Physical activity and exercise in patients with congenital heart disease

INTRODUCTION — In the past, clinicians restricted activity for patients with congenital heart disease (CHD) because of concerns that increased activity might be dangerous. However, increasingly, there is an understanding that promoting physical activity benefits the health and well-being of children and adults with CHD. The challenge for clinicians who care for these patients is to ensure safe participation in regular physical activity by tailoring recommendations to the individual's clinical status.

This topic reviews the risks and benefits of physical activity and provides an approach for the assessment and recommendation for regular physical activity in children, adolescents, and adults with CHD.

DEFINITIONS — The following definitions are used in this topic:

●Physical activity – Physical activity is defined as bodily movement produced by the contraction of skeletal muscle that increases energy expenditure above the basal level. It includes recreational and competitive sports and daily living or transportation activities. In adults, a moderate amount of physical activity is roughly equivalent to physical activity that uses approximately 150 calories (kcal) of energy per day or 1000 calories per week. Examples of moderate physical activity include leisure cycling, moderate-effort swimming, playing golf (walking), general cleaning at home, or lawn mowing (figure 1). Vigorous activity includes running, rope jumping, and calisthenics. Of note, individuals can be physically active without demonstrating significant fitness.

●Physical fitness – Physical fitness is a set of physical attributes that enables one to perform daily physical activities. Health-related physical fitness encompasses cardiorespiratory endurance, muscular strength and endurance, flexibility, and body composition.

●Exercise – Exercise is a subset of physical activity that is planned, structured, repetitive, and purposeful, with a main objective of improvement or maintenance of one or more components of physical fitness. Exercise and sports can be divided into dynamic and static components, which can be classified along a spectrum of increasing static and dynamic intensity (figure 2) [1].

•Dynamic exercise is joint movement through relatively small forces within the muscle

•Static exercise involves generating relatively large intramuscular force within the muscle with little joint movement

A specific activity will have dynamic and static components at varying levels. Some sports, such as distance running, have a high dynamic but low static component. Weight lifting and martial arts have a high static but low dynamic component. Boxing, rowing, and kayaking have both high static and dynamic demands. (See 'Classification of sport' below.)

●Competitive sport – Competitive sport is defined as an organized individual or team sport that requires regular competition with others. It typically requires systematic and intensive training and also is usually supervised by a coach [2]. (See 'Patients who wish to engage in competitive sports' below.)

BENEFITS OF PHYSICAL ACTIVITY — The need to promote regular physical activity in patients with CHD is based on the following observations [1-4]:

●Regular physical activity in healthy individuals improves cardiovascular and mental health and decreases the risk of several chronic conditions, including obesity, cancer, cardiovascular disease, and diabetes mellitus. (See "The benefits and risks of aerobic exercise".)

●Advances in CHD management have led to increased long-term survival and a greater number of patients who reach adulthood. As a result, individuals with CHD can reap the benefits of regular physical activity seen in the general healthy population.

●Recognition that previous advice of avoiding physical activity due to concerns about worsening cardiovascular status or sudden cardiac death (SCD) may not be correct.

Routine physical activity in patients with CHD is associated with better cardiorespiratory and musculoskeletal fitness, better quality of life, improved motor skills, and higher self-esteem [3,5-16]. Home-based exercise training programs and cardiac rehabilitation also have the potential to improve exercise capacity and quality of life in patients with CHD [11,13]. Routine physical activity also may have important long-term benefits of reducing obesity and cardiovascular disease, which are more prevalent in children and adults with CHD compared with the general population [17-19]. Several studies have reported that physical or exercise training improved exercise capacity and cardiovascular function in children with complex CHD with no significant adverse effects [13,20-29]. Data have also shown that physical training in children and adolescents with CHD is associated with a rise in daily physical activity and improved psychosocial well-being, with an increase in self-esteem and a decrease in withdrawal and somatic complaints [24,30-32].

LACK OF BENEFIT OF RESTRICTING ACTIVITY — Based on the available evidence, activity restriction does not appear to reduce the risk of sudden cardiac death (SCD) in most patients with CHD. However, unrestricted physical activity may be detrimental in certain conditions, as discussed below. (See 'Conditions with potential risks of exercise' below.)

Though the risk of SCD is higher in children with CHD compared with the general population, SCD is still a very rare occurrence in the CHD population. In addition, most SCD events in children with CHD occur at rest, not during exercise. Thus, activity restriction is unlikely to have a meaningful impact on SCD risk, and any potential benefit is likely outweighed by the downsides of reduced physical activity (eg, obesity, poor cardiorespiratory and musculoskeletal fitness).

The rarity of SCD in CHD, lack of association between SCD and exercise in this population, and lack of apparent benefit of physical activity restriction have been described in several observational studies [32-36]. In a retrospective study of 403 patients with congenital aortic stenosis treated with balloon aortic valvuloplasty, 45 percent of patients were managed with physical activity restriction and 55 percent were not restricted [36]. Over a mean follow-up of 13 years, outcomes were similar in both groups, with death or cardiac transplantation occurring in 6.5 percent in the restricted activity group and 3.2 percent in the unrestricted group. There was only one SCD event in this study, which occurred during rest in a child in the restricted activity group.

In a prospective population-based study of >3,500 patients with repaired CHD, there were 41 sudden deaths during 45,857 patient-years of follow-up (0.09 per 100 patient-years); only 15 percent of SCDs occurred during exercise [34]. In another population-based study of >11,000 children with CHD followed for a mean of 10.6 years, 19 children (0.2 percent) experienced sudden unexpected deaths [35]. None of the deaths occurred during physical activity; however, there were two episodes of aborted SCD that occurred during sports.

NEED FOR INDIVIDUALIZED COUNSELING — The challenge for clinicians is to balance routine daily physical activity and restricting inactivity for patients with CHD, while minimizing any potential risks from exercise. As a result, health care providers need to tailor recommendations for each individual based on a physical activity assessment and monitor the effect of routine physical activity on his or her clinical status. Adherence to an exercise program is challenging for many, including those with CHD, so clinician encouragement is critical [15]. (See 'Our approach' below.)

Virtually all patients with CHD can participate in daily physical activity and exercise, and the majority can participate in recreational sports. For example, patients who underwent an arterial switch operation for transposition of the great arteries can achieve activity levels similar to those of patients free of cardiovascular disease, even though they have a decrease in exercise capacity [37,38]. Nevertheless, certain physical activities may be detrimental to the health of a patient with CHD; this may result in limiting the intensity of the activity but should not preclude participation in physical activity most days of the week. (See 'Conditions with potential risks of exercise' below.)

Discussion with both the patient and family/caregivers is important to understand and address concerns that may reduce daily physical activity [39,40]. Several studies have shown that patients with CHD have reduced levels of physical fitness and lower levels of physical activity compared with those without CHD [2,19,23,39,41,42]. The reasons for diminished activity are multifactorial and may be changing over time, but restriction from activity likely plays an important role. Children with CHD may be needlessly limited from physical activity by worried parents/caregivers [39]. These restrictions are counterproductive and lead to unnecessary anxiety, reduced activity, and poor exercise tolerance later in life [41].

The clinician should ideally provide a management plan to the patient and family with realistic and measurable goals based on the individual's clinical status. Some providers forego specific counselling on physical activity due to limited time during visits or uncertainty about appropriate recommendations [43]. In the absence of specific counseling, many patients with CHD avoid exercise altogether. (See 'Routine physical activity' below and 'Goal setting' below.)

It is also important to review with the family differences between competitive and noncompetitive exercise. Participating and training for competitive sports typically involves high intensity levels of activity, which are usually imposed by the coach or playing conditions. Competitive sports are felt to be of higher risk because the individual may lose the ability to self-regulate effort if symptoms should arise. It is important for both the participant and supervisors to understand and implement medically needed restrictions and to understand how to regulate and monitor activity levels even when participating in "noncompetitive" physical activity. (See 'Patients who wish to engage in competitive sports' below.)

The approach to athletic eligibility is not binary. Rather, there is a continuum of risk, and some physical activities may be "reasonable" or "could be considered" for an individual patient. This is reflected in the 2015 competitive sports eligibility statement from the American Heart Association (AHA) and American College of Cardiology (ACC) [44]. An open discussion of risk between the provider, patient, and family is necessary in order to tailor exercise recommendations to the individual patient [45].

ROUTINE PHYSICAL ACTIVITY

Our approach — Our approach to promoting physical activity in patients with CHD is generally consistent with guidance issued by the American Heart Association (AHA) and European Society of Cardiology (ESC) [2,4]. It includes the following:

●Assessment and goal setting – Determine individual goals for each patient based on physical activity assessment, using history and evaluation of cardiorespiratory fitness; this may include formal exercise testing. (See 'Assessment' below and 'Goal setting' below and 'Exercise testing' below.)

●Exercise prescription and counseling:

•Clearly and specifically articulate the benefits of routine physical activity to the patient and family and the detrimental effects of prolonged sedentary periods (see 'Benefits of physical activity' above). For patients in whom activity restriction is medically necessary, ensure that the patient and family understand the reasons for restriction. Examples of acceptable activities based on the patient's current clinical status should be provided and promoted. It is important for the patient and family to understand that participation in less intensive regular activity is beneficial and should be encouraged.

•Discuss recommendations with the patient/family and provide a written plan of physical activity and restrictions to the patient and family, which are documented in the medical record. (See 'Goal setting' below.)

•Provide guidance on how to build activity time and level so that patients are able to reach a daily goal of moderate to vigorous activity most days of the week. For adults, the recommended duration of daily activity is 30 minutes and, in children, 60 minutes. Supervised exercise training programs may benefit patients with complex cardiac disease [7,46].

●Follow-up care – Assess and monitor ongoing routine physical activity and readjust goals as necessary. (See 'Follow-up' below.)

Assessment

History — The clinician should review the cardiac history to understand the patient's underlying CHD defect and cardiorespiratory status, ask about exertional symptoms, and review the patients current and prior physical activity routines.

Exertional symptoms — Presence of exertional symptoms that may limit the intensity of activity, such as angina, dyspnea, palpitations, dizziness, syncope, and increasing cyanosis. For these patients, exercise testing may be needed to better define the risk of exercising and the current level of fitness [2]. (See 'Exercise testing' below.)

Conditions with potential risks of exercise — Although the vast majority of patients with CHD can participate in daily physical activity and recreational sports with minimal risk, there are certain settings in which unrestricted intensity of physical activity may be detrimental. These include:

●Specific cardiac conditions including hereditary cardiomyopathy, long QT syndrome, other congenital channelopathies, some arrhythmias, and congenital coronary artery anomalies. (See "Overview of sudden cardiac arrest and sudden cardiac death", section on 'Heart failure and cardiomyopathy' and "Causes of wide QRS complex tachycardia in children", section on 'Congenital heart disease' and "Congenital long QT syndrome: Treatment", section on 'Physical activity and LQTS'.)

●Ventricular dysfunction – Although there is a lack of evidence, it is thought that patients with significant ventricular dysfunction are at risk for ventricular fibrillation if they participate in intense exercise and competitive sports [2]. However, these patients can still participate in a wide range of daily physical activity and recreational sports. This was illustrated in one study that reported patients with single ventricle physiology who underwent Fontan procedure from 1994 to 2001 exceeded the recommended normal daily activity level of 60 minutes per day as measured by three-day accelerometer and questionnaire [47].

●Other cardiac conditions – Although evidence is lacking, there is consensus that individuals with the following conditions should be encouraged to participate in noncompetitive activities that have low to moderate dynamic and static components (figure 2) [2].

•Coronary artery compression or insufficiency.

•Significant pulmonary hypertension – Right ventricular pressure approaching or exceeding systemic pressure with right ventricular dysfunction or symptoms.

•Severe left or right ventricular outflow tract (LVOT/RVOT) obstruction.

•Dilation of the aorta – Severe dilation of the aorta is associated with increased risk of aortic dissection. Exertion results in a rise in blood pressure that increases the wall stress of the aorta, which may increase the risk of aortic dissection [48].

●Hypoxia – Although exertion may increase cyanosis in selected patients with elevated pulmonary vascular resistance and intracardiac shunting, hypoxia should not lead to elimination of daily physical activity [2]. Rather, physical activity should be targeted to a comfortable level for each patient. A walk test with oxygen saturation monitoring can be useful for assessment and for guiding recommendations.

●Syncope – In patients with CHD and exertional syncope, further investigation is necessary to determine and manage the underlying cause (eg, arrhythmia) [2]. Patients at risk for syncope should be counseled to avoid activity that would endanger themselves or others should an event occur (eg, horseback riding, scuba diving, rock climbing, and gymnastics). Other activities, such as swimming, ice skating, and bike riding, can be done with supervision. (See "Syncope in adults: Management and prognosis" and "Emergency evaluation of syncope in children and adolescents", section on 'Diagnostic approach' and "Syncope in adults: Risk assessment and additional diagnostic evaluation", section on 'Selected additional testing'.)

●Anticoagulation – Patients who are taking anticoagulation medications should avoid sports in which body impact is an intentional aspect (eg, boxing, tackle football, ice hockey, and combative martial arts), and those in which high speed impacts could be anticipated (eg, downhill skiing) in order to avoid bruising or bleeding, specifically head injury [2]. For sports with unintentional contact, factors that are important to consider before approving participation include bleeding history, current medication and level of anticoagulation, patient skill, and specific activity.

●For patients with pacemakers or implanted cardiac defibrillators (ICD), issues to consider are:

•Protection of the device and leads, and prevention of any soft tissue injury due to impact to the device [2]. Therefore, activities with a high likelihood for direct blows to the device within the chest (eg, boxing, tackle football, ice hockey with body checking, and combative martial arts) should be avoided to prevent damage to the device and soft tissue injury to the patient.

•Appropriate shocks from ICDs appear to successfully terminate arrhythmias during exercise and this should not be a concern [49].

•Data are limited, but there appears to be no or only a small increased risk of shocks during exercise in athletes with ICDs. This was illustrated in a study of 372 patients with ICDs (10 percent with CHD) who participated in organized sports. The risk of shocks during periods of competition/practice, other physical activity, and rest were 10, 8, and 6 percent, respectively. In this cohort followed for 31 months, there was no tachyarrhythmic death or externally resuscitated tachyarrhythmia during or immediately following sports during the 31 months. Freedom from lead malfunction was 97 percent at 5 years, and 90 percent at 10 years.

Activity history — Patients with CHD exhibit a wide range of cardiorespiratory fitness. The degree of limitation is related to the severity of the underlying disease, type of repair, and, sometimes, lack of participating in regular physical activity.

Clinicians caring for patients with CHD should take an activity and inactivity history using appropriate assessment tools at each clinic visit. While direct measurement using accelerometry is more accurate, there are a number of questionnaires that can be used to assess activity and inactivity relatively quickly [50]. These include the International Physical Activity Questionnaire (IPAQ) and The Habitual Activity Estimation Scale (HAES); however, these tools have not been validated in patients with CHD.

In addition, the activity history should elicit any concerns that the patient and family may have regarding physical activity. It allows the clinician to see if the current intensity level of activity is reasonable, excessive, or can be increased. This information will be helpful in counseling and setting activity goals.

Basic cardiac assessment — The following cardiac assessment provides basic functional information needed to develop a daily physical activity regimen for the individual patient [51]:

●Echocardiography measures ventricular function (ie, ejection fraction), pulmonary artery pressure (presence of pulmonary hypertension), and the diameter of the ascending aorta, and detects the presence and degree of RVOT/LVOT obstruction.

●Electrocardiogram (ECG) as an initial screen to detect arrhythmias. In some cases, a 24-hour Holter monitor may be warranted, especially if there is a history of palpitations or syncope.

●In patients with right-to-left cardiac shunting, pulse oximetry measurement of oxygen saturation at rest measures degree of hypoxia.

As noted above, the intensity of physical activity may be limited in individuals with ventricular dysfunction, significant pulmonary artery hypertension or RVOT/LVOT obstruction, arrhythmias, and hypoxia. However, even in these settings, patients should be encouraged to participate in daily physical activity that is customized based on their cardiorespiratory status. (See 'Conditions with potential risks of exercise' above.)

Exercise testing — Exercise testing can provide useful information that can guide physical activity and exercise counseling. Formal exercise testing may uncover risks associated with exercise while the patient is still in a monitored, controlled setting.

In addition, patients with CHD are often unaware of their aerobic limitations. Because they have had abnormal physiology from birth, they do not perceive a decline or deficiency. This lack of awareness is most likely due to CHD patients adapting to their cardiovascular disease by self-regulating their aerobic participation. This is in contrast with patients with acquired heart failure who are more perceptive of their limitations [52]. As a result, exercise testing is more reliable than self-reporting in determining the functional exercise capacity of individuals with CHD.

Exercise testing uses a continuous, ramped increase in workload until the patient develops symptoms (eg, dyspnea, fatigue) that cause the patient to feel unable to exercise at a higher workload. The type of physiologic data depends on the type of equipment used for assessment. For example, treadmill exercise tests with continuous ECG and pulse oxygen monitoring, and periodic blood pressure provide important basic information of changes in ECG, blood pressure, and oxygen saturation. Cardiopulmonary exercise testing, which is more expensive and less readily available, measures ventilation and respiratory gas parameters that are used to calculate maximal oxygen uptake (consumption), ventilatory efficiency, and anaerobic threshold. (See "Exercise testing in children and adolescents: Principles and clinical application" and "Exercise ECG testing: Performing the test and interpreting the ECG results" and "Cardiopulmonary exercise testing in cardiovascular disease".)

Treadmill exercise testing — Treadmill exercise testing with continuous ECG and periodic blood pressure monitoring is less expensive and more widely available than cardiopulmonary exercise testing.

The following basic information can be determined with a standard exercise treadmill test [2]:

●Blood pressure response – A normal response is a rise in systolic blood pressure of ≥25 mmHg [51].

•Hypotensive responses with a progressive drop in blood pressure may occur in patients with significant LVOT obstruction because of limited ability to normally increase cardiac output. This may occur in patients with aortic stenosis [53-55] and hypertrophic cardiomyopathy [56]. If a hypotensive response to exercise is found in patients with outflow obstruction, the test should be terminated. Consideration should be given to relief of the LVOT obstruction. High intensity physical activity should be restricted until LVOT obstruction is relieved.

•Hypertensive response may occur in patients with coarctation of the aorta [57,58]. Those with an exaggerated hypertensive response to exercise (systolic blood pressure >230 mmHg) should have their aorta reimaged to exclude recoarctation and should possibly be placed on antihypertensive therapy prior to participation in a vigorous exercise program.

●Low heart rate response – Low heart rate response (difference between peak and resting heart rate) is associated with increased mortality in a wide variety of CHD [59-61]. It is often seen in patients on medical therapy such as beta-blockers or antiarrhythmics, in those who underwent an atrial switch (Mustard/Senning) procedure for complete transposition of the great arteries, and in those who have undergone the Fontan operation. Although not in itself an indication for exercise restriction, low heart rate response can be a suggestion to the clinician for closer electrophysiology evaluation. If heart rate is the limiting factor for exercise, pacing should be considered after electrophysiology assessment.

●Exercise-induced arrhythmias – Exercise-induced arrhythmias (ie, supraventricular and ventricular tachycardia, and ventricular fibrillation) have been associated with sudden death in CHD patients [33]. They should be identified and treated, if possible, prior to recreational sports participation.

●Myocardial ischemia – Many patients with CHD have abnormal resting ECGs with conduction delays or repolarization abnormalities that make the diagnosis of ischemia by treadmill ECG difficult. In patients with an abnormal resting ECG, and if myocardial ischemia is suspected based on symptoms and predisposing anatomy (eg, coronary artery anomalies or reimplanted coronary arteries after an arterial switch procedure) [62,63], exercise testing should be performed. The use of stress imaging modalities (such as stress echocardiography or stress nuclear testing) should be strongly considered, as in experienced hands it has higher sensitivity and specificity than treadmill ECG alone for the diagnosis of myocardial ischemia. However, assessment may be challenging in patients with CHD, particularly in those with single ventricular physiology. (See "Exercise ECG testing: Performing the test and interpreting the ECG results", section on 'ECG abnormalities during exercise' and "Selecting the optimal cardiac stress test", section on 'Stress imaging modalities'.)

Cardiopulmonary exercise testing — Although more expensive, cardiopulmonary exercise testing with gas exchange provides more information regarding exercise capacity and maximal effort than exercise ECG testing. The methodology and clinical applications of cardiopulmonary exercise testing (also referred to as functional exercise testing) are discussed in greater detail separately. (See "Cardiopulmonary exercise testing in cardiovascular disease".)

During a cardiopulmonary exercise test, the percent of oxygen, carbon dioxide, and respiratory flow is measured in both inspired and expired gas. Continuous ECG monitoring is performed, and blood pressure is periodically measured as well.

The following are derived from gas measurements during exercise testing:

●Maximal effort – The use of the respiratory exchange ratio makes it possible to determine whether the patient has given maximal effort. This is particularly useful in children who might give unreliable self-reported symptoms and have variable cooperation with the test [64].

●Peak oxygen uptake (peak VO₂) is an excellent marker of the patient's cardiovascular capabilities (aerobic capacity) [64]. Patients with reduced peak oxygen uptake have an increased risk for heart failure and sudden death [52,61]. Systematic review of the literature showed patients with valvar heart disease, coarctation of the aorta, and simple septal defects, and those who underwent an arterial switch had the best aerobic capacity, while those with pulmonary arterial hypertension, complex anatomy, and single ventricle physiology had the poorest peak oxygen uptake [52,65]. Adults appear to have greater relative impairment than children with similar diagnoses [66]. Normative tables for aerobic capacity stratified by diagnosis, age, and sex have been published [65].

●Ventilatory efficiency is the ratio of the minute ventilation and carbon dioxide production. Poor ventilatory efficiency has been associated with poor outcomes in acquired heart disease and has also been validated in many forms of CHD, including in patients with tetralogy of Fallot [67] and transposition of the great arteries [68], but not in those with Fontan [60,61] or Eisenmenger syndrome.

Goal setting — The United States Centers for Disease Control and Prevention (CDC) recommends that all children ages 6 to 17 years should obtain 60 minutes of physical activity per day [69]. This primarily includes aerobic physical activity but also some strength training and bone strengthening activity [69]. The CDC recommendation for adults is 150 minutes per week [70]. Because there is a paucity of data on the optimal types or quantity of physical activity for patients with CHD, there are no evidence-based guidelines for daily physical activity goals for this population of patients [2]. When providers are making recommendations for exercise in individuals with CHD, clinicians should start with the CDC general guidelines and adjust as needed based on individual risks, so as to aim to be as close as possible to recommended daily goals. (See "Physical activity and strength training in children and adolescents: An overview", section on 'Duration'.)

Our practice is to set individual goals that are based on the results of the physical activity assessment and are consistent with those outlined by the AHA scientific statement and the working group of the European Association for Cardiovascular Prevention and Rehabilitation (EACPR) [2,51].

●For patients with a successful repair without residual sequelae (eg, no ventricular dysfunction, normal pulmonary artery pressure, no aortic dilation, and no arrhythmias) and no cyanosis, the daily physical activity goals are the same as those of the general population. (See 'Basic cardiac assessment' above and "Physical activity and strength training in children and adolescents: An overview", section on 'Physical activity'.)

●As discussed above, the goals for daily physical activity are modified in select patients. We ensure that patients and families understand the reason for restriction and the need to participate in daily physical activity at a lower intensity than recommended for patients without CHD (see 'Conditions with potential risks of exercise' above):

•For patients with ventricular dysfunction, elevated pulmonary artery pressure, right or left ventricular outflow tract (RVOT/LVOT) obstruction, and/or dilation of the aorta, the intensity of daily physical activity is reduced to a moderate or low level depending on the severity of the abnormal cardiac condition (figure 1). The "talk test" can help these patients to monitor their intensity level so that they are able to talk comfortably. In addition, patients with any of these conditions should be counseled to avoid competitive sports.

•For patients with cyanosis, the intensity of activity is determined by a walk test with concurrent oxygen saturation monitoring.

•For patients with arrhythmias, the daily physical activity should be of low intensity, particularly the static component (figure 2).

•Patients with exertional syncope should be counseled to avoid activities that would endanger themselves or others should an event occur (eg, horseback riding, scuba diving, rock climbing, and gymnastics).

•Patients who receive anticoagulation medications should be counseled to avoid sports that involve intentional or high body impact (eg, boxing, tackle football, ice hockey, downhill skiing, and combative martial arts).

•Patients with pacemakers or implanted cardiac defibrillators (ICD) should be counseled to avoid activities that have a high likelihood for a direct blow to the device within the chest (eg, boxing, tackle football, ice hockey, and combative martial arts).

●In some cases, we use exercise testing to help determine the prescribed intensity level of activity. This information is often reassuring to the patient and families and helps them accept the proposed level of daily physical activity.

●If needed, patients are counseled to build activity time throughout the day to a daily goal of 30 minutes for adults and 60 minutes for children.

●Patients are advised to limit extended periods of sedentary activity [71]. In particular, parents/caregivers should be counseled to restrict screen time to less than two hours a day for children who are five years and older and should not allow screen time before three years of age.

Follow-up — During follow-up visits, health care providers should assess the daily physical activity of their patient and make adjustments based on activity history and the patient's clinical status [2]. Patients should be asked about any physical activity experience that raised concerns. In patients with activity restrictions, cardiorespiratory assessment of physical activity including exercise testing is suggested every three to five years or more often if there are concerns about a change in the risk of physical activity.

PATIENTS WHO WISH TO ENGAGE IN COMPETITIVE SPORTS

General considerations — Competitive sports differ fundamentally from recreational exercise and routine daily physical activity. Participating and training for competitive sports typically involves high intensity levels of activity, which are imposed by the coach or the playing conditions. Individuals have less ability to self-regulate their effort and may not be able to take breaks or decrease effort intensity in response to cardiac symptoms.

If a patient with CHD is participating in a supervised activity, it is important to determine who controls the intensity of activity for the participant. It is important to communicate to the authorized supervisor whether there are restrictions for the individual and when to stop activity if symptoms were to occur. This information is important for supervisors who control the level of activity and also for supervisors of very competitive patients who may not be able to self-limit properly [2].

Classification of sport — The 2015 scientific statement of the American Heart Association (AHA) and American College of Cardiology (ACC) provides competitive athletic participation guidelines for patients with CHD [72]. They utilize the classification of sports based on increasing static and dynamic components (figure 2). An alternative classification schema included in the 2020 European Society of Cardiology (ESC) recommendations categorizes sport as predominantly skill-based (eg, archery, golf), power-based (eg, downhill skiing, rock climbing, wrestling), mixed (eg, soccer, baseball, basketball, tennis, hockey, football), or endurance (eg, cycling, long-distance running, triathlon, cross-country skiing) [4].

Preparticipation evaluation — The evaluation prior to participation in competitive sports requires a more detailed assessment than is performed for routine physical activity or noncompetitive exercise. This is because participants in competitive sports generally exert themselves to greater extremes and are often motivated to ignore symptoms of overexertion. Making a physical activity plan in this setting should be individualized and should balance safety restrictions with the patients' priorities in a shared decision-making process.

We generally agree with the 2020 ESC guidance suggesting that, for adolescents and adults with CHD who wish to engage in competitive sports, the preparticipation evaluation include the following components [4]:

●History and physical examination

●Assessment of five parameters at rest:

•Oxygen saturation

•Ventricular structure and function, typically assessed with echocardiography, less commonly with cardiac magnetic resonance imaging (MRI)

•Estimated right ventricular pressure or pulmonary artery pressure, typically assessed with echocardiography, less commonly with cardiac catheterization

•Aortic dimension, typically measured with echocardiography, though computed tomography (CT) or MRI may be required

•Assessment of arrhythmia risk, with 12-lead ECG, ambulatory monitoring, and/or cardiovascular MRI (the latter is to identify markers of increased arrhythmic risk such as fibrosis)

●Cardiopulmonary exercise testing (see 'Exercise testing' above)

Guidance on sports participation

●According to the preparticipation evaluation — After completing the preparticipation evaluation, the clinician should make a recommendation regarding whether the athlete can participate in competitive sports and, if so, which types of activities are appropriate:

•Patients who have a reassuring history and physical examination, normal results on the five baseline parameters (oxygen saturation, ventricular function, pulmonary artery pressure, aortic size, arrythmia risk), and reassuring exercise test can participate in all competitive sports.

•Patients with abnormalities on the evaluation are progressively restricted depending on the number and severity of abnormalities:

-Patients with a single nonsevere abnormality are restricted from endurance sports (eg, cycling, long-distance running, triathlon) but may participate in skill (eg, archery, golf), power (eg, skiing, rock climbing, wrestling), or mixed sports (eg, soccer, baseball, basketball, tennis, hockey).

-Patients with multiple nonsevere findings may participate in skill sports only.

-Patients with high-risk features such as severe ventricular dysfunction, pulmonary hypertension with right ventricular dysfunction, severe aortic dilation, severe central cyanosis, or significant arrhythmia are restricted from all competitive sports.

Once the physical activity plan has been agreed on, the patient should have regular follow-up and the plan should be reassessed periodically to reflect changes in physiology and/or patient priorities.

●According to the specific heart defect — Disease-specific recommendations are challenging because of the considerable variations in physiology among different patients with the same anatomic diagnosis. For example, the risks associated with competitive sports participation for a patient with a well-repaired atrial septal defect may be negligible, while the risk in a patient with a large atrial septal defect and associated pulmonary arterial hypertension may be prohibitive.

Formal supervised exercise testing is often useful to tailor sports recommendations for individual patients. (See 'Exercise testing' above.)

The 2015 AHA/ACC scientific statement provides recommendations for patients with the following CHD defects, which are discussed in detail separately:

•Atrial septal defects (see "Isolated atrial septal defects (ASDs) in children: Management and outcome", section on 'Sports participation' and "Management of atrial septal defects in adults", section on 'Participation in sports')

•Ventricular septal defects (see "Management of isolated ventricular septal defects (VSDs) in infants and children", section on 'Health care maintenance')

•Patent ductus arteriosus (see "Management of patent ductus arteriosus (PDA) in term infants, children, and adults", section on 'Sports participation')

•Pulmonary valve stenosis (see "Pulmonic stenosis in infants and children: Management and outcome", section on 'Activity')

•Aortic stenosis (see "Subvalvar aortic stenosis (subaortic stenosis)")

•Coarctation of the aorta (see "Management of coarctation of the aorta", section on 'Competitive sports')

•Tetralogy of Fallot (see "Tetralogy of Fallot (TOF): Long-term complications and follow-up after repair", section on 'Sports participation')

•D- and L-transposition of the great arteries (see "L-transposition of the great arteries (L-TGA): Management and outcome", section on 'Activity')

•Postoperative Fontan (see "Overview of the management and prognosis of patients with Fontan circulation", section on 'Exercise')

•Ebstein anomaly of the tricuspid valve (see "Ebstein anomaly: Management and prognosis", section on 'Exercise')

•Congenital coronary anomalies (see "Congenital and pediatric coronary artery abnormalities")

•Acquired coronary anomalies in Kawasaki disease (see "Cardiovascular sequelae of Kawasaki disease: Management and prognosis", section on 'Participation in competitive sports')

Additional guidance from the AHA/ACC provide recommendations for patients with CHD who have the following conditions [72]:

•Pulmonary vascular disease/pulmonary hypertension:

-Patients with mean pulmonary artery pressure of <25 mmHg can participate in all competitive sports.

-Patients with moderate or severe pulmonary hypertension (ie, mean pulmonary artery pressure >25 mmHg) should be restricted from all competitive sports, with the possible exception of low-intensity (class IA) sports (figure 2). A complete hemodynamic evaluation should be performed prior to athletic participation.

•Ventricular dysfunction after CHD surgery:

-Before participation in competitive sports, all athletes with ventricular dysfunction after CHD surgery should undergo evaluation that includes clinical assessment, ECG, imaging assessment of ventricular function (typically with echocardiography), and exercise testing.

-Patients with normal or near-normal systemic ventricular function (ejection fraction [EF] ≥50 percent) can participate in all sports.

-Patients with mildly diminished ventricular function (EF 40 to 50 percent) may participate in low- and medium-intensity sports (classes IA, IB, IIA, and IIB) (figure 2).

-Patients with moderately to severely diminished ventricular function (EF <40 percent) should be restricted from all competitive sports, with the possible exception of low-intensity (class IA) sports.

•Unrepaired cyanotic CHD:

-In patients with unrepaired cyanotic CHD, a complete evaluation is recommended, which should involve exercise testing.

-Patients with unrepaired cyanotic heart disease who are clinically stable and without clinical symptoms of heart failure may be considered for participation in only low-intensity (class IA) sports (figure 2).

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Congenital heart disease in infants and children" and "Society guideline links: Congenital heart disease in adults".)

SUMMARY AND RECOMMENDATIONS

●Benefits of exercise – Promoting daily physical activity is beneficial for the health and well-being of children and adults with congenital heart disease (CHD). Routine physical activity and exercise in patients with CHD are associated with better quality of life, improved cardiorespiratory and musculoskeletal fitness, and improved motor skills in children. Routine physical activity reduces the risk of chronic conditions of obesity and acquired cardiovascular disease, which are prevalent in patients with CHD. (See 'Benefits of physical activity' above.)

●Routine physical activity – Most patients with CHD can participate in daily physical activity and recreational sports. Goals need to be tailored to the individual's clinical status based on a physical activity assessment. The assessment may identify conditions that may require limiting the intensity of activity but do not preclude routine daily physical activity. (See 'Need for individualized counseling' above and 'Routine physical activity' above.)

The management approach consists of assessment and goal setting, exercise prescription and counseling, and follow-up of activity and readjustment of goals as necessary.

•Assessment

-History – The history should review the underlying cardiac defect and history, with a focus on identifying conditions that may limit the intensity of activity (eg, ventricular dysfunction, arrhythmias). In addition, the clinician should ask about exertional symptoms (eg, dyspnea and syncope) and current level of physical activity. (See 'History' above and 'Conditions with potential risks of exercise' above.)

-Cardiorespiratory assessment – The cardiorespiratory assessment consists of a basic cardiac evaluation, including ECG, echocardiography to assess ventricular function, and, in patients with cyanotic heart disease, pulse oximetry. In some cases, exercise testing is used to assess risk and functional exercise capacity. (See 'Basic cardiac assessment' above and 'Exercise testing' above.)

•Prescribing a physical activity regimen

-For patients with successful repair and no residual sequelae, the physical activity goals are the same as those of the general population. This entails 60 minutes per day of physical activity for children between 6 and 17 years and 150 minutes per week for adults.

-For some patients, goals are modified in terms of the type and intensity level of activity. These include patients with compromised cardiac function (eg, ventricular dysfunction and/or outflow tract obstruction, elevated pulmonary artery pressure, or dilation of the aortic root and ascending aorta), arrhythmias, hypoxia, exertional syncope, or implantable cardiac defibrillators or pacemakers or those who receive anticoagulation therapy. (See 'Goal setting' above.)

•Follow-up – Routine follow-up care includes evaluation of daily physical activity and adjustments based on activity history and clinical status. In some patients with activity restrictions, cardiorespiratory assessment including exercise testing may be indicated. (See 'Follow-up' above.)

●Competitive sports – Patients with CHD who wish to participate in competitive sports require a more detailed assessment than is performed for routine physical activity or noncompetitive exercise. This is because participants in competitive sports generally exert themselves to greater extremes and are often motivated to ignore symptoms of overexertion. Making a physical activity plan in this setting should be individualized and should balance safety restrictions with the patients' priorities in a shared decision-making process. (See 'Patients who wish to engage in competitive sports' above.)

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Sarah D de Ferranti, MD, MPH, who contributed to earlier versions of this topic review.