Congenital pulmonary stenosis

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Congenital pulmonary stenosis (CPS) refers to congenital narrowing of the right ventricular outflow tract, pulmonary valve or pulmonary artery.

Epidemiology

The estimated incidence is 1 in 2000 births.

Pathology

Can be morphologically categorised depending on the relationship to the pulmonary valve ³

supra valvular: distal to the valve: commonest ~60% ⁴
valvular
subvalvular: infundibular

Sub-classification

Supravalvular stenoses have been traditionally classified into four types.

Associations

Generally, occurs as an isolated feature and associations are rare ³. They include:

Radiographic features

Plain radiograph

Findings on chest radiographs are not specific. Can have a normal heart size or may show evidence of right ventricular hypertrophy. May also show evidence of a dilated pulmonary trunk or a main pulmonary artery. Pulmonary vascularity is often normal in mild cases. In some cases of pulmonary valvular stenosis, the flow of blood through the stenotic valve preferentially enter the left pulmonary artery which may result to an enlarged left pulmonary artery and slightly increased left lung vascularity.

Echocardiography

May show a high flow jet with turbulent flow through the pulmonary valve or narrowed segment.

CT/CTA

Direct visualisation of stenotic segment +/- post stenotic dilatation of distal arterial segments.

MRI/MRA

Direct visualisation of stenotic segment +/- associated features. Velocity encoded phase contrast (VEC) cine sequences can assist assessing the severity of the stenosis by allowing measurement of blood flow velocities and volumes ².

-<p><strong>Congenital pulmonary stenosis</strong> <strong>(CPS)</strong> refers to congenital narrowing of the <a href="/articles/right-ventricular-outflow-tract">right ventricular outflow tract</a>, <a href="/articles/pulmonary-valve">pulmonary valve</a> or <a href="/articles/pulmonary-artery">pulmonary artery</a>.</p><h4>Epidemiology</h4><p>The estimated incidence is 1 in 2000 births.</p><h4>Pathology</h4><p>Can be morphologically categorised depending on the relationship to the <a href="/articles/pulmonary-valve">pulmonary valve</a> <sup>3</sup></p><ul>
+<p><strong>Congenital pulmonary stenosis</strong> <strong>(CPS)</strong> refers to congenital narrowing of the <a title="right ventricle" href="/articles/right-ventricle">right ventricular</a> outflow tract, <a href="/articles/pulmonary-valve">pulmonary valve</a> or <a href="/articles/pulmonary-artery">pulmonary artery</a>.</p><h4>Epidemiology</h4><p>The estimated incidence is 1 in 2000 births.</p><h4>Pathology</h4><p>Can be morphologically categorised depending on the relationship to the <a href="/articles/pulmonary-valve">pulmonary valve</a> <sup>3</sup></p><ul>
-</ul><h4>Radiographic features</h4><h5>Plain radiograph</h5><p>Findings on chest radiographs are not specific. Can have a normal heart size or may show evidence of right ventricular hypertrophy. May also show evidence of a dilated <a title="pulmonary trunk" href="/articles/pulmonary-trunk">pulmonary trunk</a> or a main pulmonary artery. Pulmonary vascularity is often normal in mild cases. In some cases of pulmonary valvular stenosis, the flow of blood through the stenotic valve preferentially enter the left pulmonary artery which may result to an enlarged left pulmonary artery and slightly increased left lung vascularity. </p><h5>Echocardiography</h5><p>May show a high flow jet with turbulent flow through the pulmonary valve or narrowed segment.</p><h5>CT/CTA</h5><p>Direct visualisation of stenotic segment +/- post stenotic dilatation of distal arterial segments.</p><h5>MRI/MRA</h5><p>Direct visualisation of stenotic segment +/- associated features. Velocity encoded phase contrast (VEC) cine sequences can assist assessing the severity of the stenosis by allowing measurement of blood flow velocities and volumes <sup>2</sup>.</p><h4>See also</h4><ul><li><a href="/articles/pulmonary-artery-atresia">pulmonary atresia</a></li></ul>
+</ul><h4>Radiographic features</h4><h5>Plain radiograph</h5><p>Findings on chest radiographs are not specific. Can have a normal heart size or may show evidence of right ventricular hypertrophy. May also show evidence of a dilated <a href="/articles/pulmonary-trunk">pulmonary trunk</a> or a main pulmonary artery. Pulmonary vascularity is often normal in mild cases. In some cases of pulmonary valvular stenosis, the flow of blood through the stenotic valve preferentially enter the left pulmonary artery which may result to an enlarged left pulmonary artery and slightly increased left lung vascularity. </p><h5>Echocardiography</h5><p>May show a high flow jet with turbulent flow through the pulmonary valve or narrowed segment.</p><h5>CT/CTA</h5><p>Direct visualisation of stenotic segment +/- post stenotic dilatation of distal arterial segments.</p><h5>MRI/MRA</h5><p>Direct visualisation of stenotic segment +/- associated features. Velocity encoded phase contrast (VEC) cine sequences can assist assessing the severity of the stenosis by allowing measurement of blood flow velocities and volumes <sup>2</sup>.</p><h4>See also</h4><ul><li><a href="/articles/pulmonary-artery-atresia">pulmonary atresia</a></li></ul>

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