/supravalvular aortic stenosis

supravalvular aortic stenosis

Rare Disease

MONDO:0008504

Also Known As

SVASsupra-valvular aortic stenosissupravalvar aortic stenosissupravalvular aortic stenosissupravalvular aortic stenosis (disease)aortic supravalvular stenosissupravalvar aortic stenosis, Eisenberg type

Definition

SupraValvar Aortic Stenosis (SVAS) is characterized by the narrowing of the aorta lumen (close to its origin) or other arteries (branch pulmonary arteries, coronary arteries). This narrowing of the aorta or pulmonary branches may impede blood flow, resulting in heart murmur and ventricular hypertrophy (in case of aorta involvement). The narrowing results from a thickening of the artery wall, which is not related to atherosclerosis.