The Fontan procedure, the last of a series of operations performed in patients with congenital heart defects, offers improved exercise capacity compared to baseline function but is still reduced compared to healthy peers. Exercise training may improve exercise tolerance but there is no consensus on the safety of this practice or the optimal training regimen. We performed a systematic literature review on the effects of exercise training in patients with a Fontan circulation.

Medline and Embase databases were systematically searched for articles regarding Fontan Procedure and cardiac rehabilitation.

A total of 23 articles met all inclusion criteria; in total, 201 Fontan subjects were included. Characteristics of the exercise training programs varied significantly. There were no adverse effects related to training programs reported in the literature. Most studies reported benefit across various exercise parameters related to exercise tolerance.

Exercise training is safe and beneficial in patients with a Fontan circulation. Exercise training should become a standard of care within this population. Physiological adaptation following exercise training needs to be investigated more extensively.