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Scimitar Syndrome

Imaging findings

Coronal CT angiogram demonstrates two large anomalous pulmonary veins draining the entire right lung into the inferior vena cava. The right lung is hypoplastic with abnormal lobation.

Key takeaways

Scimitar syndrome is a congenital anomaly characterized by anomalous venous drainage of the right lung to the IVC, right lung hypoplasia, and often systemic arterial supply. Having two separate draining scimitar veins is a rare variant.

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