Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed
Occurs at the level of the aortic valve in 60-75% of cases. It is associated with a bicuspid aortic valve in greater than 50% of cases. Supravalvular stenosis is rare, and is almost always associated with infantile hypercalcemia/Williams syndrome.
Left ventricular hypertrophy develops in response to increased ventricular end systolic pressure and dilation does not occur until the stenosis is complicated by ventricular decompensation.
The CXR is usually normal in mild cases. In more severe cases, in time, you can get left ventricular hypertrophy and then left ventricular dilation. Post stenotic dilation of the ascending aorta is the most persistent radiologic finding, due to the jet of blood shooting through the stenotic valve and striking the lateral aortic wall. "Left ventricular configuration" is when there is a concavity to the left mid-heart border combined with a prominent lower left heart border.
See References Chapter.
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