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Pulmonary vein stenosis (PVS) 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. There are many types of pulmonary vein obstruction; but the one in which stenosis occurs due to an abnormal process that thickens and narrows the walls in the veins causing blockage within the lumen of the veins is called intraluminal pulmonary vein stenosis. This type frequently recurs and 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 or underlying chronic lung disease, but it may also occur in infants with otherwise normal hearts and lungs. This is called Isolated pulmonary vein stenosis; 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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