Fontan Procedure

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Norton Children’s Heart Institute

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The Fontan procedure is the final surgery in a series to treat babies with  hypoplastic left heart syndrome (HLHS) or other complex single-ventricle conditions. Advances in treating single ventricles have given more babies the chance to live full lives. For example, HLHS was considered inoperable and fatal before surgeons developed the three-surgery series.

The goal of the Fontan procedure is 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, which previously caused lower oxygen saturations.

The board-certified and fellowship-trained 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. Our cardiothoracic surgeons are equipped to perform the Fontan procedure as part of HLHS treatment.

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 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 having to go to 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.

Fontan Clinic

Many children born with single ventricles thrive and do well after the series of heart surgeries. However, they’ll need to see their pediatric cardiologist, get echocardiograms and occasionally undergo cardiac catheterizations.

Growing into adulthood with this unique single-ventricle circulatory system can come with complications and risks affecting the liver, kidneys, heart or lungs.

The Norton Children’s Heart Institute Fontan Clinic provides regular follow-up and monitoring to help spot any emerging issue early and treat it sooner, when more options may be available.

The Fontan Clinic works in conjunction with the Norton Children’s Heart Institute’s adult congenital heart disease program to provide sophisticated care to patients born with heart conditions.

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 three heart surgeries do not completely fix all the issues with the heart, or the single ventricle can wear out because it is 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

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

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