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Fibromuscular congenital aortic stenosis

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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The anatomy varies from a localised subaortic diaphragm to a longer fibromuscular narrowing. An ejection murmur is present with no ejection sound. The stenosis is frequently mild and the murmur is noticed at routine examination.

The aortic valve is often incompetent from interference with its function by the jet from the subaortic stenosis.

Diagnosis of the subaortic site of obstruction is suggested by the absence of an ejection sound without calcification to explain this and is made by echocardiography or cardiac catheterization and angiocardiography.

A significant stenosis is treated by open resection through the aortic valve including both diaphragm and muscular component.


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