ablation in arrhythmogenic right ventricular dysplasia incorporating scar dechanneling technique. Circ Arrhythm Electrophysiol. 2012;5(1):111–121. — PubMed PMID: 22205683
  11. Cadrin-Tourigny J, Bosman LP, Nozza A, et al. A new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy. Eur Heart J. 2019;40(23):1850–1858. — PubMed PMID: 30915475
  12. Arbelo E, Protonotarios A, Gimeno JR, et al. 2023 ESC Guidelines for the management of cardiomyopathies. Eur Heart J. 2023;44(37):3503–3626. — PubMed PMID: 37622657
  13. Corrado D, Anastasakis A, Basso C, et al. Proposed diagnostic criteria for arrhythmogenic cardiomyopathy: European Task Force consensus report. Int J Cardiol. 2024;395:131447. — PubMed PMID: 37844667
  14. Geerlings L, Carreras YR, Vermeer MCSC. Disease models and AAV therapies of PKP2-induced arrhythmogenic cardiomyopathy. Mol Ther Adv. 2026;34(1):201688. — PubMed PMID: 42137277
  15. Protonotarios N, Tsatsopoulou A. Naxos disease and Carvajal syndrome: cardiocutaneous disorders that highlight the pathogenesis and broaden the spectrum of arrhythmogenic right ventricular cardiomyopathy. Cardiovasc Pathol. 2004;13(4):185–194. — PubMed PMID: 15210133

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Connections

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