Aberrant left pulmonary artery
Updates to Article Attributes
An aberrant left pulmonary artery (or pulmonary sling) represents an anatomical variant that may result in symptoms because of compression of local structures, in particular, the trachea.
Pathology
It isAberrant left pulmonary arteries are thought to arise from a failure of formation of the 6th aortic arch. They have an anomalous origin from the posterior wall of the right pulmonary artery before coursing to the left lung passing posterior to the trachea and anterior to oesophagus.
The term “sling” is best used when the proximal portion of the anomalous vessel impinges on the right main bronchus and causes air trapping of the entire right lung, or the right middle andor lower lobes, depending on the site of the compression.
The second type of anomalousaberrant left pulmonary artery, which often is fatal, is associated with long-segment tracheal stenosis. This type of tracheal stenosis is due to complete cartilagetracheal rings (rigid “O-ring" trachea without a pars membranacea).
Associations
Other anomalies that can be associated with aberrant left pulmonary artery are:
- head and neck
- absent thyroid isthmus
-
thoracic
- complete tracheal rings
- tracheal stenosis
- single lobed left lung
- bilobed right lung
-
absent thyroid isthmuscongenital lobar overinflation5
- abdominopelvic
- musculoskeletal
Radiographic features
RadiographPlain radiograph
Conventional radiographs obtained in neonates at birth may show fetal fluid retention or air, with a mediastinal shift usually to the left side.
In cases of ring sling complex, conventional radiographs often show an absence of unilateral pulmonary aeration disturbance.
Fluoroscopy
In most instances, the barium oesophagogram characteristically shows a mass between the trachea and the oesophagus just above the level of the carina.
CT/MRI
The main bronchi have horizontal courses (i.e. low T-shaped carina) detectable on high-kilovoltage radiographs, CT, and MR. Modalities such as ultrafast electron beam CT, spiral CT, and MR imaging depict the vascular anatomy is normally well and obviatedelineated on CT or MR angiography.
Treatment and prognosis
Repositioning of the artery usually reverses compression, particularly when the underlying tracheobronchial tree is normal.
The mortality rate is high in patients requiring tracheal reconstruction because the stenosis is primary and not due to the vessel.
The success of reconstructive procedures in the rigid trachea can be studied by using three-dimensional CT techniques such as virtual bronchoscopy.
-<p>An <strong>aberrant left pulmonary artery</strong> (or <strong>pulmonary sling</strong>) represents an anatomical variant that may result in symptoms because of compression of local structures, in particular, the trachea.</p><h4>Pathology</h4><p>It is thought to arise from a failure of formation of the 6<sup>th</sup> aortic arch</p><p>The term “sling” is best used when the proximal portion of the anomalous vessel impinges on the right main bronchus and causes air trapping of the entire right lung or the right middle and lower lobes, depending on the site of the compression.</p><p>The second type of anomalous left pulmonary artery, which often is fatal, is associated with long-segment tracheal stenosis. This type of tracheal stenosis is due to complete cartilage rings (rigid “O-ring" trachea without a pars membranacea).</p><h5>Associations</h5><p>Other anomalies that can be associated with aberrant left pulmonary artery are</p><ul>- +<p>An <strong>aberrant left pulmonary artery</strong> (or <strong>pulmonary sling</strong>) represents an anatomical variant that may result in symptoms because of compression of local structures, in particular, the <a href="/articles/trachea">trachea</a>.</p><h4>Pathology</h4><p>Aberrant left pulmonary arteries are thought to arise from a failure of formation of the 6<sup>th</sup> aortic arch. They have an anomalous origin from the posterior wall of the right <a href="/articles/pulmonary-artery">pulmonary artery</a> before coursing to the left <a href="/articles/lung">lung</a> passing posterior to the trachea and anterior to <a href="/articles/oesophagus">oesophagus</a>. </p><p>The term “sling” is best used when the proximal portion of the anomalous vessel impinges on the right main bronchus and causes air trapping of the entire right lung, or right middle or lower lobes. </p><p>The second type of aberrant left pulmonary artery, which often is fatal, is associated with long-segment tracheal stenosis. This type of <a href="/articles/tracheal-stenosis">tracheal stenosis</a> is due to <a href="/articles/complete-tracheal-rings">complete tracheal rings</a>. </p><h5>Associations</h5><p>Other anomalies that can be associated with aberrant left pulmonary artery are:</p><ul>
- +<li>
- +<strong>head and neck</strong><ul><li>absent thyroid isthmus</li></ul>
- +</li>
- +<li>
- +<strong>thoracic</strong><ul>
-<li>absent thyroid isthmus</li>- +<li>
- +<a title="Congenital lobar overinflation" href="/articles/congenital-lobar-overinflation">congenital lobar overinflation</a> <sup>5</sup>
- +</li>
- +</ul>
- +</li>
- +<li>
- +<strong>abdominopelvic</strong><ul>
-<li><a href="/articles/hirschsprung-disease">Hirschprung's disease</a></li>- +<li><a href="/articles/hirschsprung-disease">Hirschsprung disease</a></li>
- +<li><a href="/articles/gallbladder-agenesis">agenesis of gallbladder</a></li>
- +</ul>
- +</li>
- +<li>
- +<strong>musculoskeletal</strong><ul>
-<li><a href="/articles/gallbladder-agenesis">agenesis of gallbladder</a></li>-</ul><h4>Radiographic features</h4><h5>Radiograph</h5><p>Conventional radiographs obtained in neonates at birth may show fetal fluid retention or air, with a mediastinal shift usually to the left side.</p><p>In cases of ring sling complex, conventional radiographs often show an absence of unilateral aeration disturbance. </p><h5>Fluoroscopy</h5><p>In most instances, the barium oesophagogram characteristically shows a mass between the trachea and the oesophagus just above the level of the carina. </p><h5>CT/MRI</h5><p>The main bronchi have horizontal courses (i.e. low T-shaped carina) detectable on high-kilovoltage radiographs, CT, and MR. Modalities such as ultrafast electron beam CT, spiral CT, and MR imaging depict the vascular anatomy well and obviate angiography. </p><h4>Treatment and prognosis</h4><p>Repositioning of the artery usually reverses compression, particularly when the underlying tracheobronchial tree is normal. </p><p>The mortality rate is high in patients requiring tracheal reconstruction because the stenosis is primary and not due to the vessel.</p><p>The success of reconstructive procedures in the rigid trachea can be studied by using three-dimensional CT techniques such as virtual bronchoscopy.</p>- +</ul>
- +</li>
- +</ul><h4>Radiographic features</h4><h5>Plain radiograph</h5><p>Conventional radiographs obtained in neonates at birth may show fetal fluid retention or air, with a mediastinal shift usually to the left side.</p><p>In cases of ring sling complex, conventional radiographs often show an absence of unilateral pulmonary aeration. </p><h5>Fluoroscopy</h5><p>In most instances, the barium oesophagogram characteristically shows a mass between the trachea and the oesophagus just above the level of the carina. </p><h5>CT/MRI</h5><p>The main bronchi have horizontal courses (i.e. low T-shaped carina), and vascular anatomy is normally well delineated on CT or MR angiography. </p><h4>Treatment and prognosis</h4><p>Repositioning of the artery usually reverses compression, particularly when the underlying tracheobronchial tree is normal. </p><p>The mortality rate is high in patients requiring tracheal reconstruction because the stenosis is primary and not due to the vessel.</p><p>The success of reconstructive procedures in the rigid trachea can be studied by using three-dimensional CT techniques such as virtual bronchoscopy.</p>
References changed:
- 5. Diseases of the Heart, Chest & Breast 2011-2014. Springer. ISBN:8847019370. <a href="http://books.google.com/books?vid=ISBN8847019370">Read it at Google Books</a> - <a href="http://www.amazon.com/gp/product/8847019370">Find it at Amazon</a><span class="auto"></span>
Tags changed:
- congenital
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