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IPCCC ICD-11 Congenital Heart Atlas

You are here: Congenital supravalvar aortic stenosis Congenital anomaly of a ventriculo-arterial valve or adjacent regions Structural developmental anomaly of heart or great vessels

IPCCC Term Congenital supravalvar aortic stenosis
IPCCC Code 09.16.18
ICD-11 Code 1066595728


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


Stenosis at or above the sinotubular junction; Ascending aorta stenosis; Ascending aorta stricture

STS-EACTS-derived IPCCC term

Aortic stenosis, Supravalvar, Congenital supravalvar aortic stenosis

EPCC-derived IPCCC term

Congenital supravalvar aortic stenosis

ICD-11 MMS code or crossmap


ICD-10 MMS code or crossmap

Q25.3: Stenosis of aorta (includes "Supravalvular aortic stenosis")


Congenital anomaly of a ventriculo-arterial valve or adjacent regions


Coding Notes

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.