Orphanet: Eisenmenger syndrome

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Eisenmenger syndrome

Disease definition

A rare respiratory disease associated with unoperated congenital heart disease and characterized by congenital heart malformations with reversed or bi-directional shunting through an intra-cardiac or intervascular (usually aorto-pulmonary) communication with the development of PAH.


Classification level: Disorder
  • Synonym(s): -
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: Adolescent, Childhood, Adult
  • ICD-10: I27.2
  • OMIM: -
  • UMLS: C0013743
  • MeSH: -
  • GARD: 6323
  • MedDRA: 10058554
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