At one time, babies born with single-ventricular heart defects had low chances for recovery and a healthy future. The Fontan procedure, developed in 1971, changed that. Now, after decades of improvements to the procedure, most children survive to adulthood and enjoy a good quality of life.

If you or your child has had a Fontan procedure, lifelong monitoring is critical — not only for heart problems but also for effects on other organs, including the brain, lungs, liver and kidneys. Any possible complications need to be caught and treated as quickly as possible to prevent further damage.

If you or your child have had a Fontan procedure to correct a single-ventricle heart defect, you are invited to visit this multispecialty clinic. We accept patients from across the nation. No matter where the procedure was performed, our clinic can evaluate your needs and provide experienced, specialized care and monitoring. We can help anticipate and identify any changes or complications — so you can get the most effective treatment as quickly as possible.

Your doctor or cardiologist can refer you to the Fontan Clinic. We’ll work with your cardiologist and other healthcare providers to make sure you get all the care you need.

A Team of Specialists

The team of specialists at the Fontan Clinic at Phoenix Children’s know what to look for and how to respond effectively. No matter where the procedure was performed, we can provide follow-up testing and care, now and into the future.

The Fontan Clinic serves children and adults who have undergone the Fontan procedure and the surgeries leading up to it, the Norwood procedure and Glenn shunt. These surgeries are the mainstay of treatment for children and adults with congenital single-ventricle heart defects (also called univentricular hearts).

These rare conditions and their treatment can affect other organs, including the brain, lungs, liver and kidneys. People who have undergone the Fontan procedure may be at risk for poor growth, learning problems and mental health issues. The Fontan flow — the way your blood flows after a Fontan procedure — interacts with the entire body. We are here to help keep an eye on all the organs as they adjust to the Fontan flow. We can help detect any trouble before it becomes severe.

This procedure is the first of three surgeries — followed by the Glenn shunt and Fontan procedure — that Phoenix Children's Hospital uses to treat a severe form of congenital heart disease called hypoplastic left heart syndrome. Hypoplastic left heart syndrome occurs in babies born with a functioning right ventricle and a small, underdeveloped, nonfunctioning left ventricle.

This procedure is usually performed within the first two weeks of life and is one of the highest risk procedures in congenital heart surgery.

A physician implants a shunt, or small tube, to provide a connection for blood to flow from the heart to the blood vessels in the lungs, or pulmonary arteries. This is done so that blood can pick up oxygen and release carbon dioxide. The goal of the operation is to make the right ventricle the main pumping chamber for blood flow to the body.

During the procedure, the aorta is made larger to increase blood flow to the body. The outflow from the right ventricle (pulmonary artery) and outflow from the left ventricle (aorta) are connected side-by-side to allow all blood from either the right or left ventricles to reach the body.

A connection is also made to enable the blood traveling through the aorta toward the body to "shunt" through this connection and flow into the pulmonary artery to receive oxygen. This connection allows blood to flow to the lungs and may be accomplished with either a modified Blalock-Taussig shunt or a modified Sano procedure.

A Glenn shunt is the second of three surgeries — following the Norwood procedure and preceding the Fontan procedure — that Phoenix Children's Hospital uses to treat a severe form of congenital heart disease called hypoplastic left heart syndrome. Hypoplastic left heart syndrome occurs in babies born with a functioning right ventricle and a small, underdeveloped, nonfunctioning left ventricle.

During this operation, the Blalock-Taussig shunt that we placed during the prior Norwood procedure is removed. Then, the superior vena cava (the large vein that brings oxygen-poor blood from the head and arms back to the heart) is connected to the right pulmonary artery.

Blood from the head and arms passively flows into the pulmonary artery and proceeds to the lungs to receive oxygen. However, oxygen-poor (blue) blood returning to the heart from the lower body through the inferior vena cava will still mix with oxygen-rich (red) blood in the left heart and travel to the body. This means the child will remain cyanotic, or have a bluish or purplish discoloration of the skin.

This operation helps create some of the connections necessary for the final operation, the Fontan procedure.

The Fontan procedure is the third of three surgeries — following the Norwood procedure and Glenn shunt — that Phoenix Children's Hospital uses to treat a severe form of congenital heart disease called hypoplastic left heart syndrome. Hypoplastic left heart syndrome occurs in babies born with a functioning right ventricle and a small, underdeveloped, nonfunctioning left ventricle.

This procedure is performed about 18 to 36 months after the Glenn shunt.

During the Fontan procedure, the inferior vena cava (the blood vessel that drains deoxygenated blood from the lower part of the body into the heart) is connected to the pulmonary artery. The connection is formed by creating a channel through, or just outside, the heart to direct blood to the pulmonary artery.

This operation allows all of the oxygen-poor (blue) blood returning to the heart to flow into the pulmonary artery, greatly improving the blood’s oxygenation.

Complete Advanced Care at One Location

The Fontan Clinic was created so that all the specialists needed are available at one location. In most cases, our team members can meet with you during one visit — preventing the need for multiple trips to different locations. If needed, our social work and behavioral health specialists can help with school individual education plans (IEP) and 504 plans. This team approach helps reduce stress for your family and gives different specialists a chance to collaborate on each case.

In addition to providing advanced care, the team of specialists at the Fontan Clinic helps create educational tools for families and providers. They also help develop protocols and conduct database research to help ensure all Fontan patients receive the highest quality of care.

Let us help give your family peace of mind knowing that you’re getting the best possible care for life after a Fontan procedure.

What to Expect

Before your first visit, we will schedule you or your child for several tests. You may need tests to check your:

  • Heart and lungs: Echocardiogram, exercise testing/six-minute walk test, rhythm monitor
  • Liver function: Lab tests, ultrasound with elastography, liver magnetic resonance imaging (MRI)
  • Kidneys: Labs and renal ultrasound
  • Blood and urine
  • Neurodevelopment

Your first visit may take several hours, depending on your needs. You’ll see a number of different team members, which may include:

  • Cardiologists
  • Liver, kidney and lung specialists
  • A psychologist
  • A nutritionist
  • A social worker

After you or your child has been evaluated by all the specialists, the Fontan Clinic team will create a complete care plan and discuss it with your cardiologist. 

We’ll make plans for any medications or procedures needed. If you or your child needs any further specialty help, such as otolaryngology (ear, nose and throat) or endocrinology, we’ll coordinate the referrals.

After the first visit, you’ll need to follow up with your cardiologist every six months. You’ll meet with our team again in two years or as needed.

Locations

Phoenix Children's Hospital - Thomas Campus

Phoenix Children's Hospital - Thomas Campus

1919 E. Thomas Rd.
Phoenix, AZ 85016

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