Congenital supravalvar aortic stenosis

Description

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.

Index terms

Congenital supravalvar aortic stenosis
congenital aortic stenosis NOS
congenital aortic stricture

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congenital ascending aortic stenosis
congenital ascending aortic stricture
congenital narrowed aorta
congenital stenosis of aorta
congenital stricture of aorta
congenital supravalvular aortic stenosis

Excludes

LA8A.20 – Congenital aortic valvar stenosis

Exclusions from above levels

LA85.4 – Common arterial trunk
http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

LA8A.0 – Congenital anomaly of pulmonary valve
LA8A.1 – Congenital pulmonary atresia
LA8A.2 – Congenital anomaly of aortic valve
LA8A.4 – Aneurysm of aortic sinus of Valsalva
LA8A.5 – Congenital subaortic stenosis
LA8A.6 – Congenital subpulmonary stenosis
LA8A.Y – Other specified congenital anomaly of a ventriculo-arterial valve or adjacent regions
LA8A.Z – Congenital anomaly of a ventriculo-arterial valve or adjacent regions, unspecified

Foundation entity

Congenital supravalvar aortic stenosis – http://id.who.int/icd/entity/1066595728

Part of: LA8A: Congenital anomaly of a ventriculo-arterial valve or adjacent regions › 20: Developmental anomalies