EDITORIAL COMMENT
Athlete Screening for Occult Cardiac DiseaseNo Risk, No Fun?*
Lothar Faber, MD, FESC, FACC* and
Frank van Buuren, MD
Department of Cardiology, Heart and Diabetes Center North-Rhine Westphalia, Ruhr-University Bochum, Bad Oeynhausen, Germany.
* Reprint requests and correspondence: Dr. Lothar Faber, Department of Cardiology, Heart and Diabetes Center NRW, Ruhr-University Bochum, Georgstr. 11, D-32545 Bad Oeynhausen, Germany. (Email: lfaber{at}hdz-nrw.de).
Regular physical exercise promotes health and longevity. Young, well-conditioned competing athletes are believed to be one of the healthiest segments of society and a brilliant example of the beneficial effects of sport. Therefore, sudden cardiac death (SCD) in such persons is not only devastating to all directly involved—the athlete him- or herself who is unexpectedly converted into a victim, as well as his or her family, team, and caregivers—such a death is also disturbing to the public and regularly draws a great deal of media attention.
The current hypothesis for nontraumatic SCD in young athletes is that a fatal arrhythmia may be triggered by vigorous exercise in those who have certain occult cardiac disorders that do not necessarily interfere with exercise tolerance. Hypertrophic cardiomyopathy (HCM) has been described as the leading abnormality predisposing to SCD in young athletes in the U.S. (1). Dilated forms of cardiomyopathy are more frequent in other countries (1–3).
Sudden cardiac death is believed to be uncommon, with estimates of approximately 0.5 to 2.0 per 100,000 person-years. The true incidence, however, is unknown and may be higher for several reasons. Pathologists carrying out postmortem examinations may be inexperienced with all the cardiac conditions potentially responsible; some conditions may thus remain undiagnosed. Retrospective data from referral centers regarding the cause of SCD in athletes may be subject to bias depending on the centers’ particular expertise; comprehensive prospective data are scarce. Finally, conditions such as cardiac ion channel diseases may predispose to fatal arrhythmias but do not present as structural abnormalities.
Although a consensus has been achieved that athletes with known and significant cardiovascular abnormalities should be disqualified from competition (4–6), there is controversy over whether to actively screen for these abnormalities. Attitudes vary considerably, from no screening at all to obligatory nationwide pre-participation screening, and include both nonprofit and for-profit initiatives. Some apparently robust data concerning the problem of preventing SCD in young athletes derive from the Italian approach (2,3). This strategy consists of obligatory pre-participation screening including history, physical examination, and electrocardiogram (ECG) and proceeds to cardiac imaging in the case of abnormalities (reported in roughly 10% of participants). One of the major publications on this approach (2) reported 22 cases diagnosed with HCM in 34,000 probands screened (0.07%). Sudden cardiac death from HCM has been described as virtually absent in Italian sports venues, whereas SCD from cardiac diseases less obvious on ECG and echocardiography continued to occur, but at a significantly reduced rate. An overall disqualification rate of 2% has been reported (2,3).
The authors of the present study in this issue of the Journal (7) performed 12-channel ECG and echocardiography in 3,500 British athletes, mainly Caucasian, competing in endurance disciplines on a national level. A total of 53 probands exhibited a left ventricular wall thickness >12 mm, 50 of whom also manifested cavity dilatation and a normal transmitral flow pattern (8) suggestive of adaptive left ventricular hypertrophy (LVH). The remaining 3 probands, all of whom were also suspected of having adaptive LVH, were intensively studied (cardiac magnetic resonance imaging and genotyping) or detrained. Interestingly, 2 of these 3 probands were of African descent. No definitive diagnosis of HCM could be made. The overall yield of screening was an "electrical" diagnosis (Wolff-Parkinson-White syndrome or long-QT syndrome) in 15 participants (0.4%) and a "structural" cardiac abnormality (valvular or congenital heart disease or LVH) in 64 participants (1.8%). The authors concluded that routine echo screening of high-level athletes for the presence of HCM is not cost-effective and that intensive sporting activity naturally selects out HCM.
Overall, the data presented are welcome because they broaden the global experience on athlete screening for occult cardiac abnormalities, which were found in about 2% of subjects. On the other hand, the 0 incidence of HCM in this cohort does not really come as a surprise when compared with the Italian database, which was 10 times larger. Furthermore, all 3 cases initially suggestive of HCM would have undergone imaging anyway if examined within the Italian program because of their abnormal ECG tracings. The conclusion that echocardiographic screening is not necessary if only HCM is targeted, and that the ECG is reliable enough to pick up cases suggestive of pathological LVH, thus appears to be justified and is consistent with the Italian data. The other conclusion, that HCM is extremely uncommon in elite athletes, should be taken with caution. As the authors state in their limitations section, results may be different for non-Caucasians and for those whose sport disciplines are less dependent on cardiorespiratory endurance.
In addition, when judging the overall value of echocardiographic screening in young athletes, structural abnormalities other than HCM should also be considered. In the present study, 11 cases (0.3%) of unsuspected congenital or valvular heart disease were found, although of a mild degree and obviously not interfering with exercise tolerance. Such a finding is good to know, especially for an elite athlete. Furthermore, a negative pre-participation echo at entry, especially when archived with current digital technology, may be extremely helpful if symptoms or other health problems come up later. This indeed happens, as demonstrated by the authors of the present study, who described an experienced marathon runner who dropped dead and was found to have myocardial fibrosis on autopsy (9) or by those professional cyclists who apparently suffered cardiac damage during their careers (10).
There are some other specific aspects that deserve to be kept in mind. First, a multitude of interests and aspects may interfere with the proband/patient-physician relationship, sometimes putting into question the reliability of history data as provided by the probands. Doping affairs occur almost every other week, obviously involving some physicians and biomedical scientists. These affairs indicate that an unknown percentage of athletes and their caregivers are willing to run the risk of health damage, including premature death, in return for success, fame, and money. In addition, in contrast to the field of "normal" medicine with its increasingly restrained resources and cost-effectiveness calculations, professional sport is a growing multibillion-dollar business; screening costs appear to be absolutely negligible in this context. Even the amateur marathon runner can be reassured that the price of a screening echocardiogram is probably less than that of the latest running shoes. Societies with a public health care system might also take into account that the yearly costs of care for an occasional persistent vegetative state after a "successful" resuscitation in a victim of SCD may easily surpass thousands of screening echocardiograms. Finally, in this usually easy-to-scan population, a structured echo examination may be less time-consuming than a careful physical examination; modern echocardiography, including remote expert reading and parametric imaging, may provide objective and quantitative information on global and regional myocardial function beyond diameters and ejection fraction (11).
Our personal and institutional bias, therefore, is to routinely screen professional athletes, including performing echocardiography, in close cooperation with their teams and national sports organizations and to follow up these athletes during their careers. For nonprofessionals and nonelite athletes, we recommend, as a minimum, clinical and ECG screening as proposed in the European consensus reports (5,6). We strongly believe that the heart and circulatory system of the competing athlete deserve the same level of awareness and attention that has been directed toward the musculoskeletal system for decades. On the other hand, as demonstrated by the first marathon runner, Pheidippides, as early as 490 BC, it has to be recognized that complete risk elimination is not possible.
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Footnotes
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* Editorials published in the Journal of the American College of Cardiology reflect the views of the authors and do not necessarily represent the views of JACC or the American College of Cardiology. 
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References
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1. Maron BJ, Pellicia A. The heart of trained athletes: cardiac remodeling and the risks of sports, including sudden death Circulation 2006;114:1633-1644.[Free Full Text]2. Corrado D, Basso C, Schiavon M, Thiene G. Screening for hypertrophic cardiomyopathy in young athletes N Engl J Med 1998;339:364-369.[CrossRef][Web of Science][Medline] 3. Corrado D, Basso C, Pavei A, Michieli P, Schiavon M, Thiene G. Trends in sudden cardiovascular death in young competitive athletes after implementation of a preparticipation screening program JAMA 2006;296:1593-1601.[Abstract/Free Full Text] 4. Maron BJ, Zipes DP. 36th Bethesda conference: eligibility recommendations for competitive athletes with cardiovascular abnormalities J Am Coll Cardiol 2005;45:1312-1375.[Free Full Text] 5. Pelliccia A, Fagard R, Bjørnstad HH, et al. Recommendations for competitive sports participation in athletes with cardiovascular disease Eur Heart J 2005;14:1422-1445. 6. Corrado D, Pelliccia A, Bjørnstad HH, et al. Cardiovascular pre-participation screening of young competitive athletes for prevention of sudden death: proposal for a common European protocol Eur Heart J 2005;5:516-524. 7. Basavarajaiah S, Wilson M, Whyte G, Shah A, McKenna W, Sharma S. Prevalence of hypertrophic cardiomyopathy in highly trained athletes: relevance to pre-participation screening J Am Coll Cardiol 2008;51:1033-1039.[Abstract/Free Full Text] 8. Sharma S, Maron BJ, Whyte G, Firoozi S, Elliott PM, McKenna WJ. Physiologic limits of left ventricular hypertrophy in elite junior athletes: relevance to differential diagnosis of athlete’s heart and hypertrophic cardiomyopathy J Am Coll Cardiol 2002;40:1431-1436.[Abstract/Free Full Text] 9. Whyte GP, Sheppard M, George K, et al. Post-mortem evidence of idiopathic left ventricular hypertrophy and idiopathic interstitial myocardial fibrosis: is exercise the cause? Br J Sports Med 2008(Epub ahead of print). 10. Heidbüchel H, Hoogsteen J, Fagard R, et al. High prevalence of right ventricular involvement in endurance athletes with ventricular arrhythmias: role of an electrophysiologic study in risk stratification Eur Heart J 2003;24:1473-1480.[Abstract/Free Full Text] 11. D’Andrea A, D’Andrea L, Caso P, Scherillo M, Zeppili P, Calabro R. The usefulness of doppler myocardial imaging in the study of the athlete’s heart and in the differential diagnosis between physiological and pathological ventricular hypertrophy Echocardiography 2006;23:149-157.[CrossRef][Medline]
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