Congenital supravalvar aortic stenosis

**Definition:** A congenital cardiovascular malformation with narrowing of the aorta at the level of the sinotubular junction which may extend into the ascending aorta.

Additional information: 'Congenital supravalvar aortic stenosis' is described as three forms: an hourglass deformity, a fibrous membrane, and a diffuse narrowing of the ascending aorta. Supravalvar aortic stenosis may involve the coronary artery ostia, and the aortic leaflets may be tethered. The coronary arteries can become tortuous and dilated due to elevated pressures and early atherosclerosis may ensue.

**Exclusions:** - Congenital aortic valvar stenosis