Fontan Procedure

What is the Fontan procedure?

A Fontan procedure improves circulation in people who have only one working heart ventricle. This problem happens because of one of several congenital (since birth) heart diseases.

The Fontan procedure lets oxygen-poor blood from the lower part of your body go straight to your pulmonary artery (and then lungs) for oxygen instead of going through your heart first. This procedure usually follows a different operation (Glenn procedure) that sends oxygen-poor blood from your upper body to your pulmonary artery and lungs.

After the Fontan heart procedure, all oxygen-poor blood goes to and through your pulmonary artery to your lungs instead of going through your heart first. This blood flows in a passive way, or without a pumping ventricle to move it.

Normally, your heart has four chambers: two upper chambers (atria) and two lower chambers (ventricles). When one of your ventricles, or one of the main valves in the heart, doesn’t form adequately before birth, your other ventricle has to make up for the small or nonfunctioning ventricle.

This one ventricle has to pump:

• Oxygen-poor blood to your lungs to get oxygen.
• Oxygen-rich blood to your cells so they can use it.

When the Fontan procedure sends blood to your lungs passively through a direct route, your one functioning ventricle only has to pump oxygen-rich blood to your body.

Who needs to have a Fontan procedure?

Children between 2 and 15 (usually 3 to 5) years old who were born with a single ventricle issue may need to have a Fontan heart procedure. It’s not for everyone, though. Your child’s healthcare provider will check to make sure they’re a good candidate for the Fontan procedure. For example, their working ventricle needs to be strong enough to pump well, and the lungs have to be healthy enough to receive passive blood flow.

Why is a Fontan procedure done?

A Fontan surgical procedure is often the last of the single-ventricle procedures that eventually send all of a child’s oxygen-poor blood passively to their pulmonary artery instead of through their heart. Their pulmonary artery leads to their lungs, where their blood picks up oxygen.

Before having a Fontan heart procedure, your child usually needs to have at least one other procedure first, such as:

• Norwood procedure (letting your heart’s right side get blood to your body and your lungs).
• Glenn procedure (sending oxygen-poor blood from your upper body through the superior vena cava to your pulmonary artery).
• Blalock-Taussig-Thomas (BTT) shunt (directing blood from your subclavian artery, a major artery, to a pulmonary artery).
• Pulmonary artery band (reducing the amount of blood that goes to the lungs and increasing the amount of blood that goes to the body).

What is the Fontan procedure used for?

A Fontan procedure can treat several heart conditions that involve a ventricle or valve issue, such as:

• Hypoplastic left heart syndrome (the most common reason for a Fontan procedure).
• Hypoplastic right heart syndrome.
• Tricuspid atresia.
• Pulmonary atresia with intact ventricular septum.
• Congenitally corrected transposition of the great arteries.
• Unbalanced atrioventricular canal defects.
• Severe Ebstein’s anomaly.
• Double inlet left ventricle.
• Heterotaxy syndrome (some organs aren’t in their normal locations).

How common are Fontan procedures?

Each year, healthcare providers in the United States perform about 1,000 Fontan procedures.

An estimated 50,000 to 80,000 people worldwide have had a Fontan procedure.

What happens before a Fontan procedure?

After your child’s healthcare provider decides your child is a candidate for the Fontan procedure, they’ll do tests, which may include:

• Transthoracic echocardiogram (TTE).
• Electrocardiogram (EKG).
• Heart catheterization.
• Pulmonary assessment (checking your child’s breathing and oxygen level).
• Imaging, such as CT or MRI, in some cases.

What happens during a Fontan procedure?

In the operating room on surgery day, your child’s healthcare provider will give your child anesthesia to make them sleep deeply and without pain. The surgeon will connect your child to a machine that temporarily does the work of their heart and lungs while they connect your child’s inferior vena cava to their pulmonary artery. This sends oxygen-poor blood from your child’s lower body straight to the artery that goes to their lungs.

Sometimes a surgeon will perform additional heart procedures at the same time, such as increasing the opening between the upper chambers (atria), or repairing one of the heart valves. Depending on the health of their heart and lungs, your child may also have a small fenestration (opening) created in the tube that connects the inferior vena cava to the pulmonary artery.

How long does the Fontan procedure take?

A Fontan procedure can take four to five hours. Your child’s healthcare team will give you updates as soon as they can — and often — during the procedure.

What happens after a Fontan procedure?

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

Your healthcare provider will give you instructions about how to care for your child when it’s time to go home.

What are the advantages of a Fontan heart procedure?

A Fontan surgical procedure:

• Takes some of the workload away from a child’s one working ventricle.
• Reduces the amount of oxygen-rich blood and oxygen-poor blood blending.
• Improves a child’s oxygen level in their blood.

What are the risks or complications of a Fontan procedure?

Short-term Fontan procedure complications may include:

• Kidney disease.
• Heart failure.
• Abnormal heart rhythm.
• Liver disease.
• Pleural effusions.

Possible long-term Fontan procedure complications include:

• Protein-losing enteropathy (inability to absorb proteins during digestion).
• Thromboembolism (a blood clot that breaks off and blocks a blood vessel).
• Fontan-associated liver disease (FALD).
• Plastic bronchitis (fluid buildup blocks your airways).

Over a long period of time, cardiac output (the amount of blood your heart can pump) can be low after a Fontan procedure. When the procedure doesn’t provide results anymore, healthcare providers call it a failure or failed Fontan procedure. At that point, the person may need a heart transplant. This is common at some point after a Fontan procedure.

What is the recovery time?

Most people who have a Fontan surgical procedure stay in the hospital for an average of seven to 13 days. The first few days will be in the ICU. The other days will be in a regular hospital room.

How long do Fontan patients live?

About 90% of people who have the Fontan surgical procedure are still alive 10 years later. 30 years after surgery, depending on the initial heart and lung anatomy, 85% of people survive.

Many people who’ve had a Fontan procedure are living into their 50s and longer.

Fontan procedure life expectancy differs for the various conditions that require a Fontan procedure. Survival rates are lowest for people with hypoplastic left heart syndrome.

When should I see my healthcare provider?

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

• Blood or other fluids coming out of their chest wound.
• Fever or other signs of infection.

Your child should see their healthcare provider every six to 12 months after a Fontan heart procedure. They may also need regular testing, such as:

• Blood tests.
• Electrocardiogram (EKG).
• Chest X-ray.
• Heart MRI.
• Thoracic echocardiogram (TTE).
• Holter monitor.
• Liver ultrasound and/or magnetic resonance imaging (MRI).
• Annual liver function tests.

A note from Cleveland Clinic

In the past, the outlook for a baby born with only one working heart ventricle wasn’t good. Today, many children with this issue live past childhood and into adulthood. After their Fontan procedure, your child will need lifelong follow-ups with their cardiologist. You child’s condition is complex, so don’t be afraid to ask questions if there’s anything you don’t understand about it. Having a good understanding of your child’s condition will help you take good care of them.