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Chronic thromboembolic pulmonary hypertension

Disease definition

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by the persistence of thromboemboli in the form of organized tissue obstructing the pulmonary arteries. The consequence is an increase in pulmonary vascular resistance (PVR) resulting in pulmonary hypertension (PH) and progressive right heart failure.

ORPHA:70591

Classification level: Disorder
  • Synonym(s):
    • CTEPH
  • Prevalence: 1-9 / 100 000
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: -
  • OMIM: 612862
  • UMLS: C2363973
  • MeSH: -
  • GARD: -
  • MedDRA: 10068739

Detailed information

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