Chronic atrial and intestinal dysrhythmia syndrome

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ORPHA:435988OMIM:616201K59.8
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2Specialists8Treatment centers

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Overview

Chronic atrial and intestinal dysrhythmia syndrome (CAID) is an extremely rare genetic condition that affects both the heart and the digestive system at the same time. The name describes its two main features: abnormal heart rhythms (atrial dysrhythmia, often called sick sinus syndrome) and abnormal movement of the intestines (intestinal dysrhythmia or chronic intestinal pseudo-obstruction). In people with CAID, the heart's natural pacemaker does not work properly, leading to a slow or irregular heartbeat. At the same time, the muscles of the intestines do not contract in the normal coordinated way, which causes symptoms that look like a bowel blockage even though there is no physical obstruction. This combination of heart and gut problems typically appears in childhood and can significantly affect quality of life. Many patients require a permanent cardiac pacemaker to manage their heart rhythm problems. The intestinal symptoms can be very challenging to treat and may require specialized nutritional support, medications to help gut motility, and sometimes surgical interventions. Because CAID is so rare, treatment is largely supportive and tailored to each patient's specific symptoms. The condition was first described in recent years, and research is still ongoing to better understand its full spectrum and develop more targeted therapies.

Also known as:

Key symptoms:

Slow or irregular heartbeat (sick sinus syndrome)Severe bloating and abdominal distensionChronic constipationNausea and vomitingAbdominal pain and crampingEpisodes that mimic bowel obstruction without a physical blockagePoor appetiteWeight loss or failure to thrive in childrenFatigue and low energyDizziness or fainting from slow heart rateDifficulty tolerating normal mealsMalnutrition

Inheritance

Autosomal recessive

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

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Chronic atrial and intestinal dysrhythmia syndrome.

View clinical trials →

No actively recruiting trials found for Chronic atrial and intestinal dysrhythmia syndrome at this time.

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Specialists

2 foundView all specialists →
CM
Clair A Francomano, MD
INDIANAPOLIS, IN
Specialist
PI on 1 active trial

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 Chronic atrial and intestinal dysrhythmia syndrome.

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Community

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Latest news about Chronic atrial and intestinal dysrhythmia 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.Does my child (or do I) need a pacemaker, and when should it be placed?,What dietary changes can help manage the intestinal symptoms?,Are there medications that can improve gut motility in this condition?,How often should heart rhythm and pacemaker function be monitored?,What are the signs of a pseudo-obstruction crisis, and what should I do if one occurs?,Is genetic counseling recommended for our family, and should other family members be tested?,Are there any clinical trials or new treatments being studied for CAID?

Common questions about Chronic atrial and intestinal dysrhythmia syndrome

What is Chronic atrial and intestinal dysrhythmia syndrome?

Chronic atrial and intestinal dysrhythmia syndrome (CAID) is an extremely rare genetic condition that affects both the heart and the digestive system at the same time. The name describes its two main features: abnormal heart rhythms (atrial dysrhythmia, often called sick sinus syndrome) and abnormal movement of the intestines (intestinal dysrhythmia or chronic intestinal pseudo-obstruction). In people with CAID, the heart's natural pacemaker does not work properly, leading to a slow or irregular heartbeat. At the same time, the muscles of the intestines do not contract in the normal coordinate

How is Chronic atrial and intestinal dysrhythmia syndrome inherited?

Chronic atrial and intestinal dysrhythmia syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Chronic atrial and intestinal dysrhythmia syndrome typically begin?

Typical onset of Chronic atrial and intestinal dysrhythmia syndrome is childhood. Age of onset can vary across affected individuals.

Which specialists treat Chronic atrial and intestinal dysrhythmia syndrome?

2 specialists and care centers treating Chronic atrial and intestinal dysrhythmia syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.