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 |