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congenital total pulmonary venous return anomaly

Disease Summary
Associated Targets (1)
Tbio

1


Mondo Description Total pulmonary venous return (TAPVR) is a form of congenital pulmonary venous return where all of the pulmonary veins drain into the right atrium or one of its tributaries, instead of the left atrium, leading to various manifestations such as fatigue, exertional dyspnea, pulmonary arterial hypertension, cyanosis and progressive congestive heart failure.
Uniprot Description Rare congenital heart disease (CHD) in which the pulmonary veins fail to connect to the left atrium during cardiac development, draining instead into either the right atrium or one of its venous tributaries. This disease accounts for 1.5% of all CHDs and has a prevalence of approximately 1 out of 15'000 live births.
Mondo Term and Equivalent IDs
MONDO:0007130:  congenital total pulmonary venous return anomaly
COHD:432432: 
EFO:1001167: 
ICD9:747.41: 
NCIT:C98585: 
Orphanet:99125: 
SCTID:111323005: