Fontan Procedure

A Fontan procedure is a heart surgery. It helps children born with only one working heart ventricle.

Usually, your heart has two ventricles. One sends blood to your lungs to get oxygen. The other sends oxygen-rich blood to the rest of your body. If one ventricle doesn’t form correctly, the other has to do both jobs. That makes your heart work harder.

A Fontan procedure creates a new path for blood to reach your child’s lungs. It sends oxygen-poor blood from their lower body straight to their lungs (via their pulmonary artery). This blood doesn’t pass through their heart. It takes a kind of shortcut to avoid heavy traffic. This way, the working ventricle can focus on pumping oxygen-rich blood to their body.

This surgery is the last of several procedures to help blood reach the lungs. Most children need one or more earlier surgeries first. These may include the:

• Norwood procedure: Helps the right side of their heart pump blood to your child’s body and lungs
• Glenn procedure: Sends oxygen-poor blood from your child’s upper body to their lungs
• Blalock-Taussig-Thomas (BTT) shunt: Directs blood from an artery near the collarbone to your child’s lungs
• Pulmonary artery band: Slows down blood flow to your child’s lungs and helps more blood reach their body

What does this procedure treat?

A Fontan procedure can treat several issues with how your heart forms before birth, like:

• Hypoplastic left heart syndrome (most common)
• Hypoplastic right heart syndrome
• Tricuspid atresia
• Pulmonary atresia (in some cases)
• Transposition of the great arteries (in some cases)
• Unbalanced atrioventricular canal defects
• Severe Ebstein’s anomaly
• Double inlet left ventricle (with underdeveloped right ventricle)
• Double outlet right ventricle (in some cases)
• Heterotaxy syndrome

How common is this procedure?

Each year, healthcare providers in the U.S. perform about 1,000 Fontan procedures.

Around 50,000 to 70,000 people worldwide have had this surgery.

At what age is Fontan surgery performed?

Children with a single ventricle issue may need a Fontan procedure between ages 2 and 15, most often between 2 to 5. But this surgery isn’t right for everyone.

Your child’s healthcare provider will check to be sure the Fontan procedure is a good option. For example, their working ventricle must be strong enough to pump well. And their lungs must be healthy enough to handle blood flow without a pump.

How should I prepare my child for a Fontan procedure?

Before a Fontan operation, your child will have tests, such as:

• Transthoracic echocardiogram (TTE)
• Electrocardiogram (EKG)
• Heart catheterization
• Breathing and oxygen level checks
• Imaging tests, like CT or MRI (in some cases)

What happens during a Fontan procedure?

In the operating room:

1. Your child gets anesthesia to fall asleep without pain.
2. Their care team uses a machine to do the work of their heart and lungs.
3. The surgeon connects your child’s inferior vena cava to their pulmonary artery with a tube. This sends blood from your child’s lower body to their lungs.

Sometimes, a surgeon does other heart repairs during the same surgery. These may include fixing a valve or widening the opening between heart chambers.

How long does this procedure take?

A Fontan surgery can take four to five hours. During that time, your child’s healthcare team will check in with you often and keep you updated.

What are the potential benefits and risks of this procedure?

A Fontan operation makes the heart’s job easier. It improves oxygen levels by keeping oxygen-rich and oxygen-poor blood from mixing as much.

Short-term complications of a Fontan procedure may include:

• Kidney problems
• Heart failure
• Irregular heartbeats
• Liver problems
• Fluid buildup around your child’s lungs (pleural effusions)

Possible long-term Fontan procedure complications include:

• Trouble absorbing protein (protein-losing enteropathy)
• Blood clots (embolism)
• Fontan-associated liver disease (FALD)
• Thick secretions blocking your child’s airways (plastic bronchitis)

Do all Fontan patients need a heart transplant?

No, but it’s common for Fontan patients to need heart transplants 15 to 20 years after a Fontan operation. Over time, your child’s heart may not pump as well or the Fontan circulation may cause progressive liver damage. This is called a “failed Fontan.” This is when your child may need a heart transplant or a combined heart-liver transplant.

One study found that 1 out of 10 adults needed a heart transplant after Fontan surgery.

What happens after a Fontan procedure?

Your child’s healthcare team will take them to an intensive care unit (ICU) to watch them closely. Next, they’ll go to a regular hospital room.

Before you go home, your care team will tell you how to care for your child.

What is the recovery time?

Most children who have a Fontan operation stay in the hospital for seven to 13 days. The first few days are in the ICU.

When should I call my healthcare provider?

After your child’s Fontan procedure, call their provider if they have:

• Bleeding or fluids from their chest wound
• Fever or signs of infection
• Increasing breathing difficulty

Your child should see their provider every six to 12 months. They may also need regular heart and liver tests.

Can a Fontan patient have children?

Sometimes, yes. People without complications may be able to have children. But there is a higher risk of bleeding, pregnancy loss (miscarriage), preterm birth and other issues. Always get a full heart check-up before trying to get pregnant.

What is the life expectancy after a Fontan procedure?

Life expectancy depends on the condition people have at birth. Most people are alive 10 years after Fontan surgery. And more than 8 out of 10 people live 30 years or longer.

In fact, many people who’ve had a Fontan procedure live into their 40s and longer.

Survival rates are lowest for people with hypoplastic left heart syndrome.