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.
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Epidemiology
Associations
Williams syndrome: with supravalvular type
Pathology
Depending on location it can be classified into three types:
supravalvular stenosis
congenital aortic valve stenosis (commonest)
subvalvular stenosis
Radiographic features
Plain radiograph
Chest radiographs can be normal or may show evidence of cardiomegaly.
Ultrasound
Echocardiography
Echocardiograms may show a high flow jet through the aortic valve or a narrowed segment.
CT/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
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 2.
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