JSON

pulmonary arterial hypertension

^ http://purl.obolibrary.org/obo/MONDO_0015924

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). [ Orphanet:182090 PMID:30545973 ]

Term info

database cross reference
  • MedDRA:10064911 (Orphanet:182090/e)
  • UMLS:CN200519 (MONDO:equivalentTo)
  • SCTID:11399002 (MONDO:equivalentTo)
  • Orphanet:182090 (MONDO:equivalentTo)
  • GARD:0007501 (MONDO:equivalentTo)
  • UMLS:C2973725 (Orphanet:182090)
  • MESH:D000081029 (https://orcid.org/0000-0003-1967-3726)
Subsets

disease_grouping, ordo_group_of_disorders

abbreviation
PAH [ Orphanet:182090 ]

abbreviation
PVOD/PCH [ PMID:30545973 ]

abbreviation
PPH [ GARD:0007501 ]

IAO 0000233

https://github.com/monarch-initiative/mondo/issues/5537, https://github.com/monarch-initiative/mondo/issues/5781

closeMatch

http://identifiers.org/meddra/10064911

definition

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).

exactMatch

http://purl.obolibrary.org/obo/Orphanet_182090, http://identifiers.org/mesh/D000081029, http://linkedlifedata.com/resource/umls/id/C2973725, http://linkedlifedata.com/resource/umls/id/CN200519, http://identifiers.org/snomedct/11399002

has exact synonym

PAH, pulmonary arterial hypertension

has narrow synonym

PAH with overt features of venous/capillaries involvement, PVOD/PCH

has related synonym

PPH, idiopathic pulmonary hypertension

id

MONDO:0015924

Term relations