pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis

Go to external page http://purl.obolibrary.org/obo/MONDO_0018554


A rare subgroup of pulmonary arterial hypertension (PAH) characterized by obliterative fibrosis of the small pulmonary veins and venules and/or capillary infiltration of the pulmonary interstitium leading to increased pulmonary vascular resistance and right ventricular dysfunction. [ Orphanet:431353 https://orcid.org/0000-0001-5208-3432 ]

Synonyms: pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis PVOD and/or PCH

This is just here as a test because I lose it

Term information

database cross reference
  • EFO:0009199 (MONDO:equivalentTo)
  • Orphanet:431353 (MONDO:equivalentTo)
  • GARD:21801 (Orphanet:431353)
Subsets

gard_rare, disease_grouping, rare, nord_rare, orphanet_rare, clingen, ordo_group_of_disorders

IAO 0000233

https://github.com/monarch-initiative/mondo/issues/5781

exactMatch

http://www.orpha.net/ORDO/Orphanet_431353

id

MONDO:0018554

seeAlso

https://search.clinicalgenome.org/kb/conditions/MONDO:0018554