Scimitar syndrome

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  • Hypoplastic lung that is drained by an anomalous vein into the systemic venous system
  • Combo of pulmonary hypoplasia and partial anomalous pulmonary venous return
  • Almost exclusively occurs on the right side
  • Acyanotic left to right shunt
  • Anomalous vein most commonly drains into the IVC, right atrium or portal vein
  • Associated with congenital heart disease, ipsilateral diaphragmatic anomalies and vertebral anomalies