Scimitar syndrome
Updates to Article Attributes
Scimitar syndrome,also known as pulmonary venolobar syndrome orhypogenetic 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 several findings in pulmonary venolobar syndrome.
Pathology
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:
- inferior vena cava: most common
- right atrium
- portal vein
The lung is frequently perfused by the aorta, but the bronchial tree is still connected and thus the lung is not sequestered.
Associations
- congenital heart disease, e.g. ASD, VSD, tetralogy of Fallot, PDA
- ipsilateral diaphragmatic anomalies, e.g. accessory diaphragm, diaphragmatic hernia
- localised bronchiectasis
- horseshoe lung
- vertebral anomalies, e.g. hemivertebrae 4
- genitourinary tract abnormalities 4
- pulmonary sequestration 10
Radiographic features
The diagnosis is made by transthoracic or transesophageal echocardiography, angiography, or by CT or MR angiography.
Plain film
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 maybe blurred.
Complications
The presence of a left-to-right shunt may lead to development of pulmonary hypertension and Eisenmenger physiology.
Treatment
Surgical correction should be considered in the presence of significant left to right shunting 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 and etymology
The term was first given by C A Neill et al in 1960 5.
Differential diagnosis
General imaging differential considerations include
- pulmonary sequestration
- right middle lobe atelectasis (on AP plain film)
- unilateral absence of pulmonary artery (UAPA)
See also
-<p><strong>Scimitar syndrome</strong>,<strong> </strong>also known as <strong>pulmonary venolobar syndrome </strong>or<strong> hypogenetic lung syndrome</strong>,<sup> </sup>is characterised by a <a href="/articles/pulmonary-hypoplasia">hypoplastic lung</a> that is drained by an anomalous vein into the systemic venous system. It is a type of <a href="/articles/partial-anomalous-pulmonary-venous-return">partial anomalous pulmonary venous return</a>.</p><h4>Pathology</h4><p>It is essentially a combination of <a href="/articles/pulmonary-hypoplasia">pulmonary hypoplasia</a> and <a href="/articles/partial-anomalous-pulmonary-venous-return">partial anomalous pulmonary venous return</a> (PAPVR<a href="/articles/partial-anomalous-pulmonary-venous-return">)</a>. It almost exclusively occurs on the right side.</p><p>Haemodynamically, there is an acyanotic left to right shunt. The anomalous vein usually drains into: </p><ul>- +<p><strong>Scimitar syndrome</strong>,<strong> </strong>also known as <strong>hypogenetic lung syndrome</strong>,<sup> </sup>is characterised by a <a href="/articles/pulmonary-hypoplasia">hypoplastic lung</a> that is drained by an anomalous vein into the systemic venous system. It is a type of <a href="/articles/partial-anomalous-pulmonary-venous-return">partial anomalous pulmonary venous return</a> and is one of several findings in <strong>pulmonary venolobar syndrome.</strong></p><h4>Pathology</h4><p>It is essentially a combination of <a href="/articles/pulmonary-hypoplasia">pulmonary hypoplasia</a> and <a href="/articles/partial-anomalous-pulmonary-venous-return">partial anomalous pulmonary venous return</a> (PAPVR<a href="/articles/partial-anomalous-pulmonary-venous-return">)</a>. It almost exclusively occurs on the right side.</p><p>Haemodynamically, there is an acyanotic left to right shunt. The anomalous vein usually drains into: </p><ul>
-<a title="Pulmonary Sequestration" href="/articles/pulmonary-sequestration">pulmonary sequestration</a> <sup>10</sup>- +<a href="/articles/pulmonary-sequestration">pulmonary sequestration</a> <sup>10</sup>
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