ICD-11 code : LA8A.3;
Preferred Label : Congenital supravalvar aortic stenosis;
ICD-11 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.;
ICD-11 synonym : aortic stricture; congenital aortic stenosis NOS; congenital narrowed aorta; ascending aortic stenosis; stricture of aorta; ascending aortic stricture; supravalvular aortic stenosis; stenosis of aorta;
Origin ID : 1066595728;
Automatic exact mappings (from CISMeF team)
- ICD-10 Mapping
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.
