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Sometimes a baby is born with certain types of congenital heart problems. In these cases, a type of open heart surgery called the Fontan procedure is done as the final part of a series of surgeries needed to ensure healthy circulation in the body.

The congenital heart problems that may require the Fontan procedure include:

  • hypoplastic left heart syndrome
  • double outlet right ventricle
  • tricuspid atresia

The main purpose of the Fontan procedure is to make sure blood circulates through the lungs to acquire oxygen to supply the organs, muscles, and other tissue that need oxygen to thrive.

Babies who undergo a successful Fontan procedure often grow to adulthood. They will need lifelong monitoring by a cardiologist.

In a healthy heart, blood returning to the heart from the body enters the right atrium and then empties through the tricuspid valve into the right ventricle, which pumps blood to the lungs through the pulmonary valve and pulmonary artery.

After circulating through the lungs, oxygenated blood then returns to the heart’s left atrium via the pulmonary veins and tricuspid valve. The mitral valve then opens up, allowing blood to fill the left ventricle, which pumps it out to the rest of the body.

The Fontan procedure’s purpose is to route oxygen-depleted blood directly to the lungs so it can then enter the heart and be pumped throughout the body. It involves the surgical reconstruction of the blood vessels that supply the heart and lungs.

The procedure is needed when one of the ventricles is too small or poorly developed, or if one of the heart’s valves is missing. Conditions like this are called single ventricle defects. They can be life threatening because they limit the heart’s ability to meet the body’s constant demand for oxygenated blood.

Hypoplastic left heart syndrome (HLHS) means the left side of the heart is underdeveloped.

Specifically, the left ventricle — the heart’s main pumping chamber — and the aorta are too small to meet the body’s demands for robust circulation. The aorta is the main artery leading out of the heart, branching off to deliver blood to the brain and to the rest of the body. The aortic valve and mitral valve are usually underdeveloped, too.

HLHS is the most common type of single ventricle defect. It affects about 1 in 3,841 babies born in the United States each year, according to the Centers for Disease Control and Prevention (CDC). If not treated promptly, HLHS can be fatal within days.

In addition to HLHS, the Fontan procedure can treat other congenital heart defects. They include:

  • Double outlet right ventricle: the aorta and pulmonary valve both connect to the right ventricle (usually, the aorta connects to the left ventricle)
  • Pulmonary atresia: the pulmonary valve never forms
  • Tricuspid atresia: the tricuspid valve never forms

During the Fontan procedure, a surgeon detaches the inferior vena cava — the main vein bringing blood from the lower body back up to the heart — from the right atrium.

The vein is then attached to the pulmonary artery, the blood vessel that carries blood from the heart to the lungs. A synthetic tube called an intra-atrial baffle is used to make that connection.

As a result, blood that would normally enter the heart through the inferior vena cava will be channeled to the lungs. However, the surgeon will also create a small opening between the baffle and the right atrium to allow some returning blood to enter the heart to avoid overloading the lungs with blood.

The hole, called a fenestration, is often closed later on once the lungs get used to handling the increased blood flow.

After Fontan procedure surgery, a child typically spends up to 2 weeks recovering in the hospital. Their heart is monitored 24 hours a day. A healthcare team is always looking out for any signs of complications.

During the initial recovery period, the child will receive medications to support healthy blood flow. Some of the medications will be taken at home, too.

Follow-up care with a cardiologist may include:

The Fontan procedure usually leads to positive outcomes. However, there are risks of some serious complications.

A 2018 study suggests that between 20% and 40% of people with single ventricle defects will develop heart failure 20 years or so after undergoing the Fontan procedure.

Other possible complications may include:

  • atrial arrhythmia
  • blood clots
  • chronic liver disease
  • protein-losing enteropathy, an intestinal problem where proteins are lost
  • kidney disease

The Fontan procedure is often the third surgery a child with a congenital heart defect undergoes to improve healthy blood flow.

Though each case is unique, a child typically undergoes the Fontan procedure between the ages of 18 months and 36 months.

The first procedure a child typically gets is called the Norwood procedure. It’s often performed within a few days after birth. The Norwood procedure is appropriate in cases where the left ventricle is too small to effectively pump enough blood to meet the body’s needs.

During the Norwood procedure, a surgeon creates a larger aorta. They join it with the right ventricle so some blood can be pumped from the right ventricle to the body, while the rest of the blood is routed to the lungs for oxygen.

The second surgery that often precedes the Fontan procedure is called the hemi-Fontan procedure or the Glenn procedure. It’s usually performed when a child is between 4 and 8 months old.

In this procedure, a surgeon attaches the superior vena cava (the large vein that brings blood from the upper part of the body to the heart) to the pulmonary artery. This routes deoxygenated blood directly to the lungs instead of through the heart first.

In some cases, subsequent surgeries or procedures may be needed to address ongoing cardiac problems, such as heart failure or arrhythmia.

When to call a doctor after the Fontan procedure

Because children are usually very young when having the Fontan procedure, they may not be able to tell you about any symptoms. It’s important to pay close attention to the health of anyone who undergoes the Fontan procedure or any type of heart surgery.

Call a doctor immediately if you notice your child:

  • is breathing faster than normal, or it’s difficult for them to breathe
  • seems to have chest pain
  • is vomiting
  • is not eating
  • seems unusually irritable

Keep a pulse oximeter at home to check the oxygen saturation of your child’s blood. If you notice that oxygen levels are dropping or that your child’s skin starts to take on a bluish tint, call 911 or local emergency services.

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A 2018 research article suggests that the Fontan procedure is one of the most commonly performed surgeries to treat congenital heart disease, and has led to many successful short- and long-term results.

However, the procedure has only been around for about 50 years. Researchers are still evaluating true long-term outcomes.

A 2019 report from the American Heart Association also suggests that, based on limited long-term data, a 30-year survival rate of greater than 80% is likely.

The report also states that as many as 70,000 people around the world are alive because of the Fontan procedure. Researchers added that that figure could double by 2039.

When the Fontan procedure is performed by a surgeon with experience and the person continues to receive ongoing care from a cardiologist, the likelihood of a positive outcome is encouraging.

How do I know if my child needs the Fontan procedure?

Often, it’s not until birth that heart problems become apparent. In some cases, it may be hours or days before a problem, such as HLHS, is evident. However, congenital heart defects may be diagnosed during pregnancy using ultrasound.

Common signs and symptoms of a congenital heart defect include:

  • grayish or bluish tint to the skin
  • discolored lips and nails
  • difficulty breathing and feeding
  • lethargy
  • unusual heartbeat

How long does the Fontan procedure take?

The surgery usually takes 4 to 6 hours, but this can vary. Surgical technique and the nature of the specific heart defect can require longer operative times.

Will my child be able to grow up like other kids?

Every case is different, but with successful Fontan surgery, a child may be able to grow and develop as other children do.

Contact sports and certain activities may be off limits, but research suggests that in the 50 years since the surgery was first performed, thousands of people who have undergone the procedure have grown up and enjoyed a good quality of life.

The Fontan procedure isn’t a cure for a congenital heart defect. Rather, it’s a surgery that can boost healthy blood flow into and out of the heart.

The Fontan procedure isn’t typically the first surgery for a child born with heart problems and may not be the last. But it has saved the lives of thousands of people around the world, and the technology and techniques used in the procedure continue to improve.