A Curious Case of Scimitar Syndrome That Defies Embryology

Scimitar syndrome is a rare condition described by unique anatomic features that consist mainly of an abnormal connection of the right pulmonary veins to the inferior vena cava and right atrial junction, as well as an anomalous systemic arterial supply to the right lung. We present the case of a 60-year-old man with an atypical variant of scimitar syndrome that was embryologically perplexing and anatomically challenging to correct. We highlight key surgical and procedural considerations for a patient with scimitar syndrome presenting with this complex surgical anatomy.