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Idiopathic pulmonary arterial hypertension

Disease definition

Idiopathic pulmonary arterial hypertension (IPAH) is a sporadic form of pulmonary arterial hypertension (PAH, see this term) characterized by elevated pulmonary arterial resistance leading to right heart failure. IPAH is progressive and potentially fatal and not associated with an underlying condition or family history of PAH.

ORPHA:275766

Classification level: Subtype of disorder
  • Synonym(s):
    • IPAH
    • Primary pulmonary arterial hypertension
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: I27.0
  • OMIM: -
  • UMLS: C0152171  C3203102
  • MeSH: -
  • GARD: -
  • MedDRA: 10065151
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