Truncus Arteriosus Repair

When the fetus develops during pregnancy, the heart starts with a single large blood vessel coming from the heart called the truncus arteriosus (TA). As fetal development progresses and the heart develops normally, the truncus divides into two arteries that carry blood out of the heart: Pulmonary artery and aorta. Sometimes the single large blood vessel fails to divide during fetal development and the baby is born with a heart that has one artery carrying blood out of it. This congenital heart defect (present at birth) is known as truncus arteriosus (TA). If left untreated, truncus arteriosus can be fatal.

To address TA, open-heart surgery (involves dividing the breast bone / general anesthesia) will be performed within the first 2 weeks of your baby’s life.

Do you have a question? Request more information and we will connect you with an RWJBarnabas Health cardiovascular expert.

What to Expect Before the Procedure

Before the procedure, your doctor may perform a variety of diagnostic tests, including:

• Echocardiogram (ECHO)
• Computed tomography (CT scan)
• Magnetic resonance imaging (MRI)
• X-Rays
• Electrocardiogram (EKG/ECG)

What to Expect During the Procedure

This procedure usually takes 5 hours to complete, but preparation and recovery may add several hours. The procedure is usually performed in the cardiothoracic operating room (OR). In general, during this procedure:

• Your baby will be sedated. Then your doctor will insert a breathing tube through your baby’s throat into their lungs and connect it to a ventilator. This will breathe for your baby during surgery.
• Your doctor will administer general anesthesia (will make your baby feel sleepy).
• The procedure begins when your doctor exposes your baby’s heart by dividing the breastbone (sternum) in half. Your doctor then spreads both halves to gain access to your baby’s heart (open-heart surgery).
• For this type of surgery, the heart must be still. Prior to doing so, your doctor will place tubes into your baby’s heart so that blood can be pumped through your baby’s body by use of a heart-lung machine. This machine takes over for the heart by replacing the heart’s pumping action and the lungs by adding oxygen to the blood.
• Once the blood has been diverted into the bypass machine for pumping, your doctor will then stop the heart by injecting it with a cold solution.
• When the heart has been stopped, your doctor will start with the procedure.
• The operation uses a conduit which is a tube made of either Dacron with a pig valve inside it or a donated human valve and artery (called a homograft valve). One end of the conduit/homograft is sewn into the right ventricle and the other end is sewn into the pulmonary artery.
• The branch vessels leading to the lungs are removed from the truncus and sewn into the conduit. Keep in mind, most children with truncus arteriosus will need their conduit/homograft replaced two to three times before they reach adulthood.
• After the procedure is completed, the doctor will closely check to make sure everything is working properly. Once checked, the doctor will let the blood circulating through the bypass machine back into your baby’s heart.
• Once the procedure is complete, the machine will be turned off. The tubes will be removed and the sternum will be sewn together with the use or sutures or surgical staples.

What to Expect After the Procedure

After the procedure, your baby will be taken to the cardiothoracic intensive recovery unit (CTICU) for a few days. Your baby will also spend several days in the recovery unit. During this time the cardiac team will:

General Guidelines

• Make sure your baby’s vital signs, such as heart rate and breathing, are watched.
• Make sure your baby feels no pain by giving pain medication.
• Make sure to look after your newborn’s medications.
• Manage the tubes (drains) that were placed in your baby’s chest during surgery.
• Take care of the chest wound (sternal incision) and any other incision sites.
• Make sure that your newborn is able to feed well by mouth.
• Will give you instructions to follow during your baby’s recovery.

Do you have a question? Request more information and we will connect you with an RWJBarnabas Health cardiovascular expert.