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Start free with EleplanFamilial idiopathic dilatation of the right atrium
ORPHA:1677Morphological anomaly
What it is
A rare congenital heart malformation of unknown etiology that is characterized by an extremely dilated right atrium, and that is usually asymptomatic and fortuitously discovered by echocardiography or chest radiography, and can be sometimes associated with other anomalies such as atrial arrhythmias (e.g. atrial flutter, atrial fibrillation, supraventricular tachycardia), severe tricuspid regurgitation, or atrial thrombus that could lead to potentially life-threatening thromboembolic complications.
Key facts
- Age of onset
- All ages
- Inheritance
- Unknown
- Classified as
- Morphological anomaly
Signs and symptoms
Common30–79%
16- Abnormal cardiac ventricular function
- Abnormality of the hepatic vasculature
- Abnormal vena cava morphology
- Arrhythmia
- Atrial fibrillation
- Atrioventricular block
- Cardiomegaly
- Complete heart block with narrow QRS complexes
- Dyspnea
- Holosystolic murmur
- Midsystolic murmur
- Palpitations
- Reduced left ventricular ejection fraction
- Right ventricular hypertrophy
- Syncope
- Tricuspid regurgitation
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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