Table of ContentsView AllTable of ContentsSymptomsDiagnosisTreatment
Table of ContentsView All
View All
Table of Contents
Symptoms
Diagnosis
Treatment
Arrhythmogenic right ventricular cardiomyopathy(ARVC) is a genetic condition in which normal cardiac muscle becomes replaced with fibrous, fatty tissue, mainly in the right ventricle, one of thefour chambersof the heart. ARVC is one of the cardiac conditions associated with sudden death, especially inyoung athletes.
ARVC is uncommon, but not rare. It would be found in 1 out of every 2,000 to 5,000 adults if they were evaluated for it.The underlyinggeneticmutations that can cause ARVC may be even more common.
This article explains the genetic cause and symptoms of ARVC. It discusses how healthcare providers diagnose and treat arrhythmogenic right ventricular cardiomyopathy.
Stegerphoto / Getty Images
What Are the Symptoms of ARVC?
While ARVC is a cardiomyopathy—that is, a heart muscle disease—it only rarely causes muscle problems that are extensive enough to produceheart failure.
Rather, its clinical significance is that it can cause abnormal heartbeats (cardiac arrhythmias). In particular, ARVC can causepremature ventricular complexes,ventricular tachycardia, and sometimesventricular fibrillation.
Sudden death may also occur. Unfortunately, it may be the very first sign that there is any heart problem. In up to 10% of cases, the diagnosis only occurs after sudden cardiac death or resuscitation.
While ARVC can cause sudden death at any time, this event appears to be more likely to occur during episodes of physical exertion than at rest. This is why ARVC is one of the conditions that produces sudden death in apparently healthy, young athletes.
However, because this can also occur during routine activity or at rest, refraining from exercise is generally not enough to alleviate the risk in people with ARVC.
Between 5% to 10% of people with ARVC will have no symptoms whatsoever, or the first symptom will be death. Some of these people are diagnosed when they are screened for the disorder because a family member has been diagnosed with it.
How Is ARVC Diagnosed?
Cardiac magnetic resonance imaging (MRI)and cardiac computed tompgraphy (CT) may help, as can other tests and techniques such as:
Is ARVC Always Inherited?Between 30% and 50% of people diagnosed with ARVC have a family history of the condition.Genetic testing can be helpful because it can be inherited. Once the diagnosis is made, genetic screening is recommended for first-degree relatives, too. About one in three of them will also eventually develop this condition.
Is ARVC Always Inherited?
Between 30% and 50% of people diagnosed with ARVC have a family history of the condition.Genetic testing can be helpful because it can be inherited. Once the diagnosis is made, genetic screening is recommended for first-degree relatives, too. About one in three of them will also eventually develop this condition.
What Genomic Testing Can Tell You About Health Risks
ARVC Treatment
The main goal in treating ARVC is to prevent sudden cardiac death from ventricular tachycardia or fibrillation. This may be done with:
Activity Restrictions
Because sudden death is often associated with exercise in this condition, athletes who have ARVC should refrain from all competitive sports, with the possible exception of low-intensity activities such as golf or bowling.
Furthermore, they should refrain from any activity that produces significant palpitations or syncope.
Asymptomatic people are also warned that intense exercise increases the risk of developing ARVC symptoms including sudden death.
Medication
Most cardiologists treating this condition recommend the use ofbeta blockersto blunt the effect of adrenaline in the heart. Yet for people diagnosed with ARVC who have no high-risk features on evaluation and no symptoms, treatment may or may not include beta blockers.
The American Heart Association and American College of Cardiology suggest a beta blocker may be useful.However, the Heart Rhythm Society doesn’t believe there is enough evidence to support long-term beta-blocker treatment for unaffected carriers of the gene responsible for ARVC.
Some people may also be prescribed an antiarrhythmic drug, though evidence of benefit remains limited despite their wide use.
Defibrillators
Implantable defibrillatorsare frequently recommended for people with ARVC, especially when any of the following apply:
In people with ARVC who have had sustained ventricular arrhythmias, the long-term prognosis appears to be improved if they avoid exercise, take beta blockers, receive an implantable defibrillator,and(in some cases) take an antiarrhythmic drug. Prognosis also depends on which parts of the heart are involved and other individual factors.
Cardiac Ablation
A cardiac ablation may offer benefits, especially when other treatments for ARVC haven’t worked well.It is a procedure used to reset heart arrhythmias, and relies on a small catheter inserted into the heart tissue. Radiofrequency ablation relies on radio waves to heat the target tissue.
What Is the Life Expectancy With ARVC?A 2013 study reported the average age at death for someone with ARVC at 54 years, typically due to heart failure or sudden cardiac death.But newer treatments can mean that people achieve near-normal life expectancy.
What Is the Life Expectancy With ARVC?
A 2013 study reported the average age at death for someone with ARVC at 54 years, typically due to heart failure or sudden cardiac death.But newer treatments can mean that people achieve near-normal life expectancy.
Summary
Arrhythmogenic right ventricular cardiomyopathy is a genetic condition that can produce potentially lethal cardiac arrhythmias, including those that cause sudden death in young athletes.
In some cases, ARVC goes undetected until sudden death occurs, so it’s important to get tested if you have a history of this condition in your family. With aggressive treatment, including medication and implantable devices, people with ARVC typically do quite well.
13 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Maron BJ, Maron BA.Revisiting Athlete’s Heart Versus Pathologic Hypertrophy: ARVC and the Right Ventricle.JACC Cardiovasc Imaging. 2017 Apr;10(4):394-397. doi: 10.1016/j.jcmg.2016.05.011.Krahn AD, Wilde AAM, Calkins H, La Gerche A, Cadrin-Tourigny J, Roberts JD,et al.Arrhythmogenic Right Ventricular Cardiomyopathy.JACC Clin Electrophysiol. 2022 Apr;8(4):533-553. doi: 10.1016/j.jacep.2021.12.002.Bourfiss M, van Vugt M, Alasiri AI, Ruijsink B, van Setten J, Schmidt AF,et al.Prevalence and Disease Expression of Pathogenic and Likely Pathogenic Variants Associated With Inherited Cardiomyopathies in the General Population.Circ Genom Precis Med. 2022 Dec;15(6):e003704. doi: 10.1161/CIRCGEN.122.003704.Groeneweg JA, Bhonsale A, James CA, et al.Clinical presentation, long-term follow-up, and outcomes of 1001 arrhythmogenic right ventricular dysplasia/cardiomyopathy patients and family members.Circ Cardiovasc Genet. 2015 Mar;8(3):437-446. doi:10.1161/CIRCGENETICS.114.001003Johns Hopkins Medicine.Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C).Towbin JA, McKenna WJ, Abrams DJ, et al.2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy.Heart Rhythm. 2019 May;16(11):e301-e372. doi:10.1016/j.hrthm.2019.05.007Johns Hopkins Medicine.Genetics of Arrhythmogenic Right Ventricular Dysplasia / Cardiomyopathy (ARVDC).Al-Khatib SM, Stevenson WG, Ackerman MJ, et al.2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society.J Am Coll Cardiol. 2018 Oct;72(14):91-220. doi:10.1016/j.jacc.2017.10.054Al-Aidarous S, Protonotarios A, Elliott PM, Lambiase PD.Management of arrhythmogenic right ventricular cardiomyopathy.Heart. 2024 Jan 10;110(3):156-162. doi: 10.1136/heartjnl-2023-322612.Corrado D, Wichter T, Link MS, et al.Treatment of arrhythmogenic right ventricular cardiomyopathy/dysplasia: an international task force consensus statement.Eur Heart J. 2015 Dec;36(46):3227-3237. doi:10.1093/eurheartj/ehv162Aquaro GD, De Luca A, Cappelletto C, et al.Prognostic value of magnetic resonance phenotype in patients with arrhythmogenic right ventricular cardiomyopathy.Journal of the American College of Cardiology. 2020 Jun;75(22):2753-2765. doi:10.1016/j.jacc.2020.04.023Saguner AM, Duru F, Brunckhorst CB.Arrhythmogenic right ventricular cardiomyopathy: a challenging disease of the intercalated disc.Circulation. 2013 Sep 17;128(12):1381-6. doi: 10.1161/CIRCULATIONAHA.112.001009.Rigato I, Corrado D, Basso C, Zorzi A, Pilichou K, Bauce B,et al.Pharmacotherapy and other therapeutic modalities for managing Arrhythmogenic Right Ventricular Cardiomyopathy.Cardiovasc Drugs Ther. 2015 Apr;29(2):171-7. doi: 10.1007/s10557-015-6583-8.
13 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Maron BJ, Maron BA.Revisiting Athlete’s Heart Versus Pathologic Hypertrophy: ARVC and the Right Ventricle.JACC Cardiovasc Imaging. 2017 Apr;10(4):394-397. doi: 10.1016/j.jcmg.2016.05.011.Krahn AD, Wilde AAM, Calkins H, La Gerche A, Cadrin-Tourigny J, Roberts JD,et al.Arrhythmogenic Right Ventricular Cardiomyopathy.JACC Clin Electrophysiol. 2022 Apr;8(4):533-553. doi: 10.1016/j.jacep.2021.12.002.Bourfiss M, van Vugt M, Alasiri AI, Ruijsink B, van Setten J, Schmidt AF,et al.Prevalence and Disease Expression of Pathogenic and Likely Pathogenic Variants Associated With Inherited Cardiomyopathies in the General Population.Circ Genom Precis Med. 2022 Dec;15(6):e003704. doi: 10.1161/CIRCGEN.122.003704.Groeneweg JA, Bhonsale A, James CA, et al.Clinical presentation, long-term follow-up, and outcomes of 1001 arrhythmogenic right ventricular dysplasia/cardiomyopathy patients and family members.Circ Cardiovasc Genet. 2015 Mar;8(3):437-446. doi:10.1161/CIRCGENETICS.114.001003Johns Hopkins Medicine.Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C).Towbin JA, McKenna WJ, Abrams DJ, et al.2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy.Heart Rhythm. 2019 May;16(11):e301-e372. doi:10.1016/j.hrthm.2019.05.007Johns Hopkins Medicine.Genetics of Arrhythmogenic Right Ventricular Dysplasia / Cardiomyopathy (ARVDC).Al-Khatib SM, Stevenson WG, Ackerman MJ, et al.2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society.J Am Coll Cardiol. 2018 Oct;72(14):91-220. doi:10.1016/j.jacc.2017.10.054Al-Aidarous S, Protonotarios A, Elliott PM, Lambiase PD.Management of arrhythmogenic right ventricular cardiomyopathy.Heart. 2024 Jan 10;110(3):156-162. doi: 10.1136/heartjnl-2023-322612.Corrado D, Wichter T, Link MS, et al.Treatment of arrhythmogenic right ventricular cardiomyopathy/dysplasia: an international task force consensus statement.Eur Heart J. 2015 Dec;36(46):3227-3237. doi:10.1093/eurheartj/ehv162Aquaro GD, De Luca A, Cappelletto C, et al.Prognostic value of magnetic resonance phenotype in patients with arrhythmogenic right ventricular cardiomyopathy.Journal of the American College of Cardiology. 2020 Jun;75(22):2753-2765. doi:10.1016/j.jacc.2020.04.023Saguner AM, Duru F, Brunckhorst CB.Arrhythmogenic right ventricular cardiomyopathy: a challenging disease of the intercalated disc.Circulation. 2013 Sep 17;128(12):1381-6. doi: 10.1161/CIRCULATIONAHA.112.001009.Rigato I, Corrado D, Basso C, Zorzi A, Pilichou K, Bauce B,et al.Pharmacotherapy and other therapeutic modalities for managing Arrhythmogenic Right Ventricular Cardiomyopathy.Cardiovasc Drugs Ther. 2015 Apr;29(2):171-7. doi: 10.1007/s10557-015-6583-8.
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Maron BJ, Maron BA.Revisiting Athlete’s Heart Versus Pathologic Hypertrophy: ARVC and the Right Ventricle.JACC Cardiovasc Imaging. 2017 Apr;10(4):394-397. doi: 10.1016/j.jcmg.2016.05.011.Krahn AD, Wilde AAM, Calkins H, La Gerche A, Cadrin-Tourigny J, Roberts JD,et al.Arrhythmogenic Right Ventricular Cardiomyopathy.JACC Clin Electrophysiol. 2022 Apr;8(4):533-553. doi: 10.1016/j.jacep.2021.12.002.Bourfiss M, van Vugt M, Alasiri AI, Ruijsink B, van Setten J, Schmidt AF,et al.Prevalence and Disease Expression of Pathogenic and Likely Pathogenic Variants Associated With Inherited Cardiomyopathies in the General Population.Circ Genom Precis Med. 2022 Dec;15(6):e003704. doi: 10.1161/CIRCGEN.122.003704.Groeneweg JA, Bhonsale A, James CA, et al.Clinical presentation, long-term follow-up, and outcomes of 1001 arrhythmogenic right ventricular dysplasia/cardiomyopathy patients and family members.Circ Cardiovasc Genet. 2015 Mar;8(3):437-446. doi:10.1161/CIRCGENETICS.114.001003Johns Hopkins Medicine.Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C).Towbin JA, McKenna WJ, Abrams DJ, et al.2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy.Heart Rhythm. 2019 May;16(11):e301-e372. doi:10.1016/j.hrthm.2019.05.007Johns Hopkins Medicine.Genetics of Arrhythmogenic Right Ventricular Dysplasia / Cardiomyopathy (ARVDC).Al-Khatib SM, Stevenson WG, Ackerman MJ, et al.2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society.J Am Coll Cardiol. 2018 Oct;72(14):91-220. doi:10.1016/j.jacc.2017.10.054Al-Aidarous S, Protonotarios A, Elliott PM, Lambiase PD.Management of arrhythmogenic right ventricular cardiomyopathy.Heart. 2024 Jan 10;110(3):156-162. doi: 10.1136/heartjnl-2023-322612.Corrado D, Wichter T, Link MS, et al.Treatment of arrhythmogenic right ventricular cardiomyopathy/dysplasia: an international task force consensus statement.Eur Heart J. 2015 Dec;36(46):3227-3237. doi:10.1093/eurheartj/ehv162Aquaro GD, De Luca A, Cappelletto C, et al.Prognostic value of magnetic resonance phenotype in patients with arrhythmogenic right ventricular cardiomyopathy.Journal of the American College of Cardiology. 2020 Jun;75(22):2753-2765. doi:10.1016/j.jacc.2020.04.023Saguner AM, Duru F, Brunckhorst CB.Arrhythmogenic right ventricular cardiomyopathy: a challenging disease of the intercalated disc.Circulation. 2013 Sep 17;128(12):1381-6. doi: 10.1161/CIRCULATIONAHA.112.001009.Rigato I, Corrado D, Basso C, Zorzi A, Pilichou K, Bauce B,et al.Pharmacotherapy and other therapeutic modalities for managing Arrhythmogenic Right Ventricular Cardiomyopathy.Cardiovasc Drugs Ther. 2015 Apr;29(2):171-7. doi: 10.1007/s10557-015-6583-8.
Maron BJ, Maron BA.Revisiting Athlete’s Heart Versus Pathologic Hypertrophy: ARVC and the Right Ventricle.JACC Cardiovasc Imaging. 2017 Apr;10(4):394-397. doi: 10.1016/j.jcmg.2016.05.011.
Krahn AD, Wilde AAM, Calkins H, La Gerche A, Cadrin-Tourigny J, Roberts JD,et al.Arrhythmogenic Right Ventricular Cardiomyopathy.JACC Clin Electrophysiol. 2022 Apr;8(4):533-553. doi: 10.1016/j.jacep.2021.12.002.
Bourfiss M, van Vugt M, Alasiri AI, Ruijsink B, van Setten J, Schmidt AF,et al.Prevalence and Disease Expression of Pathogenic and Likely Pathogenic Variants Associated With Inherited Cardiomyopathies in the General Population.Circ Genom Precis Med. 2022 Dec;15(6):e003704. doi: 10.1161/CIRCGEN.122.003704.
Groeneweg JA, Bhonsale A, James CA, et al.Clinical presentation, long-term follow-up, and outcomes of 1001 arrhythmogenic right ventricular dysplasia/cardiomyopathy patients and family members.Circ Cardiovasc Genet. 2015 Mar;8(3):437-446. doi:10.1161/CIRCGENETICS.114.001003
Johns Hopkins Medicine.Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C).
Towbin JA, McKenna WJ, Abrams DJ, et al.2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy.Heart Rhythm. 2019 May;16(11):e301-e372. doi:10.1016/j.hrthm.2019.05.007
Johns Hopkins Medicine.Genetics of Arrhythmogenic Right Ventricular Dysplasia / Cardiomyopathy (ARVDC).
Al-Khatib SM, Stevenson WG, Ackerman MJ, et al.2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society.J Am Coll Cardiol. 2018 Oct;72(14):91-220. doi:10.1016/j.jacc.2017.10.054
Al-Aidarous S, Protonotarios A, Elliott PM, Lambiase PD.Management of arrhythmogenic right ventricular cardiomyopathy.Heart. 2024 Jan 10;110(3):156-162. doi: 10.1136/heartjnl-2023-322612.
Corrado D, Wichter T, Link MS, et al.Treatment of arrhythmogenic right ventricular cardiomyopathy/dysplasia: an international task force consensus statement.Eur Heart J. 2015 Dec;36(46):3227-3237. doi:10.1093/eurheartj/ehv162
Aquaro GD, De Luca A, Cappelletto C, et al.Prognostic value of magnetic resonance phenotype in patients with arrhythmogenic right ventricular cardiomyopathy.Journal of the American College of Cardiology. 2020 Jun;75(22):2753-2765. doi:10.1016/j.jacc.2020.04.023
Saguner AM, Duru F, Brunckhorst CB.Arrhythmogenic right ventricular cardiomyopathy: a challenging disease of the intercalated disc.Circulation. 2013 Sep 17;128(12):1381-6. doi: 10.1161/CIRCULATIONAHA.112.001009.
Rigato I, Corrado D, Basso C, Zorzi A, Pilichou K, Bauce B,et al.Pharmacotherapy and other therapeutic modalities for managing Arrhythmogenic Right Ventricular Cardiomyopathy.Cardiovasc Drugs Ther. 2015 Apr;29(2):171-7. doi: 10.1007/s10557-015-6583-8.
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