Cardiac anomalies-short stature-joint hypermobility-facial dysmorphism syndrome

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Overview

Cardiac anomalies-short stature-joint hypermobility-facial dysmorphism syndrome is an extremely rare genetic condition that affects multiple body systems. As the name describes, people with this syndrome typically have heart (cardiac) abnormalities, shorter-than-expected height, unusually flexible joints (joint hypermobility), and distinctive facial features (facial dysmorphism). The heart problems can range from structural defects present at birth, such as septal defects (holes between heart chambers), to other congenital cardiac malformations. The facial features may include a broad forehead, widely spaced eyes, a flat nasal bridge, and other subtle differences. Joint hypermobility means that joints can bend further than normal, which may lead to joint pain, dislocations, or instability over time. Short stature is typically noticed during childhood when growth falls below expected ranges. Because this syndrome is so rare, the treatment approach is mainly supportive and symptom-based. Heart defects may require surgical correction or ongoing monitoring by a cardiologist. Growth may be evaluated by an endocrinologist, and joint problems can be managed with physical therapy and supportive measures. Early diagnosis and coordinated care from multiple specialists are important to optimize outcomes for affected individuals.

Also known as:

Key symptoms:

Heart defects present at birthShort stature or poor growthOverly flexible or loose jointsDistinctive facial featuresBroad foreheadWidely spaced eyesFlat nasal bridgeJoint pain or instabilityDelayed growth milestonesPossible developmental delaysLow muscle toneFrequent joint dislocations or subluxations

Clinical phenotype terms (23)— hover any for plain English
Sudden deathHP:0001699
Inheritance

Autosomal dominant

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

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Cardiac anomalies-short stature-joint hypermobility-facial dysmorphism syndrome.

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No actively recruiting trials found for Cardiac anomalies-short stature-joint hypermobility-facial dysmorphism syndrome at this time.

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No specialists are currently listed for Cardiac anomalies-short stature-joint hypermobility-facial dysmorphism 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 Cardiac anomalies-short stature-joint hypermobility-facial dysmorphism syndrome.

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Community

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Caregiver Resources

NORD Caregiver Resources

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.What specific heart abnormalities does my child have, and do they need surgical treatment?,How often should we have cardiac check-ups and echocardiograms?,Is genetic testing recommended for our family, and what might the results tell us?,Would growth hormone therapy be appropriate for my child's short stature?,What physical activities are safe given the joint hypermobility and heart condition?,Are there any signs or symptoms I should watch for that would require emergency care?,Can you refer us to a physical therapist experienced with joint hypermobility?

Common questions about Cardiac anomalies-short stature-joint hypermobility-facial dysmorphism syndrome

What is Cardiac anomalies-short stature-joint hypermobility-facial dysmorphism syndrome?

Cardiac anomalies-short stature-joint hypermobility-facial dysmorphism syndrome is an extremely rare genetic condition that affects multiple body systems. As the name describes, people with this syndrome typically have heart (cardiac) abnormalities, shorter-than-expected height, unusually flexible joints (joint hypermobility), and distinctive facial features (facial dysmorphism). The heart problems can range from structural defects present at birth, such as septal defects (holes between heart chambers), to other congenital cardiac malformations. The facial features may include a broad forehead

How is Cardiac anomalies-short stature-joint hypermobility-facial dysmorphism syndrome inherited?

Cardiac anomalies-short stature-joint hypermobility-facial dysmorphism syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Cardiac anomalies-short stature-joint hypermobility-facial dysmorphism syndrome typically begin?

Typical onset of Cardiac anomalies-short stature-joint hypermobility-facial dysmorphism syndrome is neonatal. Age of onset can vary across affected individuals.