Regular Physical Activities Tied to Improved Quality of Life in Adults With Fontan Circulation

Children and adults with Fontan circulation are at risk of a sedentary lifestyle. This is due in part to physicians who advise patients restrict exercise out of concern vigorous physical activity may contribute to venous hypertension, end-organ injury and other potential dangers.

But, a sedentary lifestyle is associated with decreased quality of life, muscle loss, poor endothelial function and risks to physiologic and psychosocial health.

A recent study of 177 adult congenital heart disease patients with Fontan circulation, designed to investigate the relationship between physical activity and psychosocial health, found high rates of physical activity were associated with lower New York Heart Association heart failure classification.

Analyses in the study, published in The American Journal of Cardiology, show regular physical activities are pivotal to improved perceived health status, and perceived status — not the lower heart failure score — was tied to enhanced quality of life.

“Survivors of the Fontan procedure are among those with the most complex forms of congenital heart disease and at the greatest risk for premature morbidity and mortality,” says Jamie Jackson, PhD, a psychologist, principal investigator in the Center for Behavioral Health at the Abigail Wexner Research Institute at Nationwide Children’s Hospital and study coauthor. “The results of the current study suggest that physical activity was also associated with younger age. Therefore, we should aim to establish healthy levels of physical activity among children with Fontan circulation and continue promoting physical activity as the child ages into adolescence and beyond.”

Dr. Jackson and colleagues who treat children and adolescents with Fontan circulation encourage physical activity with a regimen that recognizes the condition.

“Regular exercise is very important for children and young adults after the Fontan operation,” says Karen Texter, MD, a pediatric cardiologist who runs the Single Ventricle Team and director of Fetal Echocardiography at The Heart Center at Nationwide Children’s. “What I tell families is ‘exercise is good for your heart, and it is even more important when you heart works less efficiently.’”

Although children with single ventricle physiology have reduced exercise capacity and maximal oxygen consumption compared to healthy kids, several studies show that exercise training has a beneficial impact on exercise tolerance in these patients, says Omar Khalid, MD, FAAP, FACC, a pediatric cardiologist who runs the Fontan Clinic at The Heart Center at Nationwide Children’s.

“Improving exercise capacity is considered a good predictor of health outcome, quality of life and survival in general in this high-risk population,” Dr. Khalid says.

He and Dr. Texter recommend pediatric cardiologists encourage exercise by explaining the following to children and their families:

• Children with a stable Fontan condition can safely participate in an exercise-training program and recreational sports. But, before considering participation in competitive sports, children should undergo full evaluation.
• Exercise requires an increase in cardiac output and though children and adolescents with Fontan circulation can have a limited ability to augment certain factors that help increase stroke volume, regular aerobic exercise along with light strength training can generate improvements in those factors.
• Exercise is important to maintain a healthy weight and lifestyle, prevent obesity and promote a healthy self-view and normalcy in other aspects of their lives.
• Beyond being good for the heart, regular exercise is good for the mind.
• Exercise, sports and physical activity can be fun, and it is good to have fun!

Holbein CE, Veldtman GR, Moons P, Kovacs AH, Apers S, Chidambarathanu S, Soufi A, Eriksen K, Jackson JL, Enomoto J, Fernandes SM, Johansson B, Alday L, Dellborg M, Berghammer M, Menahem S., Caruana M, Kutty S. Mackie AS, Thomet C, Budts W, White K, Sluman MA, Callus E, Cook SC, Khairy P, Cedars A, APPROACH-IS consortium and the International Society for Adult Congenital Heart Disease (ISACHD). Perceived health mediates effects of physical activity on quality of life in patients with a Fontan circulation. The American Journal of Cardiology.  2019 Jul 1;124(1):144-150.