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Aortic valve stenosis

  • We can really make a difference for children with aortic valve stenosis. For many kids, undergoing (catheterization) means they're very likely to get through childhood without ever needing surgery on their aortic valve, and that's tremendously satisfying.

    --Audrey Marshall, MD, cardiologist, Boston Children's Hospital

    The human heart is like a busy factory with two strong pumps: The ventricles, and two “receiving docks,” called the atria. These chambers work together to ensure that oxygen-rich blood moves out into the body, and that de-oxygenated blood comes back to the heart and lungs in return. Just like any other factory, however, the heart’s essential functions can be seriously disrupted if just one piece of machinery is compromised.

    The diagram below depicts the structures of the heart.

    Interior view of the heart

    The aortic valve:

    • is a one-way valve located between the left ventricle—the pump that pushes blood out to the body—and the aorta, the major blood vessel that carries blood to different parts of the body
    • keeps blood from leaking back into the ventricle once it’s been pumped out into the aorta
    • typically has three leaflets, or flaps of tissue that separate to allow blood out of the ventricle when the ventricle pumps—and then fall back together when the ventricle relaxes in order to keep blood from leaking backwards

    aortic stenosis

    When a child has aortic valve stenosis, the leaflets that make up his aortic valve get stuck and can’t separate fully. This causes a problematic blockage that increases the pumping work of the left ventricle, and may lessen the amount of blood that goes out of the ventricle to the body through the aortic valve.

    • In the most severe cases, aortic valve stenosis occurs before a baby is born, and may affect the baby shortly after birth. 
    • Less severe forms of the disease may be “silent”—causing no visible symptoms—until later in life. 
    • Aortic valve stenosis can occur by itself, or as part of a group of abnormalities affecting the left side of the heart.
    • Rarely, children can develop aortic stenosis as a complication of rheumatic fever.
    • Although a physical exam and an electrocardiogram may suggest a possible diagnosis of aortic valve stenosis, an echocardiogram is the definitive test to confirm the condition. Echocardiograms are painless, do not require an IV and only take about an hour.
    • Some children don’t need treatment until they are older, and some may not require any intervention at all during their childhood. Children with no outward symptoms can do very well for a long time with only regular monitoring by their clinical team.
    • Babies with severe aortic valve stenosis are likely to require interventional catheterization or valve surgery.
    • Aortic valve stenosis can affect each child differently. Your physician is always the best resource for providing detailed information about your child’s individual situation, and for recommending treatment options that best meet the needs of your child and your family.

    The detailed information on the following pages will help you, your child and your family gain a better understanding of aortic valve stenosis and a clearer picture of what to expect in the weeks and months ahead.

    How Boston Children's Hospital approaches aortic valve stenosis

    Boston Children's Hospital Cardiovascular Program team has extensive experience treating fetuses, babies, children, adolescents and adults with aortic valve stenosis. Thanks to available diagnostic procedures and imaging technology, the condition can be detected even when the only noticeable symptom is a soft heart murmur. This gives clinicians the opportunity to start monitoring kids with aortic valve stenosis right away, and to easily “stay on top of” the condition’s progression with regular echocardiograms and exams.

    Here at Boston Children’s, our specialized training in pediatric cardiology means that we understand the unique challenges, circumstances and intricacies of working with young people who have heart problems like aortic valve stenosis. 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. Every year, we treat thousands of patients who are living with aortic valve stenosis and a broad spectrum of other cardiac problems. We use sophisticated diagnostic and imaging procedures, including echocardiography and interventional catheterization, and offer dozens of specialized services in such areas as heart valve replacement, cardiac anesthesiarobotic surgery and fetal cardiology

    Our Department of Cardiology and Department of Cardiac Surgery clinicians will 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. 

    Watch a valve implantation procedure at Boston Children's
    View the webcast.

    Aortic valve stenosis: Reviewed by Audrey Marshall, MD
    © Boston Children's Hospital, 2010

  • The term “stenosis” describes an abnormal narrowing within a structure of the body. Aortic valve stenosis, therefore, refers to the narrowing of the heart’s aortic valve. 

    To picture what happens if your child has aortic valve stenosis, it’s helpful to have a solid understanding of the basic anatomy of the heart. The below diagram shows the heart's structures (click to enlarge).

    • Aortic valve stenosis is a narrowing that prevents the aortic valve from opening all the way. This can stop blood from flowing properly out of the heart, into the aorta and throughout the rest of the body.
    • In many cases, aortic valve stenosis is caused by a heart defect that is present at birth.
    • More rarely, children can develop aortic valve stenosis as a complication of rheumatic fever. Although a physical exam and an electrocardiogram may suggest a possible diagnosis of aortic valve stenosis, an echocardiogram is the definitive test to confirm the condition. Echocardiograms are painless, do not require an IV and only take about an hour.
    • Some children with aortic valve stenosis don’t need immediate treatment at all. Children with no outward symptoms can do very well for a long time with only regular monitoring by their treatment team.
    • Children with more advanced aortic valve stenosis are likely to require interventional catheterization or valve repair or replacement surgery. 

    Interior View of the Heart

    The parts of the heart affected by aortic valve stenosis are:

    • the left ventricle, one of two ventricles (chambers) that push blood to all of the body’s organs except the lungs (which are supplied by the right ventricle)
    • the aorta, the largest artery in the human body, which runs from the heart’s left ventricle all the way into the abdomen and is responsible for pumping blood out of the heart and into the rest of the body.
    • the aortic valve, which is located between the left ventricle and the aorta and directs blood from the left ventricle into the aorta (but not back the other way)

     When a child has aortic valve stenosis:

    • the heart has to work harder to pump blood into the body
    • the extra work can weaken the heart over time
    • the heart may only be able to push a reduced amount of blood through the body

    What causes aortic valve stenosis in children?

    Aortic valve stenosis in children is normally caused by either:

    • a birth defect, which can be any of the following:
      • a narrowed aortic valve
      • a condition called a bicuspid aortic valve, meaning that the child is born with an aortic valve that has only two leaflets instead of the usual three
      • valve leaflets that are fused together
      • valve leaflets that are unusually thick and do not open all the way

    Learn more about how Boston Children's treats congenital heart defects. or

    • rheumatic fever, a complicated disease that can develop in children already suffering from an infection of streptococcus bacteria, like strep throat or scarlet fever.

      Rheumatic fever is very rare in the United States today. It can lead to scarring of the tissue in the aortic valve, causing the valve to become constricted and narrowed. This scarring can also increase the likelihood of calcium deposits building up along the valve—a major risk factor for aortic valve stenosis in adulthood. 

    Types

    What are the types of aortic valve stenosis?

    The condition is classified according to its severity: mild, moderate, severe or critical.

    Mild aortic valve stenosis
    A child with mild aortic valve stenosis has very limited narrowing within the valve. These children will not show any outward symptoms; the only detectable problem is a pronounced, easily identified heart murmur. Children with this mild type of aortic valve stenosis are otherwise healthy and able to go about their daily lives without disruption.

    Moderate aortic valve stenosis
    Children with moderate aortic valve stenosis have a slightly more significant narrowing of the aortic valve, but usually show no outward symptoms and are otherwise healthy. A child with this type will have an easily detected and identified heart murmur. 

    Severe aortic valve stenosis
    A child with severe aortic valve stenosis has such an advanced degree of narrowing in the valve that the left ventricle may become very stiff.

    Children with this type may also experience atrial fibrillation, an abnormal contraction of the heart’s ventricles caused by erratic electrical signals coming from the heart’s atria. Interventional catheterization or valve repair or replacement surgery are necessary to treat severe aortic valve stenosis.

    Critical aortic valve stenosis
    This, the most serious type of aortic valve stenosis, is usually present at birth. The newborn’s aortic valve is so narrowed that the heart cannot pump enough blood to nourish the body, so immediate intervention is needed—usually by surgically replacing the aortic valve.

    Signs and symptoms

    What are the symptoms of aortic valve stenosis?

    Many children with aortic valve stenosis show no outward symptoms since they are in the mild to moderate stages of the condition. Usually, the only identifiable symptom in these cases is a pronounced heart murmur

    As aortic valve stenosis progresses, children may experience:

    • fainting
    • shortness of breath or difficulty breathing
    • irregular heartbeats or heart palpitations
    • chest pain
    • dizziness during or immediately after physical activity
    • fatigue

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

    FAQ

    Q: Will my child be OK? How much will aortic valve stenosis disrupt her life?
    A:
    Your child’s long-term health and course of treatment depend greatly on her unique circumstances, especially:

    • her age at the time of diagnosis
    • how narrowed the aortic valve has become at the time of diagnosis
    • her symptoms
    • her tolerance for specific medications or procedures
    • your family’s preferences for treatment

    Children with mild or moderate aortic valve stenosis may not need any immediate intervention, and can do well for long periods of time with regular monitoring and checkups by their treatment team.

    If your child has a serious case of aortic valve stenosis, she will need interventional catheterization or valve surgery. However, the good news is that the majority of children who undergo these procedures for aortic valve stenosis make a full recovery and go on to live healthy, normal adult lives.

    Q: Is aortic valve stenosis a common heart problem? Are some children more likely to develop it than others?
    A: Aortic valve stenosis makes up about 3 to 6 percent of all cases of congenital heart conditions. Four times as many boys as girls are born with aortic valve stenosis.

    Aortic valve stenosis caused by complications of rheumatic fever is less common, especially since rheumatic fever itself is becoming increasingly rare in the United States. 

    Q: Does my child need to take antibiotics before routine medical or dental procedures?
    A: 
    A child with aortic valve stenosis may need to periodically take antibiotics in order to prevent an infection called bacterial endocarditis, even if his aortic valve has been surgically replaced. 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 aortic valve 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 aortic valve 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?
    A: Whether your child needs to cut back significantly on activities depends greatly on her age, how narrowed the aortic valve has become at the time of diagnosis and what symptoms (if any) she is experiencing. 

    If your child has mild aortic valve stenosis with no outward symptoms, she may not need to change her daily activities and can continue to take part in sports and recreational play. If your child has more a pronounced narrowing of the aortic valve, she may need to limit activities that call for a higher level of exertion. If your child has a serious case (or is preparing to undergo interventional catheterization, surgical valve repair or valve replacement), she may need to stop certain high-impact, high-stress activities.

    Your child’s treating clinician will evaluate her individual situation and advise you of any necessary restrictions or changes in physical activity.

    Q: Is my child at risk of having a heart attack?
    A: One of the most common misconceptions about aortic valve stenosis is that a child diagnosed with the condition is at high risk of a sudden heart attack. 

    Most children with aortic valve stenosis are diagnosed when they are still comfortable and free of outward symptoms. Thanks to available diagnostic procedures and imaging technology, the condition can be detected even when the only noticeable symptom is a heart murmur. This gives clinicians the opportunity to start monitoring kids with aortic valve stenosis right away, and to easily “stay on top of” the progression of the condition with regular echocardiograms and exams.

    Newborns with critical aortic valve stenosis tend to have much more serious symptoms and much more severe narrowing of the valve at the time of diagnosis. However, sophisticated imaging and intervention procedures—including interventional catheterization and valve replacement—enable clinicians to take immediate action.

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

    You should seek medical help immediately if your child experiences:

    • fainting
    • heart palpitations or an irregular heartbeat
    • shortness of breath or difficulty breathing
    • chest pain
    • clammy skin or the feeling of a “cold sweat”
    • dizziness
    • pale, ashen color in the face
    • racing heartbeat
    • rapid breathing
    • swollen feet or legs

    Q: Can Boston Children’s treat a baby in the womb who has been diagnosed with aortic valve stenosis?
    A: Yes. Boston Children’s Fetal Cardiology Program can:

    • diagnose, or confirm an earlier diagnosis, of heart defects using fetal ultrasound, echocardiography or magnetic resonance imaging (MRI)
    • work with families to devise customized treatment plans
    • perform fetal interventions such as aortic valve dilation, atrial septum dilation and pulmonary valve dilation

    Q: Does Boston Children’s offer genetic testing for aortic valve stenosis?
    A:
    Yes. Boston Children’s  Cardiovascular Genetics Programprovides several clinical services to test for heart conditions, including aortic valve stenosis, and also offers genetic counseling. 

    Q: Is there a cure for aortic valve stenosis?
    A: In general, aortic valve stenosis is a progressive condition, meaning that the valve will continue to narrow over time and further intervention will eventually become necessary.

    The good news is that, even when aortic valve stenosis is advanced, interventional catheterization and valve surgery both have an excellent success rate for restoring normal heart function and blood flow. 

    Questions to ask your doctor

    You and your family play an essential role in your child’s treatment for aortic valve 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 aortic valve 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 will 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.)
     
    Initial questions to ask your doctor might include: 

    • How did you arrive at this diagnosis?
    • Are there any other conditions my child might have instead?
    • Does my child require further testing or procedures?
    • How advanced is the aortic valve stenosis?
    • What is my child’s prognosis?
    • What medications will you prescribe and what are possible side effects?
    • Is interventional catheterization necessary at this time?
    • Will my child need valve surgery? If so, what will the surgery entail?
    • How should I talk to my child about this condition?
    • How should I explain my child’s condition to others?
    • 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? 
  • How is aortic valve stenosis diagnosed?

    The process of arriving at a diagnosis of aortic valve stenosis usually involves several steps.

    Often, a clinician will first notice that your child has a heart murmur is simply 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 or with an electrocardiogram (EKG or ECG), a test that records the heart’s electrical activity and shows any abnormal rhythms or unusual stress. Sometimes, the murmur may emerge when the child is being tested or treated for another condition altogether.

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

    (Did you know heart defects can even be detected when a baby is still in the womb? Learn more about how Boston Children’s monitors fetal heartbeats.)

    Although exams and electrocardiograms can suggest the possibility of aortic valve stenosis, an echocardiogram is the definitive test used to confirm the diagnosis. An echocardiogram is a special procedure that uses ultrasonic sound waves to create images of the heart and its structures. Echocardiograms are painless, do not require an IV and only take about an hour to perform.

    Other tests your child’s clinician might order to make, or rule out, a diagnosis of aortic valve stenosis can include:

  • Boston Children's Hospital's cardiologists, cardiac surgeons and nurses, cardiac imaging professionals and other clinicians have years of expertise in treating all types of heart defects and heart disease, with specialized understanding of problems like aortic valve stenosis that affect the valves of the heart. 

    Our team treats every stage of aortic valve stenosis in children, adolescents and adults, as well as babies in utero. We use minimally invasive techniques—medical and surgical procedures that use small incisions and miniaturized cameras and tools—whenever we can, and are committed to repairing a child's own valve rather than resorting to an entire valve replacement whenever possible. Boston Children's Interventional Catheterization Program has made great strides in allowing children to keep their own heart valves for as long as possible during treatment.

    Your child's exact treatment plan will be determined by his age, the extent of narrowing in his aortic valve, his overall health and your and your family's preferences.

    Treatments for aortic valve stenosis

    Monitoring

    Children with mild to moderate aortic valve stenosis may not require any treatment other than routine monitoring in the short term. 

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

    Medication

    Medication is not a cure for aortic valve 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 aortic valve stenosis may also need to periodically take antibiotics in order to prevent an infection called bacterial endocarditis, even if his aortic valve has been surgically replaced. 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 aortic valve 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 aortic valve 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).

    Learn more about commonly prescribed medications.

    Interventional catheterization/balloon valvuloplasty

    Boston Children's has a comprehensive 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. This catheter can be used to fix holes in the heart, open narrowed passageways (like the aortic valve) and create new passageways.

    Recognizing the benefits of this minimally invasive treatment—less discomfort, shorter recovery periods and the use of the child's own valve, which will grow with the child after the procedure—Boston Children's considers interventional catheterization the preferred frontline approach to aortic valve stenosis.

    The most common interventional catheterization procedure used to treat aortic valve stenosis is balloon dilation, also known as balloon valvuloplasty. With a child is 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 across the aortic valve. A deflated balloon at the tip of the catheter is inflated once the tube is in place, and this balloon stretches the aortic valve open, reversing the problematic narrowing. 

    Valve replacement surgery

    For children with severe aortic valve stenosis, balloon valvuloplasty may not be able to adequately fix the narrowed valve and restore healthy heart function. In other cases, as the child grows, an aortic valve that was previously treated successfully with one or more balloon dilation procedures begins to narrow again, adding strain to the heart and affecting blood flow throughout the body. Replacement of the aortic valve is the next step in treatment for these children.

    During this procedure, cardiac surgeons will remove the damaged aortic valve and replace it with either

    • a mechanical valve, made of metal and other synthetic materials. Mechanical valves can last for more than 20 years before needing to be replaced. There is a small risk of blood clot formation associated with this procedure, so children are typically prescribed a blood-thinning medication to prevent complications.

    or

    • a donor valve taken from a human or animal donor (sometimes, new valves can even be fashioned from the child's own tissue). This type of procedure is a more recent advance, and the long-term durability of donor valves is still being determined. The results, however, look promising. Donor valve replacement does not appear to be associated with any notable risk of blood clot formation, so children normally do not need blood-thinning medications after this variation of the surgery.

    Both types of valve replacement surgery have an excellent success rate and a low incidence of complications. Children who undergo either type of valve replacement procedure are likely to enjoy normal, healthy adult lives with minimal to no restrictions on playing sports or engaging in other strenuous activities. 

    After valve replacement surgery

    Most children need to stay in the hospital for a week to 10 days after valve replacement surgery, and will need another three to six weeks of rest at home before returning to their normal daily activities.

    Children with implanted valves—just like children with aortic valve stenosis, in general—can be more susceptible to bacteria entering the bloodstream. The bacteria can cause a serious infection called bacterial endocarditis. As a result, these children might need a regimen of antibiotics before undergoing some medical procedures. If your child has undergone valve replacement surgery, you should always inform medical personnel of your child's condition before arranging for any procedure.

    It is likely that your child will need another valve replacement surgery within 20 years as the donor valve ages. 

    Coping and support

    When your child is living with aortic valve stenosis, your family may have many concerns, worries 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, 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 international.center@childrens.harvard.edu.

    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
  • 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 discovering insights into the causes and development of heart defects and disorders; paving ground for the promising new treatments and interventions; and offering ideas and discoveries that could lead to better treatments and cures.

    Learn about our ongoing research efforts. 

    Among our current research projects with promise for treating aortic valve stenosis are:

    Reversing heart damage with a simple injection

    A team of 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 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.

    The role of stem cells in the fight against heart disease

    Learn more about our research in this vital area. 

    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, Boston 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 our timeline of Boston Children’s innovations in heart care.

  • Ann Louise's story

    The first thing you notice about Ann Louise Atherton (her real name is Hazel, but everyone calls her Ann Louise) is her eyes: playful, brilliant blue and sparkling with humor. When she throws a toy and you pick it up, she beams, knowing she’s hooked you into playing fetch. Sitting in her Princess stroller and playing with her mother’s iPhone, a small catheter tube by her hip is the only sign of the risky, life-saving heart surgery that Ann Louise went through before she was even born.

    Early in her pregnancy, Mary Beth Atherton and her husband, Andrew, learned that Ann Louise’s still-developing heart was showing signs of critical aortic stenosis, a congenital defect that prevents the aortic valve from opening completely. If left unrepaired, aortic stenosis causes the left ventricle to work harder to get blood to the body. Over time, the left ventricle becomes thicker and pumps blood at a higher rate, damaging the heart while failing to pump blood efficiently. At home in North Carolina, Mary Beth’s doctors warned her that repairing her baby’s heart after birth would involve several risky surgeries, and would likely result in serious mental delays—if Ann Louise survived at all. Despite the bleak prognosis, they also advised Mary Beth and Andrew against in utero surgery, warning of low survival rates and poor quality of life for the baby. They recommended preparing for the worst.

    “We found Boston Children's Hospital on our own,” Mary Beth says. Andrew called Gerald Marx, MD, senior associate in Cardiology, directly and spoke to him for two hours about Ann Louise’s chances if they came to Children’s for in utero heart surgery. Mary Beth’s doctors advised strongly against the trip, but Marx made a convincing case. “After talking to Dr. Marx, we didn’t even need to think about it,” says Mary Beth. “He gave us his pager and said to call him directly when we made up our minds. We ended up paging him on Thanksgiving, but he called back immediately and said ‘come to Boston…now!’” Two days and one Med Flight later, Boston Children’s surgeons operated on Ann Louise in utero, repairing her aorta.

    Ann Louise was due on March 24, 2008, but she arrived a month early and spent the first five weeks of her life in the Cardiac Intensive Care Unit. It was there that she first met her brothers, Graham and Reed, who traveled to Boston Children’s to meet their new baby sister. “They had to keep her sedated because she was still healing from surgery,” says Mary Beth. “But when Graham held her hand, she squeezed back.”

    Since her first surgery, Ann Louise has returned to Boston Children’s 12 times for additional surgeries and check-ups. She and Mary Beth have spent more than 200 days in the hospital, and Ann Louise now looks forward to seeing the Cardiology team. When given a picture of Marx, she laughs and blows kisses at him. “He’s been so good to us,” she says. “Ann Louise thinks he’s part of our family.” Mary Beth also credits the Boston Children’s Center for Families with “helping keep us sane” through the long visits.

    Last month, Boston Children’s surgeons replaced Ann Louise’s mitral valve; her last major surgery, for now. The 18-month-old will need her mitral valve replaced again when she’s 10, and her pulmonary valve will need to be replaced when she’s in her early teens. “The doctors we saw before coming to Boston Children’s said Ann Louise wouldn’t live past 25 if we came to Boston for in utero surgery,” Mary Beth says. “The doctors at Boston Children’s told us there’s no realistic basis for that statistic. This surgery’s only been done for 20 years! For all anyone knows, Ann Louise could live a normal, long life.”

    For now, Mary Beth and Andrew are looking forward to bringing home a happy, healthy little girl who loves chewing on her toes, watching the Imagination Movers and flirting with doctors. “When we contacted Boston Children’s, there was such a difference in how they approached things,” says Mary Beth. “We’d been told that Ann Louise had a 25 percent chance of dying. Dr. Marx said there was a 90 percent chance that she’d live. That’s what gives you hope.”

    - Mary Beth Atherton

    Genevieve's story

    “We see something with your daughter’s heart — it is probably nothing, but we would like to refer you to Boston Children's Hospital for an echocardiogram.”

    I can still hear those words. After struggling with infertility, we were blessed with our twins and now there was potentially something wrong with them. What does this mean? Did we do something wrong? Did we cause this?  It is probably nothing, right?

    We called the Advanced Fetal Care Center to schedule an echocardiogram. The appointment seemed to go on forever — we watched the colors dancing on the screen that represented the blood flow. The ultrasound technician scanned both hearts — that of my son and my daughter — just in case. We found ourselves studying the face of the technician, hoping to see a glimpse of whether things were good or bad. My husband and I were both waiting for them to say that it was nothing.

    We then went to another room to wait for the doctor. Dr. Ronald Lacro came in to talk to us. He brought with him a book from the American Heart Association called If Your Child has a Congenital Heart Defect. He showed us pictures and went over our daughter’s diagnosis — aortic stenosis. 

    Dr. Lacro took the time to explain the diagnosis and did his best to answer our questions but a lot of it would remain unknown until after she was born. My husband liked his direct, honest approach, while I was in tears, and nothing that he could have said with the exception of “everything looks perfect” would have been a comfort to me. Dr. Lacro left us with the book, his card and an invitation to call or e-mail with anything that we wanted to discuss further. He knew that we were overwhelmed and that a lot of the questions would not come to mind until we had left the hospital.

    Hiss recommendation was to schedule a follow-up echocardiogram so they could monitor her as she got bigger. Wait and see. I was a wreck — what did this mean? And what does it mean for her twin brother?

    For the rest of the pregnancy, we had regular appointments at the Advanced Fetal Care Center. We also had regular appointments with our OB at Brigham & Women’s Hospital. She seemed genuinely surprised that the spot they had seen at our anatomy scan had turned out to be “something” and worked hard to be reassuring and supportive. It was stressful, but uneventful. No major change with her heart, no complications with the pregnancy.

    In the last month of the pregnancy, it was time to start talking about what all of this would mean. Thankfully, we did not require intervention during the pregnancy, but it looked like she would require surgery shortly after birth — a catheterization procedure to widen the narrowed valve in her heart.

    We scheduled appointments to tour the Neonatal Intensive Care Unit (NICU) at Boston Children’s as well as the NICU at Brigham & Women’s. It was heartbreaking and inspiring at the same time. The most beautiful tiny children hooked up to machines and monitors while the most dedicated and positive doctors and nurses do all they can to support life. It was amazing and gave us a bit of hope for what was to come.

    We scheduled our C-section for August 26, 2008. There was comfort in having a scheduled date. Our families could be there to support us. The cardiac team at Boston Children’s was ready and knew that our twins were going to be delivered that day. During the entire pregnancy, our son was presenting as Twin A and our daughter was Twin B. Even during a quick ultrasound right before we went into surgery, our son was Twin A. At 8:11 a.m., Genevieve Anne Norton came into the world. Determined to be born first, she slipped right past her brother — a sign of things to come with our little girl. At 8:12 a.m, Harry Francis Norton made his appearance — now the second born and little brother.

    The first few days were a blur. I saw Genevieve for a moment and then she was gone, transferred to the Cardiac Intensive Care Unit at Boston Children’s. My son Harry and I were recovering at Brigham & Women’s. My poor husband was running back and forth trying to be there for everyone. He was amazing — so strong during all of this. While time seemed to slow for me, he felt like he was in constant motion. At my bedside, holding his son and watching the Red Sox game, then back on his feet running over to Boston Children’s to check on Genevieve and meet with her doctors. And then back again.

    At just a day old, Genevieve underwent her first catheterization procedure. They had been able to open the valve more and she was recovering. Every day, I am so thankful to everyone at both Boston Children’s Hospital and Brigham & Women’s Hospital. Dr. Lacro would call me to let me know how Genevieve was doing, the staff at Boston Children’s made sure that my mom, my father-in-law and anyone else who sat at my daughter’s bedside was comfortable — and the team at Brigham & Women’s always happily took Harry to the nursery and ordered a wheelchair so that my husband could take me through the tunnel that connects the two hospitals to see Genevieve.

    I was discharged with Harry from the hospital on Saturday and Genevieve joined us at home the following Thursday. That first year was tough — a couple of hospitalizations for bronchiolitis — but we got through it.

    Genevieve continues to be monitored by Dr. Lacro and the team at Boston Children’s. She has moderate regurgitation from the valve and will require surgery to repair it. At 15 months old, she had a second catheterization procedure to check the pressure going across the valve. Her heart function continues to look good, so we are putting off the surgery a bit longer. I know that it is best to wait, to give her the chance to be bigger and stronger when going into surgery; but it is still hard to have this hanging over our heads. 

    Every day, I am thankful to be so close to Boston Children’s; to have our concern be how bad traffic will be in the morning when we have her next follow-up appointment! We have met families that come from all over the world — Genevieve’s first roommate when she got out of intensive care was a girl who had flown in from Iceland — and it reminds us that we are truly blessed to have such excellent care right in our own backyard.

    Genevieve is now 20 months old. There is still the cloud of impending surgery, but you would never know it by looking at her. Genevieve and Harry are inseparable; they are the best of friends, hitting all their milestones together and having a great time getting into all kinds of trouble. We have been blessed.

    -- Tina Norton

    Stacy's story

    My daughter, Stacy, had a brain tumor and was treated by Boston Children's Hospital from 1975–1978. Sadly, she passed away on November 1, 1978, a month before her seventh birthday. She and our whole family received wonderful care at Boston Children’s, and the numerous doctors who treated Stacy went above and beyond the call of duty while attempting to save her life.

    My other daughter, Michelle, who is now 35 years old, was born with a hole in her heart and aortic stenosis (narrowing of aortic valve). Again, we were treated with utmost care. Michelle chose to stay at Boston Children's and is currently followed there even into her adult years because of the comfort zone it provided for her.

    We had a scare with our “baby” (now 18), who was born nine weeks early and suffered bleeding in the brain. She was brought into Children's and put through various tests, after which they decided it had only been mild bleeding and reassured us that it had resolved. (Ironically, she was seen by the same neurologist and neurosurgeon that our first daughter had 20 years prior).

    She had gross motor delays, so was followed by the Early Intervention team for three years. Even though she was delayed with all her motor skills, she went on to become a championship Irish step dancer! She's now in her first year of college at the University of New Hampshire and is doing great.

    So, that's our family's history with Boston Children's Hospital. I don't hesitate to recommend it to anyone who's looking for medical help for their child.

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