Inherited isolated arrhythmogenic cardiomyopathy, dominant-left variant

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ORPHA:293888OMIM:107970I42.8
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3Active trials8Treatment centers

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Overview

Inherited isolated arrhythmogenic cardiomyopathy, dominant-left variant (also called left-dominant arrhythmogenic cardiomyopathy or ALVC) is a rare genetic heart condition. In this disease, the normal heart muscle is gradually replaced by fatty or scar tissue, primarily affecting the left ventricle — the heart's main pumping chamber. This replacement of healthy tissue weakens the heart's ability to pump blood effectively and disrupts the electrical signals that keep the heart beating in a regular rhythm. The most common symptoms include irregular heartbeats (arrhythmias), palpitations, dizziness, fainting episodes, shortness of breath, and reduced exercise tolerance. In some cases, the first sign of the disease can be a dangerous heart rhythm disturbance, which is why early detection is so important. The condition typically becomes apparent in adolescence or adulthood, though the age of onset can vary. Treatment focuses on managing symptoms and preventing life-threatening arrhythmias. This may include medications such as beta-blockers or antiarrhythmic drugs, implantable cardioverter-defibrillators (ICDs) to correct dangerous heart rhythms, and in advanced cases, heart transplantation. Exercise restriction is commonly recommended because intense physical activity can worsen the disease. Genetic testing and regular cardiac screening of family members are important parts of managing this condition, since it runs in families.

Also known as:

Key symptoms:

Irregular heartbeat or palpitationsFainting or near-fainting episodesShortness of breath, especially during exerciseChest painDizziness or lightheadednessReduced ability to exerciseFatigue and low energySwelling in the legs or anklesHeart failure symptomsSudden cardiac arrest in severe casesFluttering sensation in the chest

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

3 events
Aug 2025Long-term Follow-up Study of Gene Therapy for Arrhythmogenic Cardiomyopathy Due to a Plakophilin-2 Pathogenic Variant

Lexeo Therapeutics

TrialENROLLING BY INVITATION
Mar 2025Targeted Therapy With Glycogen Synthase Kinase-3 Inhibition for Arrhythmogenic Cardiomyopathy

Hamilton Health Sciences Corporation — PHASE2

TrialRECRUITING
Feb 2024Gene Therapy for ACM Due to a PKP2 Pathogenic Variant

Lexeo Therapeutics — PHASE1, PHASE2

TrialACTIVE NOT RECRUITING

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-left variant.

3 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

3 recruitingView all trials with filters →
Phase 21 trial
Targeted Therapy With Glycogen Synthase Kinase-3 Inhibition for Arrhythmogenic Cardiomyopathy
Phase 2
Actively Recruiting
PI: Jason D Roberts, MD MAS (McMaster University and Population Health Research) · Sites: Calgary, Alberta; Vancouver, British Columbia +15 more · Age: 1899 yrs
Other1 trial
Long-term Follow-up Study of Gene Therapy for Arrhythmogenic Cardiomyopathy Due to a Plakophilin-2 Pathogenic Variant
Enrolling by Invitation
PI: Lexeo Clinical Trials (Lexeo Therapeutics) · Sites: Ann Arbor, Michigan · Age: 1867 yrs

No specialists are currently listed for Inherited isolated arrhythmogenic cardiomyopathy, dominant-left variant.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Inherited isolated arrhythmogenic cardiomyopathy, dominant-left variant.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Inherited isolated arrhythmogenic cardiomyopathy, dominant-left variant

1 articles
ResearchBIORXIVApr 25, 2026
Preprint: Fasting reverses PLN R14del-mediated cardiomyopathy through lysosomal reactivation
Scientists discovered that fasting may help reverse heart damage caused by a specific genetic mutation in the PLN gene called R14del. This mutation causes a com
See all news about Inherited isolated arrhythmogenic cardiomyopathy, dominant-left variant

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.What is my specific genetic mutation, and what does it mean for my prognosis?,Do I need an implantable 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, and how should screening be done?,How often do I need follow-up imaging and heart rhythm monitoring?,What symptoms should prompt me to seek emergency care immediately?,Are there any clinical trials or new treatments I should know about?

Common questions about Inherited isolated arrhythmogenic cardiomyopathy, dominant-left variant

What is Inherited isolated arrhythmogenic cardiomyopathy, dominant-left variant?

Inherited isolated arrhythmogenic cardiomyopathy, dominant-left variant (also called left-dominant arrhythmogenic cardiomyopathy or ALVC) is a rare genetic heart condition. In this disease, the normal heart muscle is gradually replaced by fatty or scar tissue, primarily affecting the left ventricle — the heart's main pumping chamber. This replacement of healthy tissue weakens the heart's ability to pump blood effectively and disrupts the electrical signals that keep the heart beating in a regular rhythm. The most common symptoms include irregular heartbeats (arrhythmias), palpitations, dizzin

How is Inherited isolated arrhythmogenic cardiomyopathy, dominant-left variant inherited?

Inherited isolated arrhythmogenic cardiomyopathy, dominant-left 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-left variant?

Yes — 3 recruiting clinical trials are currently listed for Inherited isolated arrhythmogenic cardiomyopathy, dominant-left variant on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.