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absence of the pulmonary artery

Go to external page http://purl.obolibrary.org/obo/MONDO_0020007

A rare vascular anomaly characterized by congenital absence of the right or left pulmonary artery, usually ending within 2 cm of its expected origin from the pulmonary trunk. Patients often also have other cardiovascular abnormalities and respective symptoms and are then typically diagnosed in infancy or childhood, while isolated cases generally present with a mild clinical course and may go undiagnosed until adulthood. Presenting clinical features in isolated cases include hemoptysis, exertional dyspnea, and recurrent respiratory infections. The condition is typically accompanied by marked changes of lung tissue and may, if unrecognized, result in massive hemoptysis and pulmonary hypertension. [ Orphanet:980 ]

Synonyms: pulmonary artery agenesis unilateral pulmonary artery agenesis aplasia of pulmonary artery agenesis of pulmonary artery unilateral pulmonary artery absence UAPA pulmonary artery absent

This is just here as a test because I lose it

Term information

database cross reference
  • Orphanet:980 (MONDO:equivalentTo)
  • NANDO:2200282 (https://orcid.org/0000-0003-0011-764X)
  • MEDGEN:120560 (MONDO:equivalentTo)
  • GARD:18712 (MONDO:GARD)
  • SCTID:86252004 (MONDO:equivalentTo)
  • UMLS:C0265905 (MONDO:equivalentTo)
Subsets

gard_rare, ordo_disorder, rare, ordo_morphological_anomaly, nord_rare, orphanet_rare

abbreviation
UAPA [ Orphanet:980 ]

exactMatch

http://identifiers.org/medgen/120560

http://identifiers.org/snomedct/86252004

http://linkedlifedata.com/resource/umls/id/C0265905

http://www.orpha.net/ORDO/Orphanet_980

has related synonym

congenital absence of pulmonary artery

id

MONDO:0020007

term tracker item

https://github.com/monarch-initiative/mondo/issues/6876

https://github.com/monarch-initiative/mondo/issues/7171

https://github.com/monarch-initiative/mondo/issues/6740