Pediatric Fontan procedure

What is the pediatric Fontan procedure?

The Fontan procedure is the last surgery in a series of three open-heart procedures necessary to treat children born with certain types of single ventricle defects. These congenital heart diseases occur when one of the heart’s two lower chambers (ventricles) doesn’t fully develop.

With the Fontan procedure, our experienced heart surgeons make a new connection that allows blood from your child’s lower body to go directly to the pulmonary arteries in the lungs to get oxygen. Typically, blood passes through the heart before going to the lungs.

The Fontan procedure typically takes place when a child is between 18 and 36 months of age.

Your child may need the Fontan procedure if they have:

• Double outlet right ventricle, an incorrectly placed aorta (the body’s main blood vessel)

• Hypoplastic left heart syndrome (HLHS), an underdeveloped left ventricle

• Tricuspid atresia

What are the benefits of the pediatric Fontan procedure?

The Fontan procedure is part of a series of lifesaving procedures for babies with certain single ventricle defects. These defects allow blood that’s too low in oxygen to circulate throughout a child’s body. Over time, this low oxygen can lead to heart failure and other types of organ failure.

The Fontan procedure allows blood from a child’s lower body to flow directly to arteries in the lungs to get oxygen. This oxygen-rich blood then goes to the heart. Even though half of the heart still doesn’t work properly, the heart’s working chambers can pump the oxygen-rich blood to the body.

What are the risks of the pediatric Fontan procedure?

Children’s Health has deep experience in performing the Fontan procedure and minimizing risks to your child. Still, any type of heart surgery carries a risk of blood clots, infections and other problems.

What to expect with the pediatric Fontan procedure

Children’s Health is home to one of the nation’s first and most experienced dedicated Fontan Programs. Though many children enjoy active lives after the Fontan procedure, the surgery doesn’t cure the heart defect. With our program, your child receives ongoing, comprehensive care from a team of specialists dedicated to promoting an active childhood and healthy adulthood.

What to expect before the pediatric Fontan procedure

Most babies with hypoplastic left heart syndrome (HLHS) or other single left ventricle defects get the Fontan procedure when they’re between 18 and 36 months old.

At this point, your child will have already undergone two other heart procedures:

1. Norwood procedure - The Norwood proceduretakes place within the first week after birth. During this surgery, we reconstruct the aorta to allow blood to flow from the heart to the rest of the body. We also make a connection for blood to reach the lungs to pick up oxygen.

2. Glenn procedure - The Glenn procedure takes place when your baby is 4 to 6 months old. We make a connection between the superior vena cava (a large blood vessel) and blood vessels in the lungs. This procedure enables blood from the upper body to reach the lungs to get oxygen.

What to expect during the pediatric Fontan procedure

During a Fontan procedure, one of our highly skilled pediatric heart surgeons:

• Disconnects the inferior vena cava from the heart. This large vein carries oxygen-poor blood from your child’s lower body to the heart.

• Uses a tube to reattach the inferior vena cava to the heart’s pulmonary artery.

What to expect after the pediatric Fontan procedure

• Pediatric Cardiac Intensive Care Unit (CICU) - For 2 to 3 weeks, your child recovers and grows stronger in the state’s largest pediatric cardiac intensive care unit (CICU) at Children’s Health. Here, your child receives dedicated care from some of the state’s best cardiologists and cardiac nurses.

• Safe at Home program - We help you learn how to care for your child’s surgical incision and medication needs as you prepare to take your child home. Our unique Safe at Home program helps you manage your child’s care after leaving the hospital. Our medical team is available whenever you need us.

What questions should I ask my provider about the pediatric Fontan procedure?

• How many Fontan procedures have you performed?

• What is the success rate?

• Are there other treatment options for my child’s heart condition?

• Will my child need additional heart procedures? If so, when?

• How soon will I be able to see my child after surgery?

• What should I expect during my child’s recovery?