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Chronic thromboembolic pulmonary hypertension

Disease definition

A rare complication of acute pulmonary embolism (PE), either symptomatic or not, that is characterized by fibrotic intravascular material occlusion of pulmonary arteries in combination with a secondary microvasculopathy of vessels less than 500 µm. The consequence is an increase in pulmonary vascular resistance (PVR) and progressive right heart failure.

ORPHA:70591

Classification level: Disorder
  • Synonym(s):
    • CTEPH
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Elderly, Adult, Adolescent
  • ICD-10: -
  • OMIM: 612862
  • UMLS: C2363973
  • MeSH: -
  • GARD: -
  • MedDRA: 10068739

Detailed information

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