Congenital heart defect-round face-developmental delay syndrome

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:1355OMIM:270460Q87.8
Who is this for?
Show terms as
8Treatment centers

Where are you in your journey?

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

Overview

Congenital heart defect-round face-developmental delay syndrome is an extremely rare genetic condition that affects multiple body systems from birth. It is sometimes referred to by its Orphanet designation (ORPHA:1355). The hallmark features of this syndrome include heart defects present at birth (congenital heart defects), a distinctive round facial appearance, and delays in reaching developmental milestones such as sitting, walking, and talking. Children with this condition may also have intellectual disability of varying degrees, short stature, and other physical features that can help doctors recognize the syndrome. Because this syndrome is so rare, only a handful of cases have been described in the medical literature. The heart defects can range from mild to severe and may include problems with the heart valves or the walls separating the heart chambers. The round face is often noticeable in early childhood and may be accompanied by other subtle facial features such as a flat nasal bridge or widely spaced eyes. Treatment is focused on managing individual symptoms. Heart defects may require surgical repair or ongoing monitoring by a cardiologist. Developmental delays are typically addressed through early intervention programs including speech therapy, occupational therapy, and physical therapy. There is currently no cure or disease-specific treatment, so care is supportive and tailored to each patient's needs. A team of specialists working together provides the best outcomes for affected individuals.

Also known as:

Key symptoms:

Heart defects present at birthRound faceDevelopmental delayIntellectual disabilityShort statureFlat nasal bridgeWidely spaced eyesLow muscle toneDelayed speech developmentDelayed motor milestones such as walkingFeeding difficulties in infancySmall head size

Clinical phenotype terms (12)— hover any for plain English
Abnormal oral cavity morphologyHP:0000163Generalized hyperpigmentationHP:0007440
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Congenital heart defect-round face-developmental delay syndrome.

View clinical trials →

No actively recruiting trials found for Congenital heart defect-round face-developmental delay syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Congenital heart defect-round face-developmental delay syndrome community →

No specialists are currently listed for Congenital heart defect-round face-developmental delay 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 Congenital heart defect-round face-developmental delay syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Congenital heart defect-round face-developmental delay syndromeForum →

No community posts yet. Be the first to share your experience with Congenital heart defect-round face-developmental delay syndrome.

Start the conversation →

Latest news about Congenital heart defect-round face-developmental delay syndrome

No recent news articles for Congenital heart defect-round face-developmental delay syndrome.

Follow this condition to be notified when news becomes available.

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 will it need surgery?,What developmental therapies should we start, and how often?,Should we pursue genetic testing, and what kind would be most helpful?,Are there any activity restrictions my child should follow?,How often should my child see a cardiologist for follow-up?,What signs or symptoms should prompt me to seek emergency care?,Are there any other specialists my child should see?

Common questions about Congenital heart defect-round face-developmental delay syndrome

What is Congenital heart defect-round face-developmental delay syndrome?

Congenital heart defect-round face-developmental delay syndrome is an extremely rare genetic condition that affects multiple body systems from birth. It is sometimes referred to by its Orphanet designation (ORPHA:1355). The hallmark features of this syndrome include heart defects present at birth (congenital heart defects), a distinctive round facial appearance, and delays in reaching developmental milestones such as sitting, walking, and talking. Children with this condition may also have intellectual disability of varying degrees, short stature, and other physical features that can help doct

At what age does Congenital heart defect-round face-developmental delay syndrome typically begin?

Typical onset of Congenital heart defect-round face-developmental delay syndrome is neonatal. Age of onset can vary across affected individuals.