LA8A.3 Congenital supravalvar aortic stenosis

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2026-01

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  (LA8A.20)

synonyms

• Congenital supravalvar aortic stenosis
• congenital aortic stenosis NOS
• congenital aortic stricture
• congenital ascending aortic stenosis
• congenital ascending aortic stricture
• congenital narrowed aorta
• congenital stenosis of aorta
• congenital stricture of aorta
• congenital supravalvular aortic stenosis

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