supravalvular aortic stenosis

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The severity of supravalvular aortic stenosis varies from a mild narrowing above the aortic valve to a deformity which resembles an hour-glass.

Similar pathological changes are often seen throughout the large conducting arteries of the body, stenoses being found in the carotid, innominate and mesenteric arteries.

In common with all forms of aortic stenosis, there is hypertrophy of the left ventricle, but in this condition, the degree of enlargement seems out of proportion ot the degree of outflow obstruction.

The aortic stenosis is not congenital, but is acquired during early life as the result of an inherited arteriopathy.

Last reviewed 01/2018