Truncus Arteriosus
Truncus arteriosus is a congenital heart defect in which a baby is born with one artery carrying blood to the lungs and body instead of two separate arteries. This happens when a single common blood vessel fails to separate into the aorta and pulmonary arteries during fetal development. The pediatric congenital heart experts at University Hospitals Rainbow Babies & Children’s provides expert diagnosis and care for infants with truncus arteriosus.

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What is Truncus Arteriosus?
In a normal heart, there are two main blood vessels that carry blood away from the heart. The aorta carries blood to the body, while the pulmonary artery carries blood to the lungs. These arteries each have separate valves that control blood flow. With truncus arteriosus, the heart only has one great blood vessel leaving the heart, which then branches into vessels that travel to the body and the lungs. This vessel usually has one large valve to control blood flow.
Babies with truncus arteriosus usually also have a ventricular septal defect (VSD), which is a hole in the wall, or septum, between the right and left ventricles (the lower chambers of the heart).
Another congenital heart defect that nearly always happens with this condition is a ventricular septal defect (VSD). This is an abnormal hole in the wall (septum) between the two lower chambers of the heart (right and left ventricles). This hole allows oxygen-rich and oxygen-poor blood to mix together so that the body gets less oxygen than it needs.
Symptoms of Truncus Arteriosus
Symptoms of truncus arteriosus are usually noticed within the first few days after a baby is born. Signs may include:
- Breathing problems
- Cyanosis (bluish-colored skin, lips or nails)
- Excess sleepiness or fatigue
- Heart murmur (irregular sounding heart beat)
- Low blood oxygen levels
- Sweating, especially while feeding
- Trouble with feeding/poor weight gain

What Causes Truncus Arteriosus?
The cause of truncus arteriosus is not always clear. In some cases, certain heart defects can run in families. The condition is often seen in babies with 22q11.2 deletion syndrome, or DiGeorge syndrome, which is a chromosomal disorder that can cause heart defects, cleft palate, poor immune function, and other problems.
How Is Truncus Arteriosus Diagnosed?
Sometimes, truncus arteriosus may show up on a prenatal ultrasound. After birth, your child’s provider may notice signs and symptoms that could warrant referral to a pediatric heart specialist. They will examine your child and listen to their heart and lungs with a stethoscope. They may also perform diagnostic tests such as:
Chest X-ray
A chest X-ray shows the size and shape of the heart and lungs.
Electrocardiogram (ECG)
Records the heart’s electrical activity over a period of time.
Echocardiogram (echo)
Uses sound waves (ultrasound) to show blood flow patterns in the heart.
Cardiac catheterization
A noninvasive procedure that uses a catheter (a thin, flexible tube) inserted through a blood vessel in the leg or groin and guided into the heart to look at details of the heart’s structure.
Pulse oximetry
Measures blood oxygen levels through a non-invasive finger or toe monitor.
Recovering from Surgery
The hospital stay for a child recovering from truncus arteriosus surgery is typically one to three weeks. Your baby will recover from surgery in the pediatric cardiac intensive care unit (PCICU), where they will be closely monitored by a team of pediatric cardiac intensive care physicians and will have access to other pediatric experts as needed. As they recover, your child will transition from the PCICU to the pediatric cardiac stepdown unit, where they will receive continuity of care by specially trained staff. Before discharge, our cardiac nursing staff will work with families to set up follow-up appointments, educate them on how to care for their child once they return home, and ensure they have the necessary equipment and supplies.
After surgery, your child should have more energy and begin to eat better and gain weight. They may need special formula or tube feeding for several weeks as they get stronger. But most children will go on to have normal active and healthy lives.
Treating Truncus Arteriosus
Babies with truncus arteriosus will need to have open heart surgery to correct the defect. This surgery usually takes place within the first month or two of life.
With this surgery, the pediatric cardiothoracic surgeons at UH Rainbow will separate the aorta and pulmonary arteries, providing a pathway for blood to travel from the right ventricle to the lungs. They will also repair the ventricular septal defect (if there is one) with a patch and correct any other heart defects at the same time. Prior to surgery, your baby may need medications, tube feeding, nutritional supplements or other non-surgical treatments to keep them stable until surgery can take place.
Surgery for truncus arteriosus is performed in our state-of-the-art hybrid catheterization and surgical suites. Our facility offers cutting-edge technology, including the most advanced imaging available worldwide and 3-D capabilities. This helps our surgeons better understand and visualize each patient’s unique anatomy while making complex repairs, leading to improved patient safety and outcomes.
Follow-up Care
They will need to continue to be monitored by a UH Rainbow pediatric cardiologist throughout childhood and into adulthood and will likely need further surgeries as they grow. These could include replacing conduits or valves or repairing aorta or pulmonary arteries. Because infants who undergo cardiac surgery are at a higher risk of neurodevelopmental delays, UH Rainbow offers complete developmental assessments through our Cardiac Neurodevelopmental Iniatitive (CaNDI).
When your child is older, the team at UH Rainbow can help transition them to a pediatric adult congenital heart expert to continue to monitor their heart function throughout their life.