congenital aortic stenosis
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.
Pathology
Depending on location it can be classified into three types:
- supra valvular stenosis
- congenital aortic valve stenosis (commonest)
- subvalvular stenosis
Associations
- Williams syndrome: with supra valvular type
- bicuspid aortic valve
- aortic coarctation
- hypoplastic left heart
- quadricuspid aortic valve
Radiographic features
Plain radiograph
Chest radiographs can be normal or may show evidence of cardiomegaly.
Echocardiography
May show a high flow jet through the aortic valve or 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
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
See also
- aortic coarctation
- aortic stenosis
- acquired aortic stenosis
Siehe auch:
- Aortenisthmusstenose
- Bikuspidalität der Aortenklappe
- hypoplastic left heart syndrome
- quadricuspid aortic valve
- Williams syndrome
und weiter:
Assoziationen und Differentialdiagnosen zu congenital aortic stenosis:
