Congenital aortic stenosis
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- updated 19 Aug 2022:
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Congenital aortic stenosis broadly refers to a congenital narrowing of the aortic lumen. Although the term can mean narrowing at any point, it often relates to a narrowing of the aortic valve. As a broad group, there can be some overlap with ascending aortic coarctation depending on the definition used.
Epidemiology
Associations
Williams syndrome: with supravalvular type
Pathology
Depending on location it can be classified into three types:
-
supra valvularsupravalvular stenosis
congenital aortic valve stenosis (commonest)
subvalvular stenosis
Associations
Williams syndrome: with supra valvular type-
bicuspid aortic valve2,4 aortic coarctationhypoplastic left heart-
quadricuspid aortic valve4
Radiographic features
Plain radiograph
Chest radiographs can be normal or may show evidence of cardiomegaly.
Echocardiography
MayEchocardiograms may show a high flow jet through the aortic valve or a narrowed segment.
CT / MRI/MRI
Apart from showing a narrowed valve annulus and/or narrowing cross-sectional aortic segment, it may also show:
cardiomegaly with left ventricular hypertrophy
post-stenotic dilated segment of the aortic lumen
InOn MR imaging, velocity encoded phase-contrast cine sequences can assist in assessing the severity of the stenosis by allowing measurement of blood flow velocities and volumes 2.
See also
-<p><strong>Congenital aortic stenosis</strong> broadly refers to a congenital narrowing of the aortic lumen. Although the term can mean narrowing at any point, it often relates to a narrowing of the <a href="/articles/aortic-valve">aortic valve</a>. As a broad group, there can be some overlap with ascending <a href="/articles/coarctation-of-the-aorta">aortic coarctation</a> depending on the definition used. </p><h4>Pathology</h4><p>Depending on location it can be classified into three types:</p><ul>-<li>supra valvular stenosis</li>-<li>congenital aortic valve stenosis (commonest)</li>-<li>subvalvular stenosis</li>-</ul><h5>Associations</h5><ul>-<li>Williams syndrome: with supra valvular type</li>- +<p><strong>Congenital aortic stenosis</strong> broadly refers to a <a href="/articles/congenital" title="congenital (general)">congenital</a> narrowing of the <a href="/articles/aorta" title="Aorta">aortic lumen</a>. Although the term can mean narrowing at any point, it often relates to a narrowing of the <a href="/articles/aortic-valve">aortic valve</a>. As a broad group, there can be some overlap with ascending <a href="/articles/coarctation-of-the-aorta">aortic coarctation</a> depending on the definition used.</p><h4>Epidemiology</h4><h5>Associations</h5><ul>
- +<li><p>Williams syndrome: with supravalvular type</p></li>
- +<li><p><a href="/articles/bicuspid-aortic-valve">bicuspid aortic valve</a> <sup>2,4</sup></p></li>
- +<li><p><a href="/articles/coarctation-of-the-aorta">aortic coarctation</a></p></li>
- +<li><p><a href="/articles/hypoplastic-left-heart-syndrome">hypoplastic left heart</a></p></li>
- +<li><p><a href="/articles/quadricuspid-aortic-valve">quadricuspid aortic valve</a> <sup>4</sup></p></li>
- +</ul><h4>Pathology</h4><p>Depending on location it can be classified into three types:</p><ul>
- +<li><p>supravalvular stenosis</p></li>
- +<li><p>congenital aortic valve stenosis (commonest)</p></li>
- +<li><p>subvalvular stenosis</p></li>
- +</ul><h4>Radiographic features</h4><h5>Plain radiograph</h5><p><a href="/articles/chest-radiograph" title="Chest radiographs">Chest radiographs</a> can be normal or may show evidence of cardiomegaly.</p><h5>Echocardiography</h5><p><a href="/articles/echocardiography" title="Echocardiograms">Echocardiograms</a> may show a high flow jet through the aortic valve or a narrowed segment.</p><h5>CT/MRI</h5><p>Apart from showing a narrowed valve annulus and/or narrowing cross-sectional aortic segment, it may also show:</p><ul>
- +<li><p>cardiomegaly with left ventricular hypertrophy</p></li>
- +<li><p>post-stenotic dilated segment of the aortic lumen</p></li>
- +</ul><p>On MR imaging, velocity encoded phase-contrast cine sequences can assist in 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><p><a href="/articles/coarctation-of-the-aorta">aortic coarctation</a></p></li>
-<a href="/articles/bicuspid-aortic-valve">bicuspid aortic valve</a> <sup>2,4</sup>-</li>-<li><a href="/articles/coarctation-of-the-aorta">aortic coarctation</a></li>-<li><a href="/articles/hypoplastic-left-heart-syndrome">hypoplastic left heart</a></li>-<li>-<a href="/articles/quadricuspid-aortic-valve">quadricuspid aortic valve</a> <sup>4</sup>-</li>-</ul><h4>Radiographic features</h4><h5>Plain radiograph</h5><p>Chest radiographs can be normal or may show evidence of cardiomegaly.</p><h5>Echocardiography</h5><p>May show a high flow jet through the aortic valve or narrowed segment.</p><h5>CT / MRI</h5><p>Apart from showing a narrowed valve annulus and/or narrowing cross-sectional aortic segment, it may also show:</p><ul>-<li>cardiomegaly with left ventricular hypertrophy</li>-<li>post-stenotic dilated segment of the aortic lumen</li>-</ul><p>In MR imaging, velocity encoded phase-contrast cine sequences can assist in 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/coarctation-of-the-aorta">aortic coarctation</a></li>-<li>-<a href="/articles/aortic-valve-stenosis">aortic stenosis</a><ul><li><a href="/articles/acquired-aortic-stenosis">acquired aortic stenosis</a></li></ul>- +<p><a href="/articles/aortic-valve-stenosis">aortic stenosis</a></p>
- +<ul><li><p><a href="/articles/acquired-aortic-stenosis">acquired aortic stenosis</a></p></li></ul>
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Congenital aortic stenoses
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