The Fontan Procedure

What to expect

What happens during the Fontan Procedure?

A skilled congenital heart surgeon performs this surgical procedure:

• The inferior vena cava (IVC) is the large vein that brings oxygen-poor blood from the lower parts of the body back to the heart. The IVC is disconnected from the heart and reconnected to the lung arteries, so that the blood now flows towards the lungs. This can be done via several mechanisms:
  • An extra-cardiac Fontan uses a conduit or a tube-like structure outside the walls of the heart. The conduit is utilized to create a stable path for the blood to flow from the bottom half of the body to the lung arteries.
  • A lateral tunnel Fontan is created using your child’s own right atrium, or upper chamber of the heart, in addition to patch material, to create the pathway to the lung arteries. At Riley Children’s, we typically perform a lateral tunnel Fontan.

• By connecting the IVC directly to the lung arteries, blood from the lower body now flows to the lungs without needing to go through the heart. This completes the process of directing deoxygenated blood directly to the lungs and oxygenated blood directly to the body.
• During the procedure, a small hole or fenestration is typically made between the Fontan patch and the right atrium. This hole allows a small amount of blood to flow back into the heart, helping to regulate pressure within the Fontan – like a pop-off valve.

Recovery

After the Fontan is completed, children are taken to our Cardiovascular Intensive Care Unit (CVICU) where they are closely monitored by our expert physicians and our experienced nursing staff. Recovery typically takes about one week, at the end of which patients are comfortable walking, eating and have good pain management. Once they feel back to themselves, they are discharged from the hospital to continue life-long outpatient management with their cardiologist.

Benefits of the Fontan Procedure

The Fontan Procedure enriches the quality of life for children living with single ventricle heart defects by improving oxygenation levels that reach the brain, easing the volume burden on the single ventricle, and improving the pumping of oxygenated blood throughout the body.

It is not a cure for congenital heart defects, but it does help the body work more efficiently with a single ventricle. Many children can go on to live active lives, attend school, and play with friends. Many children grow into adulthood, and it is crucial to continue ongoing care with their cardiologist so they have the best chance at maximizing quality of life following surgery.

Follow-up program

As your child grows, we offer specific Fontan clinics for school-aged children and adolescents. The aim is to provide guidance on how families can assess the progression of their child’s heart disease - this includes observing any challenges at school or closely monitoring their child’s mental health following the multiple surgeries they have endured. This is a multidisciplinary clinic where you will also get to see our dietitian, social worker, and have a visit with our cardiac psychologist. We also coordinate a visit with our liver specialists, who are experts in treating patients with Fontan physiology. Even though your child will continue to have checkups with their regular cardiologist, the first visit to this specialized clinic is typically scheduled 5 years after your child’s Fontan was completed.

Management after the Fontan Procedure

Staged reconstructive surgeries are not a complete cure, and while children have fewer symptoms and improved energy initially, they may have heart rhythm problems or tire easier than their peers as years pass. Regular follow-ups with a cardiologist will continue into adulthood to help minimize and manage these problems.

When your child becomes an adult, we will help them transition their follow-up care to a cardiologist who specializes in Adult Congenital Heart Disease (ACHD). Our ACHD Program. Our ACHD Program at Riley Children’s works to help patients transition to adult care with cardiologists specifically trained on what to expect with these congenital heart problems.

Research

At Riley Children’s, the Riley Heart Center is focused on innovation in pediatric heart care. Through a grant from the National Institutes of Health, one of our congenital heart surgeons is studying ways to improve the Fontan procedure. This research aims to design a dedicated pump that could restore more normal circulatory physiology and prevent Fontan failure, an outcome we hope to eliminate.

Key points to remember

• The Fontan is an open-heart surgery where the Inferior Vena Cava (IVC) is disconnected from the heart and reconnected to the lung arteries.
• Fontan surgery is the final staged reconstructive surgery following the Norwood Procedure and Glenn Procedure for babies with Hypoplastic Left Heart Syndrome (HLHS).
• The Fontan procedure can improve the quality of life for children living with single ventricle heart defects by easing the burden on the single ventricle and improving the pumping of oxygenated blood throughout the body.