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Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed
Etiology/Pathophysiology:
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.
Pathology:
Not applicable
Imaging Findings:
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.
DDX:
References:
See References Chapter.
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