A Rare Variant of Hypogenetic Lung Syndrome Mimicking Scimitar Vein

Ann Thorac Surg. 2021 Sep;112(3):e173-e176. doi: 10.1016/j.athoracsur.2021.01.020. Epub 2021 Jan 23.


Hypogenetic lung syndrome, also known as scimitar syndrome, is a rare and well-known congenital condition that includes hypoplastic right pulmonary artery and lung, right displacement of the heart, anomalous systemic arterial supply to the lung, and a characteristically curved anomalous right pulmonary vein draining into the inferior vena cava. In exceptional cases, the anomalous pulmonary vein may drain into left atrium. We here report a case of a 17-year-old girl with a rare variant of hypogenetic lung syndrome diagnosed by means of multimodality imaging and treated with percutaneous occlusion of the aortopulmonary collateral.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple / diagnosis*
  • Adolescent
  • Diagnosis, Differential
  • Female
  • Heart Atria / abnormalities*
  • Humans
  • Pulmonary Veins / abnormalities*
  • Scimitar Syndrome / classification*
  • Scimitar Syndrome / diagnosis*
  • Vena Cava, Inferior / abnormalities*