- Pulmonary vein stenosis is a rare condition in which the veins that carry oxygen-rich blood from the lungs back to the heart are narrowed. This is a different condition than pulmonary (valve) stenosis (PVS, PS) and peripheral pulmonary stenosis.
- Pulmonary vein stenosis can occur in several forms. Many children have what is called intraluminal pulmonary vein stenosis, meaning that abnormal growth in connective tissue cells causes the walls in the pulmonary veins to become too thick.
- It is usually a progressive condition, meaning its severity and symptoms increase over time.
- Sometimes, pulmonary vein stenosis can occur as a complication of another heart or lung problem.
- The exact cause of pulmonary vein stenosis is not known.
- Some children will need many interventions—including interventional catheterization or surgery—to restore blood flow to the heart, as pulmonary vein stenosis tends to recur and even worsen over time.
- While a lung transplant does become a necessary treatment option for some children, not every child with the condition will need one.