#1 Ranked Children’s Hospital by U.S. News & World Report
MyPatients provides referring primary care providers with secure access to their patients’ information.
Boston Children's has launched the world's 1st program dedicated to offering hand transplants to children who qualify.
Innovation insider is a semi-monthly e-newsletter analyzes innovations at Boston Children’s, other academic medical centers and from industry.
Read the latest blog by a Boston Children's doctor, clinician or staff member.
There are many ways you can help children and their families get the care they need.
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:
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:
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.
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.
The exact cause of pulmonary vein stenosis is still unknown.
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:
You should seek treatment from a qualified medical professional right away if you notice any of these symptoms in your child.
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:
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:
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.
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:
Boston Children's cardiology, cardiac surgery programs ranked top in the nation by U.S. News & World Report.
Learn more about our approach to lung transplants
We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”