Fontan Program

Our Fontan Program focuses on caring for children who get the Fontan procedure. The Fontan procedure is the third operation in single ventricle palliation—a series of surgeries that treats single ventricle defects. Single ventricle palliation gives children a heart that functions with one ventricle (pumping chamber) instead of two.

Children with single ventricle defects need specialized care leading up to the Fontan procedure. Some may also need care after the surgery. Our Fontan Program gives these children intensive monitoring and expert care.

Fontan Program: Why Choose Us

At Children’s Hospital Los Angeles, your child receives surgery from the best children’s heart surgery program in the Western United States. And U.S. News & World Report consistently ranks the Heart Institute as one of the top children’s heart surgery programs nationwide. The Fontan Program offers:

  • Personalized treatment: We are one of the few pediatric hospitals with both a Single Ventricle Program and a specialized subprogram for care before and after the Fontan procedure. The Fontan Program allows us to tailor monitoring and treatment to your child’s specific anatomy and needs.
  • Comprehensive, integrated care: Our goal is to help your child have the best possible quality of life after the Fontan procedure. That’s why we have a multi-disciplinary approach where we involve gastroenterologists and pulmonologists as part of the care team
  • Expert team: Our surgeons perform more than 900 pediatric heart surgeries each year. This large number of patients means we’ve seen all types of heart defects, from the mild and rare to the severe and hard to treat. Our surgeons are also world-renowned for coming up with creative solutions to treat complex defects.
  • Advanced imaging: We use advanced imaging, such as 3D echocardiograms and cardiac MRIs. These tests help doctors see the anatomy of your child’s heart and figure out the best treatments. For complex cases, we can 3D print a model of your child’s heart. Surgeons use this model to rehearse the procedure on your child’s unique anatomy, improving the chance for a positive outcome.
  • Convenient care: All our pediatric heart programs are on one floor. This setup makes it easier for you to manage your child’s care. It also helps our doctors collaborate on treatment plans and consult experts from other subspecialties when needed.

The Fontan Procedure: What to Expect

The Fontan procedure allows blood from the lower part of the body to bypass the heart and flow directly into the lungs. This is necessary because there is no ventricle to pump this blood from the heart into the lungs.

Without a Fontan procedure, oxygen-rich and oxygen-poor blood can mix in the heart. This mixing forces the heart to work harder to pump enough oxygenated blood to the body. The Fontan procedure corrects this problem, improving circulation and heart function.

During the surgery, your child’s surgeon:

  1. Disconnects the inferior vena cava (IVC) from the heart. The IVC is the vein that usually carries blood from the lower body to the heart.
  2. Reattaches the IVC to the pulmonary artery. This step often requires a conduit (large tube) to connect the vessels. The new connection directs oxygen-poor blood into the lungs and keeps it separate from the oxygen-rich blood returning to the heart. This separation helps the heart pump enough oxygenated blood to the body.
  3. Sometimes makes a small hole (fenestration) between the Fontan conduit and right atrium. The hole allows some blood to continue flowing into the heart. This blood flow reduces pressure on the lungs as they get used to the increased blood flow from the IVC. Your child's doctor can close the hole later with cardiac catheterization.

Sometimes single ventricle palliation helps the heart function well enough for a good quality of life. In other cases, children need more treatment, such as a heart transplant.

We may use a ventricular assist device (VAD) to support your child’s heart before the Fontan procedure. This device can help improve the chance for a positive outcome after surgery. Our expert doctors work with you to identify the least invasive and most helpful treatments for your child.

Conditions We Treat

  • Double outlet right ventricle
  • Heterotaxy syndrome heart disease
  • Hypoplastic left heart syndrome
  • Pulmonary atresia with intact ventricular septum (PAIVS)
  • Tricuspid atresia