Pulmonary arterial hypertension (PAH) is a group of diseases characterized by mean pulmonary artery pressure >20 mmHg and elevated pulmonary arterial resistance leading to right heart failure. PAH is progressive and potentially fatal. PAH may be idiopathic and/ or familial, have overt features of venous/capillary involvement (pulmonary veno-occlusive disease, PVOD/pulmonary capillary hemangiomatosis, PCH), induced by drug or toxin (drug-or toxin-induced PAH), or associated with other diseases like congenital heart disease, connective tissue disease, HIV, schistosomiasis, portal hypertension (PAH associated with other disease). [ http://www.ncbi.nlm.nih.gov/pubmed/30545973 Orphanet:182090 ]
Synonyms: PAH pulmonary arterial hypertension
Term information
- GARD:7501 (Orphanet:182090)
- SCTID:11399002 (MONDO:equivalentTo)
- MESH:D000081029 (https://orcid.org/0000-0003-1967-3726)
- Orphanet:182090 (MONDO:equivalentTo)
- NORD:1634 (MONDO:NORD)
- UMLS:C2973725 (Orphanet:182090)
- MedDRA:10064911 (Orphanet:182090/e)
gard_rare, disease_grouping, rare, nord_rare, orphanet_rare, clingen, ordo_group_of_disorders
https://github.com/monarch-initiative/mondo/issues/5537
https://github.com/monarch-initiative/mondo/issues/5781
http://www.orpha.net/ORDO/Orphanet_182090
http://identifiers.org/mesh/D000081029
http://linkedlifedata.com/resource/umls/id/C2973725
http://identifiers.org/snomedct/11399002