CTICasesScimitar Syndrome (Congenital Pulmonary Venolobar Syndrome)
Imaging findings
CT in an older woman demonstrates a hypoplastic right lung and an anomalous right pulmonary vein ("scimitar vein") draining into the inferior vena cava (IVC) at the level of the diaphragm. The anomalous vein shows severe narrowing/compression as it passes between the diaphragmatic crus and the IVC, with associated hypovascularity of the right lung.
Key takeaways
Scimitar syndrome is a form of partial anomalous pulmonary venous return where the right lung veins drain anomalous systemic blood into the IVC. While it can cause significant left-to-right shunting in children, adults who present asymptomatically often have hypoplastic lungs with lower overall flow, and sometimes severe stenosis/compression of the scimitar vein at the diaphragmatic hiatus, which naturally restricts the shunt volume.
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