Pulmonary Vein Stenosis

What is pulmonary vein stenosis?

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 and peripheral pulmonary stenosis. 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.

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.

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 can worsen over time.

What are the types of pulmonary vein stenosis?

Generally, pulmonary vein stenosis is grouped into one of the following categories:

Pulmonary vein stenosis as a secondary complication

Some children develop pulmonary vein stenosis as a secondary complication of another heart or lung problem. Often, their pulmonary vein stenosis is discovered after they have already been diagnosed with a condition like complex congenital heart disease or chronic lung disease

Intraluminal pulmonary vein stenosis

A common type of pulmonary vein stenosis is intraluminal pulmonary vein stenosis, a progressive narrowing that is caused by an abnormal thickening of the walls in the pulmonary veins. (The term “intraluminal” means “within the lumen,” the central opening that allows blood to flow into the vein.)

The progressive narrowing process is believed to be linked to an abnormal overgrowth of connective tissue cells — the cells that help our bodies repair and close wounds—within the pulmonary veins. Learn more about how Boston Children’s Hospital is exploring a possible drug therapy for intraluminal pulmonary vein stenosis on the Research & Clinical Trials tab.

Isolated pulmonary vein stenosis

Babies with pulmonary vein stenosis tend to have what is called isolated pulmonary vein stenosis, meaning that they have no other defects or problems in the heart or lungs at birth, but then undergo a sudden — and often rapidly progressing — emergence of symptoms in early infancy. Babies with this type of pulmonary vein stenosis can seem healthy for several weeks before suddenly experiencing difficulty breathing and low oxygen levels.

How we care for pulmonary vein stenosis

Boston Children’s has a dedicated Pulmonary Vein Stenosis Program whose expert clinicians have years of experience treating children, adolescents and adults with this condition.

Our specialized training in pediatric cardiology means that we understand the particular challenges, circumstances and intricacies of working with young people with pulmonary vein stenosis. In addition to our medical expertise, we provide patient-centered care that always recognizes your child as an individual — and we offer resources to meet the needs of your entire family.