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Truncus Arteriosis

  • We have an unusually large, interdisciplinary cardiovascular team, including cardiologists, cardiac surgeons, anesthesiologists, intensive care specialists, nurses, respiratory therapists, social workers and other caregivers. At every step, we base our decisions on the best available evidence and ongoing research, much of which is done here.

    ––Ronald Lacro, MD, associate in Cardiology, Boston Children's Hospital

    If your infant or child has been diagnosed with truncus arteriosus, an understanding of the condition will help you to cope with this rare and complex congenital (present at birth) heart defect. While very serious, truncus arteriosus is treatable through surgery, and the outlook for most babies with this cardiac defect is very positive. 

    Truncus arteriosus is a complex defect in which a single vessel (instead of the usual two separate arteries) arises from the heart. The normal embryonic heart starts with a single great artery, the truncus arteriosus, which normally divides into two--the aorta which pumps to the body, and the pulmonary artery which pumps to the lungs. Truncus arteriosus results when the single great vessel has fails to separate completely into the aorta and the pulmonary artery, and the truncus arteriosus pumps both to the body and to the lungs.

    • Truncus arteriosus also occurs with another defect—a ventricular septal defect (VSD), a hole in the muscular wall that normally separates the heart’s two pumping chambers (left and right ventricles).
    • Having just a single vessel means that both oxygen-rich (red) blood and oxygen-poor (blue) blood are mixing and flowing out the single vessel to the lungs and the body.
    • A greater-than-normal amount of blood flow through the pulmonary arteries to the lungs can cause congestive heart failure and lung damage.
    • Signs and symptoms can include mild cyanosis (blue color); fatigue; sweating; pale or cool skin; rapid, heavy or congested breathing; and rapid heart rate.
    • Truncus arteriosus is usually diagnosed before birth or soon after a baby’s birth.
    • Most children who’ve had surgery for truncus arteriosus recover and grow normally, although they’re at risk for future arrhythmias, leaky valves and other heart issues. They may need additional procedures before they reach adulthood.
    • Truncus arteriosus is rare, affecting less than 1 out of every 10,000 babies, and it occurs equally in boys and girls.
    • The condition makes up 1 percent of congenital heart defects.
    • Truncus arteriosus can be associated with chromosomal disorders, such as DiGeorge syndrome or 22q11 deletion syndrome.

    How Boston Children's Hospital approaches truncus arteriosus

    Our team in Boston Children’s  Cardiovascular Program understands how distressing a diagnosis of truncus arteriosus 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.

    Our specialized training in pediatric cardiology means that we understand the unique challenges, circumstances and intricacies of working with young people who have heart conditions. 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.

    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 (coronary arteries)—can negatively impact surgical outcomes if not identified ahead of time. We utilize the most advanced techniques available for precisely determining the patient’s cardiac anatomy, with interpretation by highly experienced cardiologists.
    • The most sophisticated and effective therapies, available 24/7: Many babies with truncus arteriosus require complex surgery shortly after they’re born. Our cardiac intensive care unit (CICU), cardiac cath labs, and operating rooms are well-prepared to deal with the urgent needs of babies with truncus arteriosus.
    • Close, expert medical follow-up before and after surgical repair: If you live in the Boston area, one of Boston Children’s cardiologists will follow you or your child; if you live in another part of the country or the world, a designated Boston Children’s cardiologist will work closely with your local cardiologist. Adult patients with truncus arteriosus are followed by Boston Children’s cardiologists who have special training for adults with congenital heart problems.
    • An experienced and skilled team for 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. Boston Children’s Cardiac Intensive Care Unit (CICU) is one of the very first such units to be developed anywhere, and professionals from many countries visit our CICU to learn advanced techniques of post-operative care.

    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 truncus arteriosus 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 even the most complex heart problems. Each year, we treat thousands of children, adolescents and even adults with a wide variety of heart defects and disorders.

    Comparison with normal heart

    In a normal heart, oxygen-poor (blue) blood returns from the body to the right atrium, 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 atrium on the left side of the heart. The left ventricle pumps the oxygen-rich blood through the aorta out to the body.

    Visit Heart and Blood Vessels for more on how the normal heart works.

    But in truncus arteriosus, oxygen-poor (blue) and oxygen-rich (red) blood mixes in the common truncal vessel. Some of the mixed blood flows through the branch that becomes the pulmonary artery and on to the lungs, and some of it goes into the aortic branch and continue to the body.

    The mixed blood that goes to the body doesn’t have as much oxygen as the body needs, and causes varying degrees of cyanosis (blue color of the skin, lips and nailbeds).

    Open heart surgery to separate the pulmonary artery and the aorta is needed, and is usually performed in the first few weeks of the baby’s life.

    Are there additional heart defects associated with truncus arteriosus?

    • Babies with truncus arteriosus also have a ventricular septal defect (VSD), an opening in the wall of tissue (ventricular septum) that normally separates the right and left ventricles. The VSD is closed at the time of surgical correction of the truncus arteriosus.
    • Often, the valve of the single vessel is defective in form and function, with abnormal leaflets that allow leakage (regurgitation) and/or obstruction of blood flow.
    • Truncus arteriosus can be associated with chromosomal disorders such as velo-cardio-facial syndrome (also known as 22q11 deletion syndrome or DiGeorge syndrome).
       

    Are there different variations of truncus arteriosus?

    truncus arteriosusYes, there are several different variations of the defect, depending on where and how the arteries arise from the trunk, and how the aortic arch is formed. Our doctors are highly experienced in treating all forms. The diagram below shows two different systems (Collett & Edwards and Van Praagh) for classifying the defect’s variations.

    What are the signs and symptoms of truncus arteriosus?

    The most common symptoms include:

    • cyanosis (blue color of the skin, lips and nail beds)
    • fatigue
    • sweating
    • pale skin
    • cool skin
    • rapid breathing
    • heavy breathing
    • rapid heart rate
    • congested breathing
    • disinterest in feeding, or tiring while feeding
    • poor weight gain

    How do you diagnose truncus arteriosus?

    Your child's physician may have heard a heart murmur during a physical examination and referred your child to a pediatric cardiologist for a diagnosis. A heart murmur is simply a noise caused by the turbulence of blood flowing through the heart defects. Symptoms your child exhibits also help with the diagnosis.

    Diagnostic tests are needed to help confirm the diagnosis, and may include:

    In addition to the above, follow-up testing after surgery and as your child grows can include:

    How does Boston Children's treat babies with truncus arteriosus?

    Your baby most likely will be admitted to Boston Children’s Cardiac Intensive Care Unit (CICU) once her symptoms become apparent.

    Truncus arteriosus must be treated by surgical repair. Your child may need to be on medication until the operation can take place. Treatment may include:

    Before surgery:

    • medications and diuretics
    • high-calorie feedings

    Surgical repair:

    • usually performed as soon as the infant is diagnosed—before the blood vessels in the lungs are overwhelmed by extra blood flow and become diseased
    • pulmonary arteries detached from the common artery (truncus arteriosus)
    • pulmonary arteries connected to the right ventricle
    • VSD (ventricular septal defect) closed with a patch
    • truncal valve pumps to the body

    How should we care for our child after surgery?

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

    • wound care while your baby is healing
    • a nutritional program to encourage weight gain

    As your baby recovers and grows, be sure to follow a regular program of well-baby/well-child checkups.

    Will my child be OK after surgery?

    Many infants who have surgery for truncus arteriosus will grow and develop normally, with activity levels, appetite and growth eventually returning to normal. But your child will need to be followed periodically by a pediatric cardiologist, who will check for any heart-related problems.

    Future procedures may be necessary before adulthood if:

    • the pulmonary artery branches are small and don’t grow well after surgery
    • the homograft or conduit connecting the right ventricle to the pulmonary artery—as well as any patches used in the initial repair—deteriorate and need to be replaced
    • the aortic (truncal) valve begins to leak (regurgitate)

    What causes truncus arteriosus?

    The heart forms during the first eight weeks of fetal development. The problem occurs at the midpoint of this time, when the aorta and the pulmonary artery don’t divide to form two distinct vessels, but rather, remain as a single great vessel. It isn’t entirely clear what causes congenital heart malformations, including truncus arteriosus, although in most cases it appears that some combination of genetics and environmental factors is involved.

    Truncus arteriosus and some other cyanotic congenital heart defects are sometimes associated with chromosomal disorders. If your child has such a disorder, Boston Children’s will arrange for a genetics consultation and referral to other specialists as needed.

    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 Boston 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.

    Genetic syndromes are relatively common with truncus arteriosus. If your child has a syndrome, we will arrange for a genetics consultation and referral to other specialists as needed.

    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, since she will be at some risk for:

    • narrowing or deterioration of the surgical repairs (she will outgrow the homograft or conduit)
    • leaky heart valves
    • abnormal heart rhythms (arrhythmias)

    Where can my child find care and support when she 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.

    FAQ

    Q: What is truncus arteriosus?
    A:
    Everyone starts with a single vessel that divides into two; in truncus arteriosus this division or separation doesn't occur. The condition is a cyanotic congenital heart defect in which a single prenatal vessel fails to separate into the two “great” vessels that take blood away from the heart to the lungs (the pulmonary artery) or to the body (the aorta).

    Q: Are there other defects associated with truncus arteriosus?
    A:
    Yes—children with truncus arteriosus also have a ventricular septal defect (VSD), which is a hole in the wall (septum) separating the left and right pumping chambers (ventricles) of the heart, and which allows blood from both sides of the heart to mix in the one great artery or truncus.

    Q: What causes truncus arteriosus?
    A:
    The heart forms during the first eight weeks of fetal development. The problem occurs in the midpoint of that time, when the single great artery or truncus fails to divide to form two separate vessels (the pulmonary artery and aorta). It isn’t clear what causes congenital heart malformations, including truncus arteriosus, although in most cases it appears that some combination of genetics and environmental factors is involved.

    Truncus arteriosus and some other cyanotic congenital heart defects are sometimes associated with 22q11 deletion syndrome and other syndromes.

    Q: If my child has truncus arteriosus, will she be OK?
    A:
    Most infants who have truncus arteriosus surgery grow and develop normally. But your child will need to be followed periodically by a pediatric cardiologist, who will monitor her for any heart-related problems. Additional procedures may be necessary as your child grows.

    Q: What are the symptoms of truncus arteriosus?
    A:
    The most common symptoms include:

    • cyanosis (blue color of the skin, lips and nail beds)
    • fatigue
    • sweating
    • pale skin
    • cool skin
    • rapid breathing
    • heavy breathing
    • rapid heart rate
    • congested breathing
    • disinterest in feeding, or tiring while feeding
    • poor weight gain

    Q: How does Boston Children’s treat truncus arteriosus?
    A:      

    Before surgery:

    • medications and diuretics
    • high-calorie feedings

    Surgical repair:

    • usually performed as soon as the infant is diagnosed—before the blood vessels in the lungs are overwhelmed by extra blood flow and become diseased
    • pulmonary arteries detached from the common artery (truncus arteriosus)
    • pulmonary arteries connected to the right ventricle
    • VSD (ventricular septal defect) closed with a patch

    Q: Can there be complications after surgery?
    A:
    Infrequently, complications can occur, such as:

    • infection
    • respiratory problems (such as fluid in the lungs)

    Q: What should we do at home after surgery?
    A:
    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

    As your baby recovers and grows, be sure to follow a regular program of well-baby/well-child checkups.

    Q: What’s the long-term outlook for my child?
    A:
    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. After truncus arteriosus, your growing child will need periodic monitoring, since she will be at some risk for:

    • abnormal heart rhythms (arrhythmias)
    • leaky heart valves
    • narrowing or deterioration of the surgical repairs (she will outgrow the homograft or conduit)

    Q: What is Boston Children’s experience treating congenital heart defects?
    A:
    Boston Children’s 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 for large pediatric cardiac centers.

    Our experts have pioneered some of medical science’s most advanced heart treatments, now in use around the globe. Children’s is among the only major pediatric heart centers in the world performing fetal heart interventions for certain congenital defects.

    Causes

    As a congenital heart defect, truncus arteriosus is a structural heart problem that your child was born with.The heart forms during the first eight weeks of fetal development. The problem occurs in the midpoint of that time, when the aorta and the pulmonary artery don’t divide to form two separate vessels, but rather, remain as a single great vessel. It isn’t clear what causes congenital heart malformations, including truncus arteriosus, although in most cases it appears that some combination of genetics and environmental factors is involved.

    Truncus arteriosus and some other cyanotic congenital heart defects are sometimes associated with some chromosomal disorders.

    What are the signs and symptoms of truncus arteriosus?

    • cyanosis (blue color of the skin, lips and nail beds)
    • fatigue
    • sweating
    • pale skin
    • cool skin
    • rapid breathing
    • heavy breathing
    • rapid heart rate
    • congested breathing
    • disinterest in feeding, or tiring while feeding
    • poor weight gain

    When to seek medical advice

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

    Questions to ask your doctor

    If your child is diagnosed with truncus arteriosus, you may feel overwhelmed with information. It can be easy to lose track of the questions that occur to you. Lots of parents find it helpful to jot down questions as they arise—that way, when you talk to your child’s doctors, you can be sure that all your concerns are addressed.

    Some questions might include:

    • What’s happened to my child’s heart, and why?
    • Are there different forms of truncus arteriosus? What form does my child have?
    • What tests will you perform to determine a treatment for my child?
    • Is this condition associated with a genetic syndrome? How will you determine whether or not it is?
    • What are my child’s treatment options?
    • Will my child be OK if she has truncus arteriosus? How soon will she be better?
    • Will there be restrictions on my child’s activities?
    • Will she need additional procedures as she grows?
    • Will there be long-term effects?
    • How should I talk to my child about her condition when she’s old enough to understand?
    • What can we do at home?

    Who’s at risk

    Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development. So, unless the condition is part of an underlying chromosomal disorder or syndrome, it’s difficult to predict who’s at risk.

    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, counselor, parent or teacher—they’re all on your team, and they all want to help.

    For adults

    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.

    You may need lifelong monitoring and medication, since you’ll continue to be at some risk for heart problems in the future. Going forward, your cardiologist will advise you on activity levels, pregnancy issues and certain lifestyle choices.

    Fortunately, Boston Children’s can help adults with congenital heart defects. Many adults who were patients at 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 your baby is healing from surgery
    • a nutritional program to encourage weight gain

    As your baby recovers and grows, be sure to follow a regular program of well-baby/well-child checkups.

    Truncus arteriosus 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. In truncus arteriosus, it remains connected with the pulmonary artery.
    • 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/cardio-: pertaining to the heart
    • cardiac magnetic resonance imaging (MRI): provides a picture of the heart and blood vessels, but without using x-rays. MRI is rarely used to diagnose babies with truncus arteriosus, but may be helpful in follow-up to surgery to detect complications.
    • 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-ray: shows the size and spatial relationships of the heart within the child’s chest. It takes a few moments. There’s no pain or discomfort.
    • 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’s 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): evaluates the structure and function of your child’s heart using electronically recorded sound waves that produce a moving picture of the heart, heart valves and blood vessels. For more, see Tests.
    • electrocardiogram(ECG or EKG):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 truncus arteriosus. For more, see Tests
    • heart-lung machine (cardio-pulmonary bypass): machine that does the work of the baby’s heart and lungs while open heart surgery is performed. The heart-lung machine protects the heart muscle, brain and other organs during the operation. 
    • IV: intravenous, into the vein; one method of delivering medication
    • neonatologist: doctor who specializes in illnesses affecting newborns, both premature and full-term
    • 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. In truncus arteriosus, it remains connected with the aorta.
    • sporadic: occurring by chance, occasionally, 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.
    • truncus arteriosus:a complex defect in which a single vessel (normally there are two separate arteries) arising from the heart functions as both the aorta and the pulmonary artery—the single great vessel has failed to separate completely, instead leaving a connection between the aorta and pulmonary artery.
    • ventricular septal defect (VSD): a congenital (present at birth) heart defect in which there is a hole in the tissue wall (septum) that divides the two lower chambers of the heart (right and left ventricles). This occurs in all babies with truncus arteriosus. The VSD is surgically closed at the time of surgical repair of truncus arteriosus.  

    Boston Children’s heart research and innovations

    A significant amount of Boston Children’s groundbreaking cardiac research aims to refine and advance the open heart surgery and catheterization procedures that treat congenital heart defects in newborns and young children—including truncus arteriosus. Boston Children’s Cardiac Surgery Research Laboratory is studying the mechanisms of heart disease and new treatments for children with congenital heart defects.

    Read the stories of Boston Children’s heart patients …  

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

  • 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 her 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 her blood (non-invasively) and make other observations that help to determine the diagnosis.

    Your child’s cardiologist will also investigate whether she 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, gives the cardiologist a better sense of the kind of heart problem your baby may have.

    Note: If your child’s truncus arteriosus was detected prenatally through ultrasound, upon birth she’ll be immediately admitted for stabilization and surgery.

    The tests

    Some or all of the following tests may be used to confirm a diagnosis of truncus arteriosus and its related defects, and to provide detailed information on the shape and condition of your baby’s heart:

    • echocardiogram (cardiac ultrasound): An echocardiogram is the chief diagnostic test for truncus arteriosus. It 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 can reveal the single vessel (truncus arteriosus), the ventricular septal defect (VSD) and any regurgitation from the truncal valve. No discomfort is involved. It takes 30-60 minutes.

    Note: If, during your pregnancy, a routine prenatal ultrasound or other signs raise your obstetrician’s suspicion of 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 (fetal echocardiogram)—focusing exclusively on the baby’s heart—can detect many congenital heart defects.

    • 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 any abnormalities or excess fluid in the lungs. It takes a few moments. There is no pain or discomfort.
    • electrocardiogram (EKG):An EKG evaluates the electrical activity of your child’s heart. It helps to evaluate the causes of symptoms and to detect heart abnormalities, including truncus arteriosus. 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.

    Tests to monitor the condition of the truncal (aortic) valve and the conduit repair over time can include:

    • cardiac catheterization: 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.
    • Catheterization can also be used in an intervention later on—for instance, to address an obstruction of the conduit that might have developed, or a valve that has started leaking.
    • cardiac magnetic resonance imaging(MRI): provides a picture of the heart and blood vessels, but without using x-rays. MRI is rarely used in babies with truncus arteriosus, but may be helpful in follow-up to surgery to detect complications.
  • Having identified your child's heart condition, our team at Boston Children's Hospital can begin treating her.

    Treatment options

    Your newborn will be admitted to our cardiac intensive care unit (CICU). Initially, she may be placed on oxygen or a ventilator to help her breathe, and IV (intravenous) medications may be given to help her heart and lungs function more efficiently.

    Truncus arteriosus must be treated through surgery. While your baby is waiting for surgery, we treat her with diuretics to reduce fluid in the lungs, as well as high-calorie feedings to strengthen her.

    Surgical repair

    Sugery is usually performed under general anesthesia when the infant is a few days to weeks old, but before the blood vessels in the lungs are overwhelmed by extra blood flow and become diseased.

    • In the procedure, the pulmonary arteries are detached from the common artery (truncus arteriosus).
    • The pulmonary arteries are connected to the right ventricle using a homograft (a section of pulmonary artery with its valves intact from a tissue donor).
    • Occasionally, an artificial conduit (a small tube containing a valve) is used instead of a homograft (human tissue valve).
    • The ventricular septal defect (VSD) is closed with a patch.
    • During the operation, a heart-lung machine does the work of the baby's heart and lungs. The machine protects the heart muscle, brain and other organs during the operation.

    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 from the surgery
    • a nutritional program to encourage weight gain
    • an oral hygiene program to prevent infection
    • an appropriate exercise regimen

    As your baby recovers and grows, be sure to follow a regular program of well-baby/well-child checkups.

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

    Surgical techniques for truncus arteriosus and its associated defects are continually being refined, and the long-term outlook for children who have this surgery is continually improving. Most children who've had surgery recover and grow normally. Even so, your child will need periodic monitoring, since she will be at risk for arrhythmias, leaky valves, narrowing or deterioration of the repair sites and other heart issues. She may need additional procedures as she grows, since she will eventually outgrow the homograft or conduit repair.

    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 treat any complications (often detected through cardiac MRI) 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 truncus arteriosus, you'll have a lot of questions. 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 truncus arteriosus? 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: 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 her 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.

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    … by creating a free Boston Children's Carepage.

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

    The Cardiovascular Program 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.. 

    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 we are a leader in the use of this procedure.

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

    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 truncus arteriosus.

    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 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—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 current projects in cardiology research.

    Innovations in treating congenital heart defects

    The Nikaidoh procedure

    The Nikaidoh procedure is an innovative aortic translocation operation for treating transposition of the great arteries when a ventricular septal defect (VSD) and pulmonary stenosis are present—a rare combination of defects. The procedure is performed only at Children’s and a handful of other specialty centers.

    In this procedure, the aorta is switched, along with the aortic valve, and is placed in the pulmonary position.

    To follow our patients long term, Children’s uses one of the only MRI scanners dedicated to pediatric cardiology. With our scanner, we can observe features like ventricular size and function, and we can look for the development of valve leakiness, obstruction of the great arteries or coronary artery obstruction.

    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.

    cardioport

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

    History of innovation

    In 1938, Boston Children’s cardiac surgeon Robert Gross, MD, performed the world’s first successful surgery to correct a child’s heart defect. Since that time, we have gained recognition around the globe for our leadership in pediatric cardiology and continue to make critical advances in the field. In 2011, U.S. News & World Report named Boston 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.

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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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