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Ebstein's anomaly

  • Parents have peace of mind knowing that Boston Children's uses the most innovative technologies and therapies for their child.

    --David Harrild, MD, PhD, assistant in Cardiology

    If your infant or child has been diagnosed with Ebstein’s anomaly, an understanding of the condition will help you to cope with this rare and complex congenital (present at birth) heart defect. While very serious, Ebstein’s anomaly is treatable surgically at Children’s Hospital Boston, and the outlook for many babies with this cardiac defect is good.Ebsteins Anomaly

    • Ebstein’s anomaly is a rare heart defect of the tricuspid valve —affecting just one in 10,000 babies, and affecting boys and girls equally.
       
    • Ebstein’s anomaly has a wide range of severity—from mild to moderate to severe.
       
    • When Ebstein’s anomaly occurs, there are problems with the tricuspid valve.
      The valve is malformed and is positioned too low, allowing blood to leak backward from the ventricle to the atrium.
      • These abnormalities cause enlargement of the atrium and the “atrialization” of the right ventricle, leading to:
        • congestive heart failure—a back-up of blood flow that results in a fluid buildup in the lungs
        • insufficient red blood flow to the body (“blue baby” syndrome)
           
    • In addition, an opening in the wall between the two atria that occurs normally in the fetus sometimes fails to close.
      • This leads to a mixing of oxygen-rich and oxygen-poor blood, which results in poor oxygen levels in the blood that circulates through the baby’s body.
         
    • Ebstein’s anomaly often occurs with other heart defects, including atrial septal defect (ASD) and/or pulmonary (valve) stenosis (PVS, PS).
       
    • Associated arrhythmias and heart rate disturbances may also need treatment, usually by medications and/or catheter ablation.

    For more visual and audio information on Ebstein’s anomaly, visit our cardiovascular Multimedia library.

    How Boston Children's Hospital approaches Ebstein’s anomaly

    Our team in Boston Children’s Cardiac Surgery Program understands how distressing a diagnosis of Ebstein’s anomaly can be for parents. You can have peace of mind knowing that our surgeons treat some of the most complex pediatric heart conditions in the world, with overall success rates approaching 98 percent—among the highest in the nation among large pediatric cardiac centers. We use the following elements to provide the best possible outcomes:

    • accurate diagnosis and assessment: Subtle variations in heart anatomy—such as the arrangement of the arteries that feed the heart—can negatively impact surgical outcomes if not identified ahead of time. We utilize the most advanced techniques available for precisely determining your child’s heart anatomy, with interpretation by highly experienced cardiologists.
    • sophisticated and effective therapies: Babies with the most severe cases of Ebstein’s anomaly need emergency therapy. Our cardiac intensive care unit (CICU), cardiac cath labs and operating rooms deal with the urgent needs of our smallest patients 24/7. Children’s CICU was one of the first such units developed anywhere; professionals from many countries visit our CICU to learn advanced techniques of post-operative care.

      Boston Children’s Congenital Heart Valve Program cares for children with congenital heart defects that involve absent or malfunctioning heart valves. Our surgeons have a strong record of excellence in heart valve repair and replacement, including minimally invasive techniques.

      Children’s is one of just a handful of heart centers in the United States that performs the advanced and innovative cone procedure for repairing the tricuspid valve in children with Ebstein’s anomaly. (See the Patient story in this topic.)
    • experienced team of skilled professionals to perform surgery and other procedures: Boston Children’s cardiac surgeons have vast experience in the surgical procedures used to repair this defect, and they work with nurses and doctors who are focused on providing expert care after surgery.
    • close, expert medical follow-up: If you live in the Boston area, a Boston Children’s cardiologist will follow your child after surgical repair; if you live in another part of the country or the world, a Boston Children’s cardiologist will work closely with your local cardiologist. Adult patients with Ebstein’s anomaly are followed by Boston Children’s cardiologists with special training for adults with congenital heart problems.

    Ebstein’s anomaly: Reviewed by David Harrild, MD
    © Boston Children's Hospital, 2010

    Read stories of Boston Children’s heart patients…

    … and hear words of wisdom from their parents, siblings and caregivers

    Boston Children’s pediatric research

    Consistently ranked among the top pediatric hospitals in the United States, Boston Children’s is home to the world’s most extensive pediatric hospital research enterprise; and we partner with elite health care and biotech organizations around the globe. But as specialists in innovative, family-centered care, our physicians never forget that your child is precious, and not just a patient.


  • It’s natural for you to be concerned right now about your child’s health—a diagnosis of Ebstein’s anomaly can be overwhelming. But you can rest assured that at Boston Children's Hospital, your child is in expert hands.

    Our cardiologists, cardiac surgeons, cardiovascular imaging professionals and other clinicians have extensive experience with all types of heart problems. Each year, we treat thousands of children, adolescents and even adults with a wide variety of heart defects and disorders.

    How does a normal heart work?

    normal heartIn a normal heart, oxygen-poor (blue) blood returns to the right atrium from the body, and is pumped by the right ventricle through the pulmonary artery into the lungs. In the lungs the blood receives oxygen (and gives up carbon dioxide), then returns to the left side of the heart. The left ventricle pumps the oxygen-rich blood through the aorta out to the body.

    In a normal heart, the tricuspid valve controls blood flow from the right atrium, the chamber that receives blood returning from the body, to the right ventricle, the pumping chamber that pumps that blood to the lungs for oxygenation.

    What is Ebstein’s anomaly?

    But in Ebstein’s anomaly, the leaflets that make up the tricuspid valve are malformed and are malpositioned too far down into the right ventricle. Sometimes, one of the leaflets is displaced downward into the ventricle, while another leaflet is larger than usual and may be abnormally attached to the wall of the ventricle.

    When this occurs, blood leaks backward from the ventricle to the atrium. This causes enlargement of the atrium, and it can lead to congestive heart failure—a back-up of blood flow that results in a fluid buildup in the body.

    In addition, an opening in the septum between the two atria that occurs normally in the fetus, called the foramen ovale, fails to close in some newborns with Ebstein's anomaly because of the high volume of blood in the right atrium. The opening, called the patent foramen ovale (PFO), allows the mixing of oxygen-poor blue blood from the right atrium with oxygen-rich red blood in the left atrium. This results in poor oxygen levels in the blood that circulates through the body.

    Is Ebstein’s anomaly dangerous?

    There’s a very wide spectrum of variation and severity with Ebstein’s anomaly. The most severe cases are among the most challenging forms of heart disease—while the least severe forms are never even diagnosed, and children develop and function perfectly normally.

    The severe forms of the defect cause such great enlargement of the heart—even prenatally—that the heart fills the baby’s entire chest cavity and crowds out the lungs, which end up being far too small. So, extreme cases end up not being just a heart problem, but rather, a heart-lung problem. The prognosis for such cases isn’t favorable, since by the time the baby is born, there’s already irreversible lung damage.

    The good news is that such severe cases are quite uncommon. Far more common are milder forms of the disease, which are treatable, with usually favorable prognoses.

    InEbstein's Anomoly Ebstein's anomaly, the tricuspid valve is downwardly displaced and adheres to the interventricular septum. Part of the right ventricle is “atrialized,” being located above the tricuspid valve.
    (abbreviations: Ao=aorta, LA=left atrium, LV=left ventricle, PA=pulmonary artery, RA=right atrium)
     

    Are there additional defects associated with Ebstein’s anomaly?

    Often, Ebstein’s anomaly occurs with one or more other heart defects, including:

    • atrial septal defect (ASD): an opening between the right and left atria. This defect often occurs with Ebstein’s anomaly and can be easily corrected at the time of surgical repair of the tricuspid valve.
           
    • pulmonary valve stenosis: a malformation in which the pulmonary valve is unable to open completely. With pulmonary stenosis, problems with the pulmonary valve make it harder for the valve’s leaflets to open and permit blood to flow forward from the right ventricle to the lungs.

    Arrhythmias can occur, too. Patients who have Ebstein’s anomaly may also have arrhythmias and abnormal heart rates that are associated with the condition. These may need treatment—usually by medications and/or catheter ablation (targeted destruction of affected tissue).

    In 1990, Boston Children’s doctors performed the first pediatric non-surgical repair of a cardiac arrhythmia, using catheter ablation to correct a rhythm disorder called Wolff-Parkinson-White syndrome. So, today, Boston Children’s experts can call on our more than three decades’ worth of experience with this procedure.

    How common is Ebstein’s anomaly?

    While relatively rare (one in every 10,000 babies), and it affects boys and girls equally.

    Will my child be OK in the long term?

    Your cardiologist will help you create a long-term care program as your baby grows into childhood, the teen years and even adulthood. Most of our patients who’ve had congenital heart disease have an ongoing relationship with their Children’s cardiologist.

    We’ll prevent and treat complications, and will advise on daily-life issues, such as exercise and activity levels, nutrition and precautions related to pregnancy. Throughout his childhood, you can help your child—and the whole family—by making sure heeats a heart-healthy diet, exercises regularly, and in the teen years avoids smoking.

    Surgical techniques for treating congenital heart defects are continually being refined, and Boston Children’s surgical success rates are very high. Nevertheless, your child will need periodic monitoring—and perhaps medication—indefinitely, since he could be at some risk for:

    In relatively few cases, patients who’ve had surgery for Ebstein’s anomaly may need additional surgery.

    Where can my child find care and support when he grows up?

    • The Boston Adult Congenital Heart and Pulmonary Hypertension Service (BACH) provides long-term inpatient and outpatient care and advanced therapeutic options for patients with congenital heart disease and pulmonary hypertension as they reach and progress through adulthood.

      BACH is an international center for excellence, with physicians and services from Children’s, Brigham and Women’s Hospital and the Beth Israel Deaconess Medical Center. The center promotes and supports clinical and scientific research for the advancement of care of these patients, and is a leader in the education of providers caring for this unique population.
    • The Adult Congenital Heart Association (ACHA) is a national not-for-profit organization dedicated to improving and extending the life of adults with congenital heart defects, now thought to number at least 1 million. The organization serves and supports many such adults, their families and the medical community, as well as conducting research and providing advocacy, outreach and education.

    Causes

    As a congenital heart defect, Ebstein’s anomaly is a problem with your child’s heart structure that he was born with.

    The heart forms during the first eight weeks of fetal development. It isn’t clear what causes congenital heart malformations, including Ebstein’s anomaly, although research is underway to search for a genetic or environmental link.

    Signs and symptoms

    Cyanosis (blue coloration of the skin) is the most common sign of Ebstein’s anomaly in newborns and infants.

    When to seek medical advice

    Call your health care provider immediately if your baby has a bluish color, or if your child is having difficulty breathing, is breathing rapidly, or seems to tire too easily.

    Who’s at risk

    Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development. So, it’s difficult to predict who’s at risk. Familial cases have been reported, but no genetic link has been confirmed. Research is underway to search for a genetic or environmental link. In general, if you have a child with a congenital cardiac defect, the chance of more children having a defect is about 2 to 3 percent.

    For teens

    If you’re a teen with a congenital heart defect, you have a lot to cope with. Besides the typical issues any teenager faces—from social acceptance to body changes and more—you’ll also have to deal with medical appointments and procedures, some delay of your natural wish for independence, feeling different and assuming a lot of personal responsibility for maintaining your own good health.

    If you feel overwhelmed, depressed or anxious through this important time in your transition to adulthood, speak to your doctor or counselor to get help.

    For adults with congenital heart disease

    If you were treated for congenital heart disease as a child, you’re probably being followed by your cardiologist, since complications from early heart disease can arise in adulthood. In particular, if you had surgery for Ebstein’s anomaly, you need lifelong monitoring—and possibly medication—since you’ll continue to be at some risk for heart problems in the future.

    Fortunately, Boston Children’s can help adults with congenital heart defects. Many adults who were patients at Boston Children’s as babies or children continue to be monitored by the clinicians who have followed them since childhood.

    In addition, our Boston Adult Congenital Heart and Pulmonary Hypertension Service (BACH) provides long-term inpatient and outpatient care and advanced therapeutic options for patients with congenital heart disease and pulmonary hypertension as they reach and progress through adulthood.

    BACH is an international center for excellence, with physicians and services from Boston Children’s, Brigham and Women’s Hospital and the Beth Israel Deaconess Medical Center. The center promotes and supports clinical and scientific research for the advancement of care of these patients, and is a leader in the education of providers caring for this unique population.

    What you can do at home

    After surgery, your child’s cardiologist will offer recommendations for follow-up care, including:

    • wound care while the baby is healing
    • a nutritional program to encourage weight gain
    • an oral hygiene program to prevent infection
    • an appropriate exercise regimen to build body mass and achieve fitness

    As your baby recovers and grows, be sure to follow a regular program of well-baby/well-child checkups. Throughout his childhood, you can help your child—and the whole family—by making sure heeats a heart-healthy diet, exercises regularly, and in the teen years avoids smoking.

    Ebstein’s anomaly glossary

    • atrial septal defect (ASD): a congenital (present at birth) heart defect in which there is an opening in the tissue wall (septum) that divides the two upper chambers of the heart (right and left atria). This defect can be easily corrected at the time of surgical repair of the child’s tricuspid valve.
    • bioprosthetic tricuspid valve: a replacement tricuspid valve made from human tissue (from the patient or from a cadaver) or from animal tissue.
    • cardiac catheterization (cardiac cath): provides detailed visual information and measurements about the structures and pressures inside the heart. Blood pressure and oxygen measurements are taken in the four chambers of the heart, as well as the pulmonary artery and aorta.

      Cardiac catheterization can also be an interventional procedure. In Ebstein’s anomaly, a catheterization procedure called radiofrequency ablation may be performed to treat heart arrhythmias associated with the condition.
    • cardiac/cardio-: pertaining to the heart
    • cardiac surgery: surgical procedure performed on the heart or one of the blood vessels connected to the heart
    • cardiac surgeon: doctor who performs surgery on the heart. A pediatric cardiac surgeon performs surgery on the hearts of infants and children.
    • cardiologist: doctor who diagnoses and treats heart problems non-surgically. A pediatric cardiologist treats infants, children and some adults with heart problems.
    • chest x-rayshows the size and spatial relationships of the heart within the child’s chest. It takes a few moments. There’s no pain or discomfort.
    • cone procedure: In this procedure, extra tissues on the enlarged right side of the heart are folded up, and the malformed valve is surgically reshaped into a cone. The cone-shaped valve opens to a central blood flow and closes with full coaptation (joining, reuniting) of the leaflets. (See the Patient story in this topic.)

      Boston Children’s is one of just a handful of heart centers in the United States that performs the advanced and innovative cone procedure for repairing the tricuspid valve in children with Ebstein’s anomaly.
    • congenital heart defect: heart defect present at birth. The heart usually starts to form in the first eight weeks of fetal development. It’s thought that most congenital heart defects develop during this period.
    • cyanosis (cyanotic): blue color of skin, lips and nailbeds caused by a reduction in the amount of oxygen-rich (red) blood circulating in baby’s bloodstream
    • diagnosis: medical determination of illness or disease based on history, physical examinations and advanced technology diagnostic testing tools 
    • echocardiogram (echo, cardiac ultrasound): evaluates the structure and function of your child’s heart using electronically recorded sound waves that produce a moving picture of the heart and heart valves. This test confirms the diagnosis of Ebstein’s anomaly. It’s also used to check for any associated defects, such as atrial septal defects and/or ventricular septal defects. No discomfort is involved. It takes 30-60 minutes.
    • electrocardiogram (ECG or EKG): evaluates the electrical activity of your child’s heart. An EKG is used to evaluate the causes of symptoms and detecting heart abnormalities, including Ebstein’s anomaly. It’s performed by placing electrodes on the arms, legs and chest to record the electrical activity. The test takes five minutes or less and involves no pain or discomfort.
    • foramen ovale, patent foramen ovale (PFO): An opening (foramen ovale) in the septum between the two atria that occurs normally in the fetus fails to close in some newborns with Ebstein’s anomaly because of the high volume of blood in the right atrium.

      The opening, called the patent foramen ovale (PFO), allows the mixing of oxygen-poor blue blood from the right atrium with oxygen-rich red blood in the left atrium. This results in poor oxygen levels in the blood that circulates through the body.
    • neonatologist: doctor who specializes in illnesses affecting newborns, both premature and full-term
    • pediatric cardiologist: a doctor who specializes in the diagnosis and medical management of congenital heart defects in babies, as well as heart problems that may develop later in childhood.
    • pulmonary valve stenosisa malformation in which the pulmonary valve is unable to open completely. With pulmonary stenosis, problems with the pulmonary valve make it harder for the valve’s leaflets to open and permit blood to flow forward from the right ventricle to the lungs.
    • sporadic: occurring by chance, not inherited
    • signs and symptoms: the presenting reasons why a child needs medical attention. Characteristics such as onset, quality, triggers and severity help diagnosticians to determine a disease, or to decide which testing is needed to determine the disease.
    • surgical valve repair: surgeons will reposition the existing valve, reconfigure the shape of the tricuspid valve leaflets, and may suture an annular ring into place.
    • tricuspid valve replacement: Sometimes a patient’s tricuspid valve is too malformed to be repaired. In this case, the existing valve will need to be replaced with a mechanical valve or a bioprosthetic one made of human or animal tissue.

    For a more complete list of cardiovascular terms, visit our Cardiovascular glossary.

    For in-depth visual information on several of the conditions, diagnostic tools and procedures described above, including Ebstein’s anomaly, visit our Multimedia library.

  • At Boston Children's Hospital, we understand that the first step in treating your child is forming an accurate, complete and timely diagnosis

    The exam

    If your newborn baby was born with a bluish tint to his skin, or if your young child is experiencing certain symptoms, your pediatrician will immediately refer you to a pediatric cardiologist (and/or neonatologist), who will perform a physical exam. Your child’s doctor will listen to your baby’s heart and lungs, measure the oxygen level in his blood (non-invasively) and make other observations that help to determine the diagnosis.

    Your child’s cardiologist will also investigate whether he has a heart murmur—a noise heard through the stethoscope that’s caused by the turbulence of blood flow. If there’s a murmur, the location in the chest where the murmur is best heard, as well as the sound and character of the murmur itself, will give the cardiologist an initial idea of the kind of heart problem your baby may have.

    Note: If your baby’s case of Ebstein’s anomaly was detected prenatally through ultrasound, upon birth he’ll be immediately admitted for stabilization and surgery.

    The tests

    Other diagnostic tests are needed to help with the diagnosis and may include the following:

    • echocardiogram (echo, cardiac ultrasound): evaluates the structure and function of your child’s heart using electronically recorded sound waves that produce a moving picture of the heart and heart valves. This test confirms the diagnosis of Ebstein’s anomaly. It’s also used to check for any associated defects, such as atrial septal defects and/or ventricular septal defects. No discomfort is involved. It takes 30-60 minutes.
    • chest x-ray: shows the size and spatial relationships of the heart within the child’s chest. In Ebstein’s anomaly, a chest x-ray would show an enlarged heart. In severe cases involving newborn babies, the silhouette of the heart almost completely fills the chest. It takes a few moments. There’s no pain or discomfort.
    • electrocardiogram (ECG or EKG): evaluates the electrical activity of your child’s heart. An EKG may be the first test used on an older child who’s complaining of a racing heart, since it records the heart’s rhythm and will show any abnormal rhythms. The EKG is performed by placing electrodes on the arms, legs and chest to record the electrical activity. It takes five minutes or less and involves no pain or discomfort.

    For images and videos of these tools for diagnosing Ebstein’s anomaly, visit the Ebstein’s anomaly section of our cardiovascular Multimedia library.

    Watch a webcast of heart surgery at Boston Children’s
  • Having identified your child's heart condition, at Boston Children's Hospital we're able to begin the process of treating him, so that we may ultimately return him to good health.

    Treatment options

    Medication for milder cases. Most children who have a milder form of Ebstein's anomaly can be managed with medication to control:

    Surgery for more severe cases. But if your child's condition is severe—causing low levels of oxygen in the blood (cyanosis) or arrhythmia—his situation will probably be urgent, and surgery will be needed.

    Your baby will most likely be admitted to Boston Children's cardiac intensive care unit (CICU) once his symptoms become apparent. Initially, he may be placed on oxygen or a ventilator to help him breathe, and IV (intravenous) medications may be given to help his heart and lungs function more efficiently.

    There are several surgical approaches to repairing your child's tricuspid valve, closing his patent foramen ovale (PFO), and repairing other defects (such as ASD) that are associated with Ebstein's anomaly. Surgical techniques include:

    • surgical valve repair: surgeons will reposition the existing valve, reconfigure the shape of the tricuspid valve leaflets, and suture an annular ring into place.
    • cone procedure: Boston Children's is one of just a handful of heart centers in the United States that performs the advanced and innovative cone procedure for repairing the tricuspid valve in children with Ebstein's anomaly.
    • In this procedure, extra tissues on the enlarged right side of the heart are folded up, and the malformed valve is surgically reshaped into a cone. The cone-shaped valve opens to a central blood flow and closes with full coaptation (joining, reuniting) of the leaflets. (See the Patient story in this topic.)
    • tricuspid valve replacement: Sometimes a patient's tricuspid valve is too malformed to be repaired. In this case, the existing valve will need to be replaced with a mechanical valve or a bioprosthetic one made of human or animal tissue.

    Radiofrequency catheter ablation treatment for arrhythmias. For children whose condition includes significant arrhythmias and heart beat irregularities that can't be fully controlled by medication, doctors can perform a catheterization intervention called radiofrequency catheter ablation to treat these heart arrhythmias.

    In 1990, Boston Children's doctors performed the first pediatric non-surgical repair of a cardiac arrhythmia, using catheter ablation to correct a rhythm disorder called Wolff-Parkinson-White syndrome. So, today, Boston Children's experts can call on our more than three decades' worth of experience with this procedure.

    At home: caring for your child after surgery

    After surgery, your child's cardiologist will offer recommendations for follow-up care, including:

    • wound care while the baby is healing
    • a nutritional program to encourage weight gain
    • an oral hygiene program to prevent infection
    • an appropriate exercise regimen to build body mass and achieve fitness

    As your baby recovers and grows, be sure to follow a regular program of well-baby/well-child checkups. Throughout his childhood, you can help your child—and the whole family—by making sure heeats a heart-healthy diet, exercises regularly, and as he grows into the teen years avoids smoking.

    As he grows: your child's long-term outlook

    Surgical techniques for treating Ebstein's anomaly and its associated defects are continually being refined, and the long-term outlook is continually improving. Most children who've had surgery recover and grow normally.

    Even so, your child will need periodic monitoring—and possibly medication—indefinitely, since he could be at some risk for abnormal heart rhythms (arrhythmias) and heart failure. In relatively few cases, patients who have had surgery for Ebstein's anomaly may need additional surgery.

    Your child's cardiologist will help you create a long-term care program as your baby matures into childhood, the teen years and even adulthood. Most people who have had congenital heart disease repair will have an ongoing relationship with their cardiologist.

    We'll prevent and treat any complications and will advise on daily-life issues, such as activity levels, nutrition and precautions related to pregnancy.

    Coping and support

    We understand that if your child is has Ebstein's anomaly, you'll have a lot of questions. How serious is this condition? Will surgery help my child? What's the long-term outlook? Boston Children's Hospital has a variety of resources at that may be helpful to your family:

    • patient education: Our nurses will be on hand to walk you through your child's treatment and help answer any questions you may have.
    • parent-to-parent: Want to talk with someone whose child has been treated for Ebstein's anomaly? We can often put you in touch with other families who've been down a similar road and can share their experiences. To request family-to-family support, talk with your child's health care team or contact The Center for Families.
    • faith-based support: If you and your family are in need of spiritual support, we can connect you with the Boston Children's chaplaincy. Our program includes nearly a dozen clergy representing Protestant, Jewish, Muslim, Roman Catholic and other faith traditions who will listen to you, pray with you and help you observe your own faith practices during your treatment experience.
    • social work and mental health professionals: Boston Children's social workers and mental health clinicians have helped many other families in your situation. We can offer counseling and assistance with a wide variety of issues, such as coping with your child's diagnosis, stresses relating to coping with illness and dealing with financial difficulties.
    • As your child reaches adulthood, you'll want him to know about our Boston Adult Congenital Heart and Pulmonary Hypertension Service (BACH). Boston Children's is a founding institution of BACH, an international center for excellence providing long-term inpatient and outpatient care and advanced therapeutic options as needed for congenital heart disease patients as they reach and progress through adulthood.

    To find out more, visit the Family resources page of Boston Children's For Patients and Families site.

    Boston Children's Development of Interventional Catheterization

    You'll be comforted to know that Boston Children's pioneered the use of interventional catheterization for many congenital heart defects and is a leader in the use of this procedure.

    Keep family and friends up to date during your child's treatment ...

    … by creating a free Children's Carepage.

    Boston Children's Heart Care Center

    The Heart Care Center at Boston Children's is one of the largest pediatric heart programs in the United States. Our staff of more than 80 pediatric cardiac specialists cares for thousands of children and adults with congenital and acquired heart defects each year, from simple to complex cases. We have experience treating rare heart problems—with results that are among the best in the world.

  • Pediatric cardiologists and pediatric cardiovascular surgeons at Boston Children's Hospital have pioneered the interventional catheterization techniques now used widely for many congenital heart defects, such as Ebstein’s anomaly.

    And a significant amount of the groundbreaking cardiac research currently being conducted at Children’s aims to refine and advance the open heart surgery and catheterization procedures that correct congenital heart defects in newborns and young children—including Ebstein’s anomaly.

    Heart valve repair and research

    Boston Children’s  Congenital Heart Valve Center cares for children with congenital heart defects that involve absent or malfunctioning heart valves. Our surgeons have a strong record of excellence in heart valve repair and replacement, including minimally invasive techniques.

    Our field is rapidly changing. Historically, children who have heart valve conditions have had chronic problems that require lifelong follow-up and treatment. Currently, few artificial replacement heart valves are available that are designed specifically for children, so doctors are limited in their options. Further complicating matters is that when surgeons place a new valve in a child, it doesn’t expand as the child grows, so it needs to be replaced over time.

    To address these problems, Boston Children’s cardiac surgery researchers are exploring ways to reconstruct patients’ existing valves. Unlike replacement valves, a reconstructed valve can last a long time and give children an optimal quality of life.

    Valve research at Boston Children’s informs our care. Our scientists investigate every aspect of the heart, and the conditions that affect it, so we can offer new and improved treatments to our patients. The primary goal of our research is to combine our clinical and engineering expertise to advance the state of the art in surgical repair of heart valves.

    Major themes of our research include:

    • development of novel devices and methods for surgical repair of valves
    • advanced imaging and image processing to better understand and treat diseased valves and to guide minimally-invasive procedures
    • computer simulation of patient-specific valve function to help plan an individual's valve surgery

    Cardiac surgery research

    Members of Boston Children’s Cardiac Surgery Research Laboratory—a multidisciplinary team of basic and applied research investigators, all of whom hold faculty appointments at Harvard Medical School—are studying the mechanisms of heart disease and new treatments for children with congenital heart defects.

    Some principal areas of active research are:

    • surgical robotics and ultrasound-guided intracardiac surgery:The department is pioneering the use of 3-D ultrasound and laparoscopic techniques to operate on the beating heart.
    • myocardial metabolism and myocardial hypertrophy and heart failure:Researchers are exploring new methods of myocardial preservation during heart surgery and the role of angiogenic growth factors in heart failure.
    • tissue engineering to stimulate the growth of new tissue to repair congenital defects, including valve abnormalities, right ventricular defects and arrhythmias

    Learn more about Boston Children’s cardiac surgery research initiatives.

    Cardiology research

    Boston Children’s is a world leader in opening new avenues of “translational research”—bringing laboratory advances to the bedside and doctor’s office as soon as possible. Senior medical staff members of the Department of Cardiology, who hold faculty appointments at Harvard Medical School—participate in clinical research activities, and many do laboratory research, as well.

    Learn more about Boston Children’s current projects in cardiology research.

    Innovations

    Cone procedure for tricuspid valve repair

    Boston Children’s is one of just a handful of heart centers in the United States that performs the advanced and innovative Cone procedure for repairing the tricuspid valve in children with Ebstein’s anomaly.

    In this procedure, extra tissues on the enlarged right side of the heart are folded up, and the malformed valve is reshaped into a cone. (See the Patient story in this topic.)

    Creating new ways to perform surgery

    Problem: When surgeons perform heart surgery on a baby, they need to open the infant’s chest and stop her heart—an inva­sive, lengthy procedure that can cause life-threatening complications. Recognizing that there is much room for improvement, Pedro del Nido, MD, chief of Cardiac Surgery at Boston Children’s, has developed a novel research program to invent new ways to improve the way that surgeons do cardiac operations.

    Innovative solution: Del Nido decided to develop a way to perform surgery on a still-beating heart. But he needed two things that didn’t exist: superior imaging tools that could show the structures inside the heart while it’s beating, and tiny instruments to perform the intricate surgery.

    So, he bor­rowed technology from the videogame industry and developed stereo-rendered 3-D ultrasound imaging that allows surgeons to see inside the beating heart as a hologram.

    Del Nido also designed new instruments. One is a millimeter-sized tool that extends into the heart through needle-sized incisions. Using a joystick controller and real-time imaging, a surgeon can now navigate through the beating heart’s chambers in animals to remove blockages, repair faulty valves and close leaks.

    The other new instrument is a cardioportdevice that allows instruments to be safely introduced into the cardiac chambers with­out the usual risks of blood loss or an air embolism.

    cardioportResults: Del Nido’s 3-D tool appears not only to provide superior imaging, but also to yield faster surgery times. Researchers using it to operate on pigs with congenital heart disease performed the procedure 44 per­cent faster than before. Dr. Del Nido’s cardioport will soon be tested in clinical trials and will facilitate further development of similarly novel instruments for heart repair.

    Del Nido’s newly-developed cardioport will someday make possible faster, less invasive heart surgery.

    History of innovation

    In 1938, Children’s cardiac surgeon Robert Gross, MD, performed the world’s first successful surgery to correct a child’s heart defect. Since that time, we have gained recognition around the globe for our leadership in pediatric cardiology and continue to make critical advances in the field. In 2014, U.S. News & World Report named Children’s cardiology and cardiac surgery programs the best of any pediatric hospital in the country.

    Trace Boston Children’s history of innovations in pediatric heart care. 

    The Cardiac Registry

    Before operating on a congenital heart defect, cardiac surgeons need the most precise diagnosis possible. For more than 40 years, the Cardiac Registry at Boston Children’s has been a place where pathologists, cardiologists and cardiac surgeons have gone to learn more about the anatomy of heart defects. Its collection of over 3,600 specimens, some dating back as far as 1944, is irreplaceable, since large, intact examples of heart defects are no longer seen at autopsy in this country. Today, the Registry has been broadened to include developmental cardiology and molecular biology projects.

  • With healing arts, a mended heart
    A surgical procedure, rare in the U.S., has Bethlehem [PA] girl back in pink of health.

    By Steve Esack
    of The Morning Call

    Arden Ashford, feeling oddly fresh from a warm-up jog around the tennis courts, gripped her racket in both hands and waited ...

    Read the inspiring story of Ebstein's anomaly patient, Arden. This young Pennsylvania girl and her family found help, hope and expertise at Boston Children's Hospital. The family has now established the Ebstein's Anomaly Foundation to help others.

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