Congenital supravalvular aortic stenosis

Congenital supravalvular aortic stenosis is the rarest form of congenital aortic outflow tract stenosis, accounting for approximately 5-10%. The incidence is similar in males and females. Supravalvular stenosis may be accompanied by mental retardation. The stenotic lesion is located above the coronary artery ostia. The localized type is the most common form of supravalvular aortic stenosis, accounting for about 90%. The most common lesion is a diaphragm-like stenosis above the coronary sinus of Valsalva. In most cases, symptoms of aortic outflow tract stenosis do not appear until childhood. Because coronary atherosclerosis develops earlier, angina pectoris is relatively common. Some patients have a family history.