Truncus Arteriosus Repair

The skilled pediatric heart team at Children’s Health specializes in the latest procedures to treat even the rarest, most complex pediatric heart diseases. We treat truncus arteriosus, a condition in which a baby is born with only one main blood vessel leaving the heart and a hole between two heart chambers.

With advanced training and years of experience, we can repair your baby’s heart and provide ongoing care to maximize your child's heart health.

What is Pediatric Truncus Arteriosus Repair?

Truncus arteriosus repair is open-heart surgery for newborns to ensure proper blood flow to their lungs and body. Pediatric heart surgeons perform the procedure within the first few days or weeks of a baby’s life to repair the two heart defects that occur with truncus arteriosus:

  • Only one large blood vessel coming out of the heart, instead of two
  • An abnormal opening between the heart’s two lower chambers (called a ventricular septal defect)

What are the benefits of Pediatric Truncus Arteriosus Repair?

With truncus arteriosus, a baby’s heart pumps blood inefficiently, with too much blood going to the lungs and, sometimes, too little blood going to the body. The condition may prevent babies from growing and gaining weight as they should, and some babies may become very ill. Truncus arteriosus repair allows the heart to pump the right amount of blood to the body and the lungs

What are the risks of Pediatric Truncus Arteriosus Repair?

As with any open-heart surgery, there is a very small chance that the baby may not survive the surgery. This risk is higher if the child is very ill before surgery or is experiencing other major medical problems. Potential, though rare, complications include:

  • Stroke (brain injury)
  • Bleeding
  • Infection
  • Poor kidney function (temporary)

Truncus arteriosus repair involves placing a connection (called a conduit) between the right ventricle and the lung artery. Because the conduit will not grow, it will need to be replaced when the child outgrows it.

What are Children’s Health’s outcome metrics for Pediatric Truncus Arteriosus Repair?

Our outcomes for truncus arteriosus repair include a 100% survival rate and hospital stays that are shorter than national benchmark reports.

What to expect with Pediatric Truncus Arteriosus Repair

Doctors often diagnose truncus arteriosus within a few hours or days after your baby is born. In our Fetal Heart Program, pediatric cardiologists sometimes diagnose the condition before your baby is born.

We care for your newborn in our cardiac intensive care unit (CICU) until their surgery. If your baby is born at another hospital, we work with them to safely bring your baby to Children’s Health for surgery.

What to expect before Pediatric Truncus Arteriosus Repair

In our pediatric CICU, our team provides round-the-clock care and manages your baby’s condition before and after their surgery. Your baby will have high-calorie feedings to build their strength. We may also give medications before surgery to:

  • Strengthen your baby’s heart so it can pump better
  • Prevent fluid buildup throughout the body

What to expect during Pediatric Truncus Arteriosus Repair

Truncus arteriosus repair is an open-heart surgery that takes about five to six hours. Please prepare to spend the day at our Heart Center. A member of the care team will come to the family waiting room to update you about the progress.

During the surgery, a heart-lung bypass machine takes over your baby’s heart function to supply blood and oxygen to their body. Our pediatric heart surgeons will perform three procedures during the repair:

  1. Connect the right ventricle to the lung artery with a tube that has a valve in it (the conduit).
  2. Close the ventricular septal defect, an abnormal opening between the heart’s lower chambers.

What to expect after Pediatric Truncus Arteriosus Repair

Your newborn will spend about three to five weeks in the hospital after surgery. They spend the first one to two weeks in the pediatric CICU. Then they move to a regular hospital room for the remainder of their stay before going home.

Before we discharge your baby, our care team explains how to take care of your baby at home. We provide you with instructions on medications, follow-up appointments and symptoms to watch for.

As your child grows, they will need surgery or a minimally invasive procedure to replace the implanted tube and valve. This replacement may happen more than once depending on how quickly the tube and valve wear out.

What questions should I ask my provider about Pediatric Truncus Arteriosus Repair?

  • How many truncus arteriosus repairs have you performed?
  • How soon will I be able to see my child after surgery?
  • Will my child go home with any special equipment after truncus arteriosus repair?
  • What support services are available for families with a baby who has a congenital heart disease?

Truncus Arteriosus Repair Doctors and Providers

Our skilled heart surgeons, cardiologists and their teams provide exceptional care with a compassionate touch. Meet our providers.

Frequently Asked Questions

The cardiologist checks your child’s heart health using imaging and other heart diagnostic tests. With frequent monitoring, the doctor can find potential problems and treat them early. When your child becomes an adult, they can transition to our adult congenital heart disease program.

  • What other procedures will my child need?

    The tube and valve that connect the heart to the lung artery won’t grow with your child. Your child will need another open-heart surgery to replace this connection as they grow.

    Because the valve or tube wears out after the first replacement, your child may need another replacement during adolescence or young adulthood. Sometimes, we can use a minimally invasive procedure (cardiac catheterization) to replace the valve so your child won’t need open-heart surgery. Cardiac catheterization uses thin tubes (catheters) inserted through a blood vessel to access the heart. Our pediatric interventional cardiologists have extensive expertise in heart valve replacement using catheters.

  • What kind of follow-up care will my child need?

    Your child will need to see a cardiologist for regular follow-up visits for the rest of their life. The cardiologist monitors your child for signs of heart conditions, such as:

    • An irregular heart rhythm (arrhythmia)
    • A leaking heart valve
    • Weakening heart muscle