Cardiomyopathy-cataract-hip spine disease syndrome

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Overview

Cardiomyopathy-cataract-hip spine disease syndrome is an extremely rare genetic condition that affects several different parts of the body at the same time. The three main features of this syndrome are cardiomyopathy (a disease of the heart muscle that makes it harder for the heart to pump blood), cataracts (clouding of the lens in the eye that affects vision), and abnormalities of the hip joints and spine (skeletal problems). The cardiomyopathy in this condition is typically dilated cardiomyopathy, meaning the heart chambers become enlarged and weakened. Cataracts may develop early in life and can lead to significant vision problems if not treated. The hip and spine problems can include hip dysplasia, scoliosis, or other structural changes that may cause pain and difficulty with movement. Because this syndrome is so rare, with only a handful of families described in the medical literature, our understanding of the full range of symptoms and the best treatments is still limited. Treatment is mainly supportive and focuses on managing each individual symptom. Heart medications or devices may be needed for the cardiomyopathy, surgery can address cataracts, and orthopedic care can help with hip and spine issues. Regular monitoring by a team of specialists is important to catch and manage complications early.

Also known as:

Key symptoms:

Weakened or enlarged heart (dilated cardiomyopathy)Clouding of the eye lens (cataracts)Hip joint abnormalitiesSpine abnormalities such as scoliosis or kyphosisShortness of breathFatigue and low energyDifficulty with physical activity or exercise intoleranceBlurred or decreased visionJoint pain or stiffnessDifficulty walking or changes in gaitHeart rhythm problems

Clinical phenotype terms (14)— hover any for plain English
Pulmonary embolismHP:0002204Arterial thrombosisHP:0004420Abnormal intervertebral disk morphologyHP:0005108
Inheritance

Autosomal recessive

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

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Cardiomyopathy-cataract-hip spine disease syndrome.

View clinical trials →

No actively recruiting trials found for Cardiomyopathy-cataract-hip spine disease syndrome at this time.

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Search ClinicalTrials.gov ↗Join the Cardiomyopathy-cataract-hip spine disease syndrome community →

No specialists are currently listed for Cardiomyopathy-cataract-hip spine 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 Cardiomyopathy-cataract-hip spine disease syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Cardiomyopathy-cataract-hip spine 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

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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 the cardiomyopathy, and what is the current heart function?,What medications are recommended for the heart, and what side effects should I watch for?,When should cataract surgery be considered?,What type of physical activity is safe given the heart and skeletal problems?,Should genetic testing be done for other family members?,How often should follow-up appointments be scheduled with each specialist?,Are there any clinical trials or research studies we could participate in?

Common questions about Cardiomyopathy-cataract-hip spine disease syndrome

What is Cardiomyopathy-cataract-hip spine disease syndrome?

Cardiomyopathy-cataract-hip spine disease syndrome is an extremely rare genetic condition that affects several different parts of the body at the same time. The three main features of this syndrome are cardiomyopathy (a disease of the heart muscle that makes it harder for the heart to pump blood), cataracts (clouding of the lens in the eye that affects vision), and abnormalities of the hip joints and spine (skeletal problems). The cardiomyopathy in this condition is typically dilated cardiomyopathy, meaning the heart chambers become enlarged and weakened. Cataracts may develop early in life an

How is Cardiomyopathy-cataract-hip spine disease syndrome inherited?

Cardiomyopathy-cataract-hip spine disease syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.