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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.

     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.

    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.

    Did you know?

    Boston Children's is home to a non-invasive cardiac imaging program. 

    Pulmonary vein stenosis: Reviewed by Doff McElhinney, MD
    © Boston Children's Hospital, 2010

  • 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 below depicts 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

    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

    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.

    FAQ

    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

    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?

    Boston Children's cardiology, cardiac surgery programs ranked top in the nation by U.S. News & World Report

    Learn more about our ranking.

    Lung transplants at Boston Children's

    Learn more about our approach to lung transplants

  • How is pulmonary vein stenosis diagnosed?

    Pulmonary vein stenosis is typically diagnosed with one or more of the following procedures:

    • an echocardiogram, which uses sound waves to make a moving picture of the heart’s structure and function
    • cardiac catheterization, which uses a small, flexible tube threaded up to the heart to give very detailed information about the structures within the heart and lungs
    • a computed tomography (CT) scan, a non-invasive procedure using X-ray equipment and powerful computers to produce detailed, cross-sectional images of a particular area of the body (such as the chest cavity)
    • a pulmonary blood flow scan, or lung scan, which uses an injectable dye and a special camera to track the flow of blood through your child’s lungs

    Did you know?

    You can keep family and friends up to date during your child’s treatment by creating a free Boston Children’s Carepage.

  • 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

  • At Boston Children's Hospital, our care is informed by our research, and our discoveries in the laboratory strengthen the care we provide at each child's bedside. Boston Children’s scientific research program is one of the largest and most active of any pediatric hospital in the world.

    In particular, our cardiovascular and cardiac surgery researchers are yielding crucial insights into the causes and development of heart defects and disorders; paving ground for the most promising new treatments and interventions; and offering ideas and discoveries that could lead to cures.

    Among our current research projects with promise for treating pulmonary vein stenosis is an exciting study:

    Treating pulmonary vein stenosis by targeting abnormal cell growth

    Boston Children’s and the Dana-Farber Cancer Institute are conducting a research study in patients who have multi-vessel (occurring in at least two blood vessels), intraluminal (affecting the lumen, or openings into the blood vessels) pulmonary vein stenosis. They aim to determine whether two drugs that target certain tumors may also be effective in controlling the abnormal cell growth that characterizes this type of pulmonary vein stenosis.

    Did you know?

    Boston Children's helps adults with congenital heart problems, too. 

    Clinical trials

    Boston Children’s is known for pioneering some of the most effective diagnostic tools, therapies and preventive approaches in pediatric cardiology. A significant part of our success comes from our commitment to research—and to advancing the frontiers of mental health care by conducting clinical trials.

    Boston Children’s coordinates hundreds of clinical trials at any given time. Clinical trials are studies that may involve:

    • evaluating the effectiveness of a new drug therapy
    • testing a new diagnostic procedure or device
    • examining a new treatment method for a particular condition
    • taking a closer look at the causes and progression of specific diseases

    Boston Children’s is involved in several multi-site clinical trials and studies focusing on pediatric cardiology and cardiac surgery, in particular. While children must meet strict criteria in order to be eligible for a clinical trial, your child may be eligible to take part in a study. Before considering this option, you should be sure to:

    • consult with your child’s treating physician and treatment team
    • gather as much information as possible about the specific course of action outlined in the trial
    • do your own research about the latest breakthroughs relating to your child’s condition

    If your physician recommends that your child participate in a clinical trial, you can feel confident that the plan detailed for that study represents the best and most innovative care available. Taking part in a clinical trial at Boston Children’s is entirely voluntary. Our team will be sure to fully address any questions you may have, and you may remove your child from the medical study at any time.

    The role of stem cells in the fight against heart disease

    Learn more about our research in this vital area. 

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