Pulmonary Vein Stenosis

What is pulmonary vein stenosis?

Pulmonary vein stenosis (PVS) is a rare condition in which the veins that carry blood from the lungs back to the heart are narrowed. This is a different condition than pulmonary valve stenosis and peripheral pulmonary stenosis. 

Sometimes, PVS 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 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?

Pulmonary vein stenosis can occur in several forms. Generally, pulmonary vein stenosis is grouped into the following categories:

Intraluminal pulmonary vein stenosis

This is a common type of pulmonary vein stenosis 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 narrowing 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.

Isolated pulmonary vein stenosis

Isolated pulmonary vein stenosis occurs when a baby has no other defects or problems in the heart or lungs at birth, but then have sudden, often rapidly progressing, symptoms in early infancy. Babies with this type of pulmonary vein stenosis can seem healthy for several weeks before suddenly having difficulty breathing and low oxygen levels.

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

What are the symptoms of pulmonary vein stenosis? 

Symptoms of pulmonary vein stenosis can occur very suddenly, especially in infants, or may progress gradually over time. In both cases, symptoms may include:

  • shortness of breath
  • rapid heartbeat
  • rapid, shallow breathing
  • fatigue
  • poor appetite
  • pale or “washed-out” skin hue
  • blue-tinged appearance, called cyanosis, of the skin, lips or nail beds

How we care for pulmonary vein stenosis

Boston Children’s Hospital has a dedicated Pulmonary Vein Stenosis Program whose expert clinicians have decades 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.

Our areas of innovation for PVS

Over the past two decades, our team has been at the forefront of research to learn more about this rare condition and discover new treatment options for patients. In addition to developing new techniques to treat PVS in the catheterization lab and operating room, our team has started using drug therapy to treat intraluminal pulmonary vein stenosis.