Supravalvular aortic stenosis

Orpha code: 3193OMIM code: 185500

Definition

A rare aortic malformation characterized by the narrowing of the aorta lumen (close to its origin) associated or not with stenosis of 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 (left ventricle in case of aorta involvement, right ventricle in case of pulmonary artery involvement).

Disease data
Classification

Morphological anomaly

Synonyms
SVAS
Podzastawkowe zwężenie aorty
SVAS
ORPHA code
3193
OMIM code
185500
ICD10 code
Q25.3
ICD11 code
LA8A.3

No additional description.

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