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Pulmonary arterial hypertension

Disease 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).


Classification level: Group of disorders
  • Synonym(s):
    • PAH
  • Prevalence: 1-9 / 100 000
  • Inheritance: Autosomal dominant or Not applicable 
  • Age of onset: All ages
  • ICD-10: -
  • ICD-11: BB01.0
  • OMIM: -
  • UMLS: C0152171  C1701938  C2973725
  • MeSH: C536282
  • GARD: 7501
  • MedDRA: 10064911
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