KidsMD Health Topics

Truncus Arteriosis in Children

  • 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. 

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