Transposition of the Great Arteries (TGA)

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

    --David Harrild, MD, assistant in Cardiology

    This discussion refers to a heart defect known as “d-transposition of the great arteries” (“d-TGA,” “transposition,” “TGA”). Another type—“l-transposition” (“l-TGA,” “corrected transposition”)—is similar in some ways to d-TGA, but very different in others, and requires a different treatment program from what’s described here.

    If your infant or child has been diagnosed with transposition of the great arteries (TGA), an understanding of the condition will help you to cope with this rare and complex congenital (present at birth) heart defect. While very serious, TGA is treatable surgically at Children’s Hospital Boston, and the outlook for the vast majority of babies with this cardiac defect is excellent.

    In TGA, the “great” arteries are reversed in their origins from the heart, with the aorta connected to the right ventricle, and the pulmonary artery connected to the left ventricle—exactly the opposite of the normal heart’s anatomy.

    Transposition of the great arteries

    With these arteries reversed, oxygen-poor (blue) blood returns to the right atrium from the body, passes into the right ventricle, then goes into the aorta and back to the body. And oxygen-rich (red) blood returns to the left atrium from the lungs and passes into the left ventricle, which pumps it back to the lungs—the opposite of the way blood normally circulates. The oxygen-poor (blue) blood cannot meet the body’s demands, and this situation cannot support life.

    • TGA is almost always diagnosed in the first hours after birth.
    • Most babies with TGA are born with a small hole between their atria, which allows just enough red blood to get to the body to maintain life for a few hours.
    • TGA is life-threatening and in order to survive, babies with TGA need special therapy urgently soon after birth.
    • The most commonly used initial therapy is balloon atrial septostomy, where a balloon at the end of a catheter (small, flexible tube) is used to enlarge the opening between the atria.
    • Complete open heart repair generally takes place a few days later.
    • If there are no unusual risk factors, more than 98 percent of surgically treated infants survive their infancy.
    • Most children who’ve had TGA surgery recover and grow normally, although they can be at some risk in the future for arrhythmias, leaky valves and other heart issues.
    • While relatively rare (one in every 4,000 babies), TGA is among the most common cyanotic (“blue baby”) heart defects, and it affects boys more than girls.

    How Boston Children's Hospital approaches TGA

    Our team in Boston Children’s Cardiac Surgery Department understands how distressing a diagnosis of TGA 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 the patient’s cardiac anatomy, with interpretation by highly experienced cardiologists.
       
    • availability of the most sophisticated and effective types of therapy, 24/7: Many babies with TGA require emergency balloon septostomy, and a few need even more aggressive emergency therapy. Our cardiac intensive care unit (CICU), cardiac cath labs, and operating rooms are well-prepared to deal with the urgent needs of transposition patients.
       
    • close and 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 TGA are followed by Boston Children’s cardiologists who have special training for adults with congenital heart problems.
       
    • highly experienced and skilled team of 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. Boston Children’s CICU is one of the very first such units to be developed anywhere, and professionals from many countries visit the CICU to learn advanced techniques of post-operative care.

    Transposition of the great arteries: Reviewed by David Harrild, MD
    © Boston Children's Hospital, 2010

  • It’s natural for you to be concerned right now about your child’s health—a diagnosis of transposition of the great arteries 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.

    Comparison with normal heart

    In 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.

    transposition of great arteries

    But in TGA, oxygen-poor (blue) blood keeps circulating through the body, while oxygen-rich (red) blood keeps returning to the lungs—the exact opposite of the way blood is supposed to circulate.

    If there’s a large enough opening between the two atria, some of the red and blood will mix, providing enough oxygen for the baby for a period of days or weeks. A few babies will naturally have this opening, but for most patients, doctors must create this opening within the first few hours after birth. Open heart surgery, to re-connect the pulmonary artery and aorta to the appropriate ventricles, is needed for the longer run, and is usually done a few days later.

    Are there additional defects associated with TGA?

    Most babies with TGA have only that cardiac defect, but other heart defects that can occur with TGA:

    • atrial septal defect (ASD): an opening between the right and left atria. This defect occurs occasionally with TGA and is actually helpful; it can be easily corrected at the time of surgical correction.
    • ventricular septal defect (VSD): an opening in the wall of tissue (ventricular septum) separating the right and left ventricles. This occurs in about 20% of patients with TGA. If large enough, a VSD will need to be surgically closed at the time of surgical correction of the TGA.
    • patent ductus arteriosus (PDA): The ductus arteriosus is a blood vessel that’s normally open (patent) while the baby is inside the mother, but that closes naturally shortly after birth.Because keeping this vessel open helps increase the blood oxygen level in babies with TGA, doctors often give patients a medicine (prostaglandin E1) to keep the ductus open.
    • Miscellaneous other defects, such as narrowing of the aorta, infrequently occur.

    What are the signs and symptoms of TGA?

    Cyanosis (blue coloration of the skin) is the most common symptom of TGA.

    How do you diagnose TGA?

    For most patients, an echocardiogram and chest x-ray are all that’s needed to form a diagnosis. But in some circumstances, some or all of the following tests may be used to confirm a diagnosis of TGA and its related defects, and to provide detailed information on the exact shape of a patient’s heart:

    • 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.
    • 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 TGA. 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 (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. No discomfort is involved. It takes 30-60 minutes.
    • 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.

      Note: Cardiac catheterization can be an interventional tool, as well as a diagnostic tool. Most babies with TGA will require a balloon atrial septostomy as an initial therapy, performed in either the heart cath lab or the CICU. In this procedure, a balloon at the end of a catheter (small, flexible tube) is used to enlarge the opening between the atria.
    • 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 TGA, but may be helpful in follow-up to TGA surgery to detect complications.

    How do you treat TGA?

    Your baby most likely will be admitted to Boston Children’s cardiac intensive care unit (CICU) once his symptoms become apparent.

    • He may be placed on oxygen, and possibly a ventilator (breathing machine). Most babies will be given a medicine to keep the ductus arteriosus from closing (which helps increase the level of oxygen in the blood).
    • Most babies with TGA require a balloon atrial septostomy to improve mixing of oxygen-rich (red) blood and oxygen-poor (blue) blood between the right and left atria (upper chambers of the heart). The septostomy is a heart catheterization procedure, done in the heart catheterization laboratory or the CICU.
    • Within the first one to two weeks, TGA is surgically repaired through a procedure called an “arterial switch operation” (ASO).
      • A heart-lung machine does the work of the heart and lungs during the procedure.
      • The aorta and pulmonary arteries are disconnected, “switched” and reconnected to their proper ventricles.
      • The arteries that supply the heart (“coronary arteries”) must be re-connected to the newly positioned aorta so that “red” blood can supply the muscle of the heart.
      • The associated holes between the chambers of the heart are closed.
      • The heart is restarted as the heart-lung machine is withdrawn.

    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 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.

    Will my child be OK after TGA surgery?

    Most infants who have TGA surgery will grow and develop normally. But your child will need to be followed periodically by a pediatric cardiologist, who will check for any heart-related problems.

    The outlook varies from child to child, but open heart surgery at Boston Children’s has among the highest success rates in the United States among large pediatric cardiac centers.

    What causes transposition of the great arteries?

    The heart forms during the first eight weeks of fetal development. The problem occurs in the middle of these weeks, when the aorta and the pulmonary artery each attach to the incorrect heart chamber. It isn’t clear what causes congenital heart malformations, including TGA, although in most cases it appears that some combination of genetics and environment is involved.

    Few risk factors have been identified, but it appears that the risk of TGA is increased in mothers with type 1 diabetes mellitus (formerly called "insulin-dependent" or "juvenile"), and possibly with the ingestion of certain drugs, such as benzodiazepines.

    How common is TGA?

    While relatively rare (one in every 4,000 babies), TGA is among the most common cyanotic (“blue baby”) heart defects, and it affects boys more than girls.

    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.

    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 he could be at some risk for:

    • abnormal heart rhythms (arrhythmias)
    • leaky heart valves
    • narrowing of one or both switched arteries at the site(s) of the switch
    • narrowing of the coronary arteries at the site of their switch

    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.

    FAQ

    Q: What is transposition of the great arteries (TGA)?
    A:
    Transposition of the great arteries is a congenital heart defect in which the two “great” vessels that take blood away from the heart to the lungs (the pulmonary artery) or to the body (the aorta) are reversed. This means that the aorta is connected to the right ventricle, and the pulmonary artery is connected to the left ventricle—the opposite of a normal heart’s anatomy.

    Q: Are there other defects associated with TGA?
    A:
    Other heart defects occasionally occur with TGA.

    • ASD and/or VSD: Openings in the walls (septa) separating the left and right sides of the heart, called an atrial septal defect (ASD) or a ventricular septal defect (VSD), allow blood from one side to mix with blood from another.
    • Miscellaneous other defects, such as narrowing of the aorta, infrequently occur.

    Q: If my child has TGA, will he be OK?
    A:
    Most infants who have TGA surgery will grow and develop normally. But your child will need to be followed periodically by a pediatric cardiologist, who will check for any heart-related problems.

    The outlook varies from child to child, but open heart surgery at Boston Children’s has among the highest success rates in the United States among large pediatric cardiac centers.

    Q: How does Children’s treat TGA?
    A:

    • An infant with TGA may be placed on oxygen, and possibly a ventilator (breathing machine). Most babies will be given a medicine to keep the ductus arteriosus from closing (which helps increase the level of oxygen in the blood).
    • Most babies with TGA require a balloon atrial septostomy to improve mixing of oxygen-rich (red) blood and oxygen-poor (blue) blood between the right and left atria (upper chambers of the heart). The septostomy is a heart catheterization procedure, done in the heart catheterization laboratory or the CICU.
    • Within the first one to two weeks, TGA is surgically repaired through a procedure called an “arterial switch operation” (ASO).

    Q: What are the symptoms of TGA?
    A:
    Cyanosis (blue coloration of the skin) is the most common symptom of TGA.

    Q: What should we do at home after TGA 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
    • 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.

    Q: What’s the long-term outlook for TGA-affected children?
    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 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.

    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 he could be at some risk for:

    • abnormal heart rhythms (arrhythmias)
    • leaky heart valves
    • narrowing of one or both switched arteries at the site(s) of the switch
    • narrowing of the coronary arteries at the site of their switch

    Q: What causes TGA?
    A:
    The heart forms during the first eight weeks of fetal development. The problem occurs in the middle of these weeks, when the aorta and the pulmonary artery each attach to the incorrect heart chamber. It isn’t clear what causes congenital heart malformations, including TGA, although in most cases it appears that some combination of genetics and environment is involved.

    Few risk factors have been identified, but it appears that the risk of TGA is increased in mothers with type 1 diabetes mellitus, and possibly with the ingestion of certain drugs, such as benzodiazepines.

    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 among large pediatric cardiac centers.

    Our experts have pioneered some of medical science’s most advanced heart treatments, now in use around the globe. Boston 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, TGA 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. The problem occurs in the middle of these weeks, when the aorta and the pulmonary artery each attach to the incorrect heart chamber. It isn’t clear what causes congenital heart malformations, including TGA, although in most cases it appears that some combination of genetics and environment is involved.

    Few risk factors have been identified, but it appears that the risk of TGA is increased in mothers with type 1 diabetes mellitus (formerly known as "insulin-dependent" or "juvenile") , and possibly with the ingestion of certain drugs, such as benzodiazepines.

    Signs and symptoms

    Cyanosis (blue coloration of the skin) is the most common symptom of TGA.

    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 too easily.

    Questions to ask your doctor

    After your child is diagnosed with TGA, 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 happening to my child, and why?
    • What tests will you perform to determine a treatment for my child?
    • What are the treatment options?
    • Are there alternative therapies?
    • Will my child be OK if he has congenital heart disease?
    • Will there be restrictions on my child’s activities?
    • Will there be long-term effects?
    • 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, it’s difficult to predict who’s at risk. Familial cases have been reported, but no genetic link has been confirmed. 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.

    Few risk factors have been identified, but it appears that the risk of TGA is increased in mothers with insulin-dependent diabetes mellitus, and possibly with ingestion of certain drugs, such as benzodiazepines.

    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

    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 the “atrial switch” procedure for TGA (which is now infrequently used), you may be at greater risk for cardiac complications than if you had the “arterial switch” procedure more commonly in use now.

    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, 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 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.

    TGA 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 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 occurs occasionally with TGA and is actually helpful; it can be easily corrected at the time of surgical correction.
    • 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 TGA, a catheterization procedure called balloon atrial septostomy may be performed to improve the mixing of oxygen-rich (red) blood and oxygen-poor (blue) blood between the right and left atria.
    • 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 in babies with TGA, but may be helpful in follow-up to TGA 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
    • ductus arteriosis, patent ductus arteriosus: a blood vessel that connects the aorta to the pulmonary artery. Before birth, this vessel helps blood flow in the fetus. It usually closes shortly after a baby’s birth. Because an open (patent) ductus can help increase the oxygen level in the blood in a patient with TGA, a medication to keep this vessel from closing (prostaglandin E1) is often given until the great vessels can be repaired surgically.
    • 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. Can be used to understand flow in the different chambers and to estimate pressures. 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 usually the initial test for evaluating the causes of symptoms and detecting heart abnormalities, including TGA. 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.
    • 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
    • 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.
    • ventricular septal defect (VSD): a congenital (present at birth) heart defect in which there is an opening in the tissue wall (septum) that divides the two lower chambers of the heart (right and left ventricles). This occurs in about 20% of patients with TGA. If large enough, a VSD will need to be surgically closed at the time of surgical correction of the TGA.
  • 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 (there isn’t always with TGA), 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 child’s TGA was detected prenatally through ultrasound, upon birth he’ll be immediately admitted for stabilization and surgery.

    The tests

    For most patients, a echocardiogram and chest x-ray are all that’s needed to form a diagnosis. But in some circumstances, some or all of the following tests may be used to confirm a diagnosis of TGA and its related defects, and to provide detailed information on the exact shape of a patient’s heart:

    • 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. No discomfort is involved. It takes 30-60 minutes.

      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—focusing exclusively on the baby’s heart—can usually detect a congenital heart defect.
       
    • chest x-ray: A conventional chest x-ray will evaluate the size and spatial relationships of the heart within the child’s chest. It takes a few moments. There's no pain or discomfort.
       
    • electrocardiogram (EKG): An EKG is used to evaluate 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 TGA. 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.
       
    • 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.
       
    • 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 TGA, but may be helpful in follow-up to TGA surgery to detect complications.
  • 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.

    Treatment options

    Upon birth, your newborn will be admitted to Boston Children's Hospital's cardiac intensive care unit (CICU). 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.

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

    • medication: Doctors may administer an IV (intravenous) medication (prostaglandin E1) to keep open 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).
    • cardiac catheterization: Before TGA surgery, doctors may perform a cardiac catheterization procedure called 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).
    • surgery: Within a baby's first one to two weeks, transposition of the great arteries is surgically repaired through a procedure called an “arterial switch,” which roughly describes the surgical process. A heart-lung machine does the work of the heart, while the aorta and pulmonary arteries are disconnected, “switched” and reconnected to their proper ventricles.

      As part of the procedure:
    • The coronary arteries are repositioned to the newly positioned aorta so that “red” blood can supply the muscle of the heart.
    • The associated holes between the chambers of the heart are closed.
    • The heart is reactivated as the heart-lung machine is withdrawn.

    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.

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

    Surgical techniques for TGA and its associated defects are continually being refined, and the long-term outlook is continually improving. Most children who've had TGA surgery recover and grow normally. Even so, your child will need periodic monitoring, since he may be at some risk for arrhythmias, leaky valves, narrowing of the arteries at the sites of the switch, and other heart issues.

    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 (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 TGA, 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 TGA? 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). 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.
  • 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 transposition of the great arteries.

    And 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 transposition of the great arteries.

    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

    The Nikaidoh procedure

    The Nikaidoh procedure is an innovative aortic translocation operation for treating transposition of the great arteries when a VSD and pulmonary stenosis are present—a rare combination of defects. The procedure is performed at only at Boston 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—avoiding leaks of the abnormal pulmonary valve on the right side.

    To follow our patients long term, Boston 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 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.

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

  • AJ’s complex heart defect

    I was 18 weeks pregnant when we went for a routine ultrasound to check to see what gender our baby would be. It was a boy and my husband and I were thrilled to become first time parents. A few days later we got a call to go to a level 2 ultrasound because they saw a possible hole in our baby's heart.

    We were quickly whisked to a genetic counselor and she made an appointment to get an echocardiogram with the Advanced Fetal Center at Children's. We soon found out that our baby had a complex Congenital Heart Defect.

    We had no idea what a heart defect was until Dr. Wayne Tworetzky explained in detail what would happen. His diagnosis was Transposition of the Great Vessels, Ventricular Septal Defect, and Pulmonary Atresia. This was heartbreaking! We went through the rest of the pregnancy not really knowing what to expect, but I received amazing care from Brigham and Woman’s Hospital and Children’s. They prepared us with tours of the Cardiac Intensive Care Unit and talked us through the steps that would be taken when he was born.

    The day finally arrived and AJ came into this world two and a half weeks early, weighing 7lbs 2oz and 19 1/2 inches long. Since we were prepared for him to come out not crying and having trouble breathing, we were quickly relieved when he came out pink and screaming. I actually got to hold him a few seconds before he was taken to the NICU, and then over to Children's. Our journey was beginning.

    While we awaited his surgery, we met some of the most amazing surgeons, doctors, and nurses.  AJ was 4 days old when he got his repair. The morning of his surgery was nerve racking. We just wanted it to be over and for him to heal so we could take our baby home. When they took him down it was the hardest moment for us, but we knew everyone that was with him was going to take the best care of our little guy. The surgery took four hours and then his surgeon, Dr. Bacha, came to tell us how it went. He told us AJ did great and went over the logistics and what to expect once we saw him.

    During the next three weeks, we spent ever waking minute between the CICU and 8 east. It seemed as though time was going by so slowly, but each day he was making progress. We had a few bumps, but minor ones, and before we knew it, we took our baby home to begin our lives together. Over the next few weeks, we had a few more unexpected returns and overnight stays on the 8th floor.  He ended up having to get a pacemaker because the closure of his VSD was causing his top chamber to be unable to communicate with the bottom chamber. We knew this may have been a possibility. We were finally able to make our way officially home when AJ was 6 weeks old.

    Since we have been home, we’ve lived a relatively normal life. AJ is developmentally on track, which was a topic of conversation before he was born. We have to go to more appointments to see his cardiologist than a typical child, we take a few extra precautions due to his pacemaker, and he will have more heart surgeries throughout his life, but normalcy surpasses it. By looking at AJ you would never know that he had such a traumatic entrance into the world, until you lift up his shirt to see his scars.

    Without Boston Children's Hospital, AJ would not be as vibrate and amazing as he is. He is truly is miracle and each day we are reminded how thankful and blessed we are because of this remarkable hospital.

    --- Christine Sorrentino

Request an Appointment

If this is a medical emergency, please dial 9-1-1. This form should not be used in an emergency.

Patient Information
Date of Birth:
Contact Information
Appointment Details
Send RequestIf you do not see the specialty you are looking for, please call us at: 617-355-6000.International visitors should call International Health Services at +1-617-355-5209.
Please complete all required fields

This department is currently not accepting appointment requests online. Please call us at: 617-355-6000. International +1-617-355-6000.

This department is currently not accepting appointment requests online. Please call us at: 617-355-6000. International +1-617-355-6000.

Thank you.

Your request has been successfully submitted

You will be contacted within 1 business day.

If you have questions or would like more information, please call:

617-355-6000 +1-617-355-6000
close
Find a Doctor
Search by Clinician's Last Name or Specialty:
Select by Location:
Search by First Letter of Clinician's Last Name: *ABCDEFGHIJKLMNOPQRSTUVWXYZ
More optionsSearch
Condition & Treatments
Search for a Condition or Treatment:
Show Items Starting With: *ABCDEFGHIJKLMNOPQRSTUVWXYZ
View allSearch
Locations

Contact the Complex Biventricular Repair Program

Related Conditions

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.”
- Sandra L. Fenwick, President and CEO
Close