Scimitar Vein Syndrome

Alberto Iaia, MD
Francine L Jacobson, MD

October 10,1996

Presentation

Imaging Findings

On plain radiographs (notably the posteroanterior (PA) view), a tubular opacity (arrows), gently curvilinear in form, courses through the mid to lower portion of the right hemithorax. The right hemidiaphragm is elevated, and hypoplasia of the right pulmonary artery is evident.

Diagnosis

Discussion

Such an anomaly is often observed as a part of the scimitar syndrome. Although the plain film findings of scimitar sign are often diagnostic, selective pulmonary angiography can be useful in confirming the diagnosis of scimitar syndrome. This syndrome is also defined by hypoplasia of the right pulmonary artery and right lung as well as dextroposition of the heart and systemic blood supply to the right lower lobe. The clinical significance of the condition is limited to repeated bouts of pneumonia, the frequency of which increases with the severity of the right lower lung hypoplasia.

References

2. Roehm J, Jue K, Amplatz K. Radiographic features of the scimitar syndrome. Radiology 1966;86:856-859.

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