Shoulder and thorax deformity-congenital heart disease syndrome

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ORPHA:1940
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Overview

Shoulder and thorax deformity-congenital heart disease syndrome is an extremely rare condition that combines skeletal abnormalities of the shoulder and chest area with heart defects that are present from birth. This syndrome is sometimes referred to by its Orphanet designation (ORPHA:1940) and has been described in only a very small number of individuals in the medical literature. Affected individuals typically show abnormal development of the shoulder bones (such as the scapula and clavicle), chest wall deformities (like an unusually shaped ribcage or sternum), and one or more structural heart problems that develop before birth. The heart defects can range in severity and may include holes between heart chambers or abnormalities of the heart valves. Because so few cases have been reported, the full spectrum of features and the underlying cause of this syndrome are not yet well understood. Treatment is generally focused on managing symptoms, particularly addressing the heart defects, which may require surgical correction, and monitoring skeletal development. Orthopedic care may be needed for shoulder and chest abnormalities that affect function or breathing. Genetic counseling is recommended for affected families to better understand recurrence risks.

Key symptoms:

Abnormally shaped or positioned shoulder bladesChest wall deformityCongenital heart defectAbnormal collarbone developmentRib abnormalitiesLimited shoulder movementBreathing difficulties in some casesAsymmetry of the upper bodyHeart murmur detected at birthPossible feeding difficulties in infancy

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Shoulder and thorax deformity-congenital heart disease syndrome.

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No actively recruiting trials found for Shoulder and thorax deformity-congenital heart disease syndrome at this time.

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No specialists are currently listed for Shoulder and thorax deformity-congenital heart disease 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 Shoulder and thorax deformity-congenital heart disease syndrome.

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Community

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Latest news about Shoulder and thorax deformity-congenital heart disease syndrome

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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.What type of heart defect does my child have, and how severe is it?,Will my child need heart surgery, and if so, when?,How will the shoulder and chest abnormalities affect my child's growth and development?,Are there any activity restrictions my child should follow?,Should we pursue genetic testing to look for an underlying cause?,What is the chance this condition could occur again in future pregnancies?,What specialists should be part of my child's care team?

Common questions about Shoulder and thorax deformity-congenital heart disease syndrome

What is Shoulder and thorax deformity-congenital heart disease syndrome?

Shoulder and thorax deformity-congenital heart disease syndrome is an extremely rare condition that combines skeletal abnormalities of the shoulder and chest area with heart defects that are present from birth. This syndrome is sometimes referred to by its Orphanet designation (ORPHA:1940) and has been described in only a very small number of individuals in the medical literature. Affected individuals typically show abnormal development of the shoulder bones (such as the scapula and clavicle), chest wall deformities (like an unusually shaped ribcage or sternum), and one or more structural hear

At what age does Shoulder and thorax deformity-congenital heart disease syndrome typically begin?

Typical onset of Shoulder and thorax deformity-congenital heart disease syndrome is neonatal. Age of onset can vary across affected individuals.