LA8A.3
category
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
(LA8A.20)
Also indexed as
Congenital supravalvar aortic stenosiscongenital aortic stenosis NOScongenital aortic stricturecongenital ascending aortic stenosiscongenital ascending aortic stricturecongenital narrowed aortacongenital stenosis of aortacongenital stricture of aortacongenital supravalvular aortic stenosis
Nearby in Congenital anomaly of a ventriculo-arterial valve or adjacent regions
LA8A.0Congenital anomaly of pulmonary valveLA8A.1Congenital pulmonary atresiaLA8A.2Congenital anomaly of aortic valveLA8A.4Aneurysm of aortic sinus of ValsalvaLA8A.5Congenital subaortic stenosisLA8A.6Congenital subpulmonary stenosis4B4YOther specified diseases of the immune system4B4ZDiseases of the immune system, unspecified