Fontan FAQs: Is Exercise Safe or Beneficial for Individuals with a Fontan Circulation?
Growing evidence shows exercise can be safe and beneficial for most people with Fontan physiology, representing a major shift from past recommendations based on exercise restriction and limitation. A clinician and patient offer their perspectives.
A Clinician’s Perspective
By Dr. Jesse Hansen
Clinical Assistant Professor
University of Michigan C.S. Mott Children’s Hospital
Over the last several decades, improvements in single ventricle palliation have resulted in an entire generation of survivors who now have Fontan physiology, long considered the final stage of palliation for single ventricle heart disease. As this group of survivors has grown, the medical community has gained more understanding of the long-term implications of Fontan physiology.
Teens and adults with Fontan physiology live with the threat of complications and illness, many of which extend beyond the heart and lungs and can affect an individual’s entire body and mind. While some have suggested that the declines associated with Fontan physiology are inevitable, collaborations between patients, families, and researchers are beginning to demonstrate that some people with Fontan physiology defy the characteristic physical decline and socio-emotional implications of their palliated circulation.
One implication of Fontan physiology is reduced exercise performance. We measure exercise performance in a scientific way during an exercise stress test. This measure, called peak oxygen consumption or “peak VO2”, refers to how much oxygen an individual’s heart can make available to the body to use during exercise. Previous research has shown that people with Fontan physiology generally have peak oxygen consumption levels about 60 to 70 percent of the level expected when compared to people with normal hearts. We also now know that people with lower exercise performance self-report lower quality of life scores.
“...We are also learning that structured exercise programs like cardiac rehabilitation and home exercise prescriptions are safe for most people with Fontan physiology and training can improve an individual’s overall exercise performance and quality of life.”
Through the efforts of the Australia-New Zealand Fontan Registry, investigators identified people with Fontan physiology who had normal levels of oxygen consumption during exercise testing. These “super Fontans” were more likely to exercise regularly, discuss exercise with their cardiologists, and were all functioning well socially with either full-time employment or enrollment in graduate-level schoolwork. These findings were also demonstrated in a group of people with Fontan physiology studied by the Cincinnati Children’s Heart Institute where 1 in 5 participants had normal peak oxygen consumption.
Recently published data from the long-term Pediatric Heart Network study of people with Fontan physiology also found evidence that individuals in the higher exercise performance group were less likely to be overweight or obese when compared to the lower performing group. Some of the predictors of increased exercise performance are non-modifiable things that people are born with (like biologic sex and underlying heart anatomy). But we are also learning that structured exercise programs like cardiac rehabilitation and home exercise prescriptions are safe for most people with Fontan physiology and training can improve an individual’s overall exercise performance and quality of life. This is a big change from the exercise restrictions and limitations that were frequently recommended to families in the past.
Just like a healthy diet requires a wide variety of food, healthy physical activity includes a wide variety of strength, aerobic, and flexibility exercises. Emerging evidence suggests exercise plans should include lower extremity strength training to improve the “muscle pump” from the large muscles in the legs that helps return blood through the veins and lungs to the heart. It is still unclear what type, frequency, and duration of exercise is most effective for improving exercise performance and quality of life for people with single ventricle hearts.
As researchers continue to study these questions, we now have evidence that people with Fontan physiology should be regularly discussing the best way to safely exercise with their cardiologist. Most importantly, the best kind of exercise for you will include activities you enjoy doing regularly (including options for when it’s too hot or too cold to exercise outside) since many of the physical and mental health benefits start to wear off quickly once you stop regular exercise. To help get the conversation started, we’ve included a few questions you should ask your cardiologist and some resources for more information about exercise for people with Fontan circulation.
Questions to ask your cardiologist:
Is it safe for me to exercise or play sports?
How hard should I push myself when being active?
What symptoms should I be aware of when I start exercising?
References and recommended reading:
Scheffers, L. E., Berg, L. E. V., Ismailova, G., Dulfer, K., Takkenberg, J. J., & Helbing, W. A. (2020). Physical exercise training in patients with a Fontan circulation: A systematic review. Eur J Prev Cardiol, 2047487320942869.
Cordina, R., du Plessis, K., Tran, D., & d’Udekem, Y. (2018). Super-Fontan: Is it possible? J Thorac Cardiovasc Surg, 155(3), 1192-1194.
Atz, A. M., Zak, V., Mahony, L., Uzark, K., D’agincourt, N., Goldberg, D. J. et al. (2017). Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure. J Am Coll Cardiol, 69(22), 2735-2744.
Goldberg, D. J., Zak, V., McCrindle, B. W., Ni, H., Gongwer, R., Rhodes, J. et al. (2020). Exercise Capacity and Predictors of Performance After Fontan: Results from the Pediatric Heart Network Fontan 3 Study. Pediatr Cardiol.
Powell, A.W., Chin, C., Alsaied T., Rossiter, H.B., Wittekind, S., Mays, W.A., Lubert, A., Veldtman, G. (2020). The Unique Cinical Phenotype and Exercise Adaption of Fontan Patients With Normal Exercise Capacity. Can J Cardiol.
A Patient’s Perspective
By Meghan (Roswick) Didier
Patient Lead
FON Community Building & Engagement Workgroup
It is no secret that individuals with a Fontan circulation tend to experience many different physical and mental challenges throughout their lifetime. As research continues and patient input is being heard, it is becoming clearer where the priorities lie with improving quality of life. The Fontan Outcomes Network is dedicated to identifying those priorities and developing solutions to help improve patient care. One area of focus that has seen more interest from both patients and families as well as providers is the impact that exercise has on Fontan circulation.
“Learning to understand my body and my capabilities proved to be just as much of a mental challenge as a physical one. I felt like I was constantly questioning what I was capable of and if I was pushing myself too far. ”
Exercise can be an intimidating topic to discuss; not just for patients, but also for their providers. Recently, there has been a shift in the way the medical community views and treats exercise and Fontan. In the past, exercise recommendations were cautious, learning toward a more conservative approach in cardiovascular exercise and exertion. Today, there is more information and data available showing that there are benefits to exercise with a Fontan circulation. This change has led to a slew of questions and concerns surrounding what is considered safe for patients to participate in.
As a child growing up in the early 1990s, I was considered to be within one of the first waves of babies to undergo all three open heart surgeries for Hypoplastic Left Heart Syndrome. It would be an understatement to say that there were limited answers and resources for patients and families in my position at that time. I was fortunate to have a progressive team and surgeon that believed in letting their patients set their own limitations when it came to exercise and activity. As Dr. William Norwood would always say, they were “taking a good heart muscle and putting it to efficient use.”
Learning to understand my body and my capabilities proved to be just as much of a mental challenge as a physical one. I felt like I was constantly questioning what I was capable of and if I was pushing myself too far. With limited data and guidance, I struggled with my mental health as I did my best to determine where my boundaries lie.
Through trial and error over the years, I have had many opportunities to learn about how my body responds to certain activities and exercises. I am still constantly challenging myself and trying to find new ways to build strength and endurance. With challenging myself comes an understanding that there will be times when things need to be adapted. An example of something that would make a drastic difference in my ability to participate in certain activities is temperature. There are times when I will wake up incredibly early in the morning to go hiking, when it is still dark and cool, so that I am able to avoid hiking during the hottest hours of the day.
It can be difficult, especially when you feel like you need to think about every “what if” scenario and remain hypervigilant of each symptom you have. I have experienced countless panic attacks while exercising due to my own self-doubt and anxiety, but each time I would push forward and try again. That doesn’t mean there still aren’t times that I re-evaluate a situation and decide not to continue or sit out entirely. Making those decisions for yourself is important and necessary to create a healthy relationship with exercising. It is also important to remember that everyone is different when it comes to exercise–even individuals with a four chambered heart! Try not to be hard on yourself and always listen to your body.
Below I have compiled a list of five tips that have helped me navigate learning to trust myself and my capabilities:
Accept that becoming frustrated and doubting yourself are normal feelings when you are pushing yourself out of your comfort zone. Exercising is just as much of a mental game as it is physical. Practice being mindful and accepting the things you cannot control.
Create many smaller goals in addition to the big goals. This can be interpreted in different ways. Your big goal could be walking a mile or participating in a 5K. Your smaller goals could be walking one block for three days in a row and then trying to add another block a few days later. Sometimes the big goal can be so daunting that it’s hard to know where to start. Breaking your goals down into small parts can help with that.
Always keep an open line of communication between you and your care team. No conversation is too big or too small to bring up and discuss. It is important to feel as confident in yourself as possible when going outside of your comfort zone and testing your limits.
Utilize opportunities to monitor your heart while you exercise such as during a stress test or while wearing a Holter monitor. If you haven’t had one recently, don’t be afraid to ask when your next Holter monitor or stress test will be. This can also help ease stress and get you used to what your body feels like during exercising while knowing you are being monitored and are safe.
Consistency is key. I have found that consistency is incredibly important when exercising and dramatically helps build up and maintain strength/endurance.