Supra vulvar aortic stenosis, Biology

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Supra Vulvar Aortic Stenosis :  Supra valvar aortic stenosis in children with elfin facies, mental retardation, multiple peripheral pulmonary artery stenosis and hyper calcemia is known as William's Syndrome. The type of stenosis may be localized or diffuse. The localized type produces an hourglass deformity in association with some dilatqtion of sinuses of Valsalva without post stenolic dilatation of the aorta. Intimal proliferation and adhesion of aostic valve cusps may cause varying degrees of coronay ostial obstruction. Less commonly the narrowing is diffuse, extending along the whole extent of ascending aorta or even beyond the arch.

 


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