Scimitar syndrome

Case contributed by Stefan Tigges
Diagnosis certain

Presentation

Shortness of breath.

Patient Data

Age: 30 years
Gender: Female
x-ray

Right-sided scimitar vein. Lungs clear, no pleural effusion, cardiac silhouette mildly enlarged. Slightly hypoplastic right lung.

ct

The right upper lobe drains into the left atrium, but the right middle and lower lobes drain into a large scimitar vein that in turn drains into the IVC at the level of the right hemidiaphragm. The right ventricle is enlarged.

Case Discussion

Scimitar syndrome is an uncommon combination of 2 congenital anomalies: 1) hypoplastic lung and 2) partial anomalous pulmonary venous return. The syndrome is almost always right-sided.

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