Overview
Familial idiopathic dilatation of the right atrium (also called familial right atrial enlargement or isolated right atrial dilatation) is a rare heart condition where the right upper chamber of the heart becomes abnormally enlarged without a clear underlying cause like a valve problem or lung disease. 'Familial' means it tends to run in families, suggesting a genetic cause, though the exact gene responsible has not been fully identified in all cases. 'Idiopathic' simply means the enlargement happens on its own, not as a result of another known heart or lung condition. The right atrium is the chamber that receives blood returning from the body and sends it to the right ventricle, which then pumps it to the lungs. When this chamber is stretched and enlarged, it can disrupt the heart's normal electrical system, leading to irregular heartbeats (arrhythmias). Some people have no symptoms at all and are diagnosed by chance, while others experience palpitations, shortness of breath, fatigue, or even fainting. Treatment focuses on managing symptoms, especially abnormal heart rhythms. Medications to control heart rate or rhythm, blood thinners to prevent clots, and in some cases procedures like catheter ablation may be used. In severe cases, surgery to reduce the size of the right atrium has been performed. Regular monitoring by a cardiologist is essential, as the condition can progress over time.
Key symptoms:
Heart palpitations or fluttering in the chestIrregular heartbeat (arrhythmia)Shortness of breath, especially during activityFatigue or unusual tirednessDizziness or lightheadednessFainting or near-fainting episodesSwelling in the legs or anklesChest discomfort or pressureReduced ability to exercise
Clinical phenotype terms (24)— hover any for plain English
Autosomal dominant
Passed on from just one parent; each child has about a 50% chance of inheriting it
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Familial idiopathic dilatation of the right atrium.
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Specialists
View all specialists →No specialists are currently listed for Familial idiopathic dilatation of the right atrium.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Familial idiopathic dilatation of the right atrium.
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Caregiver Resources
NORD Caregiver Resources
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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 enlarged is my right atrium, and how does that compare to a normal heart?,What type of heart rhythm problems am I at risk for, and how will we monitor for them?,Do I need blood thinners, and what are the risks and benefits for me specifically?,Should my close family members (parents, siblings, children) be tested or screened?,Are there any activities or sports I should avoid?,What warning signs should prompt me to go to the emergency room immediately?,Is genetic testing available for my family, and should I see a clinical geneticist?
Common questions about Familial idiopathic dilatation of the right atrium
What is Familial idiopathic dilatation of the right atrium?
Familial idiopathic dilatation of the right atrium (also called familial right atrial enlargement or isolated right atrial dilatation) is a rare heart condition where the right upper chamber of the heart becomes abnormally enlarged without a clear underlying cause like a valve problem or lung disease. 'Familial' means it tends to run in families, suggesting a genetic cause, though the exact gene responsible has not been fully identified in all cases. 'Idiopathic' simply means the enlargement happens on its own, not as a result of another known heart or lung condition. The right atrium is the
How is Familial idiopathic dilatation of the right atrium inherited?
Familial idiopathic dilatation of the right atrium follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.