Pulmonary stenosis Supravalvular

Pulmonary stenosis is a congenital heart defect in which the lumen of the vessel is narrowed, but not closed by a membrane or valve. The pathology can be localized both above the valve of the pulmonary arteries (supravalvular pulmonary artery stenosis) and inside the valve ring Ao, narrowing it (intravenous pulmonary artery stenosis). With supravalvular stenosis of the pulmonary trunk, diffuse damage to the vessels of the pulmonary circulation can be complicated by severe pulmonary heart disease

Supravalvular pulmonary stenosis is a congenital pathology characterized by a narrowing of the pulmonary beds by more than two-thirds of the diameter. The pathology belongs to the category of vascular hypoplasia, and is also known in pediatrics as Morgagni-Adams-Stokes syndrome. The disease mainly affects children, but also occurs in adult patients. It develops due to damage to the walls of the pulmonary vessels, deformation or gross anomalies. Impaired blood circulation helps to reduce the flow of blood to the lungs, which causes respiratory disorders, changes in the functioning of the heart and increases the load on all the patient’s internal organs. The disease affects, in particular, the walls of blood vessels, the respiratory center, the heart chambers, the liver and the kidneys. Often supravalvular stenosis is combined with other cardiovascular anomalies.