Presentation
Shortness of breath.
Patient Data
Lungs clear, no pleural effusion, cardiac silhouette normal in size. Right-sided scimitar vein. Mildly hypoplastic right lung with shift of the mediastinum to the right.
Right upper and right middle pulmonary veins form a scimitar vein draining into the suprahepatic portion of the inferior vena cava at the level of the diaphragm above the IVC interruption. Right lower and left upper/lower pulmonary vein drains normally into the left atrium.
The IVC is interrupted and drains into the right SVC via azygous continuation. Hepatic veins drain to the suprahepatic portion of the inferior vena cava cephalic to the interruption.
MR confirms mild right lung hypoplasia and rightward mediastinal shift.
Case Discussion
Scimitar syndrome is an uncommon congenital anomaly combining a hypoplastic lung and partial anomalous pulmonary venous return. It almost always occurs on the right. The syndrome gets its name from the curving of the anomalous pulmonary vein that reminds some of the curve of a scimitar sword.
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