X-linked intellectual disability-cardiomegaly-congestive heart failure syndrome

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ORPHA:324410OMIM:300886Q87.8
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Overview

X-linked intellectual disability-cardiomegaly-congestive heart failure syndrome is an extremely rare genetic condition that primarily affects males. This disorder is characterized by a combination of intellectual disability (difficulty with learning, reasoning, and problem-solving) and serious heart problems, specifically cardiomegaly (an enlarged heart) and congestive heart failure (where the heart cannot pump blood effectively). Because the condition is X-linked, it is passed through families via the X chromosome, meaning mothers can be carriers while their sons are most often affected. The intellectual disability in this condition can range from mild to severe and is usually noticed in early childhood when developmental milestones such as speech and motor skills are delayed. The heart problems can develop during childhood or later and may progressively worsen over time. Congestive heart failure can cause symptoms like shortness of breath, fatigue, swelling in the legs and feet, and difficulty with physical activity. There is currently no cure for this syndrome. Treatment focuses on managing symptoms, particularly the heart-related complications. Medications commonly used for heart failure, such as ACE inhibitors, beta-blockers, and diuretics, may be prescribed. Educational support and therapies for intellectual disability, including speech therapy and occupational therapy, are also important parts of care. Because this condition is so rare, management is typically guided by a team of specialists working together.

Key symptoms:

Intellectual disabilityEnlarged heart (cardiomegaly)Congestive heart failureShortness of breathFatigue and low energySwelling in the legs, ankles, or feetDelayed speech developmentDelayed motor milestonesDifficulty with learningPoor exercise toleranceRapid or irregular heartbeatDifficulty gaining weight or poor growth

Clinical phenotype terms (30)— hover any for plain English
Contractures of the large jointsHP:0005781Abnormal atrioventricular valve morphologyHP:0006705
Inheritance

X-linked recessive

Carried on the X chromosome; typically affects males more than females

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for X-linked intellectual disability-cardiomegaly-congestive heart failure syndrome.

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No actively recruiting trials found for X-linked intellectual disability-cardiomegaly-congestive heart failure syndrome at this time.

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No specialists are currently listed for X-linked intellectual disability-cardiomegaly-congestive heart failure 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 X-linked intellectual disability-cardiomegaly-congestive heart failure 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.How severe is my child's heart enlargement, and what does this mean for their long-term health?,What medications will be needed, and what side effects should I watch for?,How often should heart function be monitored with echocardiograms?,What developmental therapies and educational supports do you recommend?,Are there any activity restrictions my child should follow?,Should other family members be tested for this condition or carrier status?,Are there any clinical trials or research studies we could participate in?

Common questions about X-linked intellectual disability-cardiomegaly-congestive heart failure syndrome

What is X-linked intellectual disability-cardiomegaly-congestive heart failure syndrome?

X-linked intellectual disability-cardiomegaly-congestive heart failure syndrome is an extremely rare genetic condition that primarily affects males. This disorder is characterized by a combination of intellectual disability (difficulty with learning, reasoning, and problem-solving) and serious heart problems, specifically cardiomegaly (an enlarged heart) and congestive heart failure (where the heart cannot pump blood effectively). Because the condition is X-linked, it is passed through families via the X chromosome, meaning mothers can be carriers while their sons are most often affected. The

How is X-linked intellectual disability-cardiomegaly-congestive heart failure syndrome inherited?

X-linked intellectual disability-cardiomegaly-congestive heart failure syndrome follows a x-linked recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does X-linked intellectual disability-cardiomegaly-congestive heart failure syndrome typically begin?

Typical onset of X-linked intellectual disability-cardiomegaly-congestive heart failure syndrome is childhood. Age of onset can vary across affected individuals.