Orphanet Rare Disease Ontology

Last uploaded: July 3, 2024
Preferred Name

Pulmonary arterial hypertension
Synonyms

PAH

Definitions

Pulmonary arterial hypertension (PAH) is a group of diseases characterized by elevated pulmonary arterial resistance leading to right heart failure. PAH is progressive and potentially fatal. PAH may be idiopathic and/ or familial, or induced by drug or toxin (drug-or toxin-induced PAH, see these terms) or associated with other diseases like congenital heart disease, connective tissue disease, HIV, schistosomiasis, portal hypertension (PAH associated with other disease, see this term).

ID

http://www.orpha.net/ORDO/Orphanet_182090

alternative_term

PAH

definition

Pulmonary arterial hypertension (PAH) is a group of diseases characterized by elevated pulmonary arterial resistance leading to right heart failure. PAH is progressive and potentially fatal. PAH may be idiopathic and/ or familial, or induced by drug or toxin (drug-or toxin-induced PAH, see these terms) or associated with other diseases like congenital heart disease, connective tissue disease, HIV, schistosomiasis, portal hypertension (PAH associated with other disease, see this term).

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=182090

has_age_of_onset

All ages

has_inheritance

Not applicable

Autosomal dominant

hasDbXref

MedDRA:10064911

MeSH:D000081029

ICD-11:BB01.0

UMLS:C2973725

label

Pulmonary arterial hypertension

notation

ORPHA:182090

Category

prefixIRI

ORDO:Orphanet_182090

prefLabel

Pulmonary arterial hypertension

present_in

France AND has_annual_incidence_average_value : 0.24 AND has_annual_incidence_range : 1-9 / 1 000 000

Spain AND has_point_prevalence_average_value : 1.6 AND has_point_prevalence_range : 1-9 / 100 000

Europe AND has_point_prevalence_average_value : 2.0 AND has_point_prevalence_range : 1-9 / 100 000

Switzerland AND has_annual_incidence_average_value : 0.35 AND has_annual_incidence_range : 1-9 / 1 000 000

Czech Republic AND has_point_prevalence_average_value : 2.24 AND has_point_prevalence_range : 1-9 / 100 000

Spain AND has_annual_incidence_average_value : 0.37 AND has_annual_incidence_range : 1-9 / 1 000 000

Switzerland AND has_point_prevalence_average_value : 1.55 AND has_point_prevalence_range : 1-9 / 100 000

United States AND has_point_prevalence_average_value : 1.09 AND has_point_prevalence_range : 1-9 / 100 000

France AND has_point_prevalence_average_value : 1.5 AND has_point_prevalence_range : 1-9 / 100 000

Czech Republic AND has_annual_incidence_average_value : 1.07 AND has_annual_incidence_range : 1-9 / 100 000

United Kingdom AND has_annual_incidence_average_value : 0.72 AND has_annual_incidence_range : 1-9 / 1 000 000

United Kingdom AND has_point_prevalence_average_value : 5.2 AND has_point_prevalence_range : 1-9 / 100 000

subClassOf

http://www.orpha.net/ORDO/Orphanet_71198

http://www.orpha.net/ORDO/Orphanet_557492

http://www.orpha.net/ORDO/Orphanet_506222

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