Orphanet: Scimitar syndrome

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Scimitar syndrome

Disease definition

Scimitar syndrome is characterized by a combination of cardiopulmonary anomalies including partial anomalous pulmonary venous return connection of the right lung to the inferior caval vein leading to the creation of a left-to-right shunt.


Classification level: Disorder
  • Synonym(s):
    • Congenital pulmonary venolobar syndrome
    • Epibronchial right pulmonary vein syndrome
    • Halasz syndrome
    • Hypogenetic lung syndrome
  • Prevalence: Unknown
  • Inheritance: -
  • Age of onset: Infancy, Neonatal
  • ICD-10: Q26.8
  • OMIM: -
  • UMLS: C0036400
  • MeSH: D012587
  • GARD: -
  • MedDRA: 10051951

Detailed information


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