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Core Information
Disease Name
Scimitar Syndrome
Standard Disease Name
scimitar syndrome
MeSH Tree
No data
ICD-10
Q26.8|Q26.2
Identifiers
DO ID
No data
MeSH ID
D012587
OMIM ID
106700|MTHU020140|MTHU019108|MTHU041722
UMLS ID
C0036400
HPO ID
No data
Description and Extensions
Description
NCI2016_02D:A rare congenital heart disorder in which all four pulmonary veins are not connected to the left atrium and drain into the systemic veins or the right atrium instead. Infants present with cyanosis, lethargy, tachypnea, respiratory infections, and poor growth.|NCI2016_02D:A rare congenital cardiopulmonary defect characterized by abnormal right-sided pulmonary venous drainage and right lung malformations.|MSH2017_2016_08_12:An anomalous pulmonary venous return in which the right PULMONARY VEIN is not connected to the LEFT ATRIUM but to the INFERIOR VENA CAVA. Scimitar syndrome is named for the crescent- or Turkish sword-like shadow in the chest radiography and is often associated with hypoplasia of the right lung and right pulmonary artery, and dextroposition of the heart.|HPO2016_07_04:Total anomalous pulmonary venous return refers to a congenital malformation in which all four pulmonary veins do not connect normally to the left atrium, but instead drain abnormally to the right atrium. [HPO:probinson]|HPO2016_07_04:Right pulmonary venous return to the inferior vena cava. [DDD:dbrown, pmid:16638549]|HPO2016_07_04:A developmental defect characterized by abnormal connection of or more pulmonary veins to the superior or inferior vena cava, the right atrium, or the coronary sinus, resulting in a left-to-right shunt of oxygenated blood. [HPO:probinson]
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