Recommendations for competitive sports participation in athletes with cardiovascular disease

A consensus document from the Study Group of Sports Cardiology of the Working Group of Cardiac Rehabilitation and Exercise Physiology and the Working Group of Myocardial and Pericardial Diseases of the European Society of Cardiology

Antonio Pelliccia1,*, Robert Fagard2, Hans Halvor Bjørnstad3, Aris Anastassakis4, Eloisa Arbustini5, Deodato Assanelli6, Alessandro Biffi1, Mats Borjesson7, François Carrè8, Domenico Corrado9, Pietro Delise10, Uwe Dorwarth11, Asle Hirth3, Hein Heidbuchel12, Ellen Hoffmann11, Klaus P. Mellwig13, Nicole Panhuyzen-Goedkoop14, Angela Pisani5, Erik E. Solberg15, Frank van-Buuren13 and Luc Vanhees2

Experts who contributed to and revised parts of these recommendations:, Carina Blomstrom-Lundqvist16, Asterios Deligiannis17, Dorian Dugmore18, Michael Glikson19, Per Ivar Hoff3, Andreas Hoffmann20, Erik Hoffmann21, Dieter Horstkotte14, Jan Erik Nordrehaug3, Jan Oudhof22, William J. McKenna23, Maria Penco24, Silvia Priori25, Tony Reybrouck2, Jeff Senden26, Antonio Spataro1, Gaetano Thiene9

1National Institute of Sports Medicine, Italian National Olympic Committee, Via dei Campi Sportivi 46, 00197 Rome, Italy
2Cardiovascular Rehabilitation Unit, KU Leuven, Leuven, Belgium
3Department of Heart Disease, Haukeland University Hospital, Bergen, Norway
4Division of Inherited Cardiovascular Diseases, University of Athens, Athens, Greece
5Department of Pathological Anatomy, University of Pavia, Pavia, Italy
6Department of Cardiology, University of Brescia, Brescia, Italy
7Department of Medicine, Sahlgrens University Hospital/Östra, Gothenburg, Sweden
8Unité Biologie et Medicine du Sport, Hopital Pontchavillon, Rennes, France
9Departments of Cardiology and Pathology, University of Padova, Padova, Italy
10Department of Cardiology, Civil Hospital, Conegliano, Italy
11Department of Cardiology, University Hospital, Munich, Germany
12University Hospital Gasthuisberg, Leuven, Belgium
13Department of Cardiology, Bad Oeynhausen, Germany
14Department of Cardiology, Nijmegen, The Netherlands
15Klinikk Ullevål Sykehus, Oslo, Norway
16Department of Cardiology, University Hospital Uppsala, Uppsala, Sweden
17Department of Sports Medicine, Aristotle University, Thessaloniki, Greece
18Wellness Medical Center, Stockport, UK
19Heart Institute, Sheba Medical Center, Tel Hashomer, Israel
20Division of Cardiology, University Hospital, Basel, Switzerland
21Children National Medical Center, Washington DC, USA
22Cardiac Rehabilitation Center, Bronovo Hospital, Gravenhage, The Netherlands
23Heart Hospital, University College London, London, UK
24Department of Cardiology, University of L'Aquila, L'Aquila, Italy
25Molecular Cardiology, Fondazione S. Maugeri, Pavia, Italy
26Department of Cardiology, Meander Medisch Centrum, Amersfoort, The Netherlands

Received 23 November 2004; revised 17 March 2005; accepted 7 April 2005; online publish-ahead-of-print 27 May 2005.

* Corresponding author. Tel: +39 6 3685 9127; fax: +39 06 36859256. E-mail address: ant.pelliccia{at}libero.it

This paper was guest edited by Prof. Hugo Saner, Inselspital, Kardiovaskulare Pravention & Rehabilitatiom, Schweizer Herz- und Gefasszentrum, Bern, Switzerland

Introduction

The rationale for offering an expert consensus document concerning the participation in competitive sports by individuals with cardiovascular (CV) disease is based on the widely accepted clinical perception, substantiated by scientific evidence,1 that athletes with underlying (even clinically silent) CV disease have an increased risk for sudden cardiac death (SCD) or clinical deterioration in comparison with normal individuals, by virtue of their regular exercise training and sports participation. Therefore, the aim of the present recommendations is to provide careful guidelines to physicians and consultant cardiologists regarding the evaluation of athletes with CV abnormalities and to suggest sports activities that can be safely performed. These recommendations assume that the cardiac diagnosis has already been made, so that the issues directly related to screening for CV disease2 are beyond the scope of this document.

Target of the recommendations

For the purpose of this document, the target of recommendations are competitive athletes, here defined as individuals of young and adult age, either amateur or professional, who are engaged in exercise training on a regular basis and participate in official sports competition. Official sports competition (local, regional, national, or international) is defined as an organized team or individual sports event that, placing a high premium on athletic excellence and achievement, is organized and scheduled in the agenda of a recognized Athletic Association.

A characteristic of competitive sports, regardless of the level of achievement, is the strong proclivity for participants to exert themselves physically until their limits and improve performance.3

Our interest in competitive sports is dictated by the awareness that competitive athletes (in particular, the elite and professional ones) represent a special subset of the society, not only for their outstanding performances, but also for the substantial economic interests they gather, as well as the intense pressure to which they are exposed by sponsors, Athletic Associations, and media.

Conversely, the present recommendations do not apply to individuals participating in a variety of recreational or leisure sports activities, from modest to vigorous, on either a regular or an inconsistent basis, not requiring systematic training or pursuit of excellence, nor the same pressure to prevail against others which is characteristic of competitive sports.

Nature of the recommendations

The present recommendations represent the consensus document of an international panel of experts appointed by the European Society of Cardiology (ESC), including clinical CV specialists with experience in exercise physiology, sports medicine, and clinical cardiology. The present recommendations are based on published scientific evidence, when available, and on personal experience and consensus of experts. However, in consideration of the scarcity of scientific investigations concerning the effect of regular sport activities on the pathophysiology and clinical course of several CV diseases, the panel acknowledges the difficulties inherent in formulating arbitrary recommendations, particularly for CV diseases in which scientific evidence is inconsistent. Therefore, caution is needed in applying the present document, and efforts should be made to tailor precise advice to each individual. The aim of the panel was to formulate indications which represent a reasonable balance between the risks and the benefits inherent with competitive sports participation, and not simply restrict sports activity that could conceivably be associated with increased risk. The present recommendations represent, therefore, a prudent, contemporary, and practical document for advising competitive sports activity in patients with CV disease.

Finally, these recommendations should be placed in perspective. The present document is based on the (scarce) scientific evidence relating the risk of death or disease progression of several CV diseases with exercise training and sports, and more information is expected to be available when a pre-participation screening programme will be widely implemented across the European countries.2 Therefore, the recommendations formulated in this document are going to be updated when larger knowledge of the natural history of CV diseases in relation to sports participation will be available.

Implementation of the recommendations within the European countries

At present, a large heterogeneity (or lack) of regulations exists in the European countries, with only a few countries requiring medical clearance of competitive athletes and, in individuals with CV disease, having implemented guidelines which are considered the standard of medical care.4 This panel believes that a common protocol for evaluation and management of competitive athletes with CV disease is now needed, in consideration of the unlimited opportunities for professional athletes to move across the European Union. Implementation of a unique and appropriate consensus document will be of relevant medical (and legal) value for physicians required to evaluate athletes with CV disease in the different European countries.

In the absence of binding requirements established by law, this panel recommends that the present recommendations represent the standard of medical care for evaluation of competitive athletes with CV disease. Adherence to these recommendations will have substantial and cost-effective impact on medical care, by enhancing the safety of athletic activities and reducing the legal controversies related to different (or lack of) regulations. This panel advises that implementation of the present recommendations will occur in the different European countries with keeping in mind the different legal and cultural backgrounds, possibly by legislative action, and with the support of the national scientific and sport organizations.

Role of the examining physician

Should the physician be the ultimate authority in determining whether an athlete with CV disease can participate in competitive sports? Alternatively, can the athlete with CV disease just sign an informed consent form and be engaged in a risky and potentially life-threatening sports activity?

Owing to the unique structure and pressures of competitive sports, individuals with CV disease may not always use proper independent judgement in assessing the overall risk associated with a competitive sports career. This panel believes that the examining physician (as well as the consultant cardiologist) has the ethical, medical, and legal obligation to exhaustively inform the candidate of the risks inherent in competitive athletic lifestyle and, when the CV risk appears to be disproportionately high, the physician should be responsible for the final decision, with the aim to prevent adverse clinical events and/or reduce the risk for disease progression. The safeguard of the athlete's health is the paramount objective of the physician, regardless of other considerations, such as the visibility and economic revenues for sponsors or Athletic Association, which may be dependent on the athlete's competitive activity. These recommendations are intended, therefore, to support the physician's decision in such difficult instances and to offer medical protection to the athlete from the unsustainable hazard of a competitive sports activity.

Search methodology

We performed systematic MEDLINE searches of the English language literature up to 2004, and reviewed abstracts of all pertinent research and review articles, and also included selected articles suggested by experts in this field.

Classification of sports

A classification of the different sports is provided in Table 1. Sports activities are classified into two main categories (i.e. dynamic and static) and intensity is roughly divided into low, moderate, and high.5 This classification is intended to provide a schematic indication of the CV demand associated with different sports, with an additional notification of those disciplines associated with increased risk of bodily collision and those associated with an enhanced risk if syncope occurs (which should be avoided in certain cardiac patients).


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Table 1 Classification of sports
 
Recommendations for participation in competitive sports in athletes with congenital heart disease

General considerations
Patients with congenital heart disease (CHD) who participate in competitive sports may expose themselves to an upper limit of physical and mental stress.6,7 Because the available literature regarding exercise and sports participation in patients with CHD is limited, a restrictive attitude seems wise. As a general recommendation, exercise physical tolerance in children with CHD is better than in adults with CHD, and dynamic exercise seems to be more suitable than static exercise.8,9 Some lesions are not compatible with competitive sports, due to their morphologic severity/complexity and tendency to serious arrhythmias, including Eisenmenger syndrome, secondary pulmonary hypertension, univentricular hearts, coronary artery abnormalities, Ebstein anomaly, congenitally corrected transposition of the great arteries, and transposition of the great arteries corrected by the Mustard, Senning, or Rastelli procedure.

Arrhythmias
As survival has improved among patients with CHD, arrhythmias become a more common problem in the long-term course of these patients. Although SCD is a feared consequence of CHD, only few cases occur during exercise.10 Patients who have undergone extensive atrial or ventricular surgery are at greater risk of arrhythmias due to scarring and ventricular dysfunction. Transventricular repair and repair late in life, both pre-dispose for arrhythmias and represent a possible reason for excluding these patients from competitive sports. In addition, the presence of ventricular dysfunction represents a serious risk for developing arrhythmias. These are important issues to consider in patients with repaired tetralogy of Fallot or corrected and uncorrected atrial septal defect (ASD), ventricular septal defect (VSD), or atrioventricular septal defect (AVSD).11 For instance, in tetralogy of Fallot, a gradual widening of the QRS duration of >160 ms might indicate an increased risk of sustained ventricular tachycardia. A history of frequent and complex tachyarrhythmias is a reason for CHD patients to refrain from participation in competitive sports.

Ventricular function
Both left ventricular (LV) and right ventricular (RV) function may be impaired due to inadequate myocardial protection during surgical repair. Because impaired ventricular function is a trigger for arrhythmias and reduced exercise tolerance, the assessment of systolic and diastolic indexes of LV and RV function is mandatory.

Pulmonary vascular resistance
Lesions with longstanding left-to-right shunt, corrected or uncorrected, may have caused persistent pulmonary hypertension.12 In addition, patients with mitral valve dysfunction are at risk for developing pulmonary hypertension. Assessment of pulmonary arterial pressure during exercise is indicated and an intermittent increase in systolic pressure to <35 mmHg may be safely tolerated.

Dysfunction of the valves
Like exercise, both valve stenosis and/or valve insufficiency result in ventricular and/or atrial overload and have to be accurately estimated before giving recommendations for competitive sports13 (see also Acquired cardiac valve diseases).

Conduits and mechanical valves
Patients with conduit should be refrained from competitive sports and patients with mechanical valves on anticoagulation should avoid sports with a risk of bodily collision.

Functioning class
A score according to the New York Heart Association (NYHA) classification can be useful. Only patients in NYHA Class I are entitled for unrestricted participation in competitive sports.

Abnormal exercise blood pressure response
An abnormal increase in systolic blood pressure (BP) during exercise is found in patients with repaired coarctation of the aorta (CoA). Whether it is of significant long-term importance in competitive athletes with CoA is not completely known. An impaired rise or even fall in BP during exercise may be seen in patients with aortic stenosis and should lead to further investigations.

Prophylaxis of endocarditis
Patients with CHD participating in competitive sports follow the same recommendations regarding endocarditis prophylaxis as non-competitors.

Unrecognized CHD
Most of CHD lesions are diagnosed during childhood (provided a good health care system); nevertheless, late diagnosis of ASD, CoA, and LV outflow tract obstructions is not uncommon. A structured screening programme of all athletes would probably identify most of these cases.2 Abnormal coronary arteries, in contrast, are unlikely to be diagnosed during life, despite extensive screening.

Evaluation
The evaluation should include a medical history, with special emphasis on surgical reports, a careful physical examination, electrocardiography, chest X-ray, and echocardiography, including an estimation of peak pulmonary artery pressure. We recommend a questionnaire to describe the symptomatic status according to the criteria of the NYHA. Treadmill or bicycle exercise testing with ergospirometry best evaluates the work capacity. The exercise testing should be standardized (e.g. the Bruce protocol) and include electrocardiogram (ECG) recording, maximal heart rate, BP, and possibly respiratory gas analysis with oxygen uptake (VO2max).

Individualized supplementary investigations
Magnetic resonance imaging (MRI) can be extremely useful in describing both functional and anatomical features, especially in cases which are inadequately visualized by echocardiography. If an arrhythmia is mentioned by the patient or common in that particular lesion, both 24 h Holter ECG monitoring and exercise testing are indicated. In some situations, e.g. when an elevated pulmonary artery pressure is suspected and cannot be determined by other methods, cardiac catheterization may be indicated.

Follow-up and re-evaluation
It is recommended to closely follow the clinical course of the subject with CHD participating in competitive sports activities, and structured reassessment may be indicated, according to the clinical judgement, every 6 or 12 months in most patients. Finally, a complete reassessment is advisable every second or third year, according to the lesion and the individual clinical course.

Conclusion
Because physical activity and sports participation have positive effects on both physical and mental health, only those patients with CHD who are likely to deteriorate as a consequence of regular physical exercise and/or those in whom exercise may trigger serious atrial/ventricular tachyarrhythmias should be restricted from sports participation. Indeed, it should be considered that the haemodynamic balance in patients with CHD varies considerably, even among patients with the same lesion. This makes it impossible to state recommendations that are valid in all cases and support the relevance of the examining cardiologist to tailor the recommendation to each individual patient.

Recommendations
See Table 2.


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Table 2 Recommendations for competitive sport participation in athletes with CHDs
 
Recommendations for participation in competitive sports in athletes with acquired cardiac valve diseases

Mitral valve stenosis
Mitral valve stenosis (MVS) is generally of rheumatic origin. This defect results in increased left atrial (LA) pressure, leading to pulmonary hypertension. The increase in heart rate and cardiac output associated with intensive exercise can markedly increase the pulmonary arterial pressure and may eventually lead to acute pulmonary oedema.14 Embolization by atrial thrombus represents a further complication, which usually occurs in the presence of atrial fibrillation (AF) and enlarged left atrium.15 To date, the long-term effects on the pulmonary circulation and on the right ventricle of repeated pulmonary arterial pressure boosts as a result of chronic physical activity are not completely known.

Evaluation
Presence of MVS can be detected by characteristic auscultation and severity can be determined by ECG, echocardiography, chest X-ray, and exercise testing.

Echocardiography16 allows assessment of valve opening area, presence of calcification, and papillary muscle function. The contribution of regurgitation should also be considered in the calculation of the valve opening area. Pulmonary systolic arterial pressure can be assessed by Doppler-echocardiography in the presence of tricuspid regurgitation, even during/after exercise. Exercise testing (or cardiopulmonary testing) can add information regarding the haemodynamic behaviour and occurrence of arrhythmias (particularly AF). Invasive testing, e.g. Swan–Ganz catheterization, is indicated only in selected cases, when accurate assessment of pressure in the pulmonary circulation is needed for therapeutic or legal purposes. Athletes who develop a pulmonary artery systolic pressure >80 mmHg during exercise are likely to develop severe adverse effects on RV function over time.

Classification
The severity of MVS can be categorized as follows:

  1. Mild=mitral valve opening area >1.5–2.5 cm2, with pulmonary systolic arterial pressure <35 mmHg, and mean gradient ≤7 mmHg;
  2. Moderate=mitral valve opening area between 1.0 and 1.5 cm2, with resting pulmonary systolic arterial pressure between 35 and 50 mmHg, and a mean gradient between 8 and 15 mmHg;
  3. Severe=mitral valve opening area <1.0 cm2, with resting pulmonary systolic arterial pressure >50 mmHg, and a mean gradient >15 mmHg.
Patients with MVS and AF must receive anticoagulation treatment (provided there are no contraindications) to avoid risk of systemic embolism.

Recommendations
See Table 3.


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Table 3 Recommendations for competitive sport participation in athletes with valvular disease
 
For subjects with MVS associated with AF, see also section Arrhythmias.

Mitral valve regurgitation
The most frequent cause of mitral valve regurgitation (MVR) is the prolapse of leaflets. Other causes include post-rheumatic fever, infectious endocarditis, coronary heart disease (ischaemic cardiomyopathy), or connective tissue disease, e.g. Marfan's syndrome (MS) or dilated cardiomyopathy. MVR is responsible for a regurgitated blood into left atrium, which causes increased LV diastolic filling and raises LA pressure.

Evaluation
MVR is detected by a characteristic auscultation. Severity of MVR can be assessed by Doppler-echocardiography,17,18 ECG, and chest X-ray. In assessing the severity of MVR, it should be considered that LV end-diastolic size is usually increased in well-trained athletes and, therefore, the definition of LV dilatation should be adjusted. In athletes, the severity of MVR should be based on LV end-systolic volume, with the cut-off of 55 mL/m2 useful to distinguish individuals with LV enlargement of clinical relevance. The extent of the LA enlargement should also be considered, because of the proclivity to AF. The 24 h Holter monitoring is recommended when arrhythmias are evident (or strongly suspected) and when MVR is due to prolapse of the leaflets.

Classification
There are several methods to classify MVR. The widely accepted PISA-method uses the width of the jet and the velocity to assess the degree of regurgitation. In addition, the vena contracta method is suitable for classification.17

  1. Mild=regurgitation width <0.3 cm;
  2. Moderate=regurgitation width 0.3–0.6 cm;
  3. Severe=regurgitation width >0.6 cm.

Recommendations
See Table 3.

Patients with AF must receive anticoagulation treatment and they should avoid sports with risk of bodily collision (see also section Arrhythmias).

Aortic valve stenosis
The most common cause for aortic valve stenosis (AVS) is a rheumatic or congenital lesion. Calcified degenerative stenosis is often associated with congenital abnormality of the aortic valve (e.g. bicuspid valve), especially when aortic stenosis is identified in young patients. Syncope can appear in a young athlete with a mild degree of AVS.19 Nevertheless, symptoms such as angina and dyspnoea usually appear in a late stage of the disease. Occurrence of SCD is far more probable if one of these symptoms is present.20

Evaluation
AVS is often detected by a characteristic auscultation. Determination of the gradient and valve opening area should be carried out by Doppler-echocardiography. Exercise testing (by bicycle ergometry) is recommended to assess LV function, development of ST segment depression, BP behaviour, and possible arrhythmias. Given that the severity of AVS is often progressive, periodical evaluation is necessary.

Classification
Classification is based on the mean aortic valve gradient and aortic valve opening area (AVA).

  1. Mild=mean gradient ≤20 mmHg (AVA >1.5 cm2);
  2. Moderate=mean gradient between 21 and 49 mmHg (AVA 1.0–1.5 cm2);
  3. Severe=mean gradient ≥50 mmHg (AVA <1.0 cm2).

Recommendations
See Table 3.

Aortic valve regurgitation
The commonest causes of aortic valve regurgitation (AVR) include congenital bicuspid aortic valve, rheumatic lesion, infectious endocarditis, MS, aortic dissection, systemic arterial hypertension, and rheumatoid spondylitis. AVR 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. Athletes with AVR in the chronic compensated phase are often asymptomatic and can remain so far for many years. As LV dysfunction proceeds, symptoms occur, typically including dyspnoea on exertion, arrhythmias and, in advanced cases, angina.21

Evaluation
AVR is often detected by a characteristic auscultation. LV dilatation can be evaluated by echocardiography. 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 AVR. Exercise testing (or cardiopulmonary testing) can be helpful in the evaluation of exercise tolerance22 and should be carried out up to the level that is consistent with the sports participation, enabling the patient tolerance to be specifically assessed. Because of possible progression of AVR over time, periodical evaluation is recommended.

Classification
The haemodynamic severity of AVR can be classified as follows:

  1. Mild=absence of peripheral signs of AVR and normal LV and atrial size and function; small dimension of the diastolic flow signal on Doppler-echocardiography.
  2. Moderate=peripheral signs of AVR, mild-to-moderate enlargement of the LV, normal systolic function, moderate dimension of the diastolic flow signal on Doppler-echocardiography.
  3. Severe=peripheral signs of AVR, marked dilatation of the LV and/or evidence of LV dysfunction; enlarged atrial size, and large dimension of the diastolic flow signal on Doppler-echocardiography.

Recommendations
See Table 3.

For athletes with Marfan's syndrome: see specific section.

Tricuspid valve stenosis
In most cases, tricuspid valve stenosis (TVS) is caused by rheumatic fever and is associated with MVS. In the presence of MVS and TVS, patients should be assessed with reference to the MVS. An isolated TVS is rare.23 If the patient is asymptomatic (no dizziness, no dyspnoea, or peripheral oedema), participation in competitive sports may be possible.

Recommendations
See Table 3.

Tricuspid valve regurgitation
Tricuspid valve regurgitation (TVR) is often the consequence of RV dilatation. Rheumatic fever and infectious endocarditis are less common causes. Primary TVR leads to volume overload of the RV, increased venous pressure, and congestive symptoms.

The severity of TVR can be determined non-invasively by physical examination, chest X-ray, and echocardiography.

Recommendations
See Table 3.

Multi-valvular diseases
Multi-valvular diseases frequently occur in connection with rheumatic fever, myxomatous valvular diseases, or infectious endocarditis. These conditions can be diagnosed by physical examination and assessed quantitatively by Doppler-echocardiography. Multi-valvular diseases of mild severity may reciprocally worsen each other for their haemodynamic effects and, therefore, great caution is needed in these athletes with regard to participation in competitive sports.

Recommendations
See Table 3.

Post-operative patients with a prosthetic/bioprosthetic heart valve
Although patients are clinically improved by heart valve replacement, the long-term mortality after operative therapy is higher than in a control population. Furthermore, many patients with normal haemodynamic pattern at rest have abnormal values under physical stress. Therefore, exercise testing (possibly integrated with cardiopulmonary analysis) should be carried out up to the limit consistent with the sport the athlete wishes to pursue. Indeed, patients with mechanical valves (or bioprosthetic valves in selected cases) need systematic anticoagulation treatment, which further limits their potential for competitive sports participation. Patients with artificial valves should undergo periodic re-evaluation.24

Recommendations
See Table 3.

Athletes with a prosthetic or bioprosthetic valves who are receiving anticoagulation treatment should not participate in sports with a risk of bodily collision (Table 1).

Athletes post-valvuloplasty
Valvuloplasty is still performed in many patients with MVS, despite the likeness of restenosis and no clear advantage in comparison to valve replacement. Aortic valvuloplasty is only rarely performed in young patients with AVS. In patients after valvulotomy, recommendations for sports participation are based on the residual degree of severity of stenosis and/or regurgitation. Exercise testing should be carried out up to the level consistent with the level reached in the sport in which the patient participates.

Recommendations
See Table 3.

Mitral valve prolapse
Mitral valve prolapse (MVP) is mostly associated with myxomatous degeneration of the valve. It preferentially occurs in patients of tall stature and shows a familial cluster.25 Ischaemic cardiomyopathy and hypertrophic obstructive cardiomyopathy are potential secondary etiologies.

Mitral regurgitation is often associated with MVP. In addition, rhythm disorders (i.e. brady- or tachyarrhythmias),26 endocarditis, syncope, or embolism can also occur.

Evaluation
The typical auscultatory finding is a late-systolic click and a murmur due to late systolic or holosystolic regurgitation. Elongation and thickening of valve leaflets, degree of mitral regurgitation and LV dimension and function should be assessed by echocardiography. Evaluation should include exercise testing and/or Holter monitoring to assess the presence of arrhythmias. Yearly cardiologic evaluation is recommended, because the regurgitation can get worse by progressive degeneration of the leaflets.

Recommendations
See Table 3.

Prophylaxis against endocarditis
Infective endocarditis (IE) is an endovascular, microbial infection of intracardiac structures facing the blood, including infections of the large intrathoracic vessels. The early lesion is a vegetation of variable size, although destruction, ulceration, or abscess may follow. The increasing accuracy of echocardiography and therapeutic progress has contributed to the prognostic improvement in the last few years.

Patients with previous history of infective endocarditis, patients with prosthetic heart valves or acquired valve disease are considered high-risk patients and should receive antibiotic prophylaxis when exposed to risk of bacteraemia in accordance with the ESC recommendations.27 Prophylaxis should be performed before dental, oral, respiratory, oesophageal, gastrointestinal, and genitourinary procedures. Dental hygiene is of relevance for prevention of IE.

As a general rule, all sports activity should be avoided when active infection with fever is present. Resumption of sport activity can be considered when the inflammatory process is completely extinguished, and systematic maintenance of endocarditis prophylaxis must be strictly observed.27

Recommendations for participation in competitive sports in athletes with cardiomyopathies, myocarditis, and pericarditis

Hypertrophic cardiomyopathy
Hypertrophic cardiomyopathy (HCM) is a primary cardiac disease, with hypertrophied and non-dilated left ventricle, in the absence of cardiac or systemic disease capable of producing LV hypertrophy of that magnitude.28 Sports participation increases the risk for SCD in HCM patients,28,29 and this disease is the most common cause of athletic field death in young athletes in the USA.29

Evaluation
Evaluation of athletes with suspected HCM includes personal and family history, physical examination, 12-lead ECG, and echocardiography.

12-Lead ECG
The majority (75–95%) of HCM patients show abnormal ECG patterns, commonly including markedly increased R- or S-wave voltages, deep and prolonged Q-waves, and deeply inverted T-waves.30 ECG abnormalities may precede the LV hypertrophy and should raise suspicion of the disease in family members of HCM patients. However, trained athletes without evident CV disease may occasionally show similar ECG abnormalities31 (see also Isolated abnormal ECGs).

Echocardiography
Classically, HCM is diagnosed when LV wall thickness is ≥13 mm, but a more substantial LV wall thickening may be found, usually with asymmetric distribution and sharp transition between contiguous segments.28 LV hypertrophy becomes evident during adolescence, in association with body growth,28 but in a few individuals, it may develop in midlife or beyond. LV end-diastolic cavity dimension is normal or even reduced, with an abnormal and sometimes bizarre shape. Diastolic LV filling (by Doppler-echocardiography)32 and tissue doppler imaging (TDI) are abnormal in the majority of HCM patients and may precede the development of LV hypertrophy. Other alterations include malformation of the mitral valve, with elongation of the leaflets, or anomalous insertion of papillary muscles.28

In contrast, distribution of LV hypertrophy is symmetric in athletes and maximum LV wall thickness does not exceed 15–16 mm.33 The LV cavity is enlarged (i.e. end-diastolic diameter ≥55 mm) with a normal shape, a normally positioned mitral valve, and no outflow tract obstruction.33 LV filling (by Doppler-echocardiography)32 and relaxation (by TDI) are normal. Most importantly, serial echocardiographic studies demonstrate reduction in LV wall thickness after complete deconditioning.34

Family screening is mandatory in borderline cases, and identification of the disease in a family member is diagnostic for HCM. Additional criteria include peak oxygen consumption (with VO2max>50 mL/kg/min being more consistent with athlete's heart35) and gender, because women athletes do not usually show LV wall thickening >12 mm.36

MRI is indicated, when echocardiography is inadequate, in identifying an atypical pattern of hypertrophy or apical HCM.

Molecular genetics
A variety of mutations of genes encoding structural and regulatory proteins of the cardiac sarcomere cause familial HCM.37,38 However, genetic testing is still not available in the current clinical practice because of the substantial genetic heterogeneity of the disease, and the complex, time consuming and expensive techniques needed.

Recommendations
See Table 4.


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Table 4 Recommendation for competitive sport participation in athletes with cardiomyopathies, myocarditis, and pericarditis
 
Isolated abnormal ECGs
Special attention should be paid to athletes with ECG abnormalities (such as markedly increased QRS voltage, diffuse T-wave inversion, deep Q-waves in precordial leads) suggestive for HCM, in the absence of familial incidence of HCM and in the absence of LV hypertrophy. Evaluation of these athletes should include complete family screening, personal history, echocardiography, and 24 h Holter ECG monitoring. When SCD or HCM in the family are excluded, and in the absence of symptoms, arrhythmias and LV hypertrophy, and with a normal diastolic filling/relaxation, there is no reason for restricting athletes from competitive sports, but periodical clinical and diagnostic follow-up is recommended.

Dilated cardiomyopathy
Dilated cardiomyopathy (DCM) is a myocardial disease characterized by LV dilatation and impaired systolic function. DCM includes disorders which are familial or genetic in origin, or secondary to infection or inflammation, exposure to toxic substances, metabolic disorders, or of idiopathic origin.39 Although not frequent, DCM represents a cause of SCD in athletes.

Evaluation
The assessment of athletes with suspected DCM includes personal and family history, physical examination, 12-lead ECG with exercise testing, echocardiography, and 24 h Holter monitoring.

Exercise testing and 24 h Holter monitoring
In young DCM patients, exercise performance may be only mildly impaired and arrhythmias are present at a very early stage of the disease, including supraventricular and ventricular tachyarrhythmias, as well as major conduction delays.

Echocardiography
The LV cavity is enlarged with respect to the LV walls, which are normal or only mildly thickened. LV shape becomes more spherical; the mitral annulus eventually enlarges with distortion of leaflets and resultant valvular regurgitation.39 Most importantly, LV systolic function is diminished (with ejection fraction <50%), segmental wall motion abnormalities may be present, and stroke volume is usually reduced.

In contrast, the physiologic LV enlargement present in athletes (mostly engaged in aerobic disciplines such as cycling, cross-country skiing, rowing, long-distance running40) is characterized by a normal systolic function, no segmental wall motion abnormalities, and normal diastolic filling and relaxation (by Doppler-echocardiography and TDI). In case of borderline ejection fraction (i.e. ≥50, <60%), it may be useful to test LV function during exercise (by echocardiography or radionuclide imaging). Absence of significant improvement of systolic function at peak exercise is in favour of a pathological dilatation.

Recommendations
See Table 4.

Arrhythmogenic RV cardiomyopathy/dysplasia
Arrhythmogenic RV cardiomyopathy/dysplasia (ARVC) is a primary myocardial disease characterized histologically by fibro-fatty replacement of the RV myocardium, and clinically by life-threatening ventricular tachyarrhythmias in young individuals.41 Sudden death may occur in young ARVC individuals in association with exercise, and this disease represents the most common cause of SCD in young athletes in Italy.42

Evaluation
Diagnosis of ARVC is based on the criteria previously proposed by an expert consensus panel.41

   12-Lead ECG
In clinical practice, the ECG is of particular value in raising suspicion for ARVC, in consideration that ECG abnormalities are present in >50% of ARVC patients. The most common abnormalities include prolonged QRS duration >110 ms (with RBBB pattern) and inverted T-waves in the right precordial leads, evidence of epsilon wave and either isolated PVCs or VT (typically with LBBB pattern and vertical axis).

   Echocardiography
In ARVC patients, echocardiography (or MRI) shows an enlarged RV cavity, segmental morphologic abnormalities (with thinning, bulging and aneurysms in the RV wall), and wall motion abnormalities. MRI may identify areas of altered signal intensity consistent with fibro-fatty replacement. Cine-MRI may be decisive for assessing wall motion anomalies. An enlarged right ventricle, in association with an enlarged left ventricle, may also be found in elite athletes (mostly engaged in endurance disciplines, such as cycling, rowing/canoeing),40 but in these instances, RV wall thickness is normal and no segmental wall motion abnormalities are present.

Recommendations
See Table 4.

Myocarditis
Myocarditis is defined as an inflammatory process of the myocardium, with histological evidence of myocyte degeneration and necrosis of non-ischaemic origin, associated with inflammatory infiltration.43

Evaluation
The assessment of athletes with suspected myocarditis includes medical history, physical examination, 12-lead ECG, and echocardiography. Additional testing may be required according to the specific case.

   History
The clinical picture usually starts with upper respiratory or gastrointestinal symptoms, but palpitations, fatigability, exertional dyspnoea, or syncope may be the clinical onset. Evidence of flu-like illness, or epidemiological circumstances supporting viral infection should be assessed.

   12-Lead ECG
The ECG abnormalities include frequent and/or complex ventricular and/or supraventricular arrhythmia, ST-segment alteration (usually depression; rarely elevation), T-wave inversion and, occasionally, LBBB or AV blocks.44

   Echocardiography
Global LV enlargement and dysfunction can be evident in certain cases;45 however, localized wall motion abnormalities (usually in the apex), mildly enlarged LV cavity, and borderline systolic dysfunction are common. Modest pericardial effusion may be present, associated with increased reflectivity of pericardial leaflets.

   Histology
Biopsy is not usually performed in the routine diagnostic course and may be reserved for selected circumstances, when needed for therapeutic or legal purposes.

Recommendations
See Table 4.

Pericarditis
Pericarditis is defined as an inflammatory process of the pericardium, which may also affect the subepicardial layers of the myocardium.

Evaluation
The assessment of athletes with suspected pericarditis includes medical history, physical examination, 12-lead ECG, and echocardiography.

   History
Pericarditis usually starts with upper respiratory or gastrointestinal symptoms, but clinical presentation may include chest pain, increased fatigability, or exertional dyspnoea. The onset of the disease may also be concealed, and the clinical course characterized by only transient fever, without significant cardiac symptoms.

   12-Lead ECG
In patients with pericarditis, a spectrum of ECG abnormalities may be present, including most commonly ST–T wave alterations mimicking ischaemic heart disease (IHD) and ventricular or supraventricular tachyarrhythmias.

   Echocardiography
Often a pericardial effusion is present at the onset of the disease, with increased reflectivity and separation of the pericardial leaflets.

Recommendations
See Table 4.

Recommendations for sports participation in patients with Marfan's syndrome (MFS)
MFS is an autosomal dominant connective tissue disorder affecting 1:5000 subjects.46 MFS is caused by fibrillin 1 gene defects (FBN1) and by mutation of transforming growth factor beta receptor 2 (TGFßR2) in a minority of patients (OMIN #154705). More than 600 mutations have been detected to date, the majority private.47 Penetrance is complete, but the involvement of different organs/tissues is variable, with large phenotypic heterogeneity. The classical phenotype includes osteo-skeletal, CV, ocular, skin, pulmonary, and nervous anomalies. The primary cause of mortality in young people and competitive athletes is aortic root dilatation, dissection, and rupture.48

Evaluation
The evaluation of an athlete with suspected MFS includes family and personal history, physical examination, echocardiography, and genetic screening.4951 Clinical diagnosis of MS is based on the Ghent criteria,52,53 namely the combination of two major criteria plus the involvement of a third organ/system.

Particular care should be paid in evaluating tall children and adolescents engaged in certain sports such as basketball and volleyball. The hypermobility of their joints, as well as their stature and osteo-skeletal aptitude are favouring pre-requisites. Once they are involved in a successful sports activity, the finding of aortic root dilatation requiring to interrupt athletic activity may raise severe psychological problems.

Recommendations
See Table 5.


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Table 5 Recommendations for competitive sport participation in athletes with MS
 
In examining patients with MFS, the physician (and consultant cardiologist) should be committed to avoid the risk of aortic dissection and to protect the aortic root from accelerated dilation. The following suggestions may help cardiologists in their recommendations:
  1. In children, offspring of patients with MFS, who present a major skeletal phenotype, an integrated educational activity can be proposed to parents, in order to dissuade children from pre-competitive commitments and direct their hobbies or physical training towards non-competitive and moderate intensity activities.
  2. In youths committed to pre-competitive activity:
    1. in case of positive family history, uncertain phenotype but positive FBN1 mutation: competetive sports participation should be strongly discouraged, and interests should be re-addressed to non-risky activities;
    2. in case of positive family history, uncertain phenotype and negative FBN1 mutation: youths may continue their activity but should undergo periodical CV monitoring;
    3. in case of negative family history (~30% of MFS is caused by de novo FBN1 or TGFßR2 mutations), unknown genotype, uncertain phenotype (especially in youths), the decision may prove particularly difficult. If clinical suspicion is strong, even in the absence of completion of the Ghent criteria, the cardiologist should be cautious and discourage the candidate from competitive sports participation.

  3. In young competitive athletes, when Ghent criteria are reached, the cardiologist should strongly discourage any competitive sports activity.
In patients with MFS, a low-intensity, leisure physical activity could be appropriate for the osteo-skeletal problems. However, these patients should avoid contact sports (Table 1), because of the risk of damaging the aorta and injuring the eyes. It is also wise to avoid strenuous physical activities to prevent any increase in aortic wall stress. Patients with MFS in the absence of aortic dilation but with MVP can perform leisure, non-contact sports with moderate intensity, such as running, cycling, swimming, and tennis (see also MVP). Patients with MFS and mechanical heart valves under anticoagulant therapy have an increased risk of haemorrhages and regular monitoring of the anticoagulation is mandatory.

Recommendations for participation in competitive sports in athletes with systemic hypertension
Hypertension is defined as systolic BP ≥140 mmHg and/or diastolic BP ≥90 mmHg, measured by conventional techniques in the seated subject according to established guidelines.5457 Isolated systolic hypertension corresponds to an elevated systolic BP with normal diastolic BP. Subjects with elevated BP in the clinic and normal out-of-office BP have white-coat or isolated clinical hypertension. The current threshold for an elevated 24 h ambulatory BP is 125/80 mmHg; the threshold for daytime ambulatory BP and home BP is 135/85 mmHg.57

Risk stratification
The severity of hypertension does not only depend on the BP level, but also on the presence of other CV risk factors, target organ damage, and CV and renal complications, i.e. the overall CV risk.54,55 The current risk stratification is based on the presence of selected risk factors, target organ damage, and/or of associated clinical conditions, as outlined in Table 6. The terms low, moderate, high, and very high added risk, in comparison with healthy normotensive subjects without risk factors, are calibrated to indicate an approximate absolute 10-year risk of CV disease of <15, 15–20, 20–30, and >30%, respectively, according to the Framingham criteria, or an approximate absolute risk of fatal CV disease of <4, 4–5, 6–8, and >8% according to the European SCORE system.56


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Table 6 Stratification of risk to quantify prognosis in patients with systemic hypertension
 
With regard to LV hypertrophy, it should be noted that sports activity itself may induce hypertrophy; the pattern of hypertrophy and assessment of diastolic LV function may help to distinguish between hypertensive heart disease and athlete's heart.58,59

Evaluation
Diagnostic procedures comprise repeated BP measurements according to established guidelines,5457 medical history, physical examination, laboratory, and instrumental investigations, of which some should be considered part of the routine approach in all subjects with high BP, and some are recommended in the hypertensive athlete. For instance, echocardiography and exercise testing (with ECG and BP monitoring), which are not always included in the evaluation of the hypertensive patient, are warranted as routine tests in the hypertensive athlete. Additional tests, such as stress echocardiography/myocardial scintigraphy and/or 24 h Holter ECG monitoring, may be recommended depending on the patient's symptoms, CV risk profile, associated clinical conditions, and the results of the first set of investigations.

Recommendations
General recommendations
Athletes with hypertension should be treated according to the general guidelines for the management of hypertension.54,55 Appropriate non-pharmacological measures should be considered in all patients. Antihypertensive drug therapy should be started promptly in patients at high or very high added risk for CV complications (Table 6). In patients at moderate added risk, drug treatment is only initiated when hypertension persists after several months despite appropriate lifestyle changes. Drug treatment is not considered mandatory in patients at low added risk. The goal of antihypertensive therapy is to reduce BP to at least <140/90 mmHg and to lower values if tolerated in all hypertensive patients, and to <130/80 mmHg in diabetics. Current evidence indicates that patients with white-coat hypertension do not have to be treated with antihypertensive drugs, unless they are at high or very high risk (Table 6), but a regular follow-up and non-pharmacological measures are recommended.60

Choice of drugs
Several drug classes can be considered for first-line antihypertensive therapy: diuretics; beta-blockers; calcium channel blockers; angiotensin converting enzyme (ACE)-inhibitors, and angiotensin II receptor blockers.54,55 However, in endurance athletes, diuretics and beta-blockers are not recommended because they may impair exercise performance and capacity and/or cause electrolyte and fluid disturbances.61,62 In addition, they are on the doping list for some sports, in which weight loss or control of tremor are of paramount importance. Calcium channel blockers and blockers of the renin–angiotensin system are the drugs of choice for the hypertensive endurance athlete63 and may be combined in case of insufficient BP control. However, the combination of an ACE inhibitor and an angiotensin II receptor blocker is currently not advocated. If a third drug is required, a low-dose thiazide-like diuretic, possibly in combination with a potassium sparing agent, is recommended. There is no unequivocal evidence that antihypertensive agents would impair performance in static sports.

Recommendations for sports participation
See Table 7.


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Table 7 Recommendations for competitive sport participation in athletes with systemic hypertension (and other risk factors) according to the CV risk profile
 
Recommendations for participation in competitive sports in athletes with hypertension are based on the risk stratification and with the understanding that the general recommendations for the management of hypertension are observed as described earlier, and the clinical condition is stable. In patients with secondary hypertension, clinical and diagnostic evaluation for sports participation is postponed to after the removal of the cause of hypertension, if possible. Patients with polycystic kidney disease or with CoA should avoid sports with danger of bodily collision.

Recommendations for participation in competitive sports in athletes with ischaemic heart disease (IHD)
IHD accounts for most exercise-related SCD, especially in individuals >35 years of age.64 The risk for triggering coronary events increases transiently during vigorous physical activity due to several mechanisms, including sympathetic drive and release of catecholamines, platelet adhesion/activation65 (with risk of thrombotic complications), electrolyte disturbances such as an increased potassium level (trigger for ventricular tachyarrhythmias), and heart-related complications (such as sub-endocardial ischaemia and necrosis).66

In younger athletes (<35 years), congenital CV abnormalities are more frequently implicated. Non-coronary anomalies may also result in acute ischaemic episodes in athletes. Abuse of certain drugs, such as cocaine, as well as risk factors for IHD, such as obesity and diabetes, may trigger myocardial ischaemia and SCD.6769 Physical inactivity is also considered a major risk factor for IHD, whereas regular physical training reduces the risk of SCD during vigorous exertion.70

The benefits of regular physical activity and sports participation are believed to outweigh the increased risk for coronary events triggered by acute, intensive exercise. However, physical activity and sports participation should be individually tailored in patients with IHD.

Athletes with evidence of IHD
These include athletes with unstable angina, stable angina, after coronary artery bypass grafting/percutaneous coronary intervention (CABG/PCI), after myocardial infarction (MI) or with silent ischaemia.

Unstable angina is here defined as recent onset angina, progressive angina (by frequency, intensity, and duration), angina at rest, or angina developing in close relation to an MI.

Stable angina is defined as angina (chest discomfort or related symptoms) experienced during/after physical exercise, temperature changes, an emotional period, a meal, or hyperdynamic circulation (such as occurs in anaemia, fever, hyperthyroidism). For a given patient, stable angina occurs with repeated progressive exercise at approximately the same rate-pressure product (i.e. ischaemia threshold).

Silent ischaemia is defined by unequivocal evidence of ischaemia on stress testing or Holter monitoring, but without clinically evident symptoms. In these patients, coronary angiography shows evidence of coronary atherosclerosis.

Evaluation
The candidate athlete with IHD should be systematically evaluated under the following criteria:

Risk stratification
On the basis of the results of diagnostic testing the risk may be stratified71 as follows.

Specific comments

Recommendations
See Table 8.


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Table 8 Recommendations for competitive sport participation in athletes with IHD
 
Athletes without evidence of IHD, but with one or more risk factors for IHD
In asymptomatic subjects without evidence of IHD, but in the presence of known risk factors, assessment of the risk profile is needed. The risk for IHD can be estimated from the presence of major risk factors including age, sex, BP, smoking, and total cholesterol level, according to the SCORE-system72 or as outlined in Table 6.

The high-risk profile for developing a fatal CV event is defined by:

The low-risk profile is defined as a 10-year risk for a fatal CV event <5%, according to the SCORE-chart, due to the absence of major risks factors.

Evaluation
Athletes with a high-risk profile should be further evaluated to rule out silent ischaemia, by history, physical examination and ECG with maximal exercise testing. In case of: negative exercise testing, the absolute risk of a major cardiac event during physical activity is considered to be low in these asymptomatic patients without evidence of IHD. For positive exercise testing, the risk for future coronary events in these individuals, although asymptomatic, is increased. They need further evaluation by stress-echocardiography/myocardial scintigraphy and/or coronary angiography to assess the presence of (even silent) IHD. Finally, athletes with evidence of ischaemia should be stratified as athletes with IHD.

Exercise testing is not routinely recommended in healthy asymptomatic athletes without classical risk factors and age <35 years for men and <45 years for women.

Recommendations
See Table 8.

Recommendations for participation in competitive sports in athletes with arrhythmias and potentially arrhythmogenic conditions
General considerations
Cardiac arrhythmias may occur not only in association with several CV abnormalities, such as genetic ionic channel diseases, anomalies of the conducting system, or structural heart diseases, but also without evidence of a morphologic substrate. The main prognostic determinant to be assessed in athletes with arrhythmias is the presence of heart disease.48,7375

The evaluation of athletes with arrhythmias, either documented or suspected, includes personal history, searching for any hints of substance abuse such as smoking, alcohol, drugs, or doping. Risk factors for IHD should be investigated particularly in adult/senior athletes. The history should explore previous CV disease and identify symptoms such as palpitations, pre-syncope or syncope, unexplained weakness, chest pain, or dyspnoea. A thorough family history should search for SCD (especially in youth and adulthood) and/or potentially arrhythmogenic conditions. The initial evaluation also includes physical examination and ECG, exercise testing, 24 h Holter monitoring, and echocardiography. In some cases, it is advisable to obtain a blood count, thyroid function markers, and electrolyte balance. When initial testing fails to demonstrate the arrhythmia, the external event recorder or loop recorder can be eventually considered; finally, an electrophysiologic study is indicated when the arrhythmia is paroxysmal and/or associated with haemodynamic impairment.

Sinus bradycardia
Asymptomatic sinus bradycardia, sinus bradyarrhythmia, wandering pacemaker (PM), and sinus pauses are common in young athletes.76 A number of studies suggest that these arrhythmias in athletes are the consequence of increased vagal tone and withdrawal of sympathetic tone. Occasionally, marked sinus bradycardia (i.e. ≤40 b.p.m.) at rest, or sinus pauses ≥3 s can be found in asymptomatic, well-trained endurance athletes. These arrhythmias are usually benign, and evaluation may be limited to history, physical examination, and ECG. Treatment is usually not necessary.4

If marked bradycardia is associated with symptoms, such as lightheadedness, pre-syncope/syncope (see also section of syncope), or exertional fatigue, 24 h Holter monitoring and exercise testing are recommended. If structural heart disease is suspected, echocardiography (or another imaging technique) is mandatory. In selected cases, a deconditioning period of 1–2 months could be useful to clarify the clinical significance of extreme bradyarrhythmias.

Recommendations
See Table 9.


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Table 9 Recommendations for competitive sport participation in athletes with arrhythmias and arrhythmogenic conditions
 
Atrioventricular blocks
In athletes, the prevalence of first-degree atrioventricular (AV) block or second-degree AV block of the Wenckebach type (Mobitz type I) is high.77,78 The AV block typically occurs during sleep or at rest. In asymptomatic athletes without structural heart disease (as assessed by echocardiography) and with resolution of the AV block during exercise (as assessed by 24 h Holter monitoring and/or exercise testing), no further investigations and no therapy are indicated.

Rarely, Mobitz type II or third-degree AV block may be observed in athletes, which require a more comprehensive clinical and diagnostic evaluation. If these findings are associated with symptoms or with structural heart disease, PM implantation is recommended.

Recommendations
See Table 9.

Supraventricular premature beats and tachycardia
Supraventricular arrhythmias may be associated with symptoms, such as palpitations, fatigue, chest discomfort or may present with dyspnoea, lightheadedness, or syncope. The patient should be encouraged to have an ECG taken during the arrhythmia, which may clarify the diagnosis. The 24 h Holter monitoring is indicated in these instances. An electrophysiologic study is indicated in patients with paroxysmal palpitations if catheter ablation may represent a therapeutic option for resolution of the arrhythmia.79

Supraventricular premature beats
Premature atrial beats are a common finding in many individuals including athletes.76,78 A careful history, physical examination, and ECG should be performed. In the absence of structural heart disease and thyroid dysfunction, with no or only mild symptoms (such as occasional palpitations), no further evaluation or therapy is required.

   Recommendation
See Table 9.

Paroxysmal supraventricular tachycardia
Paroxysmal supraventricular tachycardia may be caused by AV nodal re-entrant tachycardia (AVNRT), orthodromic AV re-entrant tachycardia (AVRT) due to an accessory pathway, or an ectopic atrial tachycardia. AVNRT is the most common form of supraventricular reciprocating tachycardia in the general population, including athletes.80 Although typically occurring in the third and fourth decade of life with a preference for women, this paroxysmal arrhythmia may be observed at any age. The arrhythmia is caused by a re-entry mechanism involving the AV node, perinodal atrium, and slow and fast pathways (which connect the AV node to the atrium). Adequate evaluation of the athlete with AVNRT includes a history with emphasis on the characteristics of the arrhythmia onset, and symptoms during tachycardia. An electrophysiologic study aimed to define the re-entry mechanism is recommended.

The catheter ablation has become the preferred therapy, especially in athletes, because antiarrhythmic drug therapy is a lifelong treatment with limited efficacy. Ablation of the slow AV nodal pathway is the elective procedure, which can be performed with success rates of>95%, and a complication rate (permanent AV block) of <1–2% in experienced centres. In selected cases with an increased risk for complete AV nodal block (i.e. para-His accessory pathway), cryo-ablation can be an alternative approach.

AVRT over a concealed accessory pathway is based on a typical electrophysiologic mechanism, i.e. the accessory pathway is able to conduct only in the retrograde way (concealed pathway). Therefore, there is no evidence of the accessory pathway on the standard ECG during sinus rhythm. Also, for the AVRT over the concealed pathway, catheter ablation is the elective therapy.

   Recommendations
See Table 9.

Ventricular pre-excitation (Wolff–Parkinson–White Syndrome)
Wolff–Parkinson–White syndrome (WPW) is defined as the presence of paroxysmal arrhythmias in a patient with overt ventricular pre-excitation. Prevalence of pre-excitation in the general population varies from 0.1 to 0.3% and it is not different in the athletic population.81,82 The tachyarrhythmias related to WPW syndrome include AV re-entry tachycardia (either orthodromic or antidromic), AF and, rarely, ventricular fibrillation.

Evaluation of the athlete with ventricular pre-excitation includes history, physical examination, ECG, and echocardiography (to exclude an associated structural cardiac disease, such as HCM or Ebstein anomaly).

WPW and paroxysmal AV re-entry tachycardia
The most common form of paroxysmal supraventricular tachycardia in the WPW syndrome is the orthodromic tachycardia with a narrow QRS complex. This AVRT involves the node-Hissian pathway anterogradely and the accessory pathway retrogradely. Occasionally, an orthodromic tachycardia may have anterograde conduction over the bypass tract and a wide QRS tachycardia is documented.

Athletes with WPW syndrome and documented symptomatic paroxysmal AV re-entry tachycardia should be preferentially treated with radiofrequency ablation of the accessory pathway.

WPW and AF or flutter
It has been estimated that one third of patients with WPW syndrome may develop AF. AF or atrial flutter in the presence of ventricular pre-excitation may result in a rapid activation of the ventricles through the accessory pathway which may degenerate into ventricular fibrillation and SCD. The risk of SCD in WPW patients/athletes varies in population-based studies from 0.15 to 0.2%, whereas in symptomatic WPW patients, the risk appears to be higher (2.2%).80 The incidence of SCD is higher in patients/athletes with an accessory pathway characterized by short refractoriness and is triggered by an episode of AF/flutter. Therefore, symptomatic patients/athletes with ventricular pre-excitation and AF or flutter should undergo catheter ablation.

Asymptomatic pre-excitation on ECG
It is generally considered that asymptomatic athletes with pre-excitation on 12-lead ECG, in the absence of structural heart disease, have a low but definite risk of SCD. Assessment of the risk profile in these athletes is based on an electrophysiologic study and includes measurement of refractoriness of the accessory pathway and induction of AF to assess the shortest pre-excitated RR interval. A short pre-excitated RR interval (currently used criteria are <240 ms during induced AF and <220 ms during effort or isoproterenol infusion), the presence of multiple accessory pathways, or even easy induction of AF83 are considered to be associated with an increased risk of SCD.

At present, given the high success rate and low incidence of complications, catheter ablation of the accessory pathway has become the first choice treatment for (even asymptomatic) athletes with pre-excitation. Therefore, these athletes should be fully advised of this preferential therapeutic option and decision should be made on an individual basis.

For those athletes who refuse the ablation, or when the procedure is associated with a high risk, competitive sports activities may nevertheless be allowed if the electrophysiologic study shows absence of risk criteria (as specified earlier) and, specifically, a short refractoriness at baseline and during effort, or isoproterenol infusion. In all other instances, catheter ablation should be performed.

To be noticed, in children <12 years of age, the risk of AF and/or SCD is considered virtual, and assessment of the risk may be postponed.

   Recommendations
See Table 9.

Atrial fibrillation
The prevalence of AF in competitive athletes is not well known, although is supposed to be higher than in the general population. In ~40% of athletes with AF, a possible substrate, such as WPW syndrome, cardiomyopathy, or silent myocarditis can be found.82,84 Use of doping substances, such as anabolic steroids, can also possibly cause AF in athletes.85

Pulmonary vein catheter ablation is not yet established as a routine procedure in focal AF, due to its limited long-term success rate (50–80%) and considerable complications (pulmonary vein stenosis, tamponade, peri-procedural stroke in 3–10%). In athletes with unsuccessful rhythm control or in athletes under rate-control therapy, anticoagulation may be necessary, depending also on the presence of risk factor for thrombo-embolic events.

Recommendations
See Table 9.

For athletes with AF and ventricular pre-excitation: see ventricular pre-excitation. Anticoagulant therapy excludes individuals from sports with a risk of bodily collision or trauma (see Table 1).

Atrial flutter
Atrial flutter is uncommon in the young healthy population. The electrophysiologic substrate for atrial flutter of the common type is a counter-clockwise re-entrant circuit around the tricuspid valve. In athletes with atrial flutter, the presence of structural heart disease, such as cardiomyopathy, should be excluded, because it is often the basis of this arrhythmia. Atrial flutter may convey an increased thrombo-embolic risk and may be life-threatening due to potential 1:1 conduction to the ventricles.

Catheter ablation of the isthmus is a highly effective and safe procedure86 and is recommended as first-line therapy in athletes. Anticoagulation therapy in atrial flutter follows the same recommendations as in AF. In the presence of combined atrial flutter and fibrillation, isthmus ablation is recommended, followed by drug therapy for AF (‘hybrid therapy’).

Recommendations
See Table 9.

Athletes with structural heart disease and atrial flutter can participate in competitive sports consistent with the limitation of the disease, only after successful catheter ablation and in the absence of recurrence of arrhythmia for >3 months. For athletes with atrial flutter and ventricular pre-excitation: see ventricular pre-excitation. Athletes who require anticoagulation therapy should not participate in sports with danger of bodily collision or trauma (Table 1).

Premature ventricular beats
Premature ventricular beats (PVBs) are a frequent finding in the athletic population.76,78 The main factor for determining prognosis and recommendation for sports participation is the presence of heart disease.87 Unfortunately, large randomized clinical trials in athletes with PVBs are uncommon. However, available studies suggest that PVBs in the absence of CV abnormalities are not associated with an increased risk of malignant ventricular arrhythmias and convey a good outcome.88 However, PVBs may also be the initial and unique manifestation of clinically silent arrhythmogenic conditions at risk of SCD (such as ARVC, HCM, myocarditis); therefore, athletes with PVBs require careful evaluation including history, physical examination, echocardiography, exercise testing, and 24 h Holter monitoring. The family history is relevant for excluding the presence of juvenile SCD or familial arrhythmogenic cardiac conditions, as well as the presence of symptoms, such as palpitations or syncope, particularly during exertion. In athletes with suspected heart disease, further testing (such as MRI, coronaro-angiography, endomyocardial biopsy) may be required in the individual case to rule out underlying disease and establish appropriate management. In selected cases, re-evaluation after 3–6 months of deconditioning may be useful.89

Recommendations
See Table 9.

Non-sustained ventricular tachycardia
Non-sustained ventricular tachycardia (NSVT), defined as ventricular tachycardia of three or more consecutive beats ≥100 b.p.m. and lasting <30 s, is an uncommon arrhythmia in healthy subjects. NSVT requires extensive clinical assessment, including echocardiography (or other imaging tests), exercise testing, and 24 h Holter monitoring to rule out underlying disease and to evaluate the mechanism of the arrhythmia. Further tests, such as an electrophysiologic study and other invasive testing, should be individually based according to the suspected cardiac lesion. Particular attention should be paid to identify those athletes with polymorphic/bi-directional NSVT which is triggered by exercise or occurs during exercise testing (‘catecholaminergic ventricular tachycardia’) which carries a high risk of SCD.

Recommendations
See Table 9.

Slow ventricular tachycardia (idio-ventricular accelerated rhythm)
This is a focal automatic ventricular rhythm with a heart rate <100 b.p.m., due to enhanced ventricular automation, which is favoured by bradycardia. Echocardiography, exercise testing, and 24 h Holter monitoring are indicated for clinical evaluation.

Recommendations
See Table 9.

Benign idiopathic ventricular tachycardia: fascicular ventricular tachycardia and RV outflow tachycardia
Fascicular ventricular tachycardia90 and automatic RV outflow tachycardia, also known as ‘RV outflow tract’ (RVOT) ventricular tachycardia91 are distinct entities which are not usually associated with heart disease, are haemodynamically well tolerated, and have a benign prognosis. Both VTs are usually induced by physical exercise. Fascicular VT originates from the distal ramifications of the left posterior bundle in the inferior part of the interventricular septum, is characteristically paroxysmal and presents with right bundle branch block QRS morphology and superior axis. RVOT originates from an automatic focus in the RVOT, may be either paroxysmal or repetitive (so called ‘repetitive monomorphic VT’) and presents with left bundle branch block QRS morphology and inferior axis. Clinical assessment including echocardiography, exercise testing, and 24 h Holter monitoring is recommended to exclude the presence of heart disease and to analyse the VT characteristics, because overlapping forms between idiopathic RVOT–VT and ARVD have been described. An electrophysiologic study may be necessary for the differential diagnosis from VT due to ARVC (see Cardiomyopathies) and supraventricular tachycardia with aberrant conduction.

Catheter ablation of the substrate is a reasonable therapeutic option for the two VTs, with high rate of success and few complications, and the athlete should be fully advised on this therapeutic option. When catheter ablation is refused or not possible, the risk must be stratified according to the presence of heart disease, the RR interval of the VT and the presence of symptoms, such as dizziness, pre-syncope and syncope.

Recommendations
See Table 9.

Malignant ventricular tachycardia
Malignant VTs include sustained ventricular tachycardia, polymorphic ventricular tachycardia, torsades de pointes, and ventricular fibrillation. These VTs are associated with haemodynamic deterioration and may lead to cardiac arrest. IHD is the most common cause of malignant VT in adult individuals; whereas in young people, a spectrum of pathologic conditions, including HCM, ARVC, and congenital coronary anomalies, have been reported. Affected individuals need a thorough clinical assessment and therapeutic options for SCD prevention, such as implantable cardioverter defibrillator (ICD). In athletes with documented malignant VT, competitive sports are contraindicated. A possible exception is ventricular arrhythmias occurring in the context of acute and transient myocardial lesions, such as myocarditis, commotio cordis, and acute electrolytic depletion, when the cause has proven to be completely resolved.

Symptoms of possible arrhythmic origin: syncope
Syncope is characterized by sudden and momentary loss of consciousness and postural tone, due to abrupt reduction of global brain blood flow, with spontaneous and complete recovery within a short time.92 Syncope may be neurocardiogenic (vasovagal, sino-carotid, environmental, or situational), orthostatic, or of cardiac origin (either arrhythmic or structural), or due to primary brain blood flow dysfunction. In young athletes, syncope is most often neurocardiogenic and seems to have a fair prognosis.92,93

Evaluation of athletes with syncope should differentiate true syncope from other conditions causing loss of consciousness (epilepsy, transitory ischaemic attack, drop attack, hypoglycaemia), and assess the presence of cardiac disease. Initial evaluation includes history (aimed at a clear description of the syncope), physical examination with BP measurement in the recumbent and standing positions, and ECG. The 24 h Holter monitoring is necessary in case of suspicion of an arrhythmic cause of the syncope; exercise testing is indicated if syncope is related to exercise, as well as in subjects with suspected IHD.92 The head-up tilt test is needed to confirm the neurocardiogenic origin of the syncope (although this test has lower specificity in athletes compared with the general population93). An electrophysiologic study is indicated when syncope is associated with palpitations as the likely expression of paroxysmal tachycardia.

Recommendations
See Table 9.

In athletes with neurocardiogenic syncope, the restriction from sports with intrinsic risk (Table 1) is dictated by the awareness that any transient loss of consciousness will convey adverse effects for the athlete and the people nearby. In athletes with syncope of arrhythmic or mechanic origin, recommendation will be granted depending on the type of arrhythmia and/or on the associated abnormal CV condition.

Arrhythmogenic disorders: ion channel disease
Sudden arrhythmic death in the athlete may be caused by inherited cardiac ion channel defects (channelopathies) which include the long QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia.

Long QT syndrome
Long QT interval is defined as heart rate corrected QT interval (according to the Bazett's formula) measured in lead II exceeding 440 ms in males and 460 ms in females.94 Congenital long QT syndrome has been associated with genetically defective cardiac K+ and Na+ channels which result in prolongation of ventricular repolarization and predispose to torsade de pointes and ventricular fibrillation. To exclude potential causes of acquired long QT interval, therefore, electrolyte depletion (i.e. hypokalaemia) or chronic intake of drugs capable to prolong repolarization (such as certain antibiotics, antihistaminics, etc.) should be first investigated. Athletes with borderline QT lengthening should be evaluated with exercise testing and 24 h Holter monitoring. Genetic testing is mandatory when definitive diagnosis for genotype-related risk stratification and therapy is required. Congenital long QT syndrome is a contraindication for any type of sports, even without documented major arrhythmic events.

Brugada syndrome
The Brugada syndrome is a genetic condition characterized by a peculiar ECG pattern in the anterior precordial leads V1–V3, i.e. ST-segment elevation >2 mm, with ‘coved type’ (either spontaneous or induced by pharmacological sodium channel blockade) in association with related arrhythmic events (syncope, cardiac arrest).95 A cardiac Na+ channel gene (SCN5A) mutation has been detected in up to 30% of cases. This syndrome is associated with risk of SCD due to malignant ventricular arrhythmias (sustained VT, VF), which usually occur at rest and often at night, as a consequence of increased vagal stimulation and/or withdrawal of sympathetic activity. Increased vagal tone as a consequence of chronic athletic conditioning may eventually enhance the propensity of athletes with Brugada syndrome to die at rest, during sleep, or during the recovery after exercise. Therefore, although no relation between exercise and arrhythmias has been found, subjects with definite diagnosis of Brugada syndrome should be restricted from competitive sports. Whether healthy genetic carriers of the Brugada syndrome without phenotypic expression should be restricted from sports participation is at present uncertain.

Catecholaminergic ventricular tachycardia
Catecholaminergic ventricular tachycardia is characterized by exercise-induced polymorphic ventricular tachycardia (most often with ‘bi-directional pattern’) which can degenerate in ventricular fibrillation. The disease has been linked to mutations of the ryanodine receptor and calcequestrin genes leading to abnormal calcium release from the sarcoplasmic reticulum. Unlike long QT syndrome and Brugada syndrome, this condition is not associated with abnormalities of the basal ECG and remains unrecognized unless the athlete undergoes exercise testing.

Catheter ablation in athletes
Catheter ablation of focal and re-entry tachyarrhythmias has become a therapeutic strategy with high success rate (>95%) and minimum risk of side effects (<1%).96,97 Lethal complications are uncommon (<1%) and usually occur when ablation is required in the left heart. The occurrence of AV blocks is rare (<1%), and limited to ablation of AV nodal re-entry tachycardia and anteroseptal accessory pathways.

In athletes, indications for catheter ablation differ from those in the general population, because the procedure is not only aimed to eliminate disabling symptoms, but also to allow resumption of competitive sports activity. When cardiac abnormalities which are per se responsible for non-eligibility are excluded, catheter ablation is recommended in athletes with the following conditions:

  1. WPW syndrome, either symptomatic or asymptomatic, with electrophysiological evidence of short anterograde refractoriness of the AV accessory pathway;
  2. supraventricular re-entry tachycardia, either paroxysmal (frequent and sustained episodes with heart rate faster than maximum heart rate by age) or incessant and iterative (with the exception of episodes with slow heart rate);
  3. typical atrial flutter, either common or non-common;
  4. symptomatic fascicular ventricular tachycardia or RV outflow ventricular tachycardia.
Three months after successful catheter ablation, patients can resume competitive sports, provided that the ECG shows no signs of ventricular pre-excitation in case of WPW and that they are asymptomatic and without recurrence of tachycardia. Repeated electrophysiologic assessment may be required in selected cases when the outcome of the procedure is considered uncertain.

Patients with PM
Patients with heart disease and PM implantation can participate only in sports consistent with the limitations of the arrhythmia and the underlying heart disease. Athletes with a PM and no signs of heart disease will be allowed to participate in competitive sports with only minor CV demand, provided that exercise testing and 24 h Holter monitoring show an appropriate increase in the paced heart rate during exercise, and no occurrence of significant arrhythmias. However, subjects with PM should be restricted from sports with a risk of body impact, because of the possible disruption of the electro-catheters and damage to the pacing unit. Furthermore, the possible risk of electromagnetic interferences should be closely evaluated.

Recommendations
See Table 9.

Patients with ICD
Most patients with ICD have a cardiac disease which represents per se a contraindication for competitive sports. Indeed, the efficacy of the ICD to interrupt malignant ventricular arrhythmias during exercise remains to be established. Because ICD patients may benefit from low-intensity and supervised exercise programmes,98 it seems wise that individuals with ICD and no evidence of structural heart disease (or with mild morphologic abnormalities) and preserved cardiac function can be allowed to participate in sports with only low dynamic or static demand, which do not pose a risk of trauma to the device, and do not specifically trigger malignant VTs (such as torsade de pointes in congenital long QT syndrome and polymorphic catecholaminergic ventricular tachycardia).

Sports participation can be allowed at least 6 months after the ICD implantation, or after the most recent arrhythmic episode requiring defibrillator intervention (including pacing, antitachycardia pacing, or shock). Furthermore, to reduce the risk of inappropriate shocks related to sinus tachycardia induced by exercise, the cut-off heart rate for the ICD needs to be appropriately set by exercise testing and 24 h Holter monitoring.

Recommendations
See Table 9.

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