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Left Scimitar Syndrome (Anomalous Pulmonary Venous Drainage to the Portal Vein)

Imaging findings

Contrast-enhanced CT and MR angiography demonstrate an aberrant right subclavian artery and a unique anomalous venous pathway draining the left lung. A large anomalous pulmonary vein arises from the left lung, courses caudally through the mediastinum, crosses the midline, and descends below the diaphragm to drain directly into the portal venous system.

Key takeaways

Anomalous pulmonary venous drainage of the left lung to the portal system represents an extremely rare, infradiaphragmatic variant of anomalous pulmonary venous return, often colloquially termed "left" Scimitar syndrome. Multiplanar vascular reconstruction on CT or MRA is essential to trace these complex, anomalous connections to plan appropriate surgical redirection.

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