CTICasesRepaired Scimitar Syndrome with Baffle Stenosis and Leak
Imaging findings
High-resolution CT and MR angiography demonstrate a hypoplastic right lung with a single upper lobe segment, anomalous airway branching, and a persistent left SVC. A surgical baffle, created to redirect the anomalous right pulmonary vein (scimitar vein) into the left atrium, demonstrates a focal stenotic kink and a small parabaffle leak, resulting in a visible jet of contrast refluxing back into the IVC and hepatic veins. Angiography shows subsequent treatment of the stenosis with balloon angioplasty and successful closure of the leak with a percutaneously placed Amplatzer septal occluder.
Key takeaways
Scimitar syndrome is a congenital cardiopulmonary anomaly characterized by anomalous pulmonary venous drainage of the right lung to the IVC, right lung hypoplasia, and systemic arterial supply. Surgical correction typically involves baffling the anomalous vein to the left atrium; late complications of this repair include baffle stenosis and leaks, which can be successfully managed using minimally invasive transcatheter techniques like balloon angioplasty and Amplatzer device closure.
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