Managing the Fontan Circulation

Fontan operations can be performed on children who are born with a heart that has just one chamber that pumps blood, instead of the normal two pumping chambers. This operation, first performed in the early 70’s, has changed the life expectancy for babies born with what’s called a single ventricle heart. The Fontan Management Clinic at Cincinnati Children’s is one of the few programs in the country that provides complete and specialized care to individuals who have had a Fontan procedure.

With the success of Fontan operations in early childhood, patients with a Fontan circulation are now living longer. However, as these children grow older, their circulations often become vulnerable to various health issues.

If you have a Fontan circulation, monitoring your health is important. We keep an eye on your heart health, and also regularly monitor the function of your liver, kidneys, and other organs. Regular Fontan-specific check-ups enable us to identify problems early. When we identify a problem early, we often have more options for treating the problem, preventing it from progressing.

In some cases, though, individuals may require more complex care, such as a heart or combined heart-liver transplant. Cincinnati Children’s has a leading heart transplant program, and has pioneered the use of heart-liver transplant for individuals with a Fontan circulation. We work in conjunction with the University of Cincinnati (UC Health) Transplant Program should it be needed.

Expertise in Fontan Management

Our adult congenital heart disease (ACHD) specialists have extensive experience in recognizing and treating issues related to congenital heart disease (CHD). These experts work closely with many other specialists including those who focus on heart failure and transplantation, arrhythmia, pulmonology, hepatology (liver), diagnostic and interventional radiology, hematology and thrombophilia (blood clotting), nephrology (kidney), endocrinology (hormones), maternal and fetal medicine (obstetrics), psychology, gynecology, cardiac and other types of surgery, and others.

The Fontan Management Clinic brings these care providers together in one place, making visits convenient for you. The team will usually coordinate your appointments so you can see all specialists in one visit, providing coordinated, comprehensive, and compassionate care.

What symptoms are causes for concern?

If you have a Fontan circulation and experience concerning symptoms such as those below, call the Fontan Management Clinic at 513-803-2243 as soon as possible:

• Breathlessness during activities that you used to be able to do without difficulty

• Leg or abdominal swelling

• Getting full quickly when eating

• Racing heartbeat while at rest or other palpitations

• Abdominal pain and loose stool or diarrhea

• Persistent coughing and wheezing

• Weight loss and fatigue

Treatment Approach

While we offer a spectrum of innovative advanced therapies, our clinic’s focus is on prevention, early detection, and proactive treatment. This takes many forms including screening for common problems. We also work closely with our team of exercise physiologists, including in the cardiac and pulmonary rehabilitation programs. We provide you with a treatment approach that addresses the complex needs of patients who have had a Fontan operation. This includes:

• Ongoing Surveillance and Care

Our surveillance program is geared to improve your quality and length of life. Routine check-ups to follow your health include the following:

• Minimally invasive hemodynamics

• Liver, lung and renal screening

• Nutritional assessment

• Early detection of obstructive sleep apnea

• Musculoskeletal strength and endurance testing

• Complex and Surgical Care

For patients with more complex needs, we provide a heart and heart-liver transplant at Cincinnati Children’s and in conjunction with the UC Health Transplant Program. In addition, the Heart Institute at Cincinnati Children’s is at the forefront of providing Fontan patients with ventricular assist devices (VADs) as a viable option when they are too sick initially to undergo a transplant. This team has made great strides in using VADs as a bridge to transplant or for chronic therapy.

• Transitioning to Adult Care

We also work closely with the Heart Institute’s Single Ventricle Interstage Clinic to provide a smooth transition for children from the pediatric to adult program. Our Adult Congenital Heart Disease Program was one of the first in the nation to be named by the Adult Congenital Heart Association as an accredited comprehensive care center. 

Heart and Mind Wellbeing Center