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Scimitar syndrome

Field	Value
name	Scimitar syndrome
image	Scimitar syndrome chest CT.jpg
caption	Scimitar syndrome chest CT

Scimitar syndrome, or congenital pulmonary venolobar syndrome, is a rare congenital heart defect characterized by anomalous venous return from the right lung (to the systemic venous drainage, rather than directly to the left atrium).{{Citation |archive-date=2009-05-30 |archive-url=https://web.archive.org/web/20090530020618/http://medind.nic.in/icb/t05/i3/icbt05i3p249.pdf |url-status=dead |archive-date=2010-12-04 |archive-url=https://web.archive.org/web/20101204202634/http://www.childrenshospital.org/az/Site2165/mainpageS2165P0.html |url-status=dead

Presentation

The anomalous venous return forms a curved shadow on chest x-ray such that it resembles a scimitar. This is called the Scimitar Sign. Associated abnormalities include right lung hypoplasia with associated dextroposition of the heart, pulmonary artery hypoplasia and pulmonary sequestration. Incidence is around 1 per 100,000 births.

Diagnosis

The diagnosis is made by transthoracic or transesophageal echocardiography and selective pulmonary angiography. More recently by CT angiography or MR Angiography.

Pulmonary angiography demonstrates anomalous arterial supply to right lower lobe.

Treatment

Surgical correction should be considered in the presence of significant left to right shunting (Qp:Qs ≥ 2:1) and pulmonary hypertension. This involves creation of an inter-atrial baffle to redirect the pulmonary venous return into the left atrium. Alternatively, the anomalous vein can be re-implanted directly into the left atrium.

History

Scimitar syndrome was first described by George Cooper and Raoul Chassinat in 1836 regarding a rare syndrome that has an abnormal pulmonary vein that drains into inferior vena cava below the diaphragm instead of draining into the left atrium.

References

Oransky, Ivan. (2006). "Catherine Neill". [[The Lancet]].
{{harvnb. Hasan. Varshney. Agarwal. 2016
(May 1966). "Radiographic Features of the Scimitar Syndrome". Radiology.
Mulligan, Michael E.. (January 1999). "History of Scimitar Syndrome". Radiology.

Info: Wikipedia Source

This article was imported from Wikipedia and is available under the Creative Commons Attribution-ShareAlike 4.0 License. Content has been adapted to SurfDoc format. Original contributors can be found on the article history page.

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