Overview
Inherited isolated arrhythmogenic cardiomyopathy, dominant-right variant (also commonly known as arrhythmogenic right ventricular cardiomyopathy or ARVC, and sometimes called arrhythmogenic right ventricular dysplasia or ARVD) is a genetic heart condition in which the muscle of the right side of the heart is gradually replaced by fatty and scar tissue. This weakens the heart wall and disrupts the normal electrical signals that keep the heart beating in a regular rhythm. As a result, people with this condition are prone to dangerous irregular heartbeats (arrhythmias), especially during exercise or physical exertion. Symptoms often begin in adolescence or early adulthood and can include palpitations (a feeling that the heart is racing or skipping beats), fainting spells, dizziness, and shortness of breath. In some cases, the first sign of the disease can be a sudden cardiac arrest, which is why early diagnosis is so important. Over time, the right ventricle may become enlarged and weakened, and in some patients the left ventricle can also be affected, leading to heart failure. Treatment focuses on managing symptoms and preventing life-threatening arrhythmias. This may include medications such as beta-blockers or antiarrhythmic drugs, lifestyle modifications including exercise restriction, and in many cases the implantation of an implantable cardioverter-defibrillator (ICD) to detect and correct dangerous heart rhythms. Catheter ablation may be used to treat recurrent arrhythmias. In advanced cases with severe heart failure, heart transplantation may be considered. There is currently no cure, but with proper management many patients can live fulfilling lives.
Also known as:
Key symptoms:
Heart palpitations or feeling like the heart is racingFainting or near-fainting episodesDizziness or lightheadednessShortness of breath, especially during exerciseChest pain or discomfortUnusual fatigue or tirednessSwelling in the legs or anklesIrregular heartbeat (arrhythmia)Exercise intolerance or reduced ability to exerciseSudden cardiac arrestHeart failure symptoms in advanced stagesFluttering sensation in the chest
Autosomal dominant
Passed on from just one parent; each child has about a 50% chance of inheriting it
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
4 eventsHospices Civils de Lyon — PHASE2
Tenaya Therapeutics — PHASE1
University Hospital, Toulouse — NA
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Inherited isolated arrhythmogenic cardiomyopathy, dominant-right variant.
4 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Specialists
View all specialists →No specialists are currently listed for Inherited isolated arrhythmogenic cardiomyopathy, dominant-right variant.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Inherited isolated arrhythmogenic cardiomyopathy, dominant-right variant.
Community
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Start the conversation →Latest news about Inherited isolated arrhythmogenic cardiomyopathy, dominant-right variant
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Questions for your doctor
Bring these to your next appointment
- Q1.What specific genetic mutation do I have, and how does it affect my prognosis?,Do I need an implantable cardioverter-defibrillator (ICD), and what are the benefits and risks?,What types and levels of physical activity are safe for me?,Should my family members be tested for this condition, and at what age should screening begin?,What medications are best for my situation, and what side effects should I watch for?,How often do I need follow-up appointments and imaging tests?,Are there any clinical trials or new treatments I should know about?
Common questions about Inherited isolated arrhythmogenic cardiomyopathy, dominant-right variant
What is Inherited isolated arrhythmogenic cardiomyopathy, dominant-right variant?
Inherited isolated arrhythmogenic cardiomyopathy, dominant-right variant (also commonly known as arrhythmogenic right ventricular cardiomyopathy or ARVC, and sometimes called arrhythmogenic right ventricular dysplasia or ARVD) is a genetic heart condition in which the muscle of the right side of the heart is gradually replaced by fatty and scar tissue. This weakens the heart wall and disrupts the normal electrical signals that keep the heart beating in a regular rhythm. As a result, people with this condition are prone to dangerous irregular heartbeats (arrhythmias), especially during exercise
How is Inherited isolated arrhythmogenic cardiomyopathy, dominant-right variant inherited?
Inherited isolated arrhythmogenic cardiomyopathy, dominant-right variant follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Are there clinical trials for Inherited isolated arrhythmogenic cardiomyopathy, dominant-right variant?
Yes — 4 recruiting clinical trials are currently listed for Inherited isolated arrhythmogenic cardiomyopathy, dominant-right variant on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.