Cardiopulmonary function, quality of life and effects of exercise in children and adolescents with Fontan circulation

Background: Survival of children and adolescents with Fontan circulation has increased over the last decades, and today a large majority survive to adult age. There is increasing concern regarding these children’s impaired physical performance and quality of life. Little is known about the impact of exercise on cardiopulmonary function and quality of life in this patient group compared with the impact on healthy children.

Aim: The aims of this thesis were to study activity, physical capacity, lung function, quality of life, and sleep before and after an exercise intervention in patients with Fontan circulation and healthy matched controls. A follow-up, one year after the training programme, was also performed. We wanted to evaluate if physical exercise could improve cardiopulmonary function and also, most importantly, quality of life in this patient group.

Method: Patients with Fontan circulation (n = 30) and healthy controls (n = 25) performed measurements and/or assessments of physical activity including accelerometer recordings, submaximal and maximal exercise capacity, lung function, sleep, and quality of life. All examinations were done prior to and after an individualised endurance training programme for 12 weeks, and also after one year.

Results: Patients with Fontan circulation reported less time in regular physical exercise than healthy controls, though the objectively measured activity was similar. Quality of life was reported significantly lower by the patients and by their parents compared with the controls. After the exercise intervention, submaximal exercise capacity had increased and quality of life was improved for the patients. However, the exercise intervention did not have an impact on maximal exercise capacity for the patients. After one year, patients showed further improvement of submaximal exercise capacity and quality of life was still improved, as after the exercise intervention. Moreover, children with Fontan circulation had impaired lung function and a reduced pulmonary diffusing capacity. Diffusing capacity increased with age in the patients, but less than in the control subjects. Moreover, endurance training improved vital capacity in the patient group. Fontan patients seemed to have a prolonged latency to sleep onset compared with healthy children. Increased time in physical activities seemed to be correlated with better sleep quality in patients with Fontan circulation.

Conclusions: Structured individualized training programmes may improve submaximal exercise capacity, lung function, and quality of life in children with Fontan circulation. Also, the improvement of quality of life appears to remain, one year after the intervention. Increased amount of physical activity may also be beneficial for sleep quality for these patients. The clinical importance of our results is that increased physical activity is likely to be beneficial for cardiopulmonary function, quality of life, and sleep quality in this patient group. Thus, patients with Fontan palliation should be encouraged to regularly engage in individually designed sports and activities. Rehabilitation programmes should include structured individualised endurance training for improved outcome in this group of patients. Impaired health-related quality of life is most likely multifactorial and further research is needed to more fully understand the effects of exercise on cardiopulmonary function, sleep, and quality of life in this patient group. Also, more research is needed on how to individualise recommendations and find optimal regimens for exercise prescriptions from early age for these patients. The important message is, though, to encourage physical exercise for these patients for better health and well-being.

List of scientific papers

I. Hedlund ER, Lundell B, Villard L and Sjoberg G. Reduced physical exercise and health-related quality of life after Fontan palliation. Acta Paediatr. 2016; 105: 1322-1328.
https://doi.org/10.1111/apa.13544

II. Hedlund ER, Lundell B, Soderstrom L and Sjoberg G. Can endurance training improve physical capacity and quality of life in young Fontan patients? Cardiol Young. 2018; 28(3): 438-446.
https://doi.org/10.1017/S1047951117002360

III. Hedlund ER, Ljungberg H, Soderstrom L, Lundell B and Sjoberg G. Impaired lung function in children and adolescents with Fontan circulation may improve after endurance training. Cardiol Young. 2018; 28(9): 1115-1122.
https://doi.org/10.1017/S1047951118000902

IV. Hedlund ER, Villard L, Lundell B and Sjoberg G. Children and adolescents with Fontan circulation have prolonged sleep onset but physical exercise may improve sleep quality. [Manuscript]