Fontan Procedure

The Fontan procedure is the final surgery in a series of operations to help the heart function better in children born with complex single-ventricle conditions such as hypoplastic left heart syndrome (HLHS). The procedure doesn’t cure the congenital heart defect. It allows the heart to work better in the absence of a second functioning ventricle.

Advances in treating babies born with just one ventricle have allowed more children to live full lives. HLHS was considered inoperable and fatal before surgeons developed the three-surgery series.

With just one ventricle, freshly oxygenated blood is diluted by mixing with deoxygenated blood, completing its trip throughout the body.

The Fontan procedure reconfigures the veins to direct blood from the lower part of the body directly to the lungs. The route bypasses the heart, preventing deoxygenated blood from mixing with oxygenated blood.

The cardiothoracic surgeons at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, are skilled at rebuilding the heart and redirecting blood flow so the heart isn’t overworked and more oxygen-rich blood is distributed throughout the body. Children from Louisville, Kentucky, Southern Indiana and beyond come to Norton Children’s Heart Institute for sophisticated treatment such as the Fontan procedure.

While the Fontan procedure and other treatments have saved lives, children with this unique blood circulation pattern are at risk for complications in other parts of the body, such as the digestive tract, as they grow into adulthood.

To monitor these patients as they grow and to catch developing complications, Norton Children’s Heart Institute has established a multidisciplinary Fontan clinic. There, a patient can see providers from cardiology, gastroenterology and other specialties in one visit. In addition to convenience, the multidisciplinary clinic provides a natural setting for specialists to compare notes and share viewpoints on a patient’s treatment plan.

How the Fontan Procedure Works

Surgeons performing the Fontan procedure disconnect the inferior vena cava (the vein that brings blood back from the lower part of the body) from the heart and connect it to the pulmonary artery.

Often, a small hole, called a fenestration, is created between the conduit and the right atrium. This allows some blood to flow directly back to the heart. It acts as a relief valve as the lungs get used to the extra flow from the lower part of the body. The fenestration can be closed later in life with a minimally invasive cardiac catheterization procedure.

With all the connections made, blood from the lower body now goes to the pulmonary artery, and then to the lungs, without passing first through the heart. The patient’s single ventricle now pumps oxygen-rich blood from the lungs out to the body and all the way back through the lungs.

With the high- and low-oxygen blood no longer mixing in the heart because of the defect, more oxygen gets out to the body.

Children who have the Fontan procedure usually spend one to two weeks in the hospital to recover. They also get medicines to help the heart and improve blood flow. The physicians and staff at Norton Children’s Hospital provide around-the-clock care and monitoring.

Learn More About Procedures Related to the Fontan Procedure

What Else You May Need to Know

Many children born with single ventricles thrive and do well after the Fontan procedure and other surgeries in the series. They’ll typically need to see their cardiologist and visit the multidisciplinary Fontan clinic, alternating every six months.

To help keep your child as healthy as possible:

  • Go to all doctor visits.
  • Give all medicines as directed.
  • Follow the guidelines from your care team.

Sometimes, the Norwood, Glenn and Fontan procedures can’t completely fix all the issues with the heart. Also, the single ventricle is at risk of wearing out after performing the workload of two ventricles. In these cases, a child may need a heart transplant.

A child with a single ventricle defect also needs the Fontan procedure because of these reasons.
An illustration shows changes to the IVC during the Fontan procedure
An illustration shows the right ventricle after the Fontan procedure

Why Choose Norton Children’s Heart Institute

The Adult Congenital Heart Association has recognized Norton Children's Heart Institute for its expertise treating adult congenital heart disease.

  • Norton Children’s Hospital has been a pioneer in pediatric cardiothoracic surgery, performing Kentucky’s first pediatric heart transplant in 1986 and becoming the second site in the United States to perform an infant heart transplant.
  • The American Board of Thoracic Surgery has certified our cardiothoracic surgeons in congenital heart surgery.
  • The Adult Congenital Heart Association has accredited Norton Children’s Heart Institute’s Adult Congenital Heart Program as the only comprehensive care center in Kentucky and Indiana treating adults born with a heart defect.
  • More than 5,000 children a year visit Norton Children’s Heart Institute for advanced heart care.
  • Norton Children’s Heart Institute has offices across Kentucky and Southern Indiana to bring quality pediatric heart care closer to home.
  • The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) at Norton Children’s Hospital is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.
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