Challenges of Exercise Recommendations and Sports Participation in Genetic Heart Disease Patients
2015; Lippincott Williams & Wilkins; Volume: 8; Issue: 1 Linguagem: Inglês
10.1161/circgenetics.114.000784
ISSN1942-325X
AutoresJ. Sweeting, Jodie Ingles, Kylie Ball, Christopher Semsarian,
Tópico(s)Cardiac pacing and defibrillation studies
ResumoHomeCirculation: Cardiovascular GeneticsVol. 8, No. 1Challenges of Exercise Recommendations and Sports Participation in Genetic Heart Disease Patients Free AccessResearch ArticlePDF/EPUBAboutView PDFSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessResearch ArticlePDF/EPUBChallenges of Exercise Recommendations and Sports Participation in Genetic Heart Disease Patients Joanna Sweeting, Jodie Ingles, Kylie Ball and Christopher Semsarian Joanna SweetingJoanna Sweeting From the Agnes Ginges Centre for Molecular Cardiology, Centenary Institute, Newtown, NSW, Australia (J.S., J.I., C.S.); Sydney Medical School, University of Sydney, Sydney, NSW, Australia (J.S., J.I., C.S.); Centre for Physical Activity and Nutrition Research, Deakin University, Melbourne, Australia (K.B.); and Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia (C.S.). , Jodie InglesJodie Ingles From the Agnes Ginges Centre for Molecular Cardiology, Centenary Institute, Newtown, NSW, Australia (J.S., J.I., C.S.); Sydney Medical School, University of Sydney, Sydney, NSW, Australia (J.S., J.I., C.S.); Centre for Physical Activity and Nutrition Research, Deakin University, Melbourne, Australia (K.B.); and Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia (C.S.). , Kylie BallKylie Ball From the Agnes Ginges Centre for Molecular Cardiology, Centenary Institute, Newtown, NSW, Australia (J.S., J.I., C.S.); Sydney Medical School, University of Sydney, Sydney, NSW, Australia (J.S., J.I., C.S.); Centre for Physical Activity and Nutrition Research, Deakin University, Melbourne, Australia (K.B.); and Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia (C.S.). and Christopher SemsarianChristopher Semsarian From the Agnes Ginges Centre for Molecular Cardiology, Centenary Institute, Newtown, NSW, Australia (J.S., J.I., C.S.); Sydney Medical School, University of Sydney, Sydney, NSW, Australia (J.S., J.I., C.S.); Centre for Physical Activity and Nutrition Research, Deakin University, Melbourne, Australia (K.B.); and Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia (C.S.). Originally published1 Feb 2015https://doi.org/10.1161/CIRCGENETICS.114.000784Circulation: Cardiovascular Genetics. 2015;8:178–186IntroductionPhysical activity has been shown to have many health benefits in the primary and secondary prevention of noncommunicable chronic diseases, including cardiovascular disease, diabetes mellitus, and certain cancers.1–3 Physical activity also has a positive effect on psychological well-being, reducing the risk and severity of depression and anxiety and improving stress levels and mood.4,5 Physical activity includes activities undertaken as part of occupational and household duties, or for transport, as well as sporting and other recreational activities.6 Exercise is a major component of physical activity, distinct in that it is generally planned, structured, repetitive and has an objective of improved physical fitness and health.6 With physical activity providing so many health benefits, it follows that physical inactivity should be a major concern for health professionals and the population as a whole. Physical inactivity is the fourth leading cause of death worldwide and has been identified as a pandemic requiring global attention.7 In addition, physical inactivity has been deemed responsible for nearly 10% of premature deaths worldwide.8Although exercise is beneficial for all age groups for both healthy people and those predisposed to chronic medical conditions, such as coronary artery disease, the role of exercise in the setting of patients with genetic heart diseases is more complex. A well described association between high-intensity exercise and sudden cardiac death (SCD) has been established,9,10 and this has historically led to blanket sport and exercise restrictions for any patient meeting diagnostic criteria for a genetic heart disease. The increased understanding of the beneficial effects of even low intensity exercise has called in to question whether current exercise and sports restrictions are too strict, and more importantly whether we are doing enough to actively encourage patients to undertake low to moderate intensity exercise. Negotiating a balance between avoiding high-intensity exercise and at the same time encouraging low-to-moderate intensity exercise for the purpose of general health is a real challenge for clinicians when discussing lifestyle advice with patients. This review focuses on the current issues related to exercise recommendations in genetic heart disease patients, with an emphasis on current recommendations, specific subgroups of patients, such as gene carriers or those who have an implantable cardioverter-defibrillator (ICD), and how this information can be integrated to provide the best possible advice about exercise in the setting of genetic heart diseases.Genetic Heart DiseasesExercise plays an important role in the management of all patients with genetic heart diseases. Genetic heart diseases include the inherited cardiomyopathies, such as hypertrophic cardiomyopathy (HCM) and arrhythmogenic right ventricular cardiomyopathy (ARVC), and primary arrhythmogenic disorders, including long QT syndrome (LQTS), Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia. Over 90% of genetic heart diseases are inherited in an autosomal dominant fashion,11 and all are characterized by both genetic and clinical heterogeneity. Over 100 genes have been implicated in various genetic heart diseases,12 and there is vast diversity of clinical outcomes, from asymptomatic to severe complications, including heart failure and SCD.Although the benefits of exercise are numerous at both an individual and a population level, individuals with genetic heart diseases are discouraged from participating in high-intensity, vigorous activities, including competitive sports, for fear of triggering sudden death events as a resut of the underlying arrhythmogenic nature of these diseases.8,13Table 1 provides examples of activities and their intensity, as well as the corresponding metabolic equivalent value (METS). The recommendation to avoid high-intensity activity (METS >6) and competitive sports is often a difficult issue for many patients. The unique and complex issues that genetic heart disease patients must face have been previously described17,18 and often relate to individuals being diagnosed in adolescence and early adulthood, many of whom have minimal cardiac limiting symptoms, though must come to terms with having a potentially life-threatening heart condition. In this setting, advising a young person that they cannot continue to engage in high-intensity or competitive sports, even though they may not have any functional limitations, can be a sensitive discussion point. Although difficult at first, most patients will adapt their lifestyle accordingly, though there are a small number who ignore the advice and continue to engage in high-intensity exercise and competitive sports, accepting the small but significant risk of an SCD event. The bigger issue, however, is whether the patients will become overly cautious and restrict themselves from undertaking low to moderate intensity exercise. Finding a balance and conveying this to the patient is a real challenge, and currently there is limited data regarding exactly how this patient group interpret and respond to these recommendations.Table 1. Recommendations for HCM and LQTS for a Selection of Exercise ActivitiesSportIntensity (METS)RecommendationHCMLQTSHorse riding (walk)Low (3.2)Assess on individual basisAssess on individual basisBrisk walking (5 km/h)Low (3.2)Probably permittedProbably permittedSnorkeling (light)Low (4)Probably permittedNot advisedGolf (pulling cart)Low (3–4)Probably permittedProbably permittedCycling (10–15 km/h)Moderate (4.8–5.9)Probably permittedProbably permittedJogging/Walking (7 km/h)Moderate (5.3)Assess on individual basisAssess on individual basisSwimming (laps)Moderate (4.3)Probably permittedNot advisedFree weightsModerate (3–7)Not advised*Not advised*SoccerHigh (10.3)Not advisedNot advisedSquashHigh (8–12)Not advisedAssess on individual basisIce hockeyHigh (12.9)Not advised*Not advised*Skiing (cross-country)High (7.7+)Assess on individual basisAssess on individual basisICD patient-specific recommendations No contact sports Restriction from sports involving ipsilateral arm movements, for example, swimming and tennis14Adapted from Maron et al.15 Authorization for this adaptation has been obtained both from the owner of the copyright in the original work and from the owner of copyright in the translation or adaptation.METS values from Jette et al.16Low intensity, 6 METS.HCM indicates hypertrophic cardiomyopathy; ICD, implantable cardioverter-defibrillator; LQTS, long QT syndrome; and METS, metabolic equivalent value.*Involves potential for traumatic injury, especially during times of impaired consciousness, for example, because of ICD shock.Exercise as a Trigger for Sudden Cardiac DeathAlthough exercise is associated with many health benefits, for those with genetic heart diseases, it may predispose to an increased risk of SCD or cardiac arrest.13 Specifically, high-intensity and competitive exercise can trigger malignant ventricular arrhythmias, leading to cardiac arrest and SCD among at-risk individuals. There is data from around the world that reports on the issue of SCD during exercise, particularly in athletes. A 21-year prospective study from Italy showed that participation in sports was associated with an increased risk of SCD in people aged 12 to 35 years,19 and the incidence was over 2-fold higher in athletes compared with nonathletes, with ARVC being the most common cause.20 Data from a large study in the United States identified a total of 1866 athletes over a period of 27 years who died suddenly (or were resuscitated) during exercise, equating to an incidence of 0.47 s in male subjects>0.44 s in male subjects>0.48 s in female subjects>0.46 s in female subjectsLow-intensity competitive sportsOnly recreational sportsPremature ventricular complexesAll competitive sports when no increase in PVCs or symptoms occur with exerciseAll competitive sports when no increase in PVCs, couplets, or symptoms occur with exerciseNonsustained ventricular tachycardiaIf no CV disease, all competitive sportsIf no CV disease, all competitive sportsIf CV disease, only low-intensity competitive sportsIf CV disease, only recreational sportsACC indicates American College of Cardiology; ARVC, arrhythmogenic right ventricular cardiomyopathy; CV, cardiovascular; DCM, dilated cardiomyopathy; ESC, European Society of Cardiology; HCM, hypertrophic cardiomyopathy; LQTS, long-QT syndrome; and PVC, premature ventricular complex.Modified from Pelliccia et al.36*Long-QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia.The ACC/AHA recommendations state, "at present, it is unresolved as to whether genotype-positive, phenotype-negative individuals warrant any restrictions from either recreational or competitive sports."15 In their 36th Bethesda recommendations for competitive athletes, it is maintained that although a resolution has not yet been achieved, no compelling data exists, which would preclude G+P− patients from either recreational or competitive sports.37 In contrast, the ESC recommends the same restrictions for G+P− individuals (referred to as silent gene carriers) as for clinically affected individuals. The ESC bases this recommendation on the fact that the natural history of G+P− individuals is at present relatively unknown and that regular exercise training could lead to alterations in heart structure similar to the HCM phenotype.36Because the ACC/AHA and ESC recommendations were released in 2004 and 2006, respectively, further studies have added to the pool of knowledge regarding G+P− individuals and exercise. A study by Gray et al34 followed a cohort of 32 G+P− HCM patients for a mean period of 4 years, and in this time-period, no G+P− individuals aged over 18 years developed clinical HCM or any adverse outcomes during the follow-up period. Maron et al presented a further 4 HCM families in which the challenges of decision-making related to exercise recommendations among G+P− HCM individuals were presented and discussed.33 A more recent study examined a cohort of 353 patients with LQTS of which 130 continued to participate in competitive sports (above the recommended intensity) after diagnosis. Of these 130 patients, 70 were identified as G+P− patients and, as such, were participating in competitive sports against the recommendations of the ESC but in line with the ACC/AHA Bethesda recommendations.38,39 No adverse events were observed in the follow-up period of ≤10 years. Collectively, these studies provide some evidence that G+P− adults may have a relatively benign clinical course, and elite-level sports participation should be permitted in some cases. These studies highlight the need for larger studies in G+P− individuals with various genetic heart diseases to evaluate the effect of high-intensity exercise and elite-level sports participation on clinical outcomes.Exercise Recommendations for Patients With Implantable Cardioverter-Defibrillator DevicesAlthough ICD therapy is established as a life-saving device in selected genetic heart disease patients at highest risk of SCD,40 how this therapy affects exercise recommendations regarding what patients can and cannot do remains less clear. In terms of exercise recommendations, the presence of an ICD creates an additional layer of complexity for several reasons. First, both arrhythmias and associated ICD shocks may lead to a temporary loss of consciousness which, depending on the situation, may be dangerous not only for the individual, for example, while swimming, but those around them, for example, while driving a car. Second, the effectiveness of ICD shocks under the conditions of exercise is less well studied.41 Third, there is the potential for lead displacement and damage to the ICD through direct force.27 Finally and importantly, exercise may increase the likelihood of inappropriate ICD shocks because of sinus tachycardia, other supraventricular arrhythmias, T-wave oversensing, or noise caused by lead failure, which may lead to significant subsequent psychological distress.27,42 Taking into account all these considerations, it is not surprising that the current exercise recommendations for those with an ICD are restrictive.The specific and important subgroup of genetic heart disease patients with an ICD is highlighted in both the ACC/AHA and ESC recommendations. The recommendations regarding exercise are significantly more restrictive because of the potential additional risk of bodily trauma, resulting in damage to the ICD or lead placement or an inappropriate shock. Furthermore, specific recommendations are made regarding involvement in sports with potential for body contact with restriction to low intensity noncontact sports recommended.14,15 The ESC also includes a recommendation regarding activities with ipsilateral arm movements, such as tennis and swimming, which are considered higher risk because of potential for lead dislocation or fracture with avoidance suggested.14As with all patients, the risks of playing sports with an ICD in place need to be balanced with the potential health gains and general psychological well-being of the patient. Interestingly, Heidbuchel and Carre27 proposed that although the current recommendations do not allow for intensive sports participation for ICD patients, recent studies suggest that more leniency may be considered in some competitive athletes and is often possible in those who want to perform low- to moderate-intensity recreational activities.27An important recent study used a prospective ICD registry and followed a cohort of 372 athletes with ICDs participating in organized or high-risk sports, such as basketball and running.43 Although a total of 77 individuals recorded 121 shock episodes, there were no occurrences of tachyarrhythmic death, externally resuscitated tachyarrhythmia during or after sports participation or severe injury as a result of syncope, or shocks during sports. The study found that more shocks did occur during sports than at other times. However, it was also observed that the majority of athletes who experienced an ICD shock during sports participation chose to continue playing. The authors suggest that the benefits of sport participation for these individuals outweighed the unpleasant experience of an ICD shock, and that although ICD shocks can decrease quality of life, so can sports restriction. The study suggests that some patients with ICDs may be able to safely engage in more vigorous competitive sports.43Once again, the multifactorial nature of the clinical setting needs to be considered with the benefits and dangers of high-intensity exercise, the underlying risk of SCD, whether the patient is an adult or a child, and the psychosocial well-being of the patient all significant factors. McDonough44 examined a sample of young (18- to 40-year-old) patients and found that some individuals experienced anxiety during exercise because of the potential for an ICD shock.44 Interestingly, some individuals chose to reduce their level of exercise, despite physician advice permitting participation, because of fear of an ICD shock. Other studies have reported similar results, reporting that a fear of exercise is common among ICD patients and that this fear negatively influences quality of life.45 Further, interventions aimed at addressing this have shown mixed results and mostly use a cardiac rehabilitation-type program to demonstrate to the patient their ability to do high-intensity exercise in a safe environment.46,47 These studies highlight the importance of how we negotiate the most effective ways to communicate the risks and benefits of exercise to this patient group, so that they can safely gain quality of life improvements.44,45The effect of functional capacity and general fitness on adjustment and anxiety levels of ICD patients has also been examined. In a sample of ARVC patients with an ICD, a reasonably good level of functional capacity (ie, ability to participate in activities, such as daily living, sports, recreational activities) was observed overall.48 The study found that higher functional capacity, represented by a higher score on the Duke Activity Status Index, was a significant predictor of better device adjustment in these ICD patients, and higher anxiety scores were associated with reduced functional capacity.48 As the Duke Activity Status Index score correlates with peak oxygen uptake and therefore fitness levels,48 this study raises the possibility that a higher level of fitness aids adjustment for patients with an ICD.Other Adverse Exercise–Induced Cardiovascular EffectsIntense exercise, such as prolonged participation in endurance sports, including ultramarathons, triathlons, and cycling, has been shown to have potential to trigger adverse cardiovascular effects.40,49–54 In some situations, particularly in endurance athletes, chronic and sustained exercise can cause patchy myocardial fibrosis and other cellular changes creating a substrate for atrial and ventricular arrhythmias, as well as coronary artery calcification, diastolic dysfunction, and large artery wall stiffening.54 Although these adverse effects were observed in endurance athletes not known to have a genetic heart disease, the intense repetitive stress for sustained periods has potential for adverse cardiac effects. The most pertinent example is found in ARVC.ARVC is a structural genetic heart disease of particular interest when considering exercise. ARVC primarily affects the right ventricle, and it is characterized by myocardial atrophy and fibro-fatty replacement of
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