Peripheral Pulmonary Stenosis

  • I've seen such a difference in pediatric cardiology over the past 25 years. Not only are kids with heart disease surviving and thriving, but they're also coming back to visit us and have children of their own. It's really wonderful.

    --Cheryl O'Connell, MBA, BSN, RN, Boston Children's Cardiovascular Program nurse

    Hearing the term peripheral pulmonary stenosis applied to your child can frightening and hard to accept. You may find yourself struggling with a seemingly endless list of questions: What caused this condition? How will it change my child's life? Is surgery necessary? How will this diagnosis affect my family? Where do we go from here? 

    Learning more about peripheral pulmonary stenosis—which is also sometimes referred to as "pulmonary branch stenosis" or "branch pulmonary artery stenosis"—can help you better understand what's next and what to expect. 

    The following pages will introduce you to the basics about the condition.

    • Peripheral pulmonary stenosis refers to narrowing in one or several areas of the branches of the pulmonary arteries. This is a different condition from pulmonary (valve) stenosis (PVS, PS) and pulmonary vein stenosis.
    • Peripheral pulmonary stenosis may be a progressive condition, meaning symptoms can increase and become more obvious and disruptive over time.
    • Many children in the mild stages of peripheral pulmonary stenosis do not show any outward symptoms.
    • Sometimes, peripheral pulmonary stenosis occurs as a complication of rare heart defects or certain genetic syndromes.
    • It can also occur if a child is born without a pulmonary valve.
    • Children with more severe peripheral pulmonary stenosis are likely to need interventional catheterization. Most do very well after undergoing this type of procedure.
    • Children with peripheral pulmonary stenosis are at risk of having insufficient oxygen within their blood, placing increased pressure on the heart. This makes the heart have to work harder.
    • Peripheral pulmonary stenosis can affect each child differently. Your physician is the best resource for providing detailed information about your child’s individual situation. Your clinician and treatment team will recommend the treatment options that best meet the needs of your child and your family.

    How Boston Children's Hospital approaches peripheral pulmonary stenosis

    The Boston Children's Hospital Cardiovascular Program team has extensive experience treating children, adolescents and adults with peripheral pulmonary stenosis.

    Our specialized training in pediatric cardiology means that we understand the particular challenges, circumstances and intricacies of working with young people with 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, Boston Children’s operates the largest pediatric heart program in the nation.
    • We use the most sophisticated diagnostic and imaging procedures, including interventional catheterization,and offer dozens of specialized services in such areas as heart valve replacement, cardiac anesthesia, robotic surgery and fetal cardiology.
    • 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.

    Boston Children's cardiology, cardiac surgery programs ranked #1 in the nation by U.S. News & World Report.

     Peripheral pulmonary stenosis: Reviewed by Doff McElhinney, MD
    © Boston Children's Hospital, 2010

  • Learning more about peripheral pulmonary stenosis can help you better understand what's next for your child and family, and what to expect in the days and weeks ahead.

    To picture what happens when a child has peripheral pulmonary stenosis, it’s helpful to have a solid understanding of the heart's anatomy. 

    interior view of the heart The pulmonary arteries:

    • are responsible for carrying blood from the heart to the lungs
    • are the only arteries in the human body (besides the umbilical cord arteries that nourish babies in the womb) carrying blood that has been de-oxygenated—meaning the oxygen has been delivered throughout the body where it is needed, and has been replaced by carbon dioxide

    You can think of the pulmonary arteries as a large tree with the following parts:

    • the main pulmonary artery, or “trunk,” that reaches from the right ventricle of the heart into the two pulmonary branches
    • the pulmonary branches—one on the left and one on the right—each of which branches out many times into the corresponding lung

    The term “stenosis” describes an abnormal narrowing within a structure of the body. Peripheral pulmonary stenosis, therefore, describes narrowing in one or more areas within the pulmonary artery branches. This condition should not be confused with pulmonary (valve) stenosis (PVS, PS) or pulmonary vein stenosis, which are different conditions.

    Boston Children with peripheral pulmonary stenosis are at risk of getting insufficient oxygen within their blood, placing increased pressure on the heart. The condition can affect each child differently, so it’s important to talk to your clinician about your child’s individual circumstances and the recommended treatment options.

    What causes peripheral pulmonary stenosis?

    Peripheral pulmonary stenosis is typically a congenital heart defect—something a child is born with, rather than a disease that is acquired later in life. It can occur:

    • as a complication of Tetralogy of Fallot, a rare condition in which four different heart defects are all present at birth
    • in conjunction with certain genetic syndromes that affect the heart, like Williams Syndrome, Alagille Syndrome and Noonan Syndrome
    • if a child is born with only one large vessel that leads out of the heart, causing the oxygen-rich blood coming from the lungs and the de-oxygenated blood going back to the heart to become mixed; this rare condition is called truncus arteriosus
    • if a child is born without a pulmonary valve; this condition is called pulmonary atresia
    • if the pulmonary valve is narrowed; this is called pulmonary (valve) stenosis (PVS, PS)
    • if the opening between the aorta and the pulmonary artery does not close soon enough after birth; this rare condition is called patent ductus arteriosus
    • as a complication of certain types of infection, like congenital rubella syndrome, that can weaken the heart

    Learn more about how Boston Children’s treats congenital heart defects

    What are the symptoms of peripheral pulmonary stenosis?

    Many children in the mild to moderate stages of peripheral pulmonary stenosis show no visible symptoms. Usually, the only identifiable symptom in these cases is a pronounced heart murmur.

    As peripheral pulmonary stenosis progresses over time, children may display one or more of the following symptoms:

    • fatigue
    • shortness of breath
    • heavy, difficult breathing
    • rapid, shallow breathing
    • rapid or fluttering heartbeat
    • swelling in the feet and ankles
    • swelling in the face
    • swelling in the abdomen

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

    The Cardiac Experience Journal 

    Read the stories of Boston Children’s patients with heart conditions and hear words of wisdom from their parents, siblings and caregivers.


    Q: Will my child be OK?
    Peripheral pulmonary stenosis can vary widely in severity and symptoms from child to child. The condition is sometimes progressive, meaning that symptoms can become more pronounced over time.

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

    • whether she has other heart problems, such as Tetralogy of Fallot
    • the extent of narrowing within the pulmonary branches
    • her tolerance for specific medications or procedures
    • your family’s preferences for treatment

    Children with mild or moderate peripheral pulmonary stenosis may not need any intervention, since they have no noticeable symptoms that disrupt their daily lives. These children can do well for long periods of time with regular monitoring and checkups by their treatment team.

    More serious cases of peripheral pulmonary stenosis will need interventional catheterization. However, the good news is that the majority of children who undergo these procedures do very well and can go on to lead healthy adult lives.

    Q: Will my child be at greater risk for contracting a bacterial infection?
    A child with peripheral pulmonary stenosis may need to periodically take antibiotics in order to prevent an infection called bacterial endocarditis. Bacterial endocarditis can cause serious damage to the inner lining of the heart and its valves.

    Children with heart conditions may be vulnerable to developing endocarditis while undergoing medical procedures that could release bacteria into the bloodstream. In these cases, a regimen of antibiotics might be prescribed before the child has the procedure. You should always let medical personnel know about your child’s peripheral pulmonary stenosis before making arrangements for a medical procedure, even if the procedure seems minor or unrelated to your child’s cardiac care.

    If your child has peripheral pulmonary stenosis, but no other cardiac problems, he probably will not need antibiotics before a routine dental procedure (for example, a teeth-cleaning). Read the American Heart Association’s latest guidelines for dental patients with heart conditions (Adobe Acrobat required).

    Q: Does my child have to cut back on physical activities?
    Whether your child needs to cut back significantly on activities—or cut back at all—depends greatly on whether he has other heart problems, how extensive the narrowing within the pulmonary branches has become and what symptoms (if any) he is experiencing. You should talk to your treating clinician about recommended exercise restrictions or practices for your child.

    Q: Is my child at serious risk of having a heart attack?
    A: One of the most common misconceptions about peripheral pulmonary stenosis is that children with the condition are automatically at high risk of suffering a heart attack. However, thanks to available diagnostic procedures and imaging technology, peripheral pulmonary stenosis can be detected even when the only noticeable symptom is a heart murmur.
    This ability to make an early diagnosis gives clinicians the opportunity to start monitoring kids with peripheral pulmonary stenosis right away, and to easily “stay on top of” the progression of the condition with regular echocardiograms and exams.

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

    You should seek medical help immediately if your child experiences:

    • fatigue
    • shortness of breath
    • rapid heartbeat
    • clammy skin or the feeling of a “cold sweat”
    • difficulty “catching a breath”
    • dizziness
    • fainting
    • heart palpitations
    • pale, ashen color in the face
    • rapid breathing
    • shallow breathing
    • swollen feet or legs
    • swollen eyelids
    • swelling in other parts of the face
    • swelling in the abdomen

    Q: Can Boston Children’s treat peripheral pulmonary stenosis while a baby is still in the womb?
    Yes. Boston Children’s Fetal Cardiology Program provides the most advanced, comprehensive care for expectant parents whose babies are diagnosed with heart defects in utero. Among the services we offer are:

    • diagnosis, or confirmation of an earlier diagnosis, of heart defects using fetal ultrasound, echocardiography or magnetic resonance imaging
    • working with the family to devise customized treatment plans
    • fetal interventions such as balloon dilation

    Q: Does Boston Children’s offer genetic testing for peripheral pulmonary stenosis?
    Yes. Boston Children’s Cardiovascular Genetics Program provides several clinical services to test for heart diseases like peripheral pulmonary stenosis and associated heart problems, such as Tetralogy of Fallot. It also offers genetic counseling.

    Q: Is there a cure for peripheral pulmonary stenosis?
    For some (though not all) children with peripheral pulmonary stenosis, the condition is progressive—meaning that the degree of narrowing within the branches can continue to increase over time, and more involved treatments—usually the use of interventional catheterization—will eventually become necessary. The good news is that interventional catheterization techniques have an excellent success rate for restoring heart and lung function and blood flow. 

    Questions to ask your doctor

    You and your family play an essential role in your child’s treatment for peripheral pulmonary 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 peripheral pulmonary 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 the peripheral pulmonary stenosis caused by another heart condition?
    • Is my child's peripheral pulmonary stenosis linked to a genetic syndrome?
    • Does my child require further testing?
    • How extensive is the narrowing within the pulmonary branches?
    • Is my child’s condition likely to be progressive, with symptoms worsening over time?
    • What is the long-term outlook for my child?
    • What medications will you prescribe and what are the possible side effects?
    • Is interventional catheterization necessary at this time?
    • 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?
    • Does my child need to take antibiotics to protect against a bacterial infection?
    • 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?

    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.

  • How is peripheral pulmonary stenosis diagnosed?

    The process of arriving at a diagnosis of peripheral pulmonary stenosis usually involves several steps. Most often, a clinician will notice that your child has a heart murmur—which is a telltale noise blood makes as it flows from the left ventricle to the aorta. Heart murmurs can be detected with a stethoscope during a routine physical examination, and can also emerge during tests or treatment for another condition or problem.

    The loudness of the murmur, where in the chest it is best heard and the types of noise it causes (e.g., gurgling, blowing) will all give your child’s clinician a better idea of the nature of your child’s heart problem.

    Sometimes, heart defects can be detected when a baby is still in the womb. Learn more about how Boston Children’s monitors fetal heartbeats.

    Upon detection of the heart murmur, your clinician can diagnose peripheral pulmonary stenosis with one or more of the following procedures:

    Minimally invasive cardiac surgery

    Learn more about what Boston Children's is doing in this essential field.

  • Boston Children's pediatric heart team has many years of expertise in treating all types of heart defects and heart disease. 

    Our clinicians have extensive experience in treating every stage of peripheral pulmonary stenosis in children, adolescents and adults, as well as in utero. 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 problems—if any—she may have
    • her symptoms
    • the extent of narrowing within the pulmonary branches
    • her overall health
    • the preferences expressed by you and your family

    Treatments for peripheral pulmonary stenosis


    Children with mild to moderate peripheral pulmonary stenosis may not require any treatment other than routine monitoring in the short term, since they have no noticeable symptoms that affect their daily lives.

    Your child's cardiologist will regularly evaluate your child for any sign of further narrowing in the pulmonary branches and related complications. Most often, routine monitoring will involve physical examinations and echocardiograms.


    Medications is not a cure for peripheral pulmonary stenosis, but can be helpful in managing specific symptoms. In some cases, your child's clinician may prescribe medication to:

    • help the heart maintain healthy function and blood flow
    • control blood pressure
    • prevent abnormal heart rhythms, called arrhythmias

    A child with peripheral pulmonary stenosis may also need to periodically take antibiotics in order to prevent an infection called bacterial endocarditis. Bacterial endocarditis can cause serious damage to the inner lining of the heart and its valves. You should always let medical personnel know about your child's peripheral pulmonary stenosis before making arrangements for a medical procedure, even if the procedure seems minor or unrelated to your child's cardiac care.

    If your child has peripheral pulmonary stenosis, but no other cardiac problems, he probably will not need antibiotics before a routine dental procedure (for example, a teeth-cleaning).

    Learn more about commonly prescribed medications.

    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 in the heart, open narrowed passageways (like those within the pulmonary branches) and create new passageways if needed.

    Many children need several interventions over time as they grow and age. The good news is that these interventional catheterization procedures are very effective and carry a low incidence of complications. Children who have these procedures are likely to enjoy healthy adult lives with minimal to no restrictions on playing sports or engaging in other activities. Since peripheral pulmonary stenosis can affect each child differently, you should always speak directly to your treating clinician for a specific treatment plan, outlook and recommendations for your child.

    The types of interventional catheterization used to treat peripheral pulmonary stenosis at Boston Children's are balloon dilation, balloon dilation and stent placement and the Cutting Balloon device.

    Balloon dilation

    The most common interventional catheterization procedure used here at 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 branches. 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 peripheral pulmonary 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 branches.

    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 branch, stretching the narrowed area and keeping it open.

    The Cutting Balloon device

    In 2008, the United States Food and Drug Administration chose Boston Children's to lead a worldwide, multi-center clinical trial, using a new device called the Cutting Balloon to treat children whose peripheral pulmonary stenosis has not been fixed by standard balloon dilation. 

    The Cutting Balloon is a balloon with three or four miniature blades attached. These blades are able to make small incisions throughout the narrowed areas of the pulmonary branches, helping to open the problematic narrowings.


    Rarely, a child's peripheral pulmonary stenosis cannot be managed adequately with interventional catheterization procedures. For these children, a heart transplant or heart-lung transplant may be necessary.

    Learn more about our transplant programs.

    Coping and support

    When your child has peripheral pulmonary stenosis, your family may have many concerns and questions. Not only are you focused on meeting all of your child's medical needs; you are also grappling with a significant emotional and psychological toll that can affect every member of the 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 Department of Psychiatry offers a free booklet, “Helping Your Child with Medical Experiences: A Practical Parent Guide” (Adobe Acrobat is required). Topics in the booklet include:
      • talking to your child about his or her condition
      • preparing for surgery and hospitalization
      • supporting siblings
      • taking care of yourself during your child's illness
      • adjusting to life after treatment
    • 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
    • Boston 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

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

    Helpful links

    Please note that neither Boston Children's Hospital nor the Department of Cardiology 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

    Did you know?

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

  • 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.

    Learn about our ongoing research efforts.

    Among our current cardiovascular research projects are:

    Reversing heart damage with a simple injection

    A team of Boston Children’s researchers—led by Bernhard Kuhn, MD, of the Department of Cardiology—has demonstrated that a growth factor called neuregulin1 (NRG1) can encourage the growth of muscle and the restoration of function after damage to the heart. Mice who received a simple injection of NRG1 have experienced considerable reversal of pre-existing damage caused by heart attacks.

    Using robotic surgery to treat congenital heart defects

    Boston Children’s cardiac surgeon-in-chief, Pedro del Nido, MD, and his team are exploring new ways of incorporating minimally invasive surgical techniques—in particular, the use of highly sensitive and precise robots in surgery—to correct congenital heart defects affecting the so-called “left heart” (the left atrium and ventricle). Watch video clips of del Nido discussing this work. 

    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 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.

    Follow Boston Children’s long history of innovation in pediatric heart care 

    In 1938, Boston Children’s cardiac surgeon Robert Gross, MD, performed the world’s first successful surgery to correct a child’s heart defect. Since that time, Children’s has gained recognition around the globe for our leadership in pediatric cardiology, and we continue to make critical advances in the field. Follow a timeline of our innovations in heart care. 

    The role of stem cells in the fight against heart disease 

    Did you know? 

    Boston Children’s was the first hospital to implant a catheter-delivered heart valve. Learn more about this achievement 

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