Congenital aortic stenosis

Last revised by Dr Bahman Rasuli on 24 Sep 2020

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.  

Depending on location it can be classified into three types:

  • supra valvular stenosis
  • congenital aortic valve stenosis (commonest)
  • subvalvular stenosis

Chest radiographs can be normal or may show evidence of cardiomegaly.

May show a high flow jet through the aortic valve or narrowed segment.

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

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 2

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Cases and figures

  • Case 1 - supravalvular aortic stenosis
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