Tetralogy of Fallot Repair

Tetralogy of Fallot is a congenital heart defect (present at birth). It is composed of 4 major characteristics: A hole in the ventricular septal wall (ventricular septal defect); aorta is on top of both ventricles, instead of just the left ventricle (overriding aorta); narrowing of the pulmonary valve or the pulmonary arteries (pulmonary stenosis); and the right ventricle becomes thicker and more muscular than normal (hypertrophy).

Together, these four defects mean that not enough blood is able to reach the lungs to get oxygen, and oxygen-poor blood flows out to the body. Tetralogy of Fallot must be repaired either soon after birth or later in infancy. The timing of the surgery depends on how severely the pulmonary valve is narrowed.

To address Tetralogy of Fallot, open-heart surgery (involves dividing the breast bone / general anesthesia) will be performed.

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 to 6 hours, 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 a 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 goal of the surgery is to improve blood flow to the lungs and to make sure that oxygen-rich and oxygen-poor blood flows to the right places.
• First, the pulmonary valve is widened or replaced, and the passage from the right ventricle to the pulmonary artery is enlarged. These procedures improve blood flow to the lungs. This allows the blood to get enough oxygen to meet the body's needs.
• Second, a patch is used to cover the hole in the ventricular septum (VSD). This patch stops oxygen-rich and oxygen-poor blood from mixing between the ventricles.
• Fixing these two defects resolves problems caused by the other two defects. When the right ventricle no longer has to work so hard to pump blood to the lungs, it will return to a normal thickness. Fixing the VSD means that only oxygen-rich blood will flow out of the left ventricle into the aorta.
• 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.