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Pulmonary artery atresia

Changed by Vincent Tatco, 7 Nov 2015

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Pulmonary artery atresia (or sometimes known as pulmonary atresia)is one of congenital cardiovascular anomaly in which there is complete disruption between the right ventricular outflow tract (RVOT) and the main pulmonary artery.

Epidemiology

The estimated incidence is 1 in 10,000 births.

Pathology

The term pulmonary atresia can cover a broad spectrum of abnormalities depending on the extent of the disruption.

Sub types

It can be classified into 3 sub-types ¹:

pulmonary atresia with intact inter ventricular septum (PA-IVS)
pulmonary atresia with VSD (PA-VSD)
complex pulmonary atresia(pulmonary atresia with complex cardiac malformation)

Variants

unilateral pulmonary artery atresia (UPAA)
- isolated unilateral pulmonary artery atresia

Associations

tetralogy of Fallot:PA-VSD is considered by some authors as a severe from of tetralogy of Fallot

Radiographic features

Chest radiograph

PA-VSD³:

normal or mildly enlarged heart with poor / diminished pulmonary arterial vascular markings
asymmetrical vascular suggest stenosis within pulmonary arterial tree
there can be plethora due to horizontal arteries forming aorto-pulmonary collaterals
mottled appearance as the lung periphery may suggest pleuro-pulmonary collateral formation

Cardiac MRI

Allows direct visualisation of anomaly. Cine sequences may show a dilated non contracting right ventricle ⁶. MR MR angiography allows detection of aortopulmonary collaterals and patent ductus arteriosus (if present).

Treatment

Management varies depending on the presence of a VSD. Prostaglandin E₁ is used to keep the ductus open ⁵.

-<p><strong>Pulmonary artery atresia </strong>(or sometimes known as <strong>pulmonary atresia</strong>)<strong> </strong>is one of <a href="/articles/congenital-cardiovascular-anomalies">congenital cardiovascular anomaly</a> in which there is complete disruption between the <a href="/articles/right-ventricular-outflow-tract">right ventricular outflow tract</a> (RVOT) and the <a href="/articles/main-pulmonary-artery">main pulmonary artery</a>. </p><h4>Epidemiology</h4><p>The estimated incidence is 1 in 10,000 births.</p><h4>Pathology</h4><p>The term pulmonary atresia can cover a broad spectrum of abnormalities depending on the extent of the disruption.</p><h5>Sub types</h5><p>It can be classified into 3 sub-types <sup>1</sup>:</p><ul>
+<p><strong>Pulmonary artery atresia </strong>(or sometimes known as <strong>pulmonary atresia</strong>)<strong> </strong>is one of <a href="/articles/congenital-cardiovascular-anomalies">congenital cardiovascular anomaly</a> in which there is complete disruption between the <a href="/articles/right-ventricular-outflow-tract">right ventricular outflow tract</a> (RVOT) and the <a href="/articles/main-pulmonary-artery">main pulmonary artery</a>. </p><h4>Epidemiology</h4><p>The estimated incidence is 1 in 10,000 births.</p><h4>Pathology</h4><p>The term pulmonary atresia can cover a broad spectrum of abnormalities depending on the extent of the disruption.</p><h5>Sub types</h5><p>It can be classified into 3 sub-types <sup>1</sup>:</p><ul>
~~-<a href="/articles/complex-pulmonary-atresia">complex pulmonary atresia</a> (pulmonary atresia with complex cardiac malformation)</li>~~
+<a href="/articles/complex-pulmonary-atresia">complex pulmonary atresia</a> (pulmonary atresia with complex cardiac malformation)</li>
-<a href="/articles/tetralogy-of-fallot">tetralogy of Fallot: </a>PA-VSD is considered by some authors as a severe from of tetralogy of Fallot</li></ul><h4>Radiographic features</h4><h5>Chest radiograph</h5><h6>PA-VSD <sup>3</sup>:</h6><ul>
+<a href="/articles/tetralogy-of-fallot">tetralogy of Fallot: </a>PA-VSD is considered by some authors as a severe from of tetralogy of Fallot</li></ul><h4>Radiographic features</h4><h5>Chest radiograph</h5><h6>PA-VSD <sup>3</sup>:</h6><ul>
-</ul><h5>Cardiac MRI</h5><p>Allows direct visualisation of anomaly. Cine sequences may show a dilated non contracting right ventricle <sup>6</sup>. MR angiography allows detection of aortopulmonary collaterals and patent ductus arteriosus (if present).</p><h4>Treatment </h4><p>Management varies depending on the presence of a VSD. Prostaglandin E<sub>1</sub> is used to keep the ductus open <sup>5</sup>.</p>
+</ul><h5>Cardiac MRI</h5><p>Allows direct visualisation of anomaly. Cine sequences may show a dilated non contracting right ventricle <sup>6</sup>. MR angiography allows detection of aortopulmonary collaterals and patent ductus arteriosus (if present).</p><h4>Treatment </h4><p>Management varies depending on the presence of a VSD. Prostaglandin E<sub>1</sub> is used to keep the ductus open <sup>5</sup>.</p>

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Image 4 CT (3D reconstruction with annotated image) ( create )

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Pulmonary artery atresia

Updates to Article Attributes

Epidemiology

Pathology

Sub types

Variants

Associations

Radiographic features

Chest radiograph

PA-VSD³:

Cardiac MRI

Treatment

Image 4 CT (3D reconstruction with annotated image) ( create )

Articles

By Section:

By System:

Cases

Radiopaedia.org

Pulmonary artery atresia

Updates to Article Attributes

Epidemiology

Pathology

Sub types

Variants

Associations

Radiographic features

Chest radiograph

PA-VSD3:

Cardiac MRI

Treatment

Image 4 CT (3D reconstruction with annotated image) ( create )

Articles

By Section:

By System:

Cases

Radiopaedia.org

PA-VSD³: