Fontan Clinic & Single Ventricle Program

SSM Health Cardinal Glennon Children’s Hospital has a multispecialty cardiology clinic dedicated to children and adults with single ventricle heart defects who have undergone the Fontan procedure. Children who undergo the procedure are those born with only one working heart ventricle instead of the normal two. Because of the risk of some complications later in life, a multidisciplinary team is available that includes:

Pediatric and adult congenital cardiologists
Heart failure specialists
Cardiovascular surgeons
Electrophysiologists
Pulmonologists
Hepatologists
Pediatric clinical dietitians
Exercise physiologists
Psychologists
Social workers

What Is the Fontan Procedure?

The Fontan procedure is an open-heart surgery that re-routes oxygen-depleted blood directly to the lungs. It is one of several surgeries that may be needed for children born with single ventricle heart defects. Congenital defects include:

Hypoplastic left heart syndrome
Double outlet right ventricle
Double inlet left ventricle
Pulmonary atresia
Heterotaxy syndrome
Tricuspid atresia

What Is a Single Ventricle Defect?

A normal heart has four chambers — the upper two are called the atria; the lower two chambers are called ventricles.

The ventricles are responsible for pumping blood out of the heart and to the rest of the body. Children born with a single ventricle defect have only one pumping chamber or one is significantly underdeveloped. The result is that a lesser amount of oxygenated blood can be pushed out to the rest of the body. Single ventricle defects are not common in children, occurring in about five out of every 100,000 live births. However, if they are left untreated, the result can be life-threatening.

These defects are usually diagnosed in infancy soon after birth and require a series of surgeries to treat effectively. Each surgery re-routes the blood flow so that the body can receive more oxygen. The final surgery, called the Fontan procedure, is normally performed on children who are between 2 and 5 years of age.

Why Is Long-Term Follow-Up Necessary?

Children and adults who have had the Fontan procedure should be followed by a congenital heart disease specialist through adulthood. That’s because they are at risk for developing serious complications as they age. Those include abnormal heart rhythms (arrhythmias), heart valve problems, chronic liver disease, peripheral vascular problems, blood clots, and sometimes heart, kidney or liver failure.

Our Expertise

Those who have had the Fontan procedure are living well into adulthood. We have congenital heart disease specialists at SSM Health Cardinal Glennon Children’s Hospital who treat both children and adults. We collaborate closely with adult specialists at SSM Health Saint Louis University Hospital. We also offer advanced imaging and lab tests to monitor heart and liver function as patients transition from adolescence and into adulthood.

We recommend that children who have undergone the Fontan procedure start receiving regular follow-up evaluations beginning 10 years after that procedure. At that time, we will assess baseline liver, pulmonary, and heart functions. Then we recommend clinic visits annually or every other year to ensure complications don’t arise, or so that they can quickly be identified and treated. We also will work closely with your child’s primary cardiologist to assess and provide care as needed.

Children and caregivers can expect to have clinic visits lasting two to three hours so that we can conduct exams and obtain any laboratory tests and imaging scans needed. The advantage of our Fontan Multispecialty Clinic is that we are a one-stop shop — in one visit to SSM Health Cardinal Glennon, we can coordinate the clinic visit and schedule any lab tests and scans to be done before you leave to minimize your time in the hospital.

Live Life to the Fullest!

The goal of our Fontan HEART team is to enable individuals to be Healthy, Empowered, Active, Resilient, and Thriving.

We can help with exercise and fitness recommendations, nutrition support, and even family planning and pregnancy-related issues in older patients. We also will educate patients and their families about some of the signs to look for that may mean a visit to a cardiologist or other specialist is needed.

We have cared for Fontan patients from infancy through 50+ years old!