Treatments for Pulmonary Vein Stenosis in Children

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Contact the Pulmonary Vein Stenosis Program

Boston Children's Pulmonary Vein Stenosis Program team has many years of expertise in treating all types and stages of this rare condition.

In our Pulmonary Vein Stenosis Program and all of our pediatric heart programs, we use minimally invasive techniques—medical and surgical procedures that use small incisions and miniaturized cameras and tools—whenever we can. In fact, Boston Children's has a Center for Minimally Invasive Surgery that is a global leader in creating and refining new surgical approaches, and our Interventional Catheterization Program has given our clinicians an important new alternative to open-heart surgery for many children with heart conditions. 

Your child's exact treatment plan will be determined by:

  • what other heart or lung problems—if any—he may have
  • his symptoms and overall health
  • how many of the four pulmonary veins are affected
  • the extent of narrowing within the pulmonary veins
  • the preferences expressed by you and your family

Children with pulmonary vein stenosis typically need one or more of the following procedures to combat the harmful narrowing process and restore adequate flow of oxygen-rich blood to the heart. In many cases, multiple interventions will be necessary over time, since the narrowing process can recur and become more pronounced even after catheterization or surgery.

Treatments for pulmonary vein stenosis

Interventional catheterization

Boston Children's has a program dedicated to interventional catheterization, the use of a thin tube called a catheter that is threaded from a vein or artery into the heart. The catheter can be used to fix holes and structural defects, open narrowed passageways (like those within the pulmonary veins) and create new passageways if needed.

The types of interventional catheterization used to treat pulmonary vein stenosis at Boston Children's are balloon dilation and balloon dilation and stent placement.

Balloon dilation

The most common interventional catheterization procedure used here at Boston Children's is balloon dilation. With the child under sedation, a small, flexible catheter is inserted into a blood vessel, most often in the groin. Using tiny, highly precise cameras and tools, clinicians guide the catheter up into the inside of the heart and then into the affected areas of the pulmonary veins. A deflated balloon at the tip of the catheter is inflated once the tube is in place, and this balloon stretches the constricted area open, reversing the problematic narrowing.

Balloon dilation and stent placement

The effects of balloon dilation can be amplified for some children with pulmonary vein stenosis by using a combination of balloon dilation and the placement of a balloon-expandable stent—a small, stainless steel tube. The stent is attached to the balloon dilation catheter as it is fed into the narrowed parts of the pulmonary veins.

The protective covering is removed when the catheter is in place, and the balloon is then inflated. The balloon affixes the stent within the pulmonary vein, stretching the narrowed area and propping it open.

Children may need several interventional catheterizations over time as they grow, since the narrowing process tends to recur and can become progressively worse.

Open-heart surgery

Some children may need open-heart surgery to sufficiently widen the narrowed areas of the pulmonary veins and allow a return of healthy oxygenated-blood flow from the lungs to the heart. Learn more about heart surgery at Boston Children's.

Lung transplants

In some cases, the extent of damage caused by pulmonary vein stenosis is too great to be reversed and cannot be managed adequately with interventional catheterization or surgical correction. For these children, a lung transplant to replace the lungs and pulmonary veins may be the only avenue of treatment. Learn more about Boston Children's Lung Transplant Program.

Coping and support

When your child has pulmonary vein stenosis, you are faced with many concerns and questions. Not only are you focused on meeting all of your child's medical needs; you are also confronting a significant emotional and psychological challenge that can affect every member of your family.

In addition to the clinical information offered on this page, Boston Children's has several other resources designed to give your family comfort, support and guidance:

  • Boston Children's Behavioral Medicine Clinic helps kids who are being treated on an outpatient basis at the hospital—as well as their families—understand and cope with their feelings about:
    • being sick
    • facing uncomfortable procedures
    • handling pain
    • taking medication
    • preparing for surgery
    • changes in friendships and family relationships
    • managing school while dealing with an illness
    • grief and loss
       
  • The Cardiac Experience Journal was designed by Boston Children's psychiatrist-in-chief David DeMaso, MD, and members of his team. This online collection features thoughts, reflections and advice from kids and caregivers about going through cardiac disease, heart transplants and many other medical experiences.
  • Boston Children's Psychiatry Consultation Service is comprised of expert and compassionate pediatric psychologists, psychiatrists, social workers and other mental health professionals who understand the unique circumstances of hospitalized children and their families. The team provides several services, including:
    • short-term therapy for children admitted to one of our inpatient units
    • parent and sibling consultations
    • teaching healthy coping skills for the whole family
    • educating members of the medical treatment team about the relationship between physical illness and psychological distress
             
  • Boston Children's Center for Families is dedicated to helping families locate the information and resources they need to better understand their child's particular condition and take part in their care. All patients, families and health professionals are welcome to use the center's services at no extra cost. The center is open Monday through Friday from 8 a.m. to 7 p.m., and on Saturdays from 9 a.m. to 1 p.m. Please call 617-355-6279 for more information.
  • The Boston Children's chaplaincy is a source of spiritual support for parents and family members. Our program includes nearly a dozen clergy members—representing Episcopal, Jewish, Lutheran, Muslim, Roman Catholic, Unitarian and United Church of Christ traditions—who will listen to you, pray with you and help you observe your own faith practices during your child's treatment.
  • For children and families affected by life-threatening illness, our Pediatric Advanced Care Team (PACT) is available to provide supportive treatments intended to optimize the quality of life and promote healing and comfort. In addition, PACT can provide emotional support and help arrange end-of-life care when necessary. Please call 617-632-5042 for more information.
  • Boston Children's Integrative Therapies Team provides a number of services for hospitalized children, their families and their caregivers, including:
    • massage therapy
    • acupuncture
    • yoga
    • therapeutic touch
       
  • Children's International Center is a dedicated resource for patients and families from countries outside the United States. The center can provide assistance with everything from reviewing medical records to setting up appointments and locating lodging. Contact the center by phone at 01-617-355-5209 or via e-mail at international.center@childrens.harvard.edu.

View a general guide for Boston Children's patients and their families.

Helpful links

Please note that neither Boston Children's Hospital nor the Pulmonary Vein Stenosis Program at Boston Children's unreservedly endorses all of the information found at the sites listed below. These links are provided as a resource.

Helpful links for parents and families

Helpful links for teens

Helpful links for younger children


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- Sandra L. Fenwick, President and CEO

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