Scimitar syndrome

Case contributed by Maxime St-Amant


Chest pain and cough.

Patient Data

Age: 60 years
Gender: Female



The right hemithorax is hypoplastic. Also, a tubular structure is seen in the lower right lung, which has the shape of a scimitar; the findings are suggestive of Scimitar's syndrome (pulmonary venolobar syndrome or hypogenetic lung syndrome), a type of partial anomalous pulmonary venous return (PAPVR). The heart is displaced to the right. The left pulmonary artery is very prominent.



CT-scan confirms the abnormal pulmonary vein communicating with the inferior vena cava.

There is dextroposition of the heart and severe hypoplasia of the right pulmonary artery. The left pulmonary artery is enlarged.

On parenchymal windows, no fissure is clearly identified.

Case Discussion

Scimitar syndrome is also known as hypogenetic lung syndrome with four features (but not all four are always present):

  • lung hypoplasia
  • hypoplasia of ipsilateral pulmonary artery
  • anomalous venous return
  • anomalous arterial supply

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