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
Term information
- EFO:0009199 (MONDO:equivalentTo)
- Orphanet:431353 (MONDO:equivalentTo)
- GARD:21801 (Orphanet:431353)
gard_rare, disease_grouping, rare, nord_rare, orphanet_rare, clingen, ordo_group_of_disorders