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Mitral Valve Stenosis

  • "When I meet a family, the first thing I say is, 'As parents and clinicians, we are your child's team.' The parents are the ones who know their child best. It's my job to educate parents so that they have the knowledge to make the right decision for their child."

    –Betsy Blume, MD, Children's cardiologist

    The human heart can be compared to a busy factory with two powerful pumps—the ventricles—and two “unloading docks,” called the atria. These chambers maintain a delicate balance, ensuring that oxygen-rich blood moves out into the body and that de-oxygenated blood comes back to the heart and lungs. Just like any other factory, however, the heart’s essential functions can be seriously disrupted if just one piece of machinery is compromised.

    heart diagram The mitral valve is a one-way valve in the left side of the heart that allows blood to fill its “pump,” the left ventricle. When a child has mitral valve stenosis (also known simply as "mitral stenosis"), her mitral valve cannot open fully—creating a problematic blockage. The blockage can stop blood from flowing freely to fill the left ventricle, and as a result, insufficient blood may be pumped out to supply the body’s needs.

    • Many children with mitral valve stenosis do not show any outward symptoms, but some will experience rapid breathing or slow growth and weight gain.
    • Often, mitral valve stenosis is caused by a heart defect present at birth.
    • Children can also develop mitral valve stenosis as a complication of rheumatic fever, though the disease is rare in the United States.
    • Children with more advanced mitral valve stenosis are likely to require interventional catheterization or valve surgery.
    • However, some kids with mitral valve stenosis don’t need any immediate treatment. Many children do very well for a long time with regular monitoring by their treatment team.
    • Mitral valve stenosis can affect each child differently. Your physician is the best resource for providing detailed information about your child’s individual situation and making recommendations about the treatment plan that best meets the needs of your child and your entire family.

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

    How Boston Children's Hospital approaches mitral valve stenosis

    The cardiologists, cardiac surgeons and nurses, cardiovascular imaging professionals and other clinicians in Boston Children’s Hospital  Cardiovascular Program have extensive experience treating mitral valve stenosis in children, adolescents and adults.

    Our specialized training in pediatric cardiology means that we understand the unique challenges, circumstances and intricacies of working with young people who have mitral valve stenosis and other 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. Every year, we treat thousands of patients who are living with mitral valve stenosis and a broad spectrum of other cardiac problems—ranging from congenital heart defects to blood vessel disorders, heart and lung disease and congestive heart failure.

    We use the most sophisticated diagnostic and imaging procedures, including echocardiography and 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 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 

    Mitral 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. Mitral valve stenosis, therefore, describes the narrowing of the heart’s mitral valve.

    To picture what happens when a child has mitral valve stenosis, it’s helpful to understand the basic anatomy of the heart. The diagram below shows the heart's internal structures (click to enlarge).

    heart diagramThe parts of the heart affected by mitral valve stenosis are:

    • the left atrium, one of two chambers that receive blood from veins in the body and then push the blood into the heart’s ventricles. The left atrium, as opposed to the right, receives oxygen-rich blood from the pulmonary veins and feeds this blood to the left ventricle.
    • the left ventricle, one of two chambers that receive blood from the atria and then push that blood further along in the circulatory system. The left ventricle, as opposed to the right, receives oxygen-rich blood from the left atrium and pumps this blood out to supply the body.
    • the mitral valve, a one-way valve with two flaps (called leaflets) that is located between the heart’s left atrium and left ventricle. The mitral valve closes when the ventricle pumps, preventing blood from backing up into the left atrium, and ensures that all of the blood moves out to the body. 

              

    When a child has mitral valve stenosis:

    • blood flow from the left ventricle into the body is limited
    • the left atrium may become enlarged due to the building pressure
    • the lungs may become congested
    • the heart may become weak over time

    Here are some other important things to know about mitral valve stenosis:

    • Many children with mitral valve stenosis do not show any outward symptoms, but some will experience rapid breathing or slow growth and weight gain.
    • Often, mitral valve stenosis is caused by a heart defect present at birth.
    • Children can also develop mitral valve stenosis as a complication of rheumatic fever, though the disease is rare in the United States.
    • Children with more advanced mitral valve stenosis are likely to require interventional catheterization or valve surgery.
    • However, some kids with mitral valve stenosis don’t need any immediate treatment. Many children do very well for a long time with regular monitoring by their treatment team.
    • Mitral valve stenosis can affect each child differently. Your physician is the best resource for providing detailed information about your child’s individual situation and making recommendations about the treatment plan that best meets the needs of your child and your entire family.

    What causes mitral valve stenosis in children?

    Mitral valve stenosis in children is normally caused by either

    • a birth defect, such as:
      • a narrowed mitral valve
      • a damaged or malformed mitral valve

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

    or

    • rheumatic fever, a complicated disease (one that is becoming increasingly rare in the United States) that can develop in children already suffering from an infection of streptococcus bacteria, like “strep throat” or scarlet fever. Rheumatic fever can lead to scarring of the leaflets that make up the mitral valve, causing the valve to become constricted and narrowed.

    In a small number of cases, children develop mitral valve stenosis as a complication of a tumor or blood clot that obstructs the valve.

    What are the symptoms of mitral valve stenosis?

    Many children with mitral valve stenosis show no outward signs, since they are in the mild to moderate stages of the condition and their symptoms have not yet progressed to a more serious level. The only identifiable symptoms in most cases are rapid breathing, slow growth/weight gain or a soft heart murmur

    As mitral valve stenosis progresses, children may show one or more of these symptoms:

    • rapid or labored breathing
    • frequent coughing or wheezing
    • slow growth/weight gain
    • feeding difficulties
    • fatigue
    • frequent respiratory infections
    • irregular heartbeats or heart palpitations
    • chest pain

    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 mitral valve stenosis disrupt her life?
    A: Your child’s long-term health  and course of treatment will depend greatly on her individual circumstances, especially:

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

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

    The most serious cases of mitral valve stenosis require interventional catheterization or surgical repair or replacement of the valve.

    Q: Does my child need to take antibiotics before routine medical or dental procedures?
    A: 
    A child with mitral valve stenosis may need to periodically take antibiotics in order to prevent an infection called bacterial endocarditis bacterial endocarditis, even if his mitral 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 like mitral valve stenosis 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 mitral 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 mitral valve stenosis, but no other cardiac problems, he probably will not need antibiotics before a routine dental procedure (for example, a teeth-cleaning).

    Q: Does my child have to cut back on physical activities?
    A:
    Whether your child needs to cut back significantly on activities—or cut back at all—depends greatly on her age, how narrowed the mitral valve has become at the time of diagnosis and what symptoms (if any) she is experiencing. 

    A child with mild mitral stenosis may not need to change his daily activities, and can continue to take part in sports and recreational play. A child with more pronounced narrowing of the mitral valve may need to limit participation in activities that call for a higher level of exertion and physical endurance. In the most serious cases (as well as when a child is preparing to undergo interventional catheterization or valve repair or replacement), the child may need to eliminate 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 serious risk of having a heart attack?
    A: One of the most common misconceptions about mitral valve stenosis is that a child diagnosed with the condition is at high risk of a sudden heart attack.

    Most children with mitral 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 soft heart murmur.

    This ability to make an early diagnosis gives clinicians the opportunity to start monitoring kids with mitral valve stenosis right away, and to easily “stay on top of” the progression of the condition with regular echocardiograms and exams.

    Newborns with critical mitral 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 repair or replacement—enable clinicians to take immediate action.

    Q: What do I need to look out for once my child has been diagnosed with mitral valve stenosis?
    A:
    Parents of children with mitral 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 any of the following:

    • fainting
    • heart palpitations or irregular heartbeat
    • racing heartbeat
    • painful coughing, especially with red- or pink-tinged discharge
    • difficulty “catching a breath”
    • rapid or shallow breathing
    • high fever
    • clammy skin or the feeling of a “cold sweat”
    • dizziness
    • pale, ashen color in the face

    Q: Are there other, non-medical precautions I can take to help my child manage her mitral valve stenosis?
    A:
    While it is essential that any child with mitral valve stenosis be treated by a qualified health professional, you and your family can help your child take steps to live a healthier life during treatment. These steps include:

    • avoiding foods that are high in sodium (salt); too much sodium can elevate blood pressure and create additional strain on the heart
    • avoiding caffeine, which can exacerbate arrhythmias (irregular heartbeats). Children should limit their intake of caffeinated beverages, like soda.
    • maintaining a healthy weight; being overweight causes the heart and lungs to work harder

    Q: Can Boston Children’s treat mitral valve stenosis when a baby is still in the womb?
    A:
    Yes. Boston Children’s Fetal Cardiology Program can:

    Learn more about these services and therapies.

    Q: Is there a cure for mitral valve stenosis?
    A:
    In general, mitral 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 mitral valve stenosis is advanced, interventional catheterization and surgical valve repair or replacement have excellent success rates 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 mitral 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 mitral 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 mitral valve stenosis?
    • What will this affect my child’s long-term health?
    • What medications will you prescribe?
    • What are the possible side effects of these medications?
    • Is interventional catheterization necessary at this time?
    • Will my child need valve replacement surgery?
    • What role should I play in my child’s treatment?
    • How should I talk to my child about this condition and the long-term outlook?
    • 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 mitral valve stenosis diagnosed?

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

    Often, a clinician will first notice that your child has a heart murmur—which 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 Children’s monitors fetal heartbeats.)

    Although exams and electrocardiograms can suggest the possibility of mitral 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 mitral valve stenosis can include:

    In order to accurately measure the degree of moderate or severe mitral stenosis, it may be necessary to do a more invasive test, such as a cardiac catheterization, which uses a small, flexible tube threaded up to the heart to give very detailed information about the structures within the heart. 

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

    Our clinicians have extensive experience in treating every stage of mitral valve 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, and are committed to repairing a child's own valve rather than resorting to an entire valve replacement whenever possible. 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 her age, the extent of narrowing in her mitral valve, her overall health and the preferences of you and your family.

    Treatments for mitral valve stenosis

    Monitoring

    In the short term, children with mild to moderate mitral valve stenosis may not need any treatment other than routine monitoring, as long as they are displaying no outward symptoms and their daily lives are not affected by the condition.

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

    Medication
    Medication is not a cure for mitral 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 mitral valve stenosis may also need to periodically take antibiotics in order to prevent an infection called Bacterial Endocarditis, even if his 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 mitral 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 mitral valve 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/balloon valvuloplasty

    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. We consider interventional catheterization the preferred way to treat children with mitral valve stenosis.

    The most common interventional catheterization procedure used to treat mitral valve stenosis is balloon dilation or balloon valvuloplasty. With the child under general anesthesia, 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 mitral valve. A deflated balloon at the tip of the catheter is inflated once the tube is in place, and this balloon stretches the mitral valve open, reversing the problematic narrowing.

    Watch a balloon valvuloplasty at Boston Children's. 

    Valve replacement surgery

    For children with severe mitral valve stenosis, balloon valvuloplasty may not adequately fix the narrowed valve. In other cases, as a child grows, an mitral 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 mitral valve is the next step in treatment for these children.

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

    • a mechanical valve, made of ceramic or other synthetic materials

    or

    • a donor valve taken from a human or animal donor (sometimes, new valves can even be fashioned from the child's own tissue)

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

    After valve replacement surgery

    Most children will 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 normal daily activities.

    Children with implanted valves—like children with mitral valve stenosis—are more susceptible to bacteria entering the bloodstream. The bacteria can cause a serious infection called bacterial endocarditis.  As a result, your child may need antibiotics before undergoing a routine medical procedure. If your child has undergone valve replacement surgery, you should always inform all medical personnel prior to any procedure.

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

    How Boston Children's used an experimental valve to treat a 4-year-old boy with a life-threatening heart defect 

    Read the story of this innovative treatment. 

  • 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 one day lead to cures.

    Learn more about our ongoing research efforts

    Among our current research projects with promise for treating mitral valve stenosis 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

    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).

    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.

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The future of pediatrics will be forged by thinking differently, breaking paradigms and joining together in a shared vision of tackling the toughest challenges before us.”
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