supravalvular aortic stenosis

types:

localized hourglass narrowing just above aortic sinuses
discrete fibrous membrane above sinuses of Valsalva
diffuse tubular hypoplasia of ascending aorta and branching arteries

associated with:

peripheral pulmonary stenosis
valvular and discrete subvalvular aortic stenosis
Marfan syndrome
Williams syndrome

findings:

dilatation and tortuosity of coronary arteries (may undergo early atherosclerotic degeneration secondary to high pressure)
narrowing of the supravalvular area (normal root diameter: 20-37mm)
normal movement of cusps

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© 2002, C.E. Kahn, Jr.
2002-04-18