This site is targeted at medical and radiology professionals, contains user contributed content, and material that may be confusing to a lay audience. Use of this site implies acceptance of our Terms of Use.

Congenital aortic stenosis

Dr Yuranga Weerakkody et al.

A congenital aortic stenosis broadly refers to a congenital narrowing of the aortic lumen. Although the term can mean narrowing at any point it often refers to a narrowing about the aortic valve. As a broad group there can be some overlap with ascending aortic coarctation depending of the definition used.

Depending on location it can be classified into 3 types.

supravalvular stenosis
congenital aortic valve stenosis (commonest)
subvalvular stenosis

Associations

Williams syndrome : with supravalvular type
bicuspid aortic valve ^2,4
aortic coarctation
hypoplastic left heart
quadricuspid aortic valve ⁴

Radiographic features

Chest radiograph

Can be normal. May show evidence of cardiomegaly

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

In MR imaging, velocity encoded phase contrast cine sequences can assist assessing the severity of the stenosis by allowing measurement of blood flow velocities and volumes ²