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Scimitar syndrome is a rare variant of partial anomalous pulmonary venous connection. The combination of scimitar syndrome with anomalous connection of the left superior pulmonary vein to the left innominate vein is extremely rare, and is yet to be documented in literature. We report the successful single-stage correction of such a rare type of anomalous pulmonary venous connection.

Scimitar syndrome with anomalous connection of left superior pulmonary vein to left innominate vein