Scimitar syndrome, or congenital pulmonary venolobar syndrome, is a rare

congenital heart defect A congenital heart defect (CHD), also known as a congenital heart anomaly and congenital heart disease, is a defect in the structure of the heart or great vessels that is present at birth. A congenital heart defect is classed as a cardiovascular ...

characterized by anomalous venous return from the right lung (to the systemic venous drainage, rather than directly to the left atrium). This anomalous pulmonary venous return can be either partial (PAPVR) or total (TAPVR). The syndrome associated with PAPVR is more commonly known as ''Scimitar syndrome'' after the curvilinear pattern created on a chest radiograph by the pulmonary veins that drain to the inferior vena cava. This radiographic density often has the shape of a

scimitar A scimitar ( or ) is a single-edged sword with a convex curved blade associated with Middle Eastern, South Asian, or North African cultures. A European term, ''scimitar'' does not refer to one specific sword type, but an assortment of different ...

, a type of curved sword. The syndrome was first described by

Catherine Neill Catherine Annie Neill (3 September 1921 – 23 February 2006) was a British pediatric cardiologist who spent the majority of her career at the Johns Hopkins Children's Center in Baltimore, where she worked alongside Helen B. Taussig. Her primar ...

in 1960.

Presentation

The anomalous venous return forms a curved shadow on chest x-ray such that it resembles a

. 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 Pulmonary angiography (or pulmonary arteriography,conventional pulmonary angiography, selective pulmonary angiography) is a medical fluoroscopic procedure used to visualize the pulmonary arteries and much less frequently, the pulmonary veins. It ...

. 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 The inferior vena cava is a large vein that carries the deoxygenated blood from the lower and middle body into the right atrium of the heart. It is formed by the joining of the right and the left common iliac veins, usually at the level of the ...

below the diaphgram instead of draining into the left atrium.

References

External links