Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 4: Congenital Heart Disease

A Scientific Statement From the American Heart Association and American College of Cardiology

Simple Shunting Lesions (Atrial Septal Defect, Ventricular Septal Defect, Patent Ductus Arteriosus), Treated and Untreated

Of the 8 most common subtypes of CHD, ventricular septal defect (VSD; 34%), atrial septal defect (ASD; 13%), and patent ductus arteriosus (PDA; 10%), respectively, are the most common (2). With rare exceptions, patients with hemodynamically insignificant CHD such as VSD, ASD, and PDA may participate competitively in all sports. There are no demonstrative data that children with hemodynamically insignificant VSD (open or after closure), ASD (open or after closure), or PDA (open or after closure) require exercise limitations or that these lesions are related to acknowledged episodes of sudden cardiac death (SCD) (3,4). Patients with associated pulmonary hypertension secondary to the above-mentioned lesions that is hemodynamically significant can develop acute symptoms, including reduced exercise capacity or, more importantly, arrhythmias, syncope, chest pain, or sudden death (5,6). For the purposes of this document, pulmonary hypertension is defined as a mean pulmonary artery pressure >25 mm Hg or a pulmonary vascular resistance index of >3 Wood units.

Patients with right-to-left shunting may become more cyanotic during exercise, at least in part because of changes in the ratio of systemic vascular resistance to pulmonary vascular resistance, which can result in increased hypoxemia. Therefore, full clinical assessment, including laboratory and exercise testing, should be considered before any physical activity, because this population represents a very high risk of sudden death (6). Additional precautions should be taken when these patients are exercising at altitude, because the pulmonary vascular resistance generally rises, thus increasing the degree of hypoxemia and cardiac workload.

Children with open or surgically closed VSDs have a normal exercise capacity despite a mild chronotropic limitation in the latter. Some data suggest that aerobic capacity is reduced in patients with open or closed VSDs, as well as in patients with closed ASDs. Abnormal right ventricular (RV) and pulmonary pressure can also occur in those with isolated VSDs; however, these findings did not impact these exercise recommendations or identify any episodes of SCD (7).

Pulmonary Valve Stenosis: Treated and Untreated

Mild valvar pulmonary stenosis (PS) is characterized by a systolic ejection murmur, a systolic ejection click that varies with respiration, and a normal ECG. Decisions are based on estimated severity by use of Doppler-derived peak instantaneous gradients. A gradient <40 mm Hg indicates mild PS, 40 to 60 mm Hg indicates moderate PS, and >60 mm Hg indicates severe PS. Treatment can be by surgery or more commonly by balloon valvuloplasty. Adequate relief means a resolution of symptoms or a reduction in gradient to <40 mm Hg.

Aortic Valve Stenosis: Treated and Untreated

Assessment of fully grown athletes with aortic stenosis (AS) is discussed in the Task Force 5 report on valvular heart disease (8). The following discussion pertains to recommendations in children and adolescents. Patients with AS are differentiated between those with mild, moderate, and severe AS by physical examination, ECG, and Doppler echocardiography. In all cases, regardless of the degree of stenosis, patients with a history of fatigue, light-headedness, dizziness, syncope, chest pain, or pallor on exercise deserve a full evaluation. Annual reevaluation is required for all patients with AS, because the disease can progress. Patients with severe AS are at risk of sudden death, particularly with exercise (9).

Mild AS is defined as a mean Doppler gradient of <25 mm Hg or a peak instantaneous Doppler gradient <40 mm Hg. On evaluation, patients should have a normal ECG, normal exercise tolerance, and no history of exercise-related chest pain, syncope, or atrial or ventricular tachyarrhythmia. Moderate AS is defined as a mean Doppler gradient of 25 to 40 mm Hg or a peak instantaneous Doppler gradient of 40 to 70 mm Hg. Patients should have only mild or no LV hypertrophy by echocardiogram and an absence of LV strain pattern on ECG, as well as a normal maximum exercise stress test without evidence of ischemia or tachyarrhythmia, with normal exercise duration and blood pressure response. Severe AS is defined as a mean Doppler gradient >40 mm Hg or a peak instantaneous Doppler gradient >70 mm Hg. Such patients may have symptoms such as exercise intolerance, chest pain, near-syncope, or syncope and likely will have LV hypertrophy with strain on ECG, as well as an abnormal blood pressure response to exercise. For cases in which symptoms for findings on ECG or exercise test appear more severe than expected for the estimated severity by Doppler, cardiac catheterization may be indicated.

Treatment may be by surgery or balloon aortic valvuloplasty. After treatment, patients may be left with residual valve gradient, aortic insufficiency, or both and may experience recurrence or progression, and thus, continued clinical follow-up is needed.

Coarctation of the Aorta: Treated and Untreated

Coarctation may be discrete or in the form of a long segment and causes hypertension in the upper limbs and hypotension in the lower limbs. The severity is determined by a clinical examination that includes the arm/leg pressure gradient, exercise testing, echocardiographic studies, and magnetic resonance imaging. Coarctation is often considered part of a more general aortopathy with a medial abnormality, particularly when associated with a bicuspid aortic valve. This renders the aorta more vulnerable to dilation, aneurysm formation, and dissection and rupture. There is a recognized association with cerebral aneurysms. Virtually all patients, except those with mild coarctation, will undergo intervention, in the form of either surgical repair or percutaneous balloon angioplasty and stenting.

Even after successful surgical repair or stent placement, residual abnormalities may persist. These include residual coarctation and aneurysm formation at the site of repair or stent. Because of the aortopathy, the ascending aorta may also dilate and even dissect and rupture. Systemic hypertension may persist and if not present at rest may also occur on exercise. Some patients may have residual LV hypertrophy, and many may have residual aortic valve disease when a concomitant bicuspid aortic valve is present. Lifetime follow-up is mandatory, and the potential for premature coronary artery disease has been reported.

Before a decision is made regarding exercise participation, a detailed evaluation should be conducted, which should include a physical examination, ECG, chest radiograph, exercise testing transthoracic echocardiographic evaluation of the aortic valve and aorta, and either magnetic resonance imaging or computed tomography angiography. Normal standards exist for peak systolic blood pressure on exercise testing, by age and sex (10,11). Magnetic resonance imaging or computed tomography imaging should be performed to evaluate the thoracic aorta in its entirety, because transthoracic echocardiographic imaging alone will not visualize the entire aorta, and both residual coarctation and aneurysm may be missed.

Elevated Pulmonary Vascular Resistance in CHD

Patients with pulmonary vascular disease and CHD are at risk of sudden death during sports activity. In those with shunts (commonly septal defects or complex CHD), cyanosis is usually present at rest (Eisenmenger syndrome) and worsens with exercise. Most of these patients self-limit their activity, and they should not participate in competitive sports, with the exception of low-intensity (class IA) sports. The benefits of a regular exercise program, however, including improved walk distance, peak oxygen consumption, quality of life, and functional class, have been demonstrated, and thus, physical activity that does not require maximal effort should be encouraged. This usually comprises physical activity that allows the patient to speak a sentence comfortably (the “talk test”), and 6-minute walk tests will facilitate guidance in this regard.

Patients with suspected residual pulmonary hypertension who have undergone prior surgical repair or catheter intervention for shunt lesions should have a complete hemodynamic evaluation by cardiac catheterization before engaging in competitive athletics. Pulmonary arterial hypertension is usually defined as a mean pulmonary artery pressure of >25 mm Hg and a pulmonary arteriolar resistance >3 Wood units. Decisions proscribing exercise for patients with mild degrees of pulmonary hypertension are quite arbitrary, and no evidence-based scientific data exist. Similarly, no data exist with regard to appropriate exercise prescriptions for patients with mild and moderate pulmonary hypertension, which emphasizes the need to collect prospective data.

Patients and families should be cautioned, however, concerning the potential effect of high altitude on the existing abnormal cardiopulmonary physiology, because this may lead to important further elevations in pulmonary vascular resistance in such patients, with adverse effects.

Ventricular Dysfunction After CHD Surgery

It is not unusual for a patient to present with significant ventricular dysfunction early or late after surgery for CHD, and this dysfunction, of course, affects exercise performance. Assessment of ventricular function is more straightforward for patients with systemic LVs than for those with systemic RVs, but the use of cardiac magnetic resonance imaging has improved the assessment of RV function (12). In general, throughout this document, severe ventricular dysfunction is defined as an ejection fraction (EF) <40%, moderate dysfunction as EF 40% to 50%, and normal as EF >50%. It should be recognized that these definitions are somewhat arbitrary. Of course, the other characteristics of the patient’s heart disease and repair should be considered as well, such as valvar stenosis and insufficiency.

Cyanotic CHD, Including Tetralogy of Fallot

Transposition of the Great Arteries: After Atrial Switch (Mustard or Senning Operation)

The atrial switch procedure was reported in 1959 and was performed frequently for transposition of the great arteries (TGA) from approximately the 1960s to the 1990s. Thus, the significant majority of patients with this anatomy are adults, because survival into the third and fourth decades occurs in most patients. Exercise tolerance is diminished in this population and correlates with clinical outcomes (13,15). Recent studies show this population may be at higher risk of sudden death than other CHD populations (19,20). The strongest predictors of sudden death are the presence of prior arrhythmia and severe systemic ventricular dysfunction, although prior VSD, age at repair, QRS duration, and heart failure symptoms may also be associated with an increased risk (19,25–29). The population with TGA with atrial switch likely has a unique response to exercise given reports that a high proportion of sudden death events occur during exertion (28). This adds complexity to the evaluation before sports participation, because the pathophysiology and prevention strategies for SCD in this population are not well understood. Unfortunately, evidence of exercise-induced arrhythmias on routine clinical testing has not been shown to reliably predict exercise-induced SCD events (28). Thus, careful evaluation of clinical status with special attention to clinical history of arrhythmias, patency and structure of the venous baffles, systemic ventricular function, coronary artery anatomy, and presence of additional obstructive lesions (e.g., PS) is recommended. Severe systemic ventricular function is defined as an EF <40%. Clinical evaluation should include cardiopulmonary exercise testing with continuous oximetry before sports participation. Restriction from high-intensity activities should be considered in the presence of severe systemic ventricular dysfunction, persistent arrhythmias, hypoxia, or inability to increase cardiac output, blood pressure, or heart rate with exertion. In the absence of these findings, moderate-intensity sports participation may be safe (30). However, the effect of long-term exercise training on the systemic RV is not known. Therefore, we recommend serial clinical evaluation during the period of sports participation, with assessment of ventricular function to evaluate the medium- and long-term effects of exercise participation. Evaluation for and optimization of pulmonary dysfunction are recommended (31).

Congenitally Corrected TGA

Patients with congenitally corrected TGA (CCTGA) are often diagnosed in childhood, usually in the presence of additional defects, including PS, VSD, or systemic atrioventricular valve abnormalities (see appropriate sections for additional recommendations). In CCTGA, exercise tolerance is limited, and both exercise tolerance and ventricular function are predictive of adverse outcomes (13,15,32,33). Systemic atrioventricular valve dysfunction is not uncommon in this population and correlates with exercise performance (33). In a recent study, patients with CCTGA and additional defects were found to have a particularly high rate of sudden death (19). However, because of the small number of patients with this anatomy, it is difficult to determine the risk factors for this outcome, although systemic ventricular dysfunction and arrhythmias may correlate with these events. When evaluating patients before competitive sports participation, we recommend assessment of clinical stability with noninvasive imaging and cardiopulmonary exercise testing. Clinical assessment should include evaluation of systemic ventricular and atrioventricular valve function and coronary artery anatomy, as well as exclusion of outflow tract obstruction. One small study found that participation of patients with CCTGA in a 3-month exercise training program of moderate to high intensity was not associated with clinical decline (30); however, the effect of long-term exercise training on the systemic RV is not known. Therefore, we recommend serial clinical evaluation during the period of sports participation, with assessment of ventricular function to evaluate the medium- and long-term effects of exercise participation.

Limited data are available to assess the risks associated with sports participation in those who have had a double-switch procedure that resulted in the redirection of pulmonary venous blood to the LV and aorta. However, assessment of the venous baffle and Rastelli or arterial switch integrity is required before consideration of sports participation.

TGA, After Arterial Switch Procedure

Significant numbers of patients have now undergone the arterial switch procedure over the past 3 decades, and thus, many are at an age when sports participation is desired. Coronary stenosis or obstruction is fortunately rare, and concerns are mainly focused on the possibility of supravalvar PS at the site of anastomosis, which is rarely significant. Patients with symptoms such as syncope or exertional chest pain should have a careful assessment of their coronary artery status, because sudden death has been reported late after arterial switch repair (34). Exercise studies are not particularly sensitive in this group of patients, and coronary angiography or other modalities such as computed tomography angiography may be necessary in those with significant symptoms (35). The issue of surveillance of asymptomatic patients after the arterial switch procedure is controversial.

Fontan Procedure

The Fontan operation, a complete redirection of systemic venous blood to the pulmonary arteries, is performed to palliate single-ventricle physiology. Patients with this circulation have significantly decreased exercise performance, and they are able to increase cardiac output during exercise through unique mechanisms (13,36,37). Limitation to exercise performance is multifactorial and correlates with morbidity and mortality (36,38). When patients are evaluated before sports participation, it is imperative to recognize that both the Fontan circulation and the underlying cardiac anatomy can be extremely variable among patients. As a result, thorough clinical assessment is recommended before sports participation. This clinical assessment should include evaluation for risk factors associated with sudden death (20,38,39). Additionally, comprehensive cardiac imaging is recommended, as well as cardiopulmonary exercise testing with continuous oximetry. If there is significant exercise intolerance during a maximal effort test, as evidenced by such things as an inability to increase blood pressure or heart rate, systemic desaturation, or development of arrhythmias or other symptomatic limitations, the healthcare provider should strongly consider restriction from participation in moderate- and high-intensity competitive sports and training. If the recommended evaluation is unremarkable, participation in moderate-intensity and moderate-duration exercise can be considered. This recommendation is based on small studies that have shown evidence of improvement in some measures of fitness without evidence of clinical deterioration in those participating in moderate-intensity exercise training and resistance training (40,41). However, the safety of participation in high-intensity or high-duration sports is unknown. Additionally, evaluation and optimization of lung function before sports participation is recommended (42). All Fontan patients requiring chronic anticoagulation should be restricted from participation in contact sports.

Ebstein Anomaly of the Tricuspid Valve

The phenotypic spectrum of this malformation is extreme, ranging from minimal to profound tricuspid regurgitation and right-sided heart enlargement. If there is an atrial shunt, cyanosis may be present. A minority of patients with Ebstein anomaly will have preexcitation that could precipitate clinically important and symptomatic arrhythmias. Physical disability and increased risk for sudden death with exercise have been reported with severe cases. Risk stratification for exercise-related arrhythmias remains imprecise for this anomaly. In patients for whom there is also evidence of Wolff-Parkinson-White syndrome or in whom a defibrillator has been implanted, the recommendations found in Task Force 9 (43) should be respected as well. Note that the recommendations below apply both before and after surgical plication and are based on the degree of valve regurgitation and existence of arrhythmias.

Congenital Coronary Anomalies

Anomalies of coronary arteries are second in frequency among identified structural causes of SCD in competitive athletes, accounting for ≈17% of such deaths in the United States (44). Anomalous origins of coronary arteries from the wrong sinus of Valsalva or from the pulmonary artery are estimated to be present in ≈1% of the overall population (45) but are proportionately far more common in athletes who die suddenly, as cited above. Although the vast majority of sudden deaths associated with coronary anomalies occur during or shortly after exercise (46), sudden death has been reported in the sedentary state (47).

The most common anomalous origin is the right coronary artery originating from the left sinus of Valsalva, but among athletes who have died suddenly, anomalous origin of the left main or left anterior descending coronary artery from the right sinus of Valsalva is far more prevalent. Furthermore, SCDs are most strongly associated with the pattern in which the anomalous left coronary artery passes between the aorta and main pulmonary artery. An anomalous origin of a coronary artery from the pulmonary artery is far less commonly observed in athletes who die suddenly and in fact often presents with myocardial infarction in infancy or early childhood. Nonetheless, some cases are not recognized until adolescence or adulthood and may be associated with sudden death in athletes, albeit rarely. Nonspecific electrocardiographic findings may be observed in adolescents with otherwise unrecognized anomalous coronary arteries arising from the pulmonary artery.

The ECG is an unreliable screening tool for suspecting or recognizing anomalous origin of coronary arteries before an event, and even stress tests are not uniformly positive among people with these anomalies (48). Clinical symptoms, such as exertional chest discomfort or dyspnea, may be helpful, but 2 reports suggest that 50% of SCDs associated with coronary artery anomalies were first events without prior symptoms (46,49). The best methods for identifying the anomaly include coronary angiography, computed tomography angiography, and magnetic resonance angiography. Although not uniformly successful, athletes undergoing echocardiographic studies for any reason should have careful attempts to identify the origins of the coronary arteries.

Surgical procedures are the only therapies available for correcting these anomalies (50), with return to intense athletic activities permitted after 3 months after the procedure with demonstration of the absence of ischemia on postoperative stress testing (51).