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Pulmonary vein stenosis is a condition in which there is an obstruction (blockage) in the blood vessels that bring oxygen-rich blood from the lungs back to the heart. Stenosis occurs due to an abnormal process that thickens and narrows the walls in the veins. Pulmonary vein stenosis frequently progresses. As a result, total obstruction or partial loss of flow to a vessel or vessels may occur.
This condition may occur as a complicating feature of complex congenital heart disease, but it may also occur in infants with otherwise normal hearts. When pulmonary vein stenosis occurs in children without congenital heart defects, it occurs in early infancy and usually progresses very rapidly. Infants with this disease may seem well for weeks before they develop difficulty breathing and low oxygen levels. They may become quite ill quickly. The effects of the disease vary in children with co-existing congenital heart defects.
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