Is being physically active the key to a good quality of life for patients living with Fontan circulation?

The pioneering Fontan procedure for children born with a complex heart defect has led to an increased rate of survival into adulthood. Several studies of the long-term outcomes for patients living with Fontan circulation have shown that they face many obstacles, including physical limitations, decreased exercise capacity and troubling symptoms. In addition, there is sometimes a general burden after undergoing innumerable medical procedures 1, 2. Despite all the negative consequences for young people living with Fontan circulation, studies have also shown that these adolescents and young adults have found ways to manage their condition in order to get the best out of life. For example, they have been shown to consider themselves as both strong and resilient 3 and our own study showed that they viewed themselves as exceptional, strong and healthy, with a special commitment to life 4. A number of studies with different approaches have investigated the life situation of patients with Fontan circulation, and the focus has ranged from physical function to quality of life (QoL). One approach has been to examine the predictive factors for their QoL. The possible connection between QoL, physical activity and exercise capacity has been discussed but not totally established. Most studies concerning physical activity have focused on examining physical performance and exercise capacity, making comparisons between patients and also comparisons with healthy peers, but few studies have focused on everyday physical activity. The study by Hedlund et al. 5, presented in this issue of Acta Paediatrica, investigated self-reported physical exercise, measured physical activity and self-reported QoL in their cohort of patients with Fontan circulation. The patients, who were aged from eight years to 19 years, were individually matched with a healthy peer they had suggested for inclusion in the control group. Comparisons with population norms were also obtained. The parents’ evaluation of their children's QoL was also obtained. The study showed that the patients spent significantly less time taking part in organised physical exercise than the controls. They also demonstrated a significantly lower average intensity level in their total activity in one week, as measured by an accelerometer, and the time spent engaged in physical exercise decreased with increased age, for both the patients and the controls. QoL, as measured by the Pediatric Quality of Life Inventory (PedsQL), including parental proxy PedsQL scores, was significantly lower for the patients than for the controls. As everyday physical activity levels are not well studied in this group of patients, and the predictive factors of QoL still need to be explored, the paper by Hedlund et al. 5 makes a valuable contribution to existing knowledge. Their findings could serve as a basis for future interventions to increase physical activity levels in patients with Fontan circulation. In line with earlier studies, the results from Hedlund et al. 5 showed that patients with Fontan circulation were less active than controls. This is an important finding, but it might also be because patients choose other interests instead of more physically demanding activities. However, the fact that adults with complex heart defects are less active has also been shown by Sandberg et al. 6, who also showed that around half of the patients – including both those with simple and complex defects – and controls did not reach the current World Health Organization recommendations on physical activity for health, including moderate and vigorous daily physical activity. The promotion of structured regular physical exercise in young patients living with Fontan circulation would be a worthwhile investment in their future health. This proposal is supported by, for example, the review by Sutherland et al. 7, which convincingly showed that cardiac rehabilitation was safe and beneficial for this group of patients. Nonetheless, it is of great importance to acknowledge the observation by Chaix et al. 8 that exercise should be tailored to the individual patient with Fontan circulation, as these patients often have exercise intolerance that is multifactorial. We also need to identify the factors that are associated both with low and high physical activity, to improve the physical activity level but also health and QoL. Hedlund et al. 5 further speculated that regular physical exercise could be one way of improving QoL. This is a good suggestion that merits further investigation. When Uzark et al. 9 investigated QoL in young patients with Fontan circulation, they found that regular exercise could be beneficial in the long run. They found that these young people were at risk of significantly impaired QoL that declined as they got older. This raises the possibility that, if the promotion of regular physical exercise starts early, there might be a better outcome for these patients later in life. Nonetheless, as QoL is a multidimensional construct, other factors besides an increased physical activity level are likely to be of benefit for a person's QoL. Studies examining QoL are a great complement to mortality and morbidity studies, because they take into consideration physical, psychological and social factors. Studies examining QoL in patients with congenital heart disease have grown exponentially in recent decades, as shown in the review by Bratt and Moons 10, which revealed major weaknesses in methodological rigour in several studies. In contrast, the study by Hedlund et al. 5 used PedsQL, an established, validated and reliability-tested instrument 11. In addition to the generic version that Hedlund et al. used, there are also disease-specific modules that can be added, for example, a cardiac module that would be of great value generating more specific information when designing interventions for different patient groups. For example, Uzark et al. 9 showed that young adults with Fontan circulation reported lower physical functioning, more symptoms related to their heart condition and more perceived treatment barriers than adolescents. The fact that many factors co-exist and influence QoL is shown in many studies. The study by Hedlund et al. 5 showed that patients with Fontan circulation presented significantly lower scores in both the physical and psychosocial dimensions. In addition, Idorn et al. 12 showed that the QoL scores in their cohort of patients with Fontan circulation were significantly lower than in controls, but after the age of 16, the physical scores were lower but the psychosocial scores were not. Furthermore, Azt et al. 13 showed results in the same vein when they presented their follow-up data from the Pediatric Heart Network Fontan Cohort. Their cohort also presented lower physical scores, but the majority of adolescents continued to score within the normal range. However, an active lifestyle is an essential component for children and adults living with Fontan circulation, with positive benefits for their health and for many other aspects of their lives. When health professionals consider the results from many studies 7, 8, and the fact that patients with Fontan circulation are less active and their activity level decreases with age, it is clear that we have a major task ahead of us. We have to convey the message, both to the parents and to the patients, that physical exercise is both important and safe. It is therefore important to work strategically and to teach these children to use their bodies as early as possible, as well as how to manage impaired exercise capacity with the physical resources they have. In this way, we can also build up their self-esteem regarding exercise and their views of themselves as physically active individuals. The result would be an improvement in their physical activity levels that could potentially contribute to their QoL, along with complementary interventions to improve, or maintain, the social or emotional aspects of QoL. The study by Hedlund et al. 5 contributes to the current understanding in this area, and it serves as a good basis and inspiration for future interventions, to improve these patients’ health and QoL.