Overview
Inherited isolated arrhythmogenic ventricular dysplasia, biventricular variant (also called biventricular arrhythmogenic cardiomyopathy or biventricular ARVC/ARVD) is a rare inherited heart condition in which the normal heart muscle in both the left and right ventricles (the two main pumping chambers) is gradually replaced by fatty or scar tissue. This replacement disrupts the heart's electrical system and weakens its ability to pump blood effectively. Unlike the more common right-dominant form of arrhythmogenic right ventricular cardiomyopathy (ARVC), the biventricular variant affects both sides of the heart, which can lead to more significant heart failure symptoms. The hallmark symptoms include abnormal heart rhythms (arrhythmias), which may cause palpitations, dizziness, fainting (syncope), and in severe cases, sudden cardiac arrest. Over time, as more heart muscle is replaced, patients may develop heart failure symptoms such as shortness of breath, swelling in the legs, and fatigue. Symptoms often first appear during adolescence or young adulthood, though the age of onset can vary. Treatment focuses on managing arrhythmias and preventing sudden cardiac death. This may include medications such as beta-blockers or antiarrhythmic drugs, implantable cardioverter-defibrillators (ICDs), and in advanced cases, heart transplantation. Patients are typically advised to avoid intense physical exercise, as vigorous activity can trigger dangerous heart rhythms and accelerate disease progression. Genetic counseling is recommended for affected families.
Key symptoms:
Heart palpitations or feeling like the heart is racing or skipping beatsFainting or near-fainting episodesDizziness or lightheadednessShortness of breath, especially during activityChest pain or discomfortUnusual fatigue or low energySwelling in the legs, ankles, or feetDifficulty exercising or reduced exercise toleranceIrregular heartbeat detected on ECGHeart failure symptomsSudden cardiac arrest (in severe cases)
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
1 eventCordarone: FDA approved
For initiation of treatment and prophylaxis of frequently recurring ventricular fibrillation and hemodynamically unstable ventricular tachycardia in patients refractory to other therapy.
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
1 availableCordarone
For initiation of treatment and prophylaxis of frequently recurring ventricular fibrillation and hemodynamically unstable ventricular tachycardia in patients refractory to other therapy.
Clinical Trials
View all trials with filters →No actively recruiting trials found for Inherited isolated arrhythmogenic ventricular dysplasia, biventricular variant at this time.
New trials open frequently. Follow this disease to get notified.
Specialists
View all specialists →No specialists are currently listed for Inherited isolated arrhythmogenic ventricular dysplasia, biventricular 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 ventricular dysplasia, biventricular variant.
Community
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.How severe is the involvement of both ventricles in my case, and what does that mean for my outlook?,Do I need an implantable cardioverter-defibrillator (ICD), and what are the risks and benefits?,What specific activities and exercise levels are safe for me?,Should my family members be tested, and what genetic testing do you recommend?,What medications will I need, and what side effects should I watch for?,How often will I need follow-up imaging and heart rhythm monitoring?,Are there any clinical trials or new treatments I should know about?
Common questions about Inherited isolated arrhythmogenic ventricular dysplasia, biventricular variant
What is Inherited isolated arrhythmogenic ventricular dysplasia, biventricular variant?
Inherited isolated arrhythmogenic ventricular dysplasia, biventricular variant (also called biventricular arrhythmogenic cardiomyopathy or biventricular ARVC/ARVD) is a rare inherited heart condition in which the normal heart muscle in both the left and right ventricles (the two main pumping chambers) is gradually replaced by fatty or scar tissue. This replacement disrupts the heart's electrical system and weakens its ability to pump blood effectively. Unlike the more common right-dominant form of arrhythmogenic right ventricular cardiomyopathy (ARVC), the biventricular variant affects both si
How is Inherited isolated arrhythmogenic ventricular dysplasia, biventricular variant inherited?
Inherited isolated arrhythmogenic ventricular dysplasia, biventricular variant follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
What treatment and support options exist for Inherited isolated arrhythmogenic ventricular dysplasia, biventricular variant?
1 patient support program are currently tracked on UniteRare for Inherited isolated arrhythmogenic ventricular dysplasia, biventricular variant. See the treatments and support programs sections for copay assistance, eligibility, and contact details.