Personalized Care That Addresses Your Complex Needs
If you have a Fontan circulation, monitoring your health is important. We keep an eye on your heart health and 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.
The Fontan Management Clinic works closely with many specialists, including a team of exercise physiologists in the cardiac and pulmonary rehabilitation programs. These specialists design exercises and rehab routines to help you improve your heart health and overall physical strength.
In some cases, though, you may need more complex care, such as a heart or combined heart-liver-transplant. We work in conjunction with the University of Cincinnati (UC Health) Transplant Program should it be needed.
Treatment Approach
We give you a personalized care plan that addresses your complex needs after a Fontan operation.
An entire team of specialists—including cardiologists, hepatologists, endocrinologists, nephrologists, pulmonologists, radiologists, and surgeons—meets weekly to review patient cases. They discuss patient imaging, treatment options, and the need for additional specialists for complex cases. These intense reviews give you the highest level of care.
Coordinated, Ongoing Surveillance and Care
Our goal is to improve your quality and length of life. Coordinated care makes this possible.
Your routine check-ups may include the following:
- Heart screenings
- Liver, lung and renal screening
- Minimally invasive hemodynamics
- Musculoskeletal strength and endurance testing
- Nutritional assessment
- Sleep studies
You’ll work with a nurse coordinator who will help you plan and manage your care. Your nurse will arrange your appointments so that you can see many specialists all on the same day, in the same place.
If needed, your nurse can connect you with a social worker to connect you and your family to additional resources.
Imaging Expertise
The Fontan Management team works closely with radiology specialists. Cincinnati Children’s is the only pediatric hospital performing heart and liver MRIs in one study. Completing these imaging tests at the same time is much easier for patients and their families.
Transitioning to Adult Care
We work closely with the Heart Institute to provide a smooth transition for teens from our pediatric to adult program.
Treatments for Fontan-Associated Liver Disease
Living with a Fontan circulation puts you at risk for liver disease. Your Fontan management care team will monitor you closely for any liver conditions. We work closely with our hepatology experts to give you the care you need.
Liver conditions associated with a Fontan circulation include:
- Cirrhosis. When liver tissue becomes inflamed or damaged, it forms scars. This scar tissue permanently damages the liver and prevents it from working as it should.
- Liver cancer. Types of liver cancer we see in Fontan patients include hepatocellular carcinoma and liver tumors.
- Protein losing enteropathy. Protein is lost from the blood into the gastrointestinal tract. This can damage the lining of the intestines, liver, and stomach and block the normal flow of lymph. This can cause diarrhea and weight loss.
Depending on your condition, treatments may include chemotherapy, medicine, surgery or a transplant.
Treatments for Fontan-Associated Heart Conditions
Being born with congenital heart disease (CHD) that requires a Fontan procedure means you may also develop other heart conditions during your lifetime.
Heart conditions associated with a Fontan circulation include:
- Arrhythmias. There are different types of abnormal heart rhythms, including fast, irregular or slow. If you are diagnosed with arrhythmia, you’ll receive care from an electrophysiologist, a doctor who specializes in the heart’s electrical impulses.
- Heart failure. Your CHD may lead to heart failure. This can be caused by cardiomyopathy, where the heart muscle becomes enlarged, thick or rigid. Congestive heart failure may also occur. When this happens, the heart muscle weakens and cannot pump enough blood.
- Plastic bronchitis. This condition frequently occurs in people with a CHD who have a Fontan procedure. Lymph fluid builds up in the airways and forms rubbery or chalky plugs called casts. The casts block the airways, making breathing difficult.
Your treatment depends on your diagnosis. Options include medicines, interventional heart procedures or a transplant.
Heart and Liver Transplants
If you need a heart or a heart and liver transplant, we provide the most advanced care. Our internationally renowned surgeons partner with transplant experts at the UC Health Transplant Program as part of our combined Heart-Liver Transplant Program.
We are experts in complex surgical care. If you are too sick to receive a transplant, we provide Fontan patients with ventricular assist devices (VADs). We specialize in using VADs as a bridge to transplant or for chronic therapy. Our VAD program is nationally recognized for mechanical circulatory support (MCS).
Cardiac Rehabilitation
Our heart rehabilitation and exercise specialists provide a robust program that will help you manage the health concerns that come with living with a Fontan circulation.
We offer cardiac rehabilitation designed just for children, teens and young adults.
Pregnancy After a Fontan Procedure
Our experts provide comprehensive consultations for people with a Fontan circulation who want to become pregnant. We’ll assess your health and the risks if you become pregnant. We’ll talk with you about the need for high-risk care during pregnancy and ongoing, close monitoring.
Pregnancy can be risky for people with a Fontan circulation, but a healthy pregnancy is possible. Talk with your doctor about your desire to have a baby and create a plan that’s right for you and your family.
Heart and Mind Wellbeing Center
You are not alone in this journey. As a Fontan management patient, you can access resources and services at our Heart and Mind Wellbeing Center.
The center is the first of its kind in the United States. Here, we provide individualized emotional support and mental health care for you and your family, including parents and siblings. You can use these services at any point in your life.
When to Call Us: Serious Symptoms
If you experience concerning symptoms, contact your care team at the Fontan Management Clinic right away.
Serious symptoms include:
- Abdominal pain and loose stool or diarrhea
- Breathlessness during activities that you used to do without difficulty
- Getting full quickly when eating
- Leg or abdominal swelling
- Persistent coughing and wheezing
- Racing heartbeat while at rest or other palpitations
- Weight loss and fatigue
