Orphanet Rare Disease Ontology

Last uploaded: July 3, 2024
Preferred Name

Pulmonary artery hypoplasia

Synonyms

PAH

Unilateral Pulmonary Artery Hypoplasia

Definitions

A rare, congenital anomaly of the great arteries characterized by various clinical signs and symptoms, including shortness of breath, recurrent lower respiratory tract infections, lung hypoplasia, pulmonary hypertension, and haemoptysis. The anomaly can be isolated or associated with congenital heart disease, such as tetralogy of Fallot, atrial septal defect, coarctation of the aorta, right aortic arch, truncus arteriosus, patent ductus arteriosus and pulmonary atresia.

ID

http://www.orpha.net/ORDO/Orphanet_99083

alternative_term

PAH

Unilateral Pulmonary Artery Hypoplasia

definition

A rare, congenital anomaly of the great arteries characterized by various clinical signs and symptoms, including shortness of breath, recurrent lower respiratory tract infections, lung hypoplasia, pulmonary hypertension, and haemoptysis. The anomaly can be isolated or associated with congenital heart disease, such as tetralogy of Fallot, atrial septal defect, coarctation of the aorta, right aortic arch, truncus arteriosus, patent ductus arteriosus and pulmonary atresia.

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=99083

hasDbXref

UMLS:C0265910

ICD-11:LA8B.1

ICD-10:Q25.7

label

Pulmonary artery hypoplasia

notation

ORPHA:99083

part_of

http://www.orpha.net/ORDO/Orphanet_182111

http://www.orpha.net/ORDO/Orphanet_98719

prefixIRI

ORDO:Orphanet_99083

prefLabel

Pulmonary artery hypoplasia

treeView

http://www.orpha.net/ORDO/Orphanet_182111

http://www.orpha.net/ORDO/Orphanet_98719

subClassOf

http://www.orpha.net/ORDO/Orphanet_557493

http://www.orpha.net/ORDO/Orphanet_377791

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