Scimitar syndrome (lungs)

Scimitar syndrome, also known as hypogenetic lung syndrome, is characterised by a hypoplastic lung that is drained by an anomalous vein into the systemic venous system. It is a type of partial anomalous pulmonary venous return and is one of the several findings in congenital pulmonary venolobar syndrome.

It is essentially a combination of pulmonary hypoplasia and partial anomalous pulmonary venous return (PAPVR). It almost exclusively occurs on the right side.

Haemodynamically, there is an acyanotic left to right shunt. The anomalous vein usually drains into:

In some variants, the abnormal lung segment may receive arterial blood supply from the aorta or its branches 1,4,11,12. Some cases may show the anomalous systemic arteries supplying an area of associated pulmonary sequestration 11

Associations

The diagnosis is made by transthoracic or transesophageal echocardiography, angiography, or by CT or MR angiography.

Plain radiograph

Chest radiographic findings are that of a small lung with ipsilateral mediastinal shift, and in one-third of cases, the anomalous draining vein may be seen as a tubular structure paralleling the right heart border in the shape of a Turkish sword (“scimitar”). The right heart border may be blurred.

Surgical correction should be considered in the presence of significant left to right shunting and pulmonary hypertension. This involves the creation of an interatrial baffle to redirect the pulmonary venous return into the left atrium. Alternatively, the anomalous vein can be re-implanted directly into the left atrium.

Complications

The presence of a left-to-right shunt may lead to a development of pulmonary hypertension and Eisenmenger physiology.

The term was first given by C A Neill et al. in 1960 5.

General imaging differential considerations include:

Inspired signs
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Article information

rID: 2027
Section: Syndromes
Synonyms or Alternate Spellings:
  • Hypogenetic lung syndrome

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