CTICasesScimitar syndrome with pulmonary venous stenosis
Imaging findings
CT shows right lung hypoplasia and an anomalous right pulmonary vein draining into the IVC. 4D flow MRI shows that a tight, narrow bend in the anomalous vein increases venous resistance, diverting 75% of pulmonary blood flow to the normal left lung.
Key takeaways
Scimitar syndrome is a form of partial anomalous pulmonary venous return. The anomalous vein can undergo localized stenosis or narrowing, which increases right lung venous pressure and limits right-sided blood flow, protecting the patient from significant left-to-right shunts.
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