In the past, babies born with a single ventricle heart defect had limited treatment options and little chance of surviving early childhood. Now, through a series of three to four palliative surgeries and ongoing disease management, many children born with single ventricular disorders can survive to adulthood and have an active life.

The Fontan procedure is the third and final surgery in the treatment of single ventricle congenital heart disease. Although this advanced procedure has increased survival rates dramatically since its introduction in 1971, it is not a cure. Patients who have the three surgeries are at higher risk for developing complications affecting multiple organ systems, making life-long management of their heart vital to their overall health.

The Fontan Clinic at Phoenix Children’s is the only center in Arizona offering a personalized, multispecialty approach for children and adults who’ve had a Fontan procedure. We know our patients require care that goes beyond cardiology. Our team of experts includes specialists to monitor and treat any issues that affect the heart, lungs, liver, kidneys and lungs – all in one convenient location.

Your doctor or your child’s doctor or a cardiologist can make a referral to the Fontan 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.

Why Choose Us?

  • Life-long condition management. We offer regular screenings and continuous monitoring to help identify potential issues before they become severe complications.
  • Coordinated multispecialty care. The specialists a patient needs to maintain optimal health after a Fontan procedure is available at one convenient location. In most cases, the patient’s healthcare team can meet with them during one visit – preventing the need for multiple trips to different locations.
  • Advanced treatment options. Phoenix Children’s Fontan Clinic is the leader in using innovative and novel tools for managing Fontan patients. The center is a national leader in the initial application of CardioMEMS™ remote monitoring device in pediatric Fontan patients for closer monitoring of Fontan pressure to improve early detection of complications.
  • Patient education and support. If needed, our social work and behavioral health specialists can help patients develop school individual education plans (IEP) and 504 plans. Our team approach helps reduce stress for patients and families and lets specialists collaborate on each case to achieve the best results.
  • Cutting edge research and innovation. We are national leaders in developing and implementing new treatment options for Fontan care through forward-thinking clinical research. Our Center for Heart Care is a pioneer in surgical techniques, such as thoracic duct decompression surgery that provides an effective alternative to heart surgery in patients with a severe complication, such as Protein Losing Enteropathy (PLE). Our team actively collaborates with the national Fontan Outcomes Network, ACTION and FORCE registries and contributes regularly to leading research in this field.
  • Transplant preparation. We collaborate with the heart transplant team to ensure patients who require a transplant receive the evaluation and preparation they need to achieve the best possible result.

Advanced Surgical Care

The Fontan Clinic offers comprehensive continuing care to children and adults who have undergone the Fontan procedure and the surgeries leading up to it, which are the Norwood procedure and Glenn shunt.

These surgeries are the mainstay of treatment for children and adults with congenital single-ventricle heart defects, such as hypoplastic left heart (HLHS) and hypoplastic right heart syndromes (HRHS). Hypoplastic right heart syndrome is a rare congenital heart issue that some babies have at birth. Parts of the right side of their hearts don’t develop fully with HRHS. This causes a low level of oxygen in their blood.

The Norwood procedure is the first of three surgeries Phoenix Children’s uses to treat a severe form of congenital single-ventricle heart defect called hypoplastic left heart syndrome.

This procedure is usually performed within the first week of life. The patient’s surgeon repairs the severely narrow aorta and implants a small tube called a shunt to create a connection that allows blood to flow from the heart to the blood vessels in your child’s lungs or pulmonary arteries. This process also allows blood to pick up oxygen and release carbon dioxide. It makes the right ventricle the main pumping chamber for blood flow to the patient’s body.

During the procedure, the aorta is made larger to increase blood flow to the patient’s body. The outflow from the right and left ventricles is connected side-by-side to allow the blood from the right or left ventricles to reach their body.

A connection may be created to enable the blood traveling through the aorta toward the body to travel through this connection and flow into the pulmonary artery and receive oxygen. This connection allows blood to flow to the lungs.

A Glenn operation (or superior cavo-pulmonary anastomosis) is the second stage of surgeries that Phoenix Children’s uses to treat congenital single-ventricle heart defects, such as hypoplastic left heart syndrome and hypoplastic right heart syndrome.

During this operation, the shunt that was placed during the first procedure is removed. Then, the large vein that brings oxygen-poor blood from the head and arms back to the heart, called the superior vena cava, is connected to the branch 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 patent will remain cyanotic or have a bluish or purplish discoloration of the skin.

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

The Fontan procedure (total cavo-pulmonary anastomosis) is the third of three surgeries Phoenix Children’s uses to treat congenital single-ventricle heart defects called hypoplastic left heart syndrome and hypoplastic right heart syndrome.

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

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

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

What to Expect

Your First Visit

Before the first visit, we will schedule several tests to test how well their heart, lungs, liver and kidney’s function. Testing may include:

  • Heart echocardiogram
  • Treadmill/bike stress test
  • Lung function test
  • Ultrasound of the liver and kidneys
  • Blood and urine lab tests
  • Six-minute walk test
  • Rhythm monitor

The first visit may take several hours, depending on the patient’s needs. They will see a number of different team members, which may include:

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

After Your Visit

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

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

After the first visit, the patient will need to follow up with their cardiologist every six months. The patient will 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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