People with Fontan surgery can be very active with few limitations; however symptoms can develop and are important to recognize. Symptoms are usually caused by a complication of the Fontan surgery, the most common being heart rhythm problems that may feel like skipped beats or a racing heart. Other symptoms might include decreased ability to exercise, fatigue, and shortness of breath. These symptoms could also be caused by heart rhythm problems however other complications like leaky heart valves, blood clots or weak heart pump muscle can also cause them. If a patient with Fontan surgery is experiencing new symptoms they should contact their congenital heart doctor to discuss them.

Regular exercise is very important for keeping healthy. Both the heart muscle and the breathing muscles help the blood flow properly in people with Fontan surgery, stronger heart and breathing muscles make for better blood flow to the body overall. People with Fontan surgery should participate in regular exercise, guided by their doctor. Sometimes people with Fontan surgery may need to exercise at their own pace, and that’s okay. For people with Fontan surgery it is important to find an exercise activity that they can do regularly. The type of exercise activity should be discussed with their congenital heart doctor.

Excellent dental hygiene is very important to keeping the heart healthy. Normal bacteria in the mouth can be very harmful if they get into the heart. The best way to protect the heart is by keeping the mouth and teeth healthy by brushing regularly, flossing and visiting the dentist at least once a year. Some patients are advised to take antibiotics before any dental procedures, people with Fontan surgery should speak to their congenital heart doctor about recommendations for antibiotics before dental procedures.

Women with Fontan surgery can become pregnant. Discussing family planning and birth control methods are an important part of healthcare for people with Fontan surgery. Some birth control methods are not safe for women with Fontan surgery. In general, progesterone only birth control methods are preferred to reduce the risk of blood clots. It is important to discuss family planning and birth control choices with a congenital heart specialist who can explain the risks and benefits of each type.
Pregnancy in women with Fontan surgery is considered higher risk and these women need to be followed by specialized heart and obstetric doctors throughout their pregnancy. Planned pregnancies with proper follow-up will usually have better outcomes for mother and baby. People with Fontan surgery should talk about family planning and pregnancy with their healthcare provider early on, to discuss the risks and help them make an informed choice about trying to become pregnant. Miscarriage and stillbirth are more likely to happen in a woman with Fontan surgery than to someone with a structurally normal heart. In addition, women with Fontan surgery may have heart related problems during their pregnancy including heart rhythm problems or blood clots which can put their health at risk. For women with Fontan surgery who become pregnant, close follow-up with specialists at an expert center is recommended.

People with complex congenital heart disease including those who have had a Fontan surgery are at risk of neurocogntive issues. Neurocognitive refers to how the nervous system affects daily behaviours and how the brain acquires knowledge. Overall it describes how an individual is able to manage emotions, learning, problem solving and other behaviours. Among individuals with a Fontan surgery, neurocognition can range from entirely normal to severely impaired.

The cause of neurocognitive issues can be complex and may be related to biology, genes, and/or environment. Factors specific to individuals with a Fontan include reduced oxygen levels and/or blood flow to the brain at any stage of development. Heart surgery, catheterizations, and anesthesia needed to repair the heart defect also impact the brain. Repeat sickness, injury and/or hospitalizations that occur due to the underlying heart condition can result in school absences, reduced socialization or isolation that can all affect cognitive, social and emotional development.

It is important to know that you or your family member are not alone. You should speak to your adult congenital heart doctor if you are worried about your neurocognitive status. Specifically, if there are challenges related to education and/or employment. There may be medications that can help, or your doctor can refer you for further assessment and management. If you believe that you are suffering from depression or anxiety, discuss this with your congenital heart doctor. This is very common among individuals with a Fontan, and treatment can help to improve mood and neurocognitive function.

People with Fontan surgery have a lifelong risk of complications including heart rhythm problems, heart failure, blood clots, liver damage, kidney damage, reduced exercise capacity and early death. New surgical techniques, and specialized care have improved survival of people with Fontan surgery over the past 20 years, however it is important for people with Fontan surgery to understand the possible long-term complications and limitations that may affect them. It is also important for people with Fontan surgery to discuss future complications and life expectancy openly with their healthcare provider. Having a good understanding about possible future complications can help people with Fontan surgery make informed life decisions including those involving their career, family planning and end-of-life wishes.
Complications from Fontan surgery can significantly impair quality of life for some people. Medications or repeat heart procedures/surgeries are sometimes needed. Some people with Fontan surgery may be evaluated for possible heart transplant, however transplant is high risk and not possible or appropriate for everyone. For some patients there are no medications or other procedures that are felt to help improve their symptoms or survival, for these people the focus of medical care is to improve symptoms and not try to fix the underlying disease. This approach is called palliative care, it does not necessarily mean end-of-life care, but rather changing the focus of treatments to making people feel better and improve their quality of life as opposed to making them live longer.
Regardless of the clinical course people with Fontan surgery should discuss their wishes with their family, friends and care provided when they are well enough to do so.

In Canada there are two types of health insurance— public and private. As a Canadian, you have access to public health insurance, provided by the province you live in. This type of insurance covers:

– Treatment, diagnostics and health exams provided by a medical doctor
– Treatment provided in a hospital emergency room
– Hospitalization
– Any other health services provided by a medical doctor or surgeon

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