Short stature-valvular heart disease-characteristic facies syndrome

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ORPHA:2868OMIM:126190Q87.1
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Overview

Short stature-valvular heart disease-characteristic facies syndrome is an extremely rare genetic condition that affects multiple parts of the body. People with this syndrome are notably shorter than average and have heart valve problems, which means the valves that control blood flow through the heart do not work properly. They also have distinctive facial features that doctors can recognize, sometimes described as a characteristic facial appearance. These facial features may include a broad forehead, widely spaced eyes, a flat nasal bridge, and other subtle differences in facial structure. Because the heart valves are affected, individuals may experience symptoms related to heart function such as heart murmurs, fatigue, or shortness of breath. The short stature is usually apparent from early childhood and may be one of the first signs that leads families to seek medical evaluation. Additional features can include skeletal abnormalities and other developmental differences. There is currently no cure for this syndrome. Treatment focuses on managing symptoms, particularly monitoring and treating heart valve problems, supporting growth, and addressing any other medical issues that arise. Regular follow-up with a team of specialists is important to ensure the best possible quality of life.

Key symptoms:

Short stature or growth delayHeart valve problems (valvular heart disease)Distinctive facial featuresBroad foreheadWidely spaced eyesFlat nasal bridgeHeart murmurShortness of breath with activitySkeletal abnormalitiesFatigue or low energyDelayed growth milestones

Clinical phenotype terms (14)— hover any for plain English
Large faceHP:0100729
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Short stature-valvular heart disease-characteristic facies syndrome.

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No actively recruiting trials found for Short stature-valvular heart disease-characteristic facies syndrome at this time.

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No specialists are currently listed for Short stature-valvular heart disease-characteristic facies syndrome.

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 Short stature-valvular heart disease-characteristic facies syndrome.

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Community

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Caregiver Resources

NORD Caregiver Resources

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Mental Health Support

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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 child's heart valve problem, and will it need surgery?,How often should we have cardiac check-ups and echocardiograms?,Are there any activity restrictions my child should follow?,Would growth hormone therapy be appropriate for my child?,Should we pursue genetic testing, and what type would be most helpful?,Are there any signs or symptoms I should watch for that would require emergency care?,Can you refer us to a genetic counselor to discuss family planning implications?

Common questions about Short stature-valvular heart disease-characteristic facies syndrome

What is Short stature-valvular heart disease-characteristic facies syndrome?

Short stature-valvular heart disease-characteristic facies syndrome is an extremely rare genetic condition that affects multiple parts of the body. People with this syndrome are notably shorter than average and have heart valve problems, which means the valves that control blood flow through the heart do not work properly. They also have distinctive facial features that doctors can recognize, sometimes described as a characteristic facial appearance. These facial features may include a broad forehead, widely spaced eyes, a flat nasal bridge, and other subtle differences in facial structure. B

How is Short stature-valvular heart disease-characteristic facies syndrome inherited?

Short stature-valvular heart disease-characteristic facies syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Short stature-valvular heart disease-characteristic facies syndrome typically begin?

Typical onset of Short stature-valvular heart disease-characteristic facies syndrome is childhood. Age of onset can vary across affected individuals.