--- title: Arrhythmogenic right ventricular cardiomyopathy description: What arrhythmogenic right ventricular cardiomyopathy is, why heart muscle is replaced by fatty and fibrous tissue, how the disease is detected, which treatment approaches clinical guidelines describe and when urgent help is needed. type: disease aliases: - ARVC - arrhythmogenic right ventricular dysplasia - ARVD - arrhythmogenic cardiomyopathy sources: - tier: guideline org: minzdrav-rf title: Желудочковые нарушения ритма сердца. Внезапная сердечная смерть year: 2025 doi: 10.15829/1560-4071-2025-6670 url: https://cr.minzdrav.gov.ru/view-cr/569_2 - tier: guideline org: esc title: 2023 ESC Guidelines for the management of cardiomyopathies year: 2023 doi: 10.1093/eurheartj/ehad194 url: https://doi.org/10.1093/eurheartj/ehad194 - tier: reference org: msd-manuals title: Arrhythmogenic Right Ventricular Cardiomyopathy and Variants year: 2026 url: https://www.msdmanuals.com/professional/cardiovascular-disorders/arrhythmogenic-cardiac-disorders/arrhythmogenic-right-ventricular-cardiomyopathy-and-variants - tier: guideline org: nice title: Implantable cardioverter defibrillators and cardiac resynchronisation therapy for arrhythmias and heart failure (TA314) year: 2014 url: https://www.nice.org.uk/guidance/ta314 - tier: guideline org: nice title: Transient loss of consciousness ('blackouts') in over 16s (CG109) year: 2023 url: https://www.nice.org.uk/guidance/cg109 sources_checked: 2026-10-12 review: null --- Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease in which heart muscle is replaced by fibrofatty tissue; it shows itself as ventricular arrhythmias and heart failure and can cause sudden cardiac death. It used to be called arrhythmogenic right ventricular dysplasia; it mainly affects the right ventricle, causes [ventricular tachycardia](../ventricular-tachycardia/) and other ventricular arrhythmias, and increases the risk of sudden death. ARVC is one of the cardiomyopathies, like [dilated](../dilated-cardiomyopathy/) and [hypertrophic](../hypertrophic-cardiomyopathy/) cardiomyopathy. According to the European Society of Cardiology, its prevalence in adults is about 0.078%, and it is very rare in infancy and early childhood.

Normally the wall of the right ventricle (RV) is made of heart muscle. Its cells are held together by special junctions called desmosomes, which connect cells in tissues that undergo mechanical stress. The dotted line shows the normal size of the RV in both states.

In ARVC heart muscle cells are replaced by fibrofatty tissue, mostly in the "triangle" between the outflow part, the inflow part and the apex of the RV, and sometimes also in the posterolateral wall of the left ventricle. At first the disease shows itself as extra beats and ventricular arrhythmias; later the RV becomes dilated and thin and heart failure develops. The diagram shows this late stage; in advanced disease the ventricles can have systolic failure, so the RV in the diagram contracts more weakly.

## Why it happens ARVC is a genetic disease. Changes (mutations) in genes most often disturb desmosomes, the junctions that hold heart muscle cells together. These cells are damaged more easily by mechanical strain on the heart, for example by prolonged exercise, and where the damage heals the muscle is replaced by fatty and fibrous tissue. Most often the disease is passed on from one parent (autosomal dominant inheritance), but rare forms with a different type of inheritance are also known. Sustained heavy exertion, such as endurance sport, is thought to bring on the disease earlier and speed it up. Arrhythmogenic cardiomyopathy is usually characterised by fibrous or fibrofatty replacement of the myocardium, leading to progressive impairment of contractility and a high frequency of ventricular arrhythmias; three phenotypes are recognised: predominantly right ventricular ("classic" ARVC), biventricular and predominantly left ventricular. Most often the mutations affect the desmosome of the intercalated disc; the desmosomal proteins that may be affected include plakophilin, desmoplakin and desmoglein. The abnormal proteins are prone to damage by mechanical stress, and as the damage heals, myocytes are replaced by fibrofatty tissue; the manifestations of the disease result from both electrophysiological and structural changes. Inheritance is most often autosomal dominant with variable penetrance, and autosomal recessive forms are also known. ARVC and its variants account for about 10% of sudden cardiac deaths not explained by coronary or other structural heart disease. ## Symptoms The disease may cause no symptoms. Palpitations and fainting are possible. In some people the first sign is fainting, an attack of ventricular tachycardia, ventricular fibrillation or sudden death. Dangerous arrhythmias are especially likely during emotional stress or physical exertion. As the disease progresses, signs of right ventricular failure appear, a form of [heart failure](../heart-failure/). Atrial fibrillation and signs of right and/or left ventricular systolic failure are usually manifestations of advanced disease. According to the ESC, the prevalence of [atrial fibrillation](../atrial-fibrillation/) in ARVC is 9–30%. ## How it is diagnosed ARVC is suspected when a person, especially a young one (often under 40), has palpitations, fainting caused by the heart, recorded ventricular arrhythmias or a cardiac arrest without a clear cause, while tests show no obvious heart disease. The assessment starts with questions, including about illness in the family, an [ECG](../../diagnostic/ecg/), [echocardiography](../../diagnostic/echocardiography/) and cardiac MRI. An ECG can help to raise suspicion of inherited diseases linked to arrhythmias and sudden death: cardiomyopathies, including ARVC, and channelopathies such as [Brugada syndrome](../brugada-syndrome/). If an arrhythmia has not yet been recorded, an exercise test, long-term ECG recording or an electrophysiological study may be needed. The European guideline advises yearly [Holter monitoring](../../diagnostic/holter-monitoring/) for people with ARVC. No single test settles the diagnosis: it is based on a combination of findings, namely changes on the ECG and on heart imaging, recorded arrhythmias, cases of the disease or of sudden death in the family, and the results of genetic testing. A similar picture can occur in cardiac sarcoidosis, [myocarditis](../myocarditis/) and in athletes ("athlete's heart"), and the doctor tells ARVC apart from these conditions. Genetic testing is recommended when ARVC is suspected and when the diagnosis is established. First-degree relatives are advised to have an ECG and echocardiography. If a mutation is found in the patient, all close relatives are tested for it, even if they have no signs of the disease. No single test is diagnostic; the diagnosis is based on major and minor criteria proposed by an international task force, and their combinations define possible, borderline or definite disease of the right, left or both ventricles. The criteria include evidence of ventricular disease on imaging (often fibrous or fatty replacement of myocardium), replacement of myocytes by fibrous and/or fatty tissue on biopsy, ECG repolarisation changes (including right precordial T-wave inversion), depolarisation changes (including right precordial epsilon waves), late potentials on signal-averaged ECG, documented ventricular arrhythmias originating from ventricular scar (most commonly from the RV), a family history of ARVC or of sudden death, and identification of a disease-associated mutation. Identification of a high-class pathogenicity variant (pathogenic or likely pathogenic) is a major diagnostic criterion for all types of arrhythmogenic cardiomyopathy. In the ESC table of example ECG findings in the different cardiomyopathies, ARVC is listed with T-wave inversion in V1–V3 and terminal activation delay, sometimes with low right ventricular voltages and atypical right bundle branch block. Ventricular arrhythmias of RV origin typically have a left bundle branch block–shaped QRS with a superior frontal plane axis; the more benign idiopathic right ventricular outflow tract VT usually has an inferior axis. The Russian Ministry of Health guideline, which grades recommendations on the ESC scale, recommends cardiac MRI when ARVC is suspected (class I, level B) and genetic testing when the diagnosis is suspected or established (class I, level B). When the diagnostic criteria are met, the yield of genetic testing is about 50–70%. The Russian guideline recommends contrast ventriculography and/or right ventricular myocardial biopsy if ARVC or myocarditis cannot be confirmed or excluded by non-invasive tests; biopsy findings are often nonspecific, and biopsy is rarely performed. The differential diagnosis includes cardiac sarcoidosis, athlete's heart, myocarditis and idiopathic right ventricular outflow tract VT. ## How it is treated Treatment aims to prevent sudden death and attacks of arrhythmia. People with ARVC are advised to avoid intense physical exercise and competitive sport, and the European guideline also advises against moderate-intensity exercise. People who carry the mutation but have no signs of the disease may also be advised to limit exercise. Most people with ARVC are prescribed beta-blockers. If these do not keep the attacks under control, the doctor may add other antiarrhythmic medicines, but they work only to a limited extent in ARVC and do not replace a defibrillator. The main protection against sudden death is an implantable cardioverter-defibrillator (ICD); how it works is explained in the article on [ventricular tachycardia](../ventricular-tachycardia/). An ICD is recommended for people who have survived a cardiac arrest or an arrhythmia that disrupted the circulation. For others, the doctor and the patient decide together about an ICD, taking into account fainting, markedly reduced ventricular function and other risk factors; a dedicated risk calculator helps. If attacks of tachycardia or ICD shocks keep coming back despite medicines, catheter ablation in an experienced centre may be recommended: treatment of the area of the heart that sustains the arrhythmia. If ventricular function falls markedly, heart failure is treated, up to and including heart transplantation. The Russian Ministry of Health guideline advises refraining from intense exercise (class I, level B), and the 2023 ESC guideline does not recommend moderate- and/or high-intensity exercise, including competitive sport (class III, level B). For genotype-positive/phenotype-negative family members, both guidelines suggest that limiting exercise may be considered (class IIb). The ESC recommends beta-blockers in ARVC patients with ventricular ectopy, NSVT and VT (class I, level C). The Russian guideline names sustained VT or NSVT as an indication (class I, level C) and suggests that beta-blockers may be considered in all patients with an established diagnosis (class IIb, level C). For recurrent symptomatic VT when beta-blockers are ineffective, the Russian guideline recommends antiarrhythmic drugs (class IIa, level C), although their efficacy in ARVC is limited. The ESC recommends an ICD to reduce the risk of sudden death and all-cause mortality in patients who have survived a cardiac arrest or a ventricular arrhythmia causing haemodynamic instability (class I, level A); it should be considered after haemodynamically tolerated VT (class IIa, level B). For primary prevention the Russian guideline lists as indications, among others, arrhythmic syncope (class IIa, level B) and severe systolic dysfunction of the right or left ventricle (class IIa, level C). The ESC suggests taking into account high-risk features and the updated 2019 ARVC risk calculator (class IIa, level B). NICE includes arrhythmogenic right ventricular dysplasia among familial cardiac conditions with a high risk of sudden death in which ICDs are recommended as a treatment option. The Russian guideline recommends catheter ablation in an experienced centre for recurrent symptomatic sustained monomorphic VT or ICD shocks despite beta-blockers (class IIa, level C). The ESC suggests considering it for incessant VT or frequent appropriate ICD interventions for VT despite beta-blockers, with availability of an epicardial approach (class IIa, level C). Significant ventricular dysfunction requires heart failure therapy, including transplantation. During pregnancy, beta-blocker treatment is recommended for women with ARVC (class IIa, level C). ## When to call an ambulance In ARVC the first sign of the disease can be fainting, ventricular fibrillation or sudden cardiac arrest, especially during physical or emotional stress. If someone has lost consciousness, call your local emergency number (112 in the EU, 911 in the US and Canada, 999 in the UK). The Russian Ministry of Health guideline requires immediate cardiopulmonary resuscitation in cardiac arrest, with emergency defibrillation as its key element: with emergency defibrillation the chances of restoring a normal rhythm are much higher than with delayed defibrillation. That is why automated external defibrillators are recommended in places where many people gather, such as schools, airports, railway stations and stadiums. The Russian guideline advises calling an ambulance if an episode of arrhythmia comes with dizziness, weakness, feeling faint, loss of consciousness or a racing heartbeat. An ambulance should also be called after an ICD has delivered a shock. NICE treats fainting during exertion as a red flag, as well as fainting in a person with a family history of sudden cardiac death under the age of 40 or of an inherited heart condition: such a person is referred urgently for cardiovascular assessment, and a specialist reviews the referral within 24 hours (the recommendation covers people over 16).