Athletes with valvular heart disease and competitive sports: a position statement of the Sport Cardiology Section of the European Association of Preventive Cardiology

Abstract

This article provides an overview of the recommendations from the Sports Cardiology section of the European Association of Preventive Cardiology on sports participation in individuals with valvular heart disease (VHD). The aim of these recommendations is to encourage regular physical activity including sports participation, with reasonable precaution to ensure a high level of safety for all affected individuals. Valvular heart disease is usually an age-related degenerative process, predominantly affecting individuals in their fifth decade and onwards. However, there is an increasing group of younger individuals with valvular defects. The diagnosis of cardiac disorders during routine cardiac examination often raises questions about on-going participation in competitive sport with a high dynamic or static component and the level of permissible physical effort during recreational exercise. Although the natural history of several valvular diseases has been reported in the general population, little is known about the potential influence of chronic intensive physical activity on valve function, left ventricular remodelling pulmonary artery pressure, and risk of arrhythmia. Due to the sparsity of data on the effects of exercise on VHD, the present document is largely based on clinical experience and expert opinion.

Introduction

Valvular heart disease (VHD) is usually an age-related degenerative process, predominantly affecting individuals in their fifth decade and onwards. Regular exercise should be encouraged in the elderly population with VHD to avoid physical deconditioning and frailty whilst balancing the risks and consequences of the possibility of accelerated progression of VHD. However, there is a pertinent population of younger individuals with congenital VHD, many of whom are asymptomatic, and some aspire to participate in competitive and leisure exercise.¹ Middle-aged and older individuals constitute up to 40% of participants in mass endurance events and a significant proportion will have asymptomatic VHD. The diagnosis of VHD in an athlete during routine or pre-participation cardiac examination often raises questions about on-going participation in competitive sport with a high dynamic or static component and the level of permissible physical effort during recreational exercise. Although the natural history of several valvular diseases has been reported in the general population, little is known about the potential influence of chronic intensive physical activity on the progression of VHD. It is theoretically possible; however, that an increased cardiac preload and afterload during exercise could accelerate valvular dysfunction, resulting in adverse cardiac remodelling, pulmonary hypertension, and a greater propensity to cardiac arrhythmias. Since the previous European Society of Cardiology recommendations on the management of VHD over a decade ago,^2,3 there have been a plethora of novel reports on the investigation and risk stratification of athletes with VHD which necessitates an update.^4–11

These recommendations for exercise in athletes with VHD focus on competitive athletes. Practical guidance for recreational athletes and mildly symptomatic patients with mild and moderate VHD is presented elsewhere.^12,13 The present document is based on available scientific knowledge in the literature, clinical experience, and expert opinion.

Classification of sport

For the purposes of this document and consistent with exercise recommendations in athletes with other cardiovascular diseases, sport disciplines have been divided into four groups, notably skill sports, power sports, mixed sports, and endurance sports in accordance with their haemodynamic responses and their long-term impact on cardiac output and remodelling (Figure 1).^14–16 Although purely dynamic exercise is associated with a predominant increase in volume load to the ventricle and static exercise results in a predominant pressure load, most sport and the training associated with these disciplines consist of both dynamic and static components.¹⁷ It is also true that even within a given sporting discipline, the cardiovascular load varies based on the intensity and duration of exercise, the environmental conditions, and the different roles of the athlete in an organized team. The classification of sports described in Figure 1 is used to provide exercise recommendations for the different valvular defects.

General evaluation of athletes with valvular heart disease

The majority of athletes with VHD are asymptomatic and detected during physical examination or after investigation for an abnormal electrocardiogram (ECG). Athletes with advanced VHD may present with chest discomfort during exertion, breathlessness that is disproportionate to the amount of exercise being performed, exertional dizziness, palpitation, or syncope (Figure 2). Valve defects with a predominantly regurgitant component are often better tolerated than stenotic lesions.¹⁸ Athletes with VHD should be evaluated 6 monthly to 2-yearly based on symptoms and/or severity of valve disease. Transthoracic echocardiography (TTE) is the first-line investigation to confirm the diagnosis of VHD and provides essential prognostic information in relation to the severity of valve dysfunction, ventricular function, and pulmonary artery pressure.^19,20

All athletes with mild to moderate VHD should have an exercise stress test or cardiopulmonary exercise test using a protocol that closely resembles the level of exertion expected from the type of sport wished to be engaged in to assess functional capacity, blood pressure response to exercise, and the presence or absence of myocardial ischaemia and arrhythmias. Exercise echocardiography and 3D echocardiography may provide helpful additional information to quantify the severity of valve dysfunction and may be used if echocardiography produces equivocal results.

Cardiac magnetic resonance (CMR) can also contribute additional value as it can quantify regurgitant volumes, identify the presence of left ventricular myocardial fibrosis^4,5 and provide accurate quantification of biventricular volumes and function. Transoesophageal echocardiography should also be considered when TTE is of suboptimal quality and in athletes with prosthetic valve dysfunction.

Mitral valve stenosis

Mitral valve stenosis (MS) is mostly due to rheumatic heart disease. During exercise, the increase in heart rate and left atrial filling is associated with a high-pressure gradient across the mitral valve which may result in dyspnoea and may even cause acute pulmonary oedema in advanced disease. Chronic elevation of pulmonary venous pressure is associated with pulmonary hypertension and right ventricular hypertrophy.^19,21 The long-term effects of regular intensive exercise on the progression of MS, the pulmonary vasculature, and the right ventricle are unknown although exercise may exacerbate left atrial enlargement predisposing to atrial fibrillation (AF).²²

Evaluation

The presence of MS may be detected by a characteristic mid-diastolic rumbling murmur during auscultation. The severity of MS is generally determined by TTE. The severity of MS is defined by the mitral valve area and pulmonary artery pressure (Appendix Table 1). Valve area measurement using planimetry is the reference technique for the evaluation of MS, taking into consideration the challenges for its assessment,¹⁹ whereas mean transvalvular gradient and pulmonary pressure reflect its consequences and have a greater prognostic value.¹⁹ Pulmonary systolic arterial pressure is assessed by Doppler-echocardiography in the presence of tricuspid regurgitation. Exercise testing (or cardiopulmonary testing) can provide additional information regarding haemodynamic response and the occurrence of arrhythmias (particularly AF). Stress echocardiography provides additional objective information by assessing changes in mitral gradient and pulmonary artery pressure.^3,19,23 Individuals who develop a severe increase of pulmonary artery systolic pressure (sPAP) i.e. > 40mmHg during exercise are likely to develop adverse effects on right ventricular (RV) function over time. Invasive pressure monitoring is indicated only in selected cases when accurate assessment of pressure in the pulmonary circulation is needed for therapeutic purposes. Individuals with severe symptomatic MS require percutaneous mitral commissurotomy as first-line treatment in cases of favourable anatomy or alternatively mitral valve replacement. The likeliness of restenosis after valvuloplasty is low particularly in young individuals and demonstrates favourable outcomes in comparison with prosthesis-related complications.²⁴ For classification of severity of MS, see Appendix Table 1.

Recommendations

Asymptomatic athletes with mild MS may compete in all sports if the resting sPAP is <40 mmHg (Figure 3). Asymptomatic athletes with moderate MS (MVA 1.0–1.5 cm²) may participate in competitive sports involving low intensity and recreational sport involving low and moderate intensity when the resting sPAP is <40 mmHg and an exercise stress test shows good functional capacity and normal blood pressure response.^12,25 Individuals with severe MS or those with a resting sPAP >40 mmHg should avoid all competitive sport. Athletes with MS who are anticoagulated for AF should not participate in collision sports. Recommendations for sports participation in individuals who have undergone mitral valve annuloplasty are based on the residual degree of severity of stenosis. Asymptomatic individuals with mitral valve replacement may participate in skill and mixed sporting disciplines provided valve function is satisfactory and there is no evidence of haemodynamic disturbance.

Mitral regurgitation

Primary mitral regurgitation (MR) is often due to mitral valve prolapse (MVP), degenerative mitral valve disease, infective endocarditis, or rheumatic heart disease.

Secondary MR may also occur due to failure of coaptation of the valve leaflets in cardiomyopathy and ischaemic heart disease (secondary MR). Mitral regurgitation is associated with an elevated left atrial pressure, and increased preload on the left ventricle resulting in a progressive compensatory increase in left ventricular (LV) size as the regurgitant jet becomes more severe.^22,26

Mitral valve prolapse

Mitral valve prolapse is the most frequent cause of primary MR and may be associated with single or bi-leaflet protrusion into the left atrium of at least 2 mm beyond the long-axis annular plane.^22,27 Rhythm disorders (i.e. brady- or tachyarrhythmias), endocarditis, syncope, or systemic thromboembolism are more frequent in these individuals than the general population.¹⁹ Arrhythmic sudden cardiac death (SCD) in MVP is rare (0.2–0.4% per annum) and more common in women, occurs regardless of the degree of regurgitation, and is often associated with bileaflet prolapse, mitral annulus disjunction (MAD), and myocardial fibrosis in the papillary muscles.^4,5 The presence of syncope, T-wave inversion in the inferior leads and ventricular extrasystoles conduction with right bundle branch block (RBBB) morphology and a superior axis should raise suspicion for myocardial fibrosis and along with MAD, are an indication for CMR.⁴

Evaluation

The majority of individuals with mild or moderate MR are asymptomatic²³ and identified during routine auscultation or incidentally during echocardiography. The severity of MR can be assessed by two-dimensional echocardiography with colour flow and Doppler studies. Three-dimensional echo can provide additional information in patients with complex valve lesions. Transoesophageal echocardiography or cardiac CMR are advocated when TTE cannot quantify the severity of MR accurately.^23,28 The severity of MR is classified according to the regurgitant jet (Appendix Table 2). Exercise recommendations in athletes should also consider LV size and function and pulmonary artery pressure. It is important to consider that some athletes, particularly males participating in endurance sport may show a physiologically enlarged left ventricular cavity size that would raise suspicion of severe volume overload in the context of MR. In such cases, decision-making should be based upon functional capacity, myocardial reserve, and pulmonary artery pressure. CMR may be more useful for quantifying the regurgitant volume in such instances and is helpful to detect associated myocardial fibrosis.

In athletes with primary MR an LVEDD with a cut-off of 35 mm/m² in men and 40 mm/m² in women turned out to be useful to identify individuals with LV enlargement of clinical relevance;²⁹ however, a case by case assessment is recommended in such instances when prescribing exercise. A significant increase of pulmonary artery pressure with the exercise of >50 mmHg has been reported to be of prognostic value.²¹ An exercise stress test is recommended in athletes with MR to check functional capacity, haemodynamic response, and complex arrhythmias.

Twenty-four-hour Holter monitoring is recommended to identify complex ventricular arrhythmias in MVP. The risk of SCD is independent of the severity of MR, therefore, risk stratification in these individuals is challenging. Risk factors for arrhythmic SCD in MVP family history of premature sudden cardiac death, inverted T waves in the inferior leads, ventricular premature beats (VPBs) with RBBB morphology at rest or during exercise, MAD, non-sustained ventricular tachycardia (NSVT), and in some cases late Gadolinium enhancement in the basal inferior wall on CMR. For classification of severity of MR, see Appendix Table 2.

Recommendations

Asymptomatic athletes with mild MR may participate in all competitive sports (Figure 3). Asymptomatic athletes with moderate MR may also compete in all sports if the left ventricular end-diastolic dimension (LVEDD) is ≤ 60 mm (or 35 mm/m² in males and 40 mm/m² in females), the LV ejection fraction is ≥60%, and resting pulmonary artery pressure is <50 mmHg and a maximal exercise stress test shows excellent functional capacity, normal haemodynamic response, and absence of significant ventricular arrhythmias during an exercise test.

Athletes with MVP and mild to moderate regurgitation can engage in all competitive sport in the absence of T-wave inversion in the inferior leads on the 12-lead ECG, ventricular arrhythmias on 24 h-Holter ECG, and family history of SCD athletes anticoagulated for AF should avoid collision sports.

Aortic valve stenosis

Aortic stenosis (AS) is the most commonly due to degenerative calcific stenosis and usually affects the elderly population. Progressive disease causes mechanical obstruction to LV outflow resulting in compensatory LV hypertrophy and impaired diastolic function. LV size is usually within normal limits although an enlarged left atrial diameter may reflect haemodynamic burden in patients with asymptomatic severe aortic stenosis.^30,31 Severity of AS is determined by the pressure gradient across the aortic valve as well as valve opening area.³² Symptoms of syncope or presyncope usually precede angina pectoris or dyspnoea which usually appear in a late stage of the disease. The occurrence of SCD is far more probable in symptomatic athletes.

Bicuspid aortic valve

Congenital bicuspid aortic valve (BAV) affects 1–2% of the general population with a higher male predisposition and may be complicated by AS or aortic regurgitation (AR) in young individuals.^13,33–35 Approximately 50% of individuals with a BAV are also at increased risk of aortopathy.³⁴ Over one-third of BAV individuals develop significant AS and/or AR in the fifth decade. In patients with aortopathy the risk of aortic aneurysm, dissection or rupture is 0.1% per annum.³⁴ The long-term significance of intensive exercise on the aortic valve (AV) and aortic root are unknown. Medium-term studies have shown that competitive exercise does not appear to have a detrimental effect on the AV or LV morphology and function.^32,36 Similarly, a medium-term (5-year follow-up) study in a small cohort of athletes showed no differences in aortic root dimensions in athletes with BAV compared with athletes with a trileaflet AV.⁷ In a study with BAV elite athletes, the annual growth rates for sinuses of Valsalva were 0.11 ± 0.59 mm and 0.21 ± 0.44 mm for proximal ascending aorta over 3-year follow-up.¹¹ Moreover, AR was the only functional abnormality, but no significant progression was found. A normally functioning BAV usually does not represent a limit for competitive sport.³⁷ However, affected athletes require regular clinical surveillance to monitor for progressive valvular dysfunction and aortic root dilatation. Athletes with a BAV and associated AS/AR should be managed similar to individuals with trileaflet AS or AR. Several factors influence aortic root dimensions, including body size, age, height, gender, and blood pressure control³⁸ An abnormally enlarged aortic root is defined as a range as an aortic root with a Z-score ≥2 which takes these factors into consideration.³⁶ Raw and corrected aortic measures at all levels were significantly greater in sports with a high dynamic component in both sexes. Among athletes, an aortic root >40 mm in males (1.8%) and >34 mm in females (1.5%) is uncommon and unlikely to represent the physiological consequence of exercise training.^10,39,40 Another study from the British athletes suggests that the upper limit for aortic diameter (sinuses of Valsalva) in females is 38 mm.³⁹ A rapid increase in aortic dilatation (≥0.5 cm/year) may also be suggestive of an aortopathy.⁴¹

Evaluation

Aortic stenosis is frequently detected by auscultation. Determination of the pressure gradient across the aortic valve and the aortic valve area are initially assessed by Doppler-echocardiography. Exercise testing is recommended in individuals with mild to moderate or moderate AS assess for myocardial ischaemia, haemodynamic response, and arrhythmias. Stress echocardiography to evaluate the dynamic component of valvular abnormalities and to unmask subclinical myocardial dysfunction that could be missed at rest is of utmost importance in individuals with at least moderate AS.^42–44 In patients with reduced systolic function and a valve opening area < 1.5 cm², the use of stress echocardiography is often helpful when attempting to differentiate true severe AS from pseudo-severe AS and provides guidance for adequate therapy through evaluating the contractile reserve of the left ventricle.^32,45 For classification of severity of AS, see Appendix Table 1.

Recommendations

Asymptomatic athletes with mild AS and normal LV size and LV function may participate in all competitive sports (Figure 3). Asymptomatic athletes with moderate AS and normal LV function, who show good functional capacity, normal haemodynamic response, and absence of complex arrhythmias during a maximal exercise test, may also engage in all competitive sport. Athletes with moderate AS and coexisting symptoms, LV dysfunction at rest or under stress, and complex ventricular arrhythmias should be advised to refrain from both competitive and recreational sport but may perform the usual physical activity recommendations to maintain cardiovascular health.

In the context of BAV, individuals with an aortic root above the normal range or ≥43 mm in tall men and ≥39 mm in tall women should not participate in sports associated with increased loading conditions on the aorta such as power lifting and isometric exercises^12,46 The general recommendations advise that in individuals with BAV and an aortic root dimension of 45–50 mm only skilled or mixed or low-intensity endurance sports are recommended.¹²

Aortic valve regurgitation

Recognized causes of AR include BAV, rheumatic fever, infective endocarditis, Marfan's syndrome, aortic dissection, systemic arterial hypertension, and rheumatoid spondylitis. AR causes dilatation of the LV cavity with increases in LV diastolic and systolic volumes. Bradycardia can worsen the haemodynamic pattern, due to lengthening of the diastolic duration and increase of the regurgitant volume. AR causes both pressure and volume loading of the left ventricle. Isometric exercise is associated with increased aortic wall tension and may worsen AR.^47,48 Athletes with chronic AR can remain asymptomatic for many years. Once LV function deteriorates, there may be a rapid onset of symptoms including dyspnoea on exertion, arrhythmias and, in advanced cases, angina. In patients with AR, static exercise causes a reduction of preload resulting in a reduction in LV stroke volume and regurgitant volume.⁴⁹ Assessment of LV enlargement can be challenging in athletes with AR as the valve defect itself and also training can induce an enlarged cavity size. As LV dysfunction proceeds, symptoms occur, typically including dyspnoea on exertion, arrhythmias and, in advanced cases, angina.^18,50

Evaluation

AR severity is determined by the colour flow regurgitant jet and continuous wave signal jet (see Appendix Table 3 for classification of severity of AR) and flow reversal with the descending aorta. CMR is recommended as a complementary tool for the accurate assessment of AR severity and AV morphology in cases where image quality is suboptimal by echocardiography.^51,52 Furthermore, tissue characterization by cardiac CMR may offer incremental diagnostic information.⁵³ LV size is also generally assessed by TTE. In consideration that LV cavity dimension is increased in healthy athletes as a consequence of training, this should be considered when assessing LV size in the presence of AR.

Exercise testing (or cardiopulmonary testing) can be helpful for evaluating functional capacity, haemodynamic response, and inducible cardiac arrhythmias.

Recommendations

Athletes with mild AR, normal LV size and function, and normal exercise testing with no arrhythmia can do all sports (Figure 1). Those with moderate AR with a non-dilated LV and a LV ejection fraction >50%, a good functional capacity, and an aortic root <43 mm may engage in moderate-intensity sport. Athletes with severe AR may engage in low- to moderate-intensity sport. However, a case by case evaluation is recommended in such instances as individuals with a mildly enlarged left ventricular cavity (LVEDD 55–65 mm) and normal functional capacity may be permitted to do moderate-intensity sport. Individuals with severe AR with progressive LV dilatation or ventricular arrhythmia at rest or under stress should not engage in competitive sports.

Tricuspid valve stenosis

Tricuspid valve stenosis (TS) is commonly due to rheumatic fever and is frequently associated with MS.⁵⁴ Isolated TS is rare. In athletes with coexisting MS and TS, recommendations should be based on the severity of MS. Asymptomatic athletes with mild TS may participate in all sports. Grading of TS is challenging. However, a mean gradient pressure gradient across the tricuspid valve ≥5 mmHg at normal heart rate is considered indicative of clinically significant TS.¹⁹

Recommendations

Asymptomatic athletes with mild TS and normal ventricular function may participate in all sports. Individuals with moderate or severe stenosis should avoid sports.

Tricuspid valve regurgitation

Tricuspid regurgitation (TR) may be due to acquired disease such as rheumatic fever, infective endocarditis, and carcinoid syndrome or congenital disease such as Ebstein’s anomaly. TR in most cases co-exists or is functional secondary to RV dilatation from other cardiac pathologies including left-sided VHD with co-existent raised sPAP >40 mmHg and systolic dysfunction. The severity of TR is generally determined by TTE. Dense and triangular early peaking regurgitant signal on colour flow, vena contracta flow, proximal isovelocity surface area (PISA), early diastolic filling velocity, and RV outflow velocity are established echocardiographic parameters to assess the severity of TR^3,55 (see Appendix Table 4 for classification of its severity).

Recommendations

Individuals with mild TR may participate in all sports. Individuals with moderate TR with normal biventricular systolic function, normal exercise testing, and a systolic pressure (PAP) < 40 mmHg at rest, all sports are allowed. Individuals with any degree of TR, sPAP > 50 mmHg at rest and RV dysfunction may participate in low-intensity sport only. Individuals with a right atrial pressure of more than 20 mmHg and any degree of TR should avoid competitive sports.^3,56

Multi-valvular diseases

Multi-valvular diseases frequently occur in connection with rheumatic fever, myxomatous valvular diseases or infective endocarditis. These conditions can be assessed quantitatively by Doppler-echocardiography. Deterioration of valvular disease entity may impact on other abnormal valves with unfavourable haemodynamic effects.^19,57 Therefore, close follow-up is warranted in these athletes with regard to participation in competitive sports. Exercise recommendations are governed by the most severe valvular abnormality.

Prosthetic/bioprosthetic heart valves and/or valve repair in exercising individuals

Although patients improve clinically after heart valve replacement, the long-term mortality is higher than in a healthy control population. Furthermore, patients with normal haemodynamic patterns at rest may have abnormal values under physical stress. A proportion of individuals warranting surgical correction for mitral regurgitation may undergo valve repair instead of replacement. There are no data on the natural history of a valve replacement or repair in individuals who exercise intensively, therefore the current consensus recommendations are relatively conservative. Therefore, exercise testing should be performed up to the intensity consistent with that of the sport the athlete wishes to pursue. Given that artificial valves are associated with some flow limitation, we recommend that athletes who have had valve replacements have the same exercise limitations as asymptomatic athletes with moderate native valve disease provided ventricular function is preserved and pulmonary artery pressure is within normal limits.^57,58 Often anticoagulation is mandatory for mechanical prosthesis and those with AF which further limits their choice of competitive sports. As with native valves, athletes with prosthetic valves or valve repair should undergo annual re-evaluation (Figure 2).

Prophylaxis for infective endocarditis

Patients with a previous history of infective endocarditis, patients with prosthetic heart valves, or congenital valve disease are considered high risk patients and should receive antibiotic prophylaxis when exposed to the risk of bacteraemia in accordance with the ESC recommendations⁵⁹ and systematic maintenance of endocarditis prophylaxis must be strictly observed. Non-specific hygiene measures should be applied in all patients with VHD.

Conflict of interest: none declared.

Appendix

References

Author notes