loading...| Preferred Name | Right atrial isomerism | |
| Synonyms |
RAI |
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| Definitions |
(RAI) - A severe complex congenital heart defect resulting from embryonic disruption of proper left-right axis determination. RAI is usually characterized by complete atrioventricular septal defect with a common atrium and univentricular AV connection, total anomalous pulmonary drainage, and transposition or malposition of the great arteries. Affected individuals present at birth with severe cardiac failure. Other associated abnormalities include bilateral trilobed lungs, midline liver, and asplenia, as well as situs inversus affecting other organs. |
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| ID |
http://identifiers.org/omim/208530 |
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| altLabel |
RAI |
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| definition |
(RAI) - A severe complex congenital heart defect resulting from embryonic disruption of proper left-right axis determination. RAI is usually characterized by complete atrioventricular septal defect with a common atrium and univentricular AV connection, total anomalous pulmonary drainage, and transposition or malposition of the great arteries. Affected individuals present at birth with severe cardiac failure. Other associated abnormalities include bilateral trilobed lungs, midline liver, and asplenia, as well as situs inversus affecting other organs. |
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| id |
OMIM:208530 |
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| notation |
OMIM:208530 |
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| prefLabel |
Right atrial isomerism |
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| subClassOf |