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Tricuspid Atresia

  • If your infant or child has been diagnosed with tricuspid atresia (TA), an understanding of the condition will help you to cope with this very rare congenital (present at birth) heart defect.

    • In tricuspid atresia, the tricuspid valve—which is normally located between the right atrium and the right ventricle—is abnormal and does not open.
    • The failure of the tricuspid valve to develop results in a small (or missing) right ventricle that can’t adequately pump blood to the lungs.
    • While very serious, tricuspid atresia is treatable surgically, and surgical advances have greatly improved children’s outcomes.

    Comparison with normal heart

    In a normal heart, oxygen-poor (blue) blood returns to the right atrium from the body, travels to the right ventricle, then is pumped through the pulmonary artery into the lungs, where it receives oxygen. And oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left ventricle, then is pumped through the aorta out to the body.

           

    click on images to enlarge

    In tricuspid atresia, improper development of the tricuspid valve prevents oxygen-poor (blue) blood from passing from the right atrium to the right ventricle and on to the lungs as it should. As a result, the right ventricle is underdeveloped (hypoplastic).

    Additional defects

    Like many congenital heart conditions, tricuspid atresia isn’t a single defect, but rather a cluster of associated defects in various combinations and with varying degrees of severity:

    • In addition to a missing tricuspid valve and underdeveloped right ventricle, most TA defects are accompanied by secondary defects called atrial septal defect (ASD) and ventricular septal defect (VSD), which are essentially holes in the tissue walls (septa) that normally separate the right and left atria (ASD) and the right and left ventricles (VSD).
      • These ASD and VSD holes are actually useful in this condition, since they allow oxygen-poor (blue) blood and oxygen-rich (red) blood to mix, providing at least some oxygen supply to circulate. But the heart often has to work extra hard to carry enough oxygen through the body.
    • A third defect is called the patent ductus arteriosus (PDA). In this condition, a blood vessel (ductus arteriosus) which connects the two great arteries (aorta and pulmonary artery), and which usually closes soon after birth, fails to do so. The PDA remains open (patent), allowing blood to pass from the aorta to the pulmonary artery.
      • This opening allows some oxygen-rich blood to circulate to the lungs to pick up oxygen. However, it can strain the heart, as well as increasing blood pressure in the lung arteries.
    • A narrowing or blockage of the passageway to the lungs (pulmonary valve stenosis) may also be present with tricuspid atresia. And occasionally, when the VSD is very large, there is also transposition of the great arteries (TGA), in which the normal positions of the aorta and pulmonary artery are reversed.

    The Boston Children's Hospital approach

    The experienced surgeons in Boston Children’s Cardiac Surgery Department understand how distressing a diagnosis of a tricuspid atresia 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. For tricuspid atresia, our 30-day hospital survival rate for the past five years for each of the three necessary operations has been higher than 98 percent.

    At Boston Children’s, we provide families with a wealth of information, resources, programs and support—before, during and after your child’s treatment. With our compassionate, family-centered approach to expert treatment and care, you and your child are in the best possible hands.

    Tricuspid atresia: Reviewed by Michael Freed, MD, and Thomas Kulik, MD
    © Boston Children's Hospital, 2010

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

    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.
     

    Why is tricuspid atresia a concern?

    This heart defect causes children to be cyanotic (blue), since a mixture of oxygen-poor (blue) and oxygen-rich (red) blood leaves the heart and goes to the body. Just how much or how little oxygen will be in the bloodstream depends on a number of variables. Some children will be only mildly cyanotic, while others won't have enough oxygen in the blood to meet the body’s needs.

    How common or rare is TA?

    Tricuspid atresia occurs in two out of every 10,000 live births. While relatively rare, it accounts for 1 to 2 percent of all cases of congenital heart disease, and is the third most common form of cyanotic congenital heart disease. TA occurs equally in boys and girls.

    What are the forms of tricuspid atresia?

    There are three major variations in tricuspid atresia, usually characterized by the size of the hole (VSD) between the right and left ventricles:

    • If the VSD is large, the baby (newborn or in the first few weeks of life) might have congestive heart failure (CHF), in which the heart pumps too much blood to the lungs and has difficulty pumping enough blood to the rest of the body. If the left side of the heart fails, the lungs can get flooded with too much blood and fluid; the child breathes rapidly, sweats and has difficulty gaining weight. An initial surgical procedure called pulmonary artery banding will usually be performed in the first few weeks of life to reduce the amount of blood to the lungs.
    • If the VSD is small or absent, the right ventricle will be severely underdeveloped, and, in addition, the pulmonary valve may be obstructed (pulmonary valve stenosis). As a result, not enough blood will be able to pump through to the lungs and pick up oxygen. Without sufficient oxygen, the baby’s skin will turn blue (cyanotic). This condition is usually recognized in the hospital after birth or soon after going home.
      • In this case, to stabilize the baby before surgery, medication will keep the PDA open, allowing blood to pass from the aorta to the pulmonary artery so that some oxygen-rich blood can circulate. Initial surgery will usually be performed early in the baby’s life.
    • If the VSD is moderate sized, the amount of blood going to the lungs is more balanced, causing less distress in the baby and reasonable levels of oxygen in the blood. Initial surgery will usually be performed when the baby is about six months old, or even older.
       

    Will my child be OK?

    Open heart surgery at Boston Children’s has among the highest success rates in the United States among large pediatric cardiac centers. With our advanced surgical techniques and timely, family-centered care, the likelihood is very good that your child will undergo successful surgery.

    Note: Infants who’ve had initial surgery for a single ventricle defect are typically enrolled in Boston Children’s Home Monitoring Program between the baby’s Stage I and Stage II operations in the Fontan sequence.
     

    What about later in life?

    Your cardiologist will help you create a long-term care program as your baby grows into childhood, the teen years and even adulthood. Most people who've had congenital heart disease repair will have an ongoing relationship with their cardiologist; we'll treat complications, and will advise on daily-life issues such as exercise and activity levels, nutrition and precautions related to pregnancy.

    Surgical techniques for TA and its associated defects are continually being refined, and long-term outcomes are continually improving. Nevertheless, patients will need lifelong monitoring and medication, since they will always be at some risk for arrhythmias, infections, heart failure or stroke.
     

    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 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 the quality of life—and extending the life—of adults with congenital heart defects. The organization serves and supports many of the adults with congenital heart defects (thought at this time to be as many as one million), their families and the medical community, as well as conducting research and providing advocacy, outreach and education.

    Causes

    It’s important for parents to understand that you’ve done nothing to cause your baby’s tricuspid atresia and its accompanying defects. Some congenital heart defects may have a genetic link, causing heart problems to occur more often in certain families. Most often, though, this heart defect occurs sporadically (by chance), with no clear reason for its development.

    Symptoms

    • rapid breathing
    • rapid heartbeat
    • blue color of the skin, lips and nailbeds (cyanosis)
    • sweating
    • disinterest in feeding or tiring while feeding
    • poor weight gain
    • heart murmur (detected by doctor)

    When to seek medical advice

    Call your health care provider immediately if your baby or child is having difficulty breathing or is breathing rapidly, has a bluish color, tires easily or is uninterested in eating.

    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. TA occurs equally in boys and girls. In general, if you have a child with a congenital cardiac defect, the chance of later children having a defect is about 2 to 3 percent.

    Complications

    The first children who underwent the staged surgical repairs for TA are now in their 20s and largely doing well. However, complications can occur, including arrhythmias, blood clots, infections within the heart (endocarditis), easy tiring and loss of protein from the digestive tract. As he grows, it’s important for your child to be monitored closely by his cardiologist.

    Long-term outlook

    Surgical techniques for TA and its associated defects are continually being refined, and long-term outcomes are continually improving. But patients will still need lifelong monitoring and medication, since they'll always be at some risk for arrhythmias, blood clots, infections, heart failure or stroke.

    What you can do at home

    Your child’s cardiologist will offer recommendations for post-operative follow-up care, including:

    • wound care
    • 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.

    Prevention

    It’s important to understand that as parents, you’ve done nothing to cause TA and its accompanying defects, and there are no precautions you could have taken to prevent it. Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development.

    TA Glossary

    • aorta: one of the heart’s two great arteries. In a normal heart, the aorta arises from the left ventricle and carries oxygen-rich blood out to the body.
    • atrial septal defect (ASD): a congenital (present at birth) heart defect that is present in all children with TA, in which there's an opening in the tissue wall (septum) that divides the two upper chambers of the heart (right and left atria)
    • arterial switch procedure: a surgical procedure necessary if the aorta and pulmonary artery are reversed in relation to each other (transposition of the great arteries [TGA]). In TGA, the aorta and pulmonary artery are divided and “switched” so that the blue blood goes out the pulmonary artery and the red blood goes out the aorta.
    • bi-directional Glenn: surgical procedure that frequently replaces the Blalock-Taussig shunt with another connection to the pulmonary artery to provide a path for blue blood to go out to the lungs. The superior vena cava is surgically connected to the right pulmonary artery to direct blood from the upper part of the body to the lungs to receive oxygen. This is the second procedure of the Fontan Sequence.
    • Blalock-Taussig shunt: a surgical procedure to create a pathway for blood to reach the lungs. A shunt (tube) may be inserted between the aorta or one of its branches and the pulmonary artery to increase blood flow. This is frequently the first procedure of the Fontan Sequence.
    • cardiac catheterization: an invasive diagnostic procedure performed under sedation to obtain detailed visual information and measurements about the structures inside the baby’s heart. Blood pressure and oxygen measurements are taken in the four chambers of the heart, as well as the pulmonary artery and aorta.
    • cardiac/cardio-: pertaining to the heart
    • cardiac magnetic resonance imaging (MRI): a non-invasive diagnostic tool using 3-D imaging technology produced by magnets to accurately determine the blood flow and functioning of your child's heart
    • cardiac surgery: any 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.
    • The Center for Families at Boston Children’s: dedicated to helping families find the information, services and resources they need to understand their child’s medical condition and take part in their care
    • chest x-ray: a diagnostic tool chest to evaluate the size and spatial relationships of the heart within the child’s chest, as well as the presence of TA and its associated defects
    • 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
    • congenital heart defect: heart defect present at birth. The heart usually starts to form in the first eight weeks of fetal development. It is thought that most congenital heart defects develop during this period.
    • diagnosis: medical determination of illness or disease based on history, physical examinations and advanced technology diagnostic testing tools
    • echocardiogram (echo, cardiac ultrasound): a diagnostic tool that evaluates the structure and function of the heart using sound waves that produce a moving picture of your child’s heart and heart valves. The ultrasound can be used to understand flow in the different chambers and to estimate pressures.
    • electrocardiogram (ECG, EKG): a diagnostic tool that evaluates the electrical activity of your child’s heart. An EKG is usually the initial test for evaluating the causes of symptoms and detecting heart abnormalities, including TA.
    • Fontan sequence: a series of three operations performed on children who have just one full-sized functioning ventricle (a type of single ventricle defect). The Fontan operations are usually performed at intervals starting within days or months after birth and ending at a few years of age. The Fontan operation is the third procedure in the Fontan Sequence.
    • pulmonary artery: one of the heart’s two great arteries, which normally arises from the right ventricle and carries oxygen-poor blood to the lungs, where it receives oxygen
    • pulmonary artery banding (PAB): a repair in which a band is secured around the pulmonary artery to limit blood flow to the lungs
    • pulmonary valve stenosis (PVS, PS): a congenital (present at birth) heart defect sometimes associated with TA in which the pulmonary valve is unable to open completely, making it harder for blood to flow from the right ventricle to the lungs
    • shunt: an artificial connection of blood vessels in order to redirect blood to the lungs in children with inadequate flow for oxygenation
    • single ventricle defect (SVD): one of several congenital (present at birth) heart defects in which the heart has only one fully functioning ventricle. TA is considered a single ventricle defect.
    • sporadic: occurring by chance, occasionally, not inherited
    • symptoms: the presenting reasons why a child needs medical attention. A symptom’s 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.
    • transposition of the great arteries (TGA): an uncommon defect associated with TA, in which the aorta and pulmonary artery are reversed in relation to each other. In TGA, the aorta comes off the right ventricle and the pulmonary artery arises from the left ventricle.
    • tricuspid atresia: a rare congenital (present at birth) heart defect in which the tricuspid valve is absent or blocked off, resulting in a small or absent right ventricle that cannot adequately pump blood to the lungs.
    • ventricular septal defect (VSD): a congenital (present at birth) heart defect associated with TA in which there's an opening in the tissue wall (septum) that divides the two lower chambers of the heart (right and left ventricles)

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

  • The exams

    If your newborn baby was born with a bluish tint to his skin, or if your young child is experiencing symptoms, your pediatrician will refer you to a pediatric cardiologist, who will perform a physical exam. Your cardiologist 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 Boston Children's Hospital 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. 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.

    The tests

    Some combination (not necessarily all) of the following medical tests will also used to diagnose TA and its related defects:

    • electrocardiogram (EKG): An EKG is used to evaluate the electrical activity of your child’s heart. It's usually the initial test for evaluating the causes of symptoms and for detecting heart abnormalities, including TA. 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.
    • echocardiogram (cardiac ultrasound): An echocardiogram 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. If your baby has TA, the ultrasound will reveal the absence of a tricuspid valve and an underdeveloped right ventricle. No discomfort is involved. It takes 30-60 minutes. Some younger children may need to be sedated.

      If, during your pregnancy, a routine prenatal ultrasound or other signs cause your obstetrician to suspect a congenital heart defect in the fetus, a cardiac ultrasound (described in the paragraph above) of the baby in utero will usually be the next step. The cardiac ultrasound—focusing exclusively on the baby’s heart—can usually detect whether a congenital heart defect is present.
    • cardiac magnetic resonance imaging (MRI): An MRI is a non-invasive test using 3-D imaging technology produced by magnets to accurately determine the blood flow and functioning of your child’s heart. No pain is involved, but an IV may be needed. It takes about an hour. Children under 10 years of age usually need anesthesia.
    • chest x-ray: A conventional chest x-ray will evaluate the size and spatial relationships of the heart within the child’s chest, as well as the presence of TA and associated defects. It takes a few moments. There's no pain or discomfort.
    • cardiac catheterization: This invasive procedure performed under sedation provides detailed visual information and measurements about the structures 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.
  • Having identified your child's heart condition, we're able to begin the process of treating him, so that we may ultimately return him to good health.

    Specific treatments for tricuspid atresia depend on the extent of the disease and other variables. Most likely, he will be admitted to Boston Children's Hospital's intensive care unit (ICU) or special care nursery. Initially, your child 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.

    Once he's stabilized, your baby's treatments will probably include:

    • cardiac catheterization: Prior to the initial TA surgery, or between staged operations (see below), doctors occasionally need to perform a cardiac catheterization procedure called a balloon atrial septostomy to improve the mixing of oxygen-rich (red) blood and oxygen-poor (blue) blood. A special catheter with a balloon in the tip is used to create or enlarge an opening in the atrial septum (wall between the left and right atria).

      You'll be comforted to know that Boston Children's pioneered interventional catheterization for many congenital heart defects.
       
    • medication: Doctors may administer an IV (intravenous) medication to prevent the closing of the infant's ductus arteriosus—the prenatal connection between the aorta and the pulmonary artery, which usually closes shortly after birth, but which is now important as a temporary alternative opening for blood flow.
    • surgery: Since tricuspid atresia with an underdeveloped right ventricle is considered a single ventricle defect (where the defect results in just one fully functioning ventricle), it's usually treated using the Fontan Sequence—a staged series of three operations performed between the first few days or months and the first few years of life.
    • The first stage is to optimize the blood flow to the lungs, whether it's too much or too little. If the blood flow is too little, a Blalock-Taussig shunt is usually performed. If it's too much, the pulmonary artery may be banded to control blood flow. If it's neither too little nor too much, the first stage may be skipped, and the second stage—the bi-directional Glenn—may be performed at 4 to 8 months of age.
    • Blalock-Taussig shunt: This first operation, done when the blood flow to the lungs is inadequate, is usually performed soon after birth to create a pathway for blood to reach the lungs. A connection is made between the first artery off the aorta (right subclavian artery) and the right pulmonary artery. Some of the blood traveling through the aorta towards the body will “shunt” through this connection and flow into the pulmonary artery to receive oxygen.

      Following the procedure, your child will still have some degree of cyanosis, since oxygen-poor (blue) blood from the right atrium and oxygen-rich (red) blood from the left side of the heart will mix and flow through the aorta to the body.
    • pulmonary artery banding (PAB): This alternative first operation, done when the blood flow to the lungs is excessive, is performed to reduce and limit pulmonary artery blood flow and to protect the pulmonary vessels from hypertrophy and pulmonary hypertension.
    • bi-directional Glenn: The second operation, often performed when a child is between 4 and 12 months old, reduces the left ventricle's workload, and thus the risk of damage, and sets the stage for the Fontan Procedure to come. This procedure replaces the Blalock-Taussig shunt (which the baby's heart will outgrow) with another connection to the pulmonary artery.

      The superior vena cava (the large vein that returns oxygen-poor blood from the head and arms back to the heart) is surgically connected to the right pulmonary artery so that blood can proceed to the lungs to receive oxygen.
    • Fontan procedure: This final operation in the sequence, done in the first few years of life, is performed for treatment of children with a single functioning ventricle, such as in TA.

      The principle of the procedure is that it's not necessary to have a ventricle that pumps to the lungs so long as the lung arteries have a low resistance, are well developed and are of good size, and so long as the single ventricle fills at a low pressure.

      Surgery involves directly connecting the returning blue blood into the pulmonary arteries. This can be achieved in a number of different ways—sometimes with, and sometimes without, the use of synthetic tubes (conduits).

    At home: caring for your child after tricuspid atresia surgery

    Babies usually remain cyanotic after the first two operations and until the final (Fontan) operation is performed. After the Fontan procedure, you can expect your child's oxygen levels to improve. In fact, many children experience major improvements in growth and development after Fontan, eventually catching up to other children.

    After each operation, your child will need to be followed by a pediatric cardiologist who will adjust your child's medications, measure his oxygen levels and determine when it's time for the next operation.

    Your child's cardiologist will also offer recommendations for post-operative follow-up care, including:

    • wound care
    • 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. And to the greatest extent possible, encourage your child to live normally. Even if some physical activities are limited, your child and your family can enjoy a full life together.

    Boston Children's Home Monitoring Program for your child

    An infant with single ventricle anatomy needs support with shunt-dependent blood flow between his Stage I and Stage II surgical repairs (see surgery descriptions above on this page).

    The results of the Stage I surgery have improved—with nearly 90% of infants who are cared for in experienced centers discharged home after the first stage. So we can now focus new attention on reducing the known mortality of 10 to15 percent for these infants between their Stage I and Stage II surgical repairs.

    Research shows the vital importance of a Home Monitoring Program, including daily at-home assessments of oxygen saturations and weight between the Stage I and Stage II surgeries.

    Checking your baby's daily weight:

    • alerts you and your child's providers to dehydration
    • enables appropriate immediate intervention
    • decreases the risk of sudden death at this very fragile stage in your infant's multiple-surgery sequence

    During this period between the Stage I and Stage II surgeries, your child's pediatric cardiologist and pediatrician will be in close contact with you as your child's primary home caregiver. (After your child's Stage II repair, this intensive level of home monitoring will no longer be necessary.)

    In Boston Children's Home Monitoring Program:

    • You'll be given detailed guidelines and goals for your child's:
    • growth
    • weight gain
    • oxygen saturations
    • You'll be instructed to call your child's cardiologist if his goals for growth, weight gain and oxygen saturation aren't met, or if:
      • your child is breathing harder, faster or is fussy beyond his baseline
      • you have any questions or concerns
    • You'll be supplied with a pulse oximeter and a baby scale and shown how to use both instruments. You'll measure your child's saturations twice a day and weigh the baby daily—checking against given guidelines and goals.
    • You'll record your baby's weights, oxygen saturations and general observations in a daily log, and will share this log with the pediatrician/cardiologist during check ups.
    • You'll consult your pediatrician if your child:
    • is experiencing nausea/vomiting/diarrhea
    • is not having sufficient wet diapers
    • is feverish
    • You'll have weekly calls with your designated pediatric nurse practitioner, who will consult with you, and who will notify your child's cardiologist of any concerns that arise.

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

    Surgical techniques for TA and its associated defects are continually being refined, with long-term outcomes continually improving. Nevertheless, TA patients will need lifelong monitoring and medication, since they will always be at some risk for arrhythmias, infections, heart failure or stroke.

    Your cardiologist will help you create a long-term care program as your baby grows into childhood, the teen years and even adulthood. Most people who've had congenital heart disease repair will have an ongoing relationship with their cardiologist. We'll prevent and treat complications, and will advise on daily-life issues, such as activity levels, nutrition and precautions related to pregnancy.

    Coping and support

    At Boston Children's, we understand that a hospital visit can be difficult, and sometimes overwhelming. So, we offer many amenities to make your child's—and your own—hospital experience as pleasant as possible. Visit The Center for Families for all you need to know about:

    • getting to Boston Children's
    • accommodations
    • navigating the hospital experience
    • resources that are available for your family

    In particular, we understand that you may have a lot of questions if your child is diagnosed with TA. How will it affect my child long term? What do we do next? We can connect you with a number of resources to help you and your family through this difficult time, including:

    • patient education: From the office visit to pre-op to the recovery room, our nurses will be on hand to walk you through your child's treatment and help answer any questions you may have—How long will I be separated from my child during surgery? What will the operating room be like? They'll also reach out to you by phone, continuing the care and support you received while at Boston Children's.
    • parent-to-parent: Want to talk with someone whose child has been treated for TA? We can often put you in touch with other families who've been through the same procedure that you and your child are facing, and who will share their experiences.
    • faith-based support: If you're in need of spiritual support, we'll connect you with the Boston Children's chaplaincy. Our program includes nearly a dozen clergy— 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 hospital experience.
    • social work: Our social workers and mental health clinicians have helped many families in your situation. We can offer counseling and assistance with 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 the Boston Adult Congenital Heart (BACH) and Pulmonary Hypertension Service. Boston Children's is a founding institution of BACH—an international center for excellence that provides long-term inpatient and outpatient care and advanced therapeutic options as needed for congenital heart disease patients as they reach and progress through adulthood.

    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 repair techniques now used widely for many congenital heart defects, including tricuspid atresia.

    A significant amount of the groundbreaking cardiac research currently being conducted at Boston 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 tricuspid atresia.

    Cardiac surgery research

    Members of the Boston Children’s Cardiac Surgery Research Laboratory—a multidisciplinary team of basic and applied research investigators who 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 research.

    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—all of whom 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 cardiology research.

    Innovations: 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. Pedro del Nido, MD, chief of Cardiac Surgery at Children’s, had to perform surgery on his tiny patients using this method, or come up with a way to improve it.

    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 to remove blockages, repair faulty valves and close leaks.

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

    Results: 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.

    cardioport

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

  • melvinWhen 9-year-old Melvin Trujillo flew to Boston Children's Hospital from his home in Acajutla, El Salvador, for open-heart surgery in February, nobody expected that the lethargic, blue—in color and in spirits—boy would end up stealing the hearts of so many of our staff. Extremely weak, weighing only 39 pounds, and standing at a mere 3 feet, 4 inches, Melvin was barely able to walk.

    Melvin was born with a congenital heart defect called tricuspid atresia, meaning that the right side of his heart didn't develop symmetrically with the left side. El Salvadorian hospitals aren't equipped to perform the surgery Melvin needed. But at Boston Children's, he got that surgery soon after he arrived, as Emile Bacha, MD, volunteered his time to perform a Fontan procedure that vastly improved his quality of life and increased Melvin's life expectancy by decades.

    Melvin had been registered with the organization Gift of Life New England, and two years of their fundraising, in conjunction with Boston University student members of the rotary club, paid off. They had enough money to sponsor a child with a life-threatening heart condition and fly that child to Boston for surgery—and Melvin's name was at the top of the list. Karen Jacobs, clinical professor at B.U. and Rotary advisor, says, "The Cardiology staff and Boston Children's donated all of their time, energy and love to make a difference in this child's life."

    After the surgery, Melvin's color returned to normal and his spirits soared. He was able to tackle his next hurdle: getting over his fascination with American TV, which had taken hold in the few days while he awaited surgery. Soon, Melvin began spending every possible moment in the playroom on 8 East. "He was riding Big Wheels down the hallway, giving high-fives and saying, 'thank you,' to everyone," says Lynne Polsi, Child Life specialist in Cardiology. "He even tried to say 'Sponge Bob Square Pants' in English before he left." Melvin also made friends with the B.U. students who'd worked hard to get him here, and after days of playing together, they all decorated a ceiling tile on 8 East with their handprints.

    Melvin's instant transformation into a spirited, outgoing boy was shocking for his mother, Maria Amaya. She'd been carrying him everywhere in El Salvador, since he was too weak to walk and his family couldn't afford a stroller. "He was so ill and now he is running around; I honestly feel like this is a miracle," she says in Spanish. Amaya had been frightened and overwhelmed when they arrived in America, but was soon put at ease. "The doctors and nurses impressed me because they treated me so well," she says. "Some of the staff spoke Spanish, so when I spoke in Spanish, I felt very comfortable." When Felicia Perez, an environmental assistant, noticed Amaya and Melvin speaking Spanish, she asked if Amaya had eaten anything. When she answered 'no,' Perez brought them in a dish of rice, beans, chicken and plantains that night after work. "I'm an immigrant in this country, too, so I know how it feels," says Perez.

    Clinical assistant in Cardiology Michelle Fernandez also put her Spanish skills to use, taking Melvin and Amaya through the cafeteria and distracting Melvin in Spanish during procedures. "He was so strong, even during painful procedures like getting his chest tubes removed," she says. "He had so much energy—he couldn't wait to play soccer when he got home." Melvin's goals don't stop at sports, though. He's also eager to go back to school, and even told Jacobs that he wants to be a doctor some day.

    Melvin was excited to go home at the end of February after being granted a clean bill of health. He'd missed his brother, sister, father and school pals. But, believe it or not, he was sad to leave the Boston winter. "The snow was my favorite part!" he said, in Spanish. "It's very beautiful, and I've never seen anything like it. My father asked me to bring him some." While Melvin will remember Boston for its snow flurries, his mom will never forget Boston for another reason. "It was in this city that the miracle happened, so my son can live," she says.

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