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Pulmonary arterial hypertension associated with portal hypertension

Disease definition

Pulmonary arterial hypertension associated with portal hypertension (PAH-PH) is a form of pulmonary arterial hypertension (PAH), characterized by an elevated pulmonary arterial resistance leading to right heart failure observed as a complication of portal hypertension.


  • Synonym(s):
    • PAH associated with portal hypertension
    • POPH
    • Portopulmonary hypertension
  • Prevalence: Unknown
  • Inheritance: No data available 
  • Age of onset: Adult
  • ICD-10: I27.2
  • OMIM: -
  • UMLS: C1868851
  • MeSH: -
  • GARD: -
  • MedDRA: -

Additional information

Further information on this disease

Specialised Social Services

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