PVS The Term:
The term “stenosis” describes an abnormal narrowing within a structure of the body. Pulmonary vein stenosis, therefore, refers to narrowing in one or more areas within the body’s four pulmonary veins.
Pulmonary Vein Stenosis is a rare and serious cardiovascular condition in which there is an obstruction in the blood vessels that bring oxygen-rich blood from the lungs back to the heart. It is exceptionally hard to manage & has a very poor prognosis and high mortality rate. It can be isolated to a single pulmonary vein, but most often occurs in multiple veins simultaneously. Pulmonary vein stenosis can occur in two forms- Congenital- where the child is born with it and Acquired- where a child develops PVS as a result of a surgical procedure. It is usually a progressive condition, meaning it’s severity and symptoms increase over time.
The exact cause of Pulmonary Vein Stenosis is unknown. Some children will need many interventions—including cardiac 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 or be a candidate for one. Children that have developed PVS due to other heart conditions are usually denied lung transplant, due to an unhealthy heart. In very rare cases children can receive a heart and double lung transplant.
Babies with Congenital PVS are usually born appearing completely healthy. After a few weeks or even months the baby will start to develop breathing problems. Most often this goes undiagnosed or mistaken for asthma. If it is not discovered quickly the baby will eventually be unable to breathe. Best way of diagnosis is through an echo of the heart and lungs or a cardiac catheterization. In most cases congenital PVS is considered terminal.
Babies or children with acquired PVS were born with some form of a Congenital Heart Defect (CHD) that requires surgical intervention. In very unfortunate cases, the much needed surgical intervention causes Pulmonary Vein Stenosis. This can occur in one or all four of the veins causing the child to need further surgical interventions. Some of these babies loose a lung due to one sided PVS. Without continued surgical intervention the child will loose their life.
Pulmonary Vein Stenosis currently has no cure and very little funding for research. These babies and children’s only chance of survival is through life long hospital support and procedures.