Pulmonary Vein Stenosis

"We're thinking not only in terms of getting the child through a procedure today, but also of how life might be for that child at age 50, 60, 70. How do we plan their management over many decades? It's such an important part of making decisions about every child's care."

--Pedro del Nido, MD, Boston Children's chief of Cardiac Surgery

The news that your child has pulmonary vein stenosis can be hard to hear and difficult to accept. Not only are you grappling with all of your child’s immediate medical needs; you may also be wondering what the diagnosis means for the future, and how it will affect your family over the long term.

What is pulmonary vein stenosis?

Learning the basics about pulmonary vein stenosis—what it involves, how it develops and how it can be treated—can be a helpful first step in better understanding what to expect in the weeks and months to come.

  • 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.

Chris Baird, MD - Director of Congenital Heart Valve Program

How Boston Children's Hospital approaches pulmonary vein stenosis

Boston Children’s has a dedicated Pulmonary Vein Stenosis Program whose expert clinicians have many years of experience treating children, adolescents and adults with the 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 and other rare and serious heart problems. 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.

  • With more than 80 cardiac experts on our staff, Children’s operates the largest pediatric heart program in the nation.
  • We use the most sophisticated diagnostic and imaging procedures, including cardiac catheterization, and offer dozens of specialized services in such areas as cardiac anesthesia and robotic surgery.
  • Our Department of Cardiology and Department of Cardiac Surgery clinicians work closely with you to determine the right treatment plan for your child. We consider you an invaluable member of the treatment team, and always welcome your input and questions.