Developments in cardiovascular (CV) surgery and related care have led to the improved survival of patients with congenital heart disease (CHD) and more teenagers and adults living with congenital heart disease (ACHD). Traditionally, patients with CHD were advised to limit their physical activity on the basis of a concern that exercise carries excessive risk. These restrictions were often based on minimal data but had lasting effects on patients' physical and psychological well-being. The results of recent studies, however, have highlighted the benefits and safety of routine aerobic exercise in the CHD patient population. Additionally, exercise capacity has been shown to correlate with duration and quality of life in patients with ACHD. Health care providers now support patients with CHD in achieving an active lifestyle. Assessing individual risk and personalizing exercise recommendations can be challenging. This review will outline a general approach to allow patients with ACHD to safely derive all the benefits of exercise training and sports participation (Figure 1).

Figure 1: General Approach to Exercise and Sports Participation for Patients With CHD

General Approach to Allowing Patients With ACHD to Participate in Exercise Training and Sports

1. Resting assessment of the underlying CHD. Providing personalized recommendations for exercise and sports participation first requires consideration of the physiological and hemodynamic limitations and risk factors related to a patient's CHD. These include underlying specific CV anatomy, ventricular function, arrhythmia history, and factors that predispose the patient to ventricular or vascular hypertension. Consideration of these factors is paramount because, even among individuals with similar cases of CHD, marked variation in severity of condition exists.
2. Exercise testing assessment of the underlying CHD. In addition to a resting assessment of the underlying CHD, the increased metabolic demand and hemodynamic stress during exercise merits dynamic assessment in individuals with CHD. Cardiopulmonary exercise testing (CPET) is the best method to assess one's CV risk specifically during exercise to help in determining risk with exercise and sports. A full CPET is useful not only for CV assessments but also because patients with CHD frequently have limitations in pulmonary mechanics, right-to-left shunting, and restrictive lung disease. In addition to evaluating oxygen (O₂) uptake, peak heart rate (HR), blood pressure response, ventilatory response, and exercise O₂ levels, it is critical to evaluate for arrhythmias during exercise. Current guidelines recommend obtaining routine surveillance exercise stress testing between 1 and 2 years for patients who are significantly symptomatic or between every 3 and 5 years for those who have minimal symptoms. Having patients' actual exercise data is critical because many patients with CHD have a nonstandard HR response to exercise, and therefore standard prediction equations and exercise prescriptions will be inaccurate. Additionally, if patients have pacemakers, consideration of the accelerometer type is indicated, as some pacemakers have an inadequate response to the cycle ergometer (e.g., some Medtronic pacemakers require a treadmill because of accelerometer configuration to mount an HR response). Rarely, stress echocardiography can be used to assess pulmonary artery (PA) pressure or for coronary ischemia.
3. Personalized exercise prescription. CPET is the basis for creating an individualized exercise plan. For aerobic exercise, the authors recommend aiming for 150 min of moderate-intensity exercise per week. The exercise prescription includes HR goals (e.g., 60-80% of maximum HR based on CPET) and commensurate rated perceived exertion–based recommendations (13-16 for moderate intensity). In select patients, the recommendation is 75 min of high-intensity exercise per week when possible according to the American Heart Association (AHA). Intensity and duration can then be increased on the basis of patient progress.¹

Sports Participation

This expert analysis focuses on a few patient populations to discuss considerations when evaluating the appropriateness of sports participation in patients with CHD. This analysis discusses representative lesions in simple, moderate-complexity, and high-complexity CHD cases. In addition to considering the demands of each sport under consideration, considering the training regimen each sport demands is equally important. Noting that each patient has a unique set of risks and aspirations is important, and ultimately a shared decision-making mindset should be used. All patients with CHD should be advised that participation in competitive sports should occur only after adequate exercise training, as nonhabituated athletes carry additional risk of adverse CV events.

Simple Complexity (Representative Lesions: Atrial Septal Defect/Ventricular Septal Defect)

For simple lesions such as atrial and ventricular septal defects, minimal testing is required. The goal of testing in these patients is to rule out prohibitive complications, such as severe pulmonary hypertension (in the context of an ongoing shunt) or significant postoperative arrhythmia. If prohibitive complications are ruled out by echocardiography and CPET, these patients should generally not have activity restrictions.

Moderate Complexity (Representative Lesion: Tetralogy of Fallot)

Tetralogy of Fallot (ToF) is the most common "complex" CHD lesion. Repair occurs in infancy, and advancements in surgical technique have led to most patients living into adulthood. Guidelines suggest that patients with repaired ToF without symptoms can exercise without significant restriction. The authors suggest consideration of moderate to high static exercises only in those without significant aortic dilation, valve dysfunction, right ventricular obstruction, ventricular dysfunction, or sustained arrhythmias. For those with significant ventricular dysfunction or sustained arrhythmias, consideration of lower dynamic and static exercises is recommended.^2,3

Severe Complexity (Representative Lesion: Fontan)

The Fontan procedure is a palliation for patients born with single-ventricle physiology, including those with hypoplastic left heart syndrome. The end result of the Fontan procedure consists of the single ventricle connecting to the aorta while the superior and inferior vena cavae passively drain deoxygenated blood directly to the PA. Patients with Fontan circulation have decreased exercise capacity, decreased stroke volume, chronotropic limitations due to sinus node dysfunction, limited peak minute ventilation, and remarkable spirometry findings.⁴ A decrease in peak oxygen consumption (pVO₂) by more than 3% per year or overall by 10% has been shown to be associated with death, transplantation, or need for surgical intervention in the population of patients with Fontan circulation;⁵ subsequently, many providers encourage these patients to undergo a routine exercise regimen. Although there is limited evidence for types of exercise training in the population of patients with Fontan circulation, these patients can increase muscle mass and pVO₂ with resistance training; similarly, cardiac rehabilitation programs in patients with Fontan circulation have shown similar benefits in increasing pVO₂.⁶ Given the complex anatomy of patients with Fontan circulation, many have limitations that preclude competitive sports. As a result, the American College of Cardiology (ACC) and AHA guidelines predominantly recommend low dynamic, low static sports.⁷ However, for consideration of moderate-intensity dynamic sports, the patient with Fontan circulation should be well conditioned, without cyanosis, outflow tract obstruction, ventricular dysfunction, and/or sustained arrhythmias. Consideration of aortic dimensions is critical when assessing static sports, especially in patients with neoaortic root dilation.

References

1. Buber J, Shafer K. Cardiopulmonary exercise testing and sports participation in adults with congenital heart disease. Heart 2019;105:1670-9.
2. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2019;73:e81-e192.
3. Schuermans A, Boerma M, Sansoni GA, et al. Exercise in patients with repaired tetralogy of Fallot: a systematic review and meta-analysis. Heart 2023;109:984-91.
4. Egbe AC, Driscoll DJ, Khan AR, et al. Cardiopulmonary exercise test in adults with prior Fontan operation: the prognostic value of serial testing. Int J Cardiol 2017;235:6-10.
5. Cunningham JW, Nathan AS, Rhodes J, Shafer K, Landzberg MJ, Opotowsky AR. Decline in peak oxygen consumption over time predicts death or transplantation in adults with a Fontan circulation. Am Heart J 2017;189:184-92.
6. Rychik J, Atz AM, Celermajer DS, et al.; American Heart Association Council on Cardiovascular Disease in the Young and Council on Cardiovascular and Stroke Nursing. Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association. Circulation 2019;140:e234-e284.
7. Mitchell JH, Haskell W, Snell P, Van Camp SP. Task Force 8: classification of sports. J Am Coll Cardiol 2005;45:1364-7.