Fontan Procedure

Hypoplastic Left Heart Syndrome (HLHS) is a severe congenital heart defect (present at birth) in which the left side of the heart is underdeveloped. In a normal heart, the heart's left side has the job of pumping oxygenated blood into the aorta (large artery that carries blood to the body). In a child with HLHS, there are several problems: The mitral valve is too small or completely closed; the left ventricle is very small; and the aortic valve is too small or completely closed. This results in a situation where the left side of the heart is completely unable to support the circulation needed by the body's organs. Without treatment, HLHS is fatal, often within the first hours or days of life.

To address HLHS, three types of open-heart surgeries (involves dividing the breast bone / general anesthesia) must take place. These must follow a specific order: Norwood procedure (within several days of birth), Glenn Operation (within six months of birth), and the Fontan procedure (approximately at 2 to 4 years of age).

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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 Fontan 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 child will be sedated and then the 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 child sleep).
• 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 is to redirect blood flow from the lower body to the lungs. To do so, the inferior vena cava (IVC) is disconnected from the heart and rerouted directly to the pulmonary artery. Usually a large tube called a “conduit” is added to make the connection.
• Often, a small hole or “fenestration,” is created between the Fontan conduit and the right atrium. This lets some blood still flow directly back to the heart and acts as a "pop-off" valve as the lungs get used to the extra flow from the lower part of the body. This hole can be closed later with a cardiac catheterization.
• 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.