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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).

ORPHA:182090

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

Additional information

Further information on this disease

Specialised Social Services

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