Exercise may bring a wide range of benefits to adult and pediatric patients with Fontan circulation, according to study results intended to be presented at the annual meeting of the American College of Cardiology (ACC.20).1

In patients with Fontan circulation, the systemic venous pressure is elevated, and pulmonary resistance can also increase. Resistance training in individuals with Fontan circulation was found to improve cardiac filling, cardiac output, and exercise capacity on constant positive airway pressure.2

Some patients with Fontan circulation also exhibit myopenia,3 and a subset of adults with Fontan and New York Heart Association Class I/II heart failure may have reduced skeletal muscle mass compared with healthy individuals (mean z-score, -1.5; 70% with z-score <1; 30% with a reduction in z-score in the sarcopenic range).4

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In a study in which adult patients with Fontan circulation were enrolled, muscle mass and peak VO2 increased in participants who followed a high-intensity resistance training regimen (n=6) vs no exercise (n=5) during a 20-week period (P =.003 and P =.02, respectively).2

Individuals with Fontan circulation between the ages of 6 and 20 participating in sports club activities had higher peak VO2 compared with their less active counterparts.5 In another study, patients who had undergone Fontan surgery (n=17; mean age, 15±3) and followed an 8-week-long exercise training program at home or the hospital, had improved oxygen consumption at anaerobic threshold (P =.02) and peak oxygen pulse (P =.049) at the end of the training program compared with baseline.6

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Additional benefits recorded in patients with Fontan circulation engaging in specific types of exercise regimens include improved ventilator efficiency and stroke volume with inspiratory muscle training and strengthening of respiratory muscles with traditional types of exercises.1

“In the setting of Fontan physiology, exercise improves body composition, preload, stroke volume, and exercise capacity,” concluded the presenter, Rachael Cordina, MD, staff specialist in cardiology at the Royal Prince Alfred Hospital in Sydney, Australia. “Exercise is great therapy for mental health and quality of life…[and] observational data suggest that exercise training may translate to improved clinical outcomes but prospective data are lacking.”


  1. Cordina R. Exercise in the Fontan patient: The other medication. Intended to be presented at: American College of Cardiology 69th Annual Scientific Session; March 28-30, 2020; Chicago, IL.
  2. Cordina RL, O’meagher S, Karmali A, et al. Resistance training improves cardiac output, exercise capacity and tolerance to positive airway pressure in Fontan physiology. Int J Cardiol. 2013;168(2):780-788.
  3. Shiina Y, Murakami T, Matsumoto N, et al. Body composition, appetite-related hormones, adipocytokines, and heart failure in adult patients with congenital heart disease: A preliminary study. Congenit Heart Dis. 2018;13(1):79-84.
  4. Cordina R, O’meagher S, Gould H, et al. Skeletal muscle abnormalities and exercise capacity in adults with a Fontan circulation. Heart. 2013;99(20):1530-1534.