Left ventricular noncompaction

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ORPHA:54260OMIM:615373I42.8
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1Active trials17Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Left ventricular noncompaction (LVNC), also called noncompaction cardiomyopathy or spongy myocardium, is a rare heart condition where the left ventricle — the main pumping chamber of the heart — does not develop properly. Normally, the heart muscle becomes smooth and compact before birth. In LVNC, part of the muscle stays spongy and trabeculated, meaning it has deep ridges and pockets instead of a firm, solid wall. This makes it harder for the heart to pump blood efficiently. The condition can cause a wide range of problems. Some people have few or no symptoms for years, while others develop serious heart failure, dangerous heart rhythm problems (arrhythmias), or blood clots that can travel to the brain and cause a stroke. Symptoms often include shortness of breath, extreme tiredness, swelling in the legs, and palpitations. In children, LVNC can cause poor growth and feeding difficulties. Treatment focuses on managing symptoms and preventing complications. Medications such as beta-blockers, ACE inhibitors, and blood thinners are commonly used. Some patients may need a pacemaker or implantable defibrillator. In severe cases, a heart transplant may be considered. There is currently no cure, but early diagnosis and careful management can significantly improve quality of life and outcomes.

Also known as:

Key symptoms:

Shortness of breath, especially during activity or when lying flatExtreme tiredness or fatigue even with mild exertionSwelling in the legs, ankles, or feetHeart palpitations or a racing, fluttering heartbeatDizziness or lightheadednessFainting or near-fainting episodesChest pain or tightnessStroke or mini-stroke symptoms such as sudden weakness or speech problemsPoor feeding and slow weight gain in infantsBluish skin color (cyanosis) in newborns or infantsReduced ability to exercise or keep up with peersIrregular heartbeat detected on an ECG

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

1 event
Sep 2023Predictors of Risk in Left Ventricular Non-Compaction

London Health Sciences Centre Research Institute OR Lawson Research Institute of St. Joseph's

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Left ventricular noncompaction.

1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

1 recruitingView all trials with filters →
Other1 trial
Predictors of Risk in Left Ventricular Non-Compaction
Actively Recruiting
· Sites: London, Ontario · Age: 1899 yrs

Specialists

17 foundView all specialists →
SF
Sanjay Sharma, MD FRCP FESC
Specialist
PI on 1 active trial
JR
José F Rodríguez-Palomares
Specialist
2 Left ventricular noncompaction publications
KH
Keiichi Hirono
Specialist
2 Left ventricular noncompaction publications
FM
Felix Tanner, Prof. MD
Specialist
PI on 1 active trial
BS
Ben Statton
Specialist
1 Left ventricular noncompaction publication
BB
Beatrice Boschi
Specialist
1 Left ventricular noncompaction publication
FG
Francesca Girolami
Specialist
1 Left ventricular noncompaction publication
AR
Angharad M Roberts
Specialist
1 Left ventricular noncompaction publication
EL
Elisabeth M Lodder
Specialist
1 Left ventricular noncompaction publication
MA
Mona Allouba
Specialist
1 Left ventricular noncompaction publication
SR
Soha Romeih
Specialist
1 Left ventricular noncompaction publication
YA
Yasmine Aguib
Specialist
1 Left ventricular noncompaction publication
KM
Kathryn A McGurk
TOLEDO, OH
Specialist
1 Left ventricular noncompaction publication
FM
Francesco Mazzarotto
Specialist
1 Left ventricular noncompaction publication
MB
Matteo Beltrami
Specialist
1 Left ventricular noncompaction publication
LB
Leander Beekman
Specialist
1 Left ventricular noncompaction publication
MH
Megan H Hawley
Specialist
1 Left ventricular noncompaction publication

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 Left ventricular noncompaction.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Left ventricular noncompaction

Disease timeline:

New recruiting trial: Predictors of Risk in Left Ventricular Non-Compaction

A new clinical trial is recruiting patients for Left ventricular noncompaction

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 my LVNC, and what does that mean for my daily life and long-term outlook?,Should I have genetic testing, and should my family members be screened even if they feel fine?,Do I need blood thinners to prevent a stroke, and what are the risks and benefits for me specifically?,Should I avoid certain types of exercise or physical activity, and are there activities that are safe?,Do I need an implantable defibrillator or pacemaker, and how will I know if that changes in the future?,How often should I have echocardiograms or cardiac MRIs to monitor my heart?,What symptoms should prompt me to go to the emergency room immediately?

Common questions about Left ventricular noncompaction

What is Left ventricular noncompaction?

Left ventricular noncompaction (LVNC), also called noncompaction cardiomyopathy or spongy myocardium, is a rare heart condition where the left ventricle — the main pumping chamber of the heart — does not develop properly. Normally, the heart muscle becomes smooth and compact before birth. In LVNC, part of the muscle stays spongy and trabeculated, meaning it has deep ridges and pockets instead of a firm, solid wall. This makes it harder for the heart to pump blood efficiently. The condition can cause a wide range of problems. Some people have few or no symptoms for years, while others develop

Are there clinical trials for Left ventricular noncompaction?

Yes — 1 recruiting clinical trial is currently listed for Left ventricular noncompaction on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Left ventricular noncompaction?

17 specialists and care centers treating Left ventricular noncompaction are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.