Pulmonary Vein Stenosis Symptoms & Causes

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Contact the Pulmonary Vein Stenosis Program

Learning more about pulmonary vein stenosis can help you better understand what's next for your child and your family.

To picture what happens when a child has pulmonary vein stenosis, it’s good to know the basic anatomy of the heart and circulatory system. The diagram belowdepicts the heart’s structures (click to enlarge).

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. 

The pulmonary veins:

  • are four large blood vessels (two connected to each lung) that carry oxygen-rich blood from the lungs into the left atrium (blood-collection chamber) of the heart
  • are among the only veins in the body that carry oxygenated blood

Diagram of a normal human heart

In pulmonary vein stenosis, one or multiple areas within one or more pulmonary veins become narrowed, depriving the heart of essential oxygen. Pulmonary vein stenosis can cause dangerously low oxygen levels in the body, and can cause symptoms including:

  • labored breathing
  • a blue coloring throughout the child’s skin, called cyanosis
  • a rapid heartbeat

In the most severe cases, children with pulmonary vein stenosis may be at risk for complete obstruction of blood flow to the heart. The condition is always serious and will require medical or surgical intervention.

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.

What causes pulmonary vein stenosis?

The exact cause of pulmonary vein stenosis is still unknown.

  • Sometimes, it can occur as a complication of another heart or lung problem, such as complex congenital heart disease or chronic lung disease.
  • The intraluminal form of pulmonary vein stenosis may be linked to an overgrowth of connective tissue cells. These are the cells that are normally responsible for closing and healing wounds within the body.

What are the symptoms of pulmonary vein stenosis?

Symptoms of pulmonary vein stenosis can emerge very suddenly—especially in infants—or it can also progress gradually over time. The warning signs in both cases 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

You should seek treatment from a qualified medical professional right away if you notice any of these symptoms in your child.

Frequently Asked Questions (FAQ) about Pulmonary Vein Stenosis

Q: How serious is pulmonary vein stenosis? Is my child going to be OK?
A: Pulmonary vein stenosis is a rare condition that is usually progressive and serious. That said, the severity of symptoms can vary from child to child. Pulmonary vein stenosis may emerge very quickly—this is usually the case in infants—or a child may display signs gradually over time.

A child’s long-term health depends greatly on his individual circumstances, especially:

  • his overall well-being at the time of diagnosis
  • whether he has other heart or lung problems
  • how many of the four pulmonary veins are affected
  • the extent of narrowing within the affected areas
  • his tolerance for specific procedures

Q: Are there different types of pulmonary vein stenosis?
A:
Yes. Pulmonary vein stenosis can affect different children in very different ways. Some only have narrowing in one pulmonary vein; others have narrowing in multiple areas throughout all four pulmonary veins.

Q: Does my child have to cut back on physical activities?
A: Whether your child needs to cut back significantly on activities depends greatly on whether she has other heart or lung problems, how widespread the narrowing process is within the pulmonary veins and what symptoms she is experiencing. You should always talk to your treating clinician about recommended exercise restrictions or practices for your child.

Q: Is my child going to need a lung transplant?
A: While a lung transplant does become a necessary treatment option for some children with pulmonary vein stenosis, not every child with the condition will need one.

Q: What do I need to look out for once my child has been diagnosed with pulmonary vein stenosis?
A:
Parents of children with pulmonary vein stenosis should always be watchful for changes in their child’s breathing, appearance, activity level and appetite.

You should seek medical help immediately if your child experiences:

  • fatigue
  • shortness of breath
  • rapid heartbeat
  • difficulty “catching a breath”
  • heart palpitations
  • pale, ashen color in the face
  • rapid breathing
  • shallow breathing
  • a bluish tinge in the skin, lips or nail beds

Q: Is there a cure for pulmonary vein stenosis?
A: Pulmonary vein stenosis is usually a progressive condition, meaning that the degree of narrowing within the pulmonary veins will continue to increase over time, and more involved treatments, such as interventional catheterization or surgery, will become necessary. Children with pulmonary vein stenosis may need multiple catheterizations or surgeries throughout their lives, since the narrowing process tends to recur within a relatively short period of time.

For some children, the extent of damage caused by the pulmonary vein stenosis is so great that a lung transplant to replace the lungs and pulmonary veins is the only treatment option. 

Questions to ask your doctor about pulmonary vein stenosis

You and your family play an essential role in your child’s treatment for pulmonary vein stenosis. It’s important that you share your observations and ideas with your child’s treating physician, and that you have all the information you need to fully understand the treatment team’s explanations and recommendations.

You’ve probably thought of many questions to ask about your child’s pulmonary vein stenosis. It’s often very helpful to jot down your thoughts and questions ahead of time and bring them with you, along with a notebook, to your child’s appointment. That way, you’ll have all of your questions in front of you when you meet with your child’s treating clinician and can make notes to take home with you. (If your child is old enough, you can encourage him or her to write down questions, too.)

Some questions to ask your doctor might include:

  • How did you arrive at this diagnosis?
  • Are there any other conditions my child might have instead?
  • Is my child’s pulmonary vein stenosis linked to another heart or lung problem?
  • Does my child require further testing?
  • How extensive is the narrowing within the pulmonary veins?
  • What is the long-term outlook for my child?
  • Will you prescribe any medications? If so, what are the possible side effects?
  • Is interventional catheterization necessary at this time?
  • Will my child require open-heart surgery?
  • Will my child need a lung transplant?
  • How should I talk to my child about this condition and her long-term health?
  • Do I need to restrict my child’s physical activity?
  • Do I need to make any other changes to my child’s home and school routines?
  • What other resources can you point me to for more information?

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