- Email: [email protected]
Sports-related sudden cardiac death: How to prove an effect of preparticipation screening?
Author’s Accepted Manuscript Sports-Related Sudden Cardiac Death: How to Prove an Effect of Pre-Participation Screening? Bjarke Risgaard, Jacob Tfelt-Hansen, Bo Gregers Winkel www.elsevier.com/locate/buildenv
PII: DOI: Reference:
S1547-5271(16)30087-X http://dx.doi.org/10.1016/j.hrthm.2016.03.043 HRTHM6674
To appear in: Heart Rhythm Received date: 11 January 2016 Cite this article as: Bjarke Risgaard, Jacob Tfelt-Hansen and Bo Gregers Winkel, Sports-Related Sudden Cardiac Death: How to Prove an Effect of PreParticipation Screening?, Heart Rhythm, http://dx.doi.org/10.1016/j.hrthm.2016.03.043 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Sports-related sudden cardiac death: How to prove an effect of pre-participation screening?
Bjarke Risgaard, MD, PhD; Jacob Tfelt-Hansen, MD, DMSc; Bo Gregers Winkel, MD, PhD Affiliations: The Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark;
Author contributions: Bjarke Risgaard designed and drafted the manuscript. Jacob Tfelt-Hansen and Bo Gregers Winkel designed and revised the manuscript critically.
Conflicts of interest: None to declare
Running Title: Sports-related sudden cardiac death
Word count: 1452
Corresponding author: Corresponding author: Bjarke Risgaard, MD, PHD. Address for correspondence: The Heart Centre, Copenhagen University Hospital, Rigshospitalet, Afnist 2143, Blegdamsvej 9, 2100 Copenhagen Ø. Tel: (+45) 35 45 35 45 Email: [email protected]
1
Viewpoint: Sports-related sudden cardiac deaths (SrSCD) are tragic events that occur in individuals who are believed to be in good health prior to death. These deaths are highly visible in the public health debate due to significant media attention. The reported annual incidence rate of SrSCD remains low at 0.93 and 0.70 per 100,000 participants in Minnesota high school athletes1,2; 0.87 in the Veneto region3; and 0.61 for the entire United States4. However, recently published data from the United States suggest that the incidence rate of SCD in athletes in the National Collegiate Athletic Association is higher (1.86 per 100,000 athlete-years)5. Whether we should implement mandatory pre-participation screening programs to lower the burden of SrSCD among competitive athletes is hotly debated. Pre-participation screening programs have been mandated in several places. In the United States, screening consists of a history and a physical examination, whereas in Italy and Israel, the pre-participation screening programs additionally include a 12-lead electrocardiogram (ECG)1,6–8. Data that favor pre-participation screening mainly come from the Veneto region in Italy3. Corrado and colleagues3 showed a declining incidence rate of SrSCD from 4.19 (1979-1981) to 0.87 (19932004) per 100,000 athletes following the implementation of these programs in Italy. Today, the incidence rate of SrSCD in Italy is lower than the incidence rate of SCD in the general population3. Several other studies, however, have challenged these findings by suggesting that pre-participation screening is of limited value2,4,9–12. The American Heart Association argues against routine ECG screening of all competitive athletes and highlights the many obstacles they may introduce 13–15. The low specificity and sensitivity of an ECG for detecting the diseases in question, together with the associated economic challenges, has led to a discussion of how best to bridge current knowledge with practice16. In addition, it has been argued that mandatory pre-participation screening of young competitive athletes also may introduce certain ethical dilemmas6,17. Despite the level of evidence, pre-participation screening has been implemented on very different levels throughout the world, and it has been used most extensively in Italy and Israel3,9. In Denmark, the Danish Society of Cardiology has decided to not recommend mandatory screening of competitive athletes, which makes the country suitable for investigating the incidence rate of SrSCD in a pre-screening era18. Among competitive athletes 12-35 years of age, we previously reported incidence rates of SrSCD of 1.21 (95% CI 0.73-2.01) (2000-2006) and 0.47 (95% CI 0.101.14)(2007-2009) per 100,000 athlete person-years11,10 corresponding to 1-2 cases of SrSCD per 2
year in Denmark in the 12-35-year age group. Young non-competitive athletes had a similarly risk to that of competitive athletes (incidence rates of 0.43 vs. 0.47), and competitive athletes 36-49 years of age had a much higher risk than competitive athletes 12-35 years of age (incidence rates of 6.64 vs. 0.47 per 100,000 athlete person-years)10. Most importantly, we have shown that despite the lack of mandatory screening programs in Denmark, the incidence rate of SrSCD is nearly 3 times lower than that of SCD in the general population19–21. As described above, the current literature highlights uncertainty with regard to the efficacy and cost-effectiveness of screening and also questions whether these screening programs should include ECGs. Importantly, no solid evidence from large randomized trials has been published due to low event rates and ethical considerations. Hence, the question remains whether we can show that screening all competitive athletes is beneficial with regard to reducing mortality. Is a gold standard prospective randomized controlled trial feasible? The calculation for a randomized controlled trial would be as follows. The highest incidence rate of SrSCD among competitive athletes in the 12-35-year age group in Denmark (where screening is not performed routinely) was 1.21 per 100,000 person-years. Theoretically, this means that among 100 athletes followed for one year, we would expect 0.00121 (0.00121%) cases of SrSCD. The lowest incidence rate of SrSCD in a screened population (without ECG) is reported to be 0.61 per 100,000 person-years4. This corresponds to 0.00061 (0.00061%) deaths among 100 athletes followed for one year, and if we assume that we will never eliminate all cases of SrSCD, this may be regarded as the lowest possible incidence rate. As the incidence rates are low, so are the absolute numbers. In Denmark, Italy and within the state of Minnesota, the numbers of SCD among the young has been estimated to be 1-2 deaths, 2 deaths and <1 death per year, respectively1,10,11. According to an internet-based sample size calculator, the numbers of individuals required, assuming an 80% chance of detecting a change in the endpoint, is nearly 8 million athletes22. However, using the incidence rate of SCD in the pre-screening era in Italy (4.19/100,000), the number of athletes required would be reduced (588,066 athletes). Nevertheless, due to the large numbers of athletes, it is very doubtful that such a study will be performed. Another question is whether the screening of young competitive athletes with or without ECGs fulfills the general criteria for a screening program. In 1968, the World Health Organization published a guideline with 10 criteria, referred to as the Wilson criteria, that outline the principles of screening (table 1)23. In 2009, a National Screening Committee in the UK published new criteria for
3
appraising the viability, effectiveness and appropriateness of a screening program24. The key message was that screening should be performed only if the disease in question is an important health problem. Furthermore, the epidemiology should be adequately understood and should have an identifiable risk factor. The screening test should be simple, safe, precise, and validated. In addition, it should be acceptable to the population. Finally, there should be an available intervention or treatment. The UK National Screening Committee24 also argues that the evidence for the entire program should come from high-quality randomized controlled trials that address efficacy in reducing mortality. There should be evidence that the complete screening program is ethical and acceptable and that the beneficial effects outweigh any physical or psychological harm. It is emphasized that the costs should be economical and that all other options for managing the disease should have been considered24. In our opinion, most of the criteria described above for implementing a screening program are not met with regards to SrSCD among young athletes. First, we do not believe that SrSCD constitutes a significant public health issue, as we have shown that SrSCD is rare even in an unscreened population, while SCD is much more common in the general population10,11. Second, although screening with ECG may be acceptable to the public (no adverse effects), sensitivity, specificity and interobserver variability are poor25. Third, the high numbers of false positive and false negative results, as well as the negative effects of sports restriction (both psychological and health), should be considered. Fourth, screening that is restricted to a subset of individuals with the lowest risk (competitive athletes age 12-35 years) is questionable from an ethical perspective. Fifth, costs of targeted screening with ECG have been shown to be high compared with the potential beneficial effect26. Finally, sixth, no randomized clinical controlled trial has been or is likely to be performed. Thus, overall, we believe that pre-participation screening of young athletes is questionable, and we particularly believe that the additional use of ECG in its current form is not justified. Other options for managing SrSCD should be considered. Athletes and coaches should be thoroughly educated with regard to which symptoms warrant a clinical evaluation. Cardiac symptoms prior to SCD or SrSCD are common, and identification of these patients may be an alternative approach to identifying patients at risk10,21,27,28. As secondary prevention, automated external defibrillators (AEDs) should be widely distributed and accessible in strategic areas29 to increase the chance of survival in cases of out-of-hospital cardiac arrest. Education of the general population in bystander
4
cardiopulmonary resuscitation should be performed and continued. This has proven very beneficial in Denmark, where survival rates have increased30. Several other governmental and private health initiatives have also been implemented over the past 2 decades in Denmark to address the prevention of sudden death. These programs have included large populations instead of targeting only those engaged in competitive sports. As a consequence, for example, prevention of suicide and motor vehicle accidents has been associated with saving 220 lives per year in young people aged 15-25 years31, whereas only 1-2 athletes die each year10,11. Pre-participation screening programs are currently in place to various extents in many countries. To eliminate established programs seems virtually impossible. It is our belief that other strategies should be considered to prevent SrSCD and to reduce the burden of SCD in the general population. Based on the Danish experience and the Wilson criteria, we believe that pre-participation screening programs with or without ECGs should not currently be recommended.
Tables Table 1. The key principles of screening according to the Wilson criteria*. Principles of screening 1) The condition should be an important health problem. 2) There should be a treatment for the condition. 3) Facilities for diagnosis and treatment should be available 4) There should be a latent stage of the disease 5) There should be a test or examination for the condition. 6) The test should be acceptable to the population 7) The natural history of the disease should be adequately understood 8) There should be an agreed policy on whom to treat. 9) The total cost of finding a case should be economically balanced in relation to medical expenditure as a whole 10) Case-finding should be a continuous process, not just a "once and for all" project *Wilson JMG, Jungner G. Principles and practice of screening for disease. Public Health Paper Number 34. Geneva: WHO, 1968 (available at http://whqlibdoc.who.int/php/WHO_PHP_34.pdf) 23
5
References 1.
Maron BJ, Haas TS, Doerer JJ, Thompson PD, Hodges JS: Comparison of U.S. and Italian experiences with sudden cardiac deaths in young competitive athletes and implications for preparticipation screening strategies. Am J Cardiol 2009; 104:276–280.
2.
Maron BJ, Haas TS, Ahluwalia A, Rutten-Ramos SC: Incidence of cardiovascular sudden deaths in Minnesota high school athletes. Heart Rhythm 2012; .
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: The Journal of the American Medical Association 2006; 296:1593–1601.
4.
Maron BJ, Doerer JJ, Haas TS, Tierney DM, Mueller FO: Sudden Deaths in Young Competitive Athletes. Circulation 2009; 119:1085–1092.
5.
Harmon KG, Asif IM, Maleszewski JJ, Owens DS, Prutkin JM, Salerno JC, Zigman ML, Ellenbogen R, Rao AL, Ackerman MJ, Drezner JA: Incidence, Cause, and Comparative Frequency of Sudden Cardiac Death in National Collegiate Athletic Association Athletes: A Decade in Review. Circulation 2015; 132:10–19.
6.
Maron BJ: Diversity of views from Europe on national preparticipation screening for competitive athletes. Heart Rhythm 2010; 7:1372–1373.
7.
Thiene G, Corrado D, Schiavon M, Basso C: Screening of competitive athletes to prevent sudden death: implement programmes now. Heart 2013; 99:304–306.
8.
Lawless CE: Minnesota high school athletes 1993-2012: evidence that American screening strategies and sideline preparedness are associated with very low rates of sudden cardiac deaths. J Am Coll Cardiol 2013; 62:1302–1303.
9.
Steinvil A, Chundadze T, Zeltser D, Rogowski O, Halkin A, Galily Y, Perluk H, Viskin S: Mandatory electrocardiographic screening of athletes to reduce their risk for sudden death proven fact or wishful thinking? J Am Coll Cardiol 2011; 57:1291–1296.
10. Risgaard B, Winkel BG, Jabbari R, Glinge C, Ingemann-Hansen O, Thomsen JL, Ottesen GL, Haunsø S, Holst AG, Tfelt-Hansen J: Sports-related sudden cardiac death in a competitive and a noncompetitive athlete population aged 12 to 49 years: data from an unselected nationwide study in Denmark. Heart Rhythm 2014; 11:1673–1681. 11. Holst AG, Winkel BG, Theilade J, Kristensen IB, Thomsen JL, Ottesen GL, Svendsen JH, Haunsø S, Prescott E, Tfelt-Hansen J: Incidence and etiology of sports-related sudden cardiac death in Denmark--implications for preparticipation screening. Heart Rhythm 2010; 7:1365– 1371. 12. Viskin S: Antagonist: routine screening of all athletes prior to participation in competitive sports should be mandatory to prevent sudden cardiac death. Heart Rhythm 2007; 4:525–528.
6
13. Maron BJ, Thompson PD, Ackerman MJ, et al.: Recommendations and considerations related to preparticipation screening for cardiovascular abnormalities in competitive athletes: 2007 update: a scientific statement from the American Heart Association Council on Nutrition, Physical Activity, and Metabolism: endorsed by the American College of Cardiology Foundation. Circulation 2007; 115:1643–1455. 14. Maron BJ, Thompson PD, Puffer JC, McGrew CA, Strong WB, Douglas PS, Clark LT, Mitten MJ, Crawford MH, Atkins DL, Driscoll DJ, Epstein AE: Cardiovascular preparticipation screening of competitive athletes. A statement for health professionals from the Sudden Death Committee (clinical cardiology) and Congenital Cardiac Defects Committee (cardiovascular disease in the young), American Heart Association. Circulation 1996; 94:850–856. 15. Maron BJ, Friedman RA, Kligfield P, et al.: Assessment of the 12-lead electrocardiogram as a screening test for detection of cardiovascular disease in healthy general populations of young people (12-25 years of age): a scientific statement from the American Heart Association and the American College of Cardiology. J Am Coll Cardiol 2014; 64:1479–1514. 16. Link MS, Estes NAM 3rd: Sudden cardiac death in the athlete: bridging the gaps between evidence, policy, and practice. Circulation 2012; 125:2511–2516. 17. Maron BJ, Friedman RA, Caplan A: Ethics of preparticipation cardiovascular screening for athletes. Nat Rev Cardiol 2015; 12:375–378. 18. Dansk Cardiologisk Selskab - Holdningspapirer2 [Internet]. [cited 2013 Jun 12],. Available from: http://www.cardio.dk/rapporter/holdningspapir-menu 19. Winkel BG, Holst AG, Theilade J, Kristensen IB, Thomsen JL, Ottesen GL, Bundgaard H, Svendsen JH, Haunsø S, Tfelt-Hansen J: Nationwide study of sudden cardiac death in persons aged 1-35 years. Eur Heart J 2011; 32:983–990. 20. Risgaard B, Winkel BG, Jabbari R, Behr ER, Ingemann-Hansen O, Thomsen JL, Ottesen GL, Gislason GH, Bundgaard H, Haunsø S, Holst AG, Tfelt-Hansen J: Burden of sudden cardiac death in persons aged 1 to 49 years: nationwide study in Denmark. Circ Arrhythm Electrophysiol 2014; 7:205–211. 21. Winkel BG, Risgaard B, Sadjadieh G, Bundgaard H, Haunsø S, Tfelt-Hansen J: Sudden cardiac death in children (1-18 years): symptoms and causes of death in a nationwide setting. Eur Heart J 2014; 35:868–875. 22. Sealed Envelope | Power calculator for binary outcome superiority trial [Internet]. [cited 2015 Jan 18],. Available from: https://www.sealedenvelope.com/power/binary-superiority/ 23. WHO_PHP_34.pdf - WHO_PHP_34.pdf [Internet]. [cited 2015 Jan 7],. Available from: http://whqlibdoc.who.int/php/WHO_PHP_34.pdf 24. Programme appraisal criteria [Internet]. [cited 2015 Jan 6],. Available from: http://www.screening.nhs.uk/criteria
7
25. Brosnan M, La Gerche A, Kumar S, Lo W, Kalman J, Prior D: Modest agreement in ECG interpretation limits the application of ECG screening in young athletes. Heart Rhythm 2015; 12:130–136. 26. Leslie LK, Cohen JT, Newburger JW, Alexander ME, Wong JB, Sherwin ED, Rodday AM, Parsons SK, Triedman JK: Costs and benefits of targeted screening for causes of sudden cardiac death in children and adolescents. Circulation 2012; 125:2621–2629. 27. Jabbari R, Risgaard B, Holst AG, Nielsen JB, Glinge C, Engstrøm T, Bundgaard H, Svendsen JH, Haunsø S, Winkel BG, Tfelt-Hansen J: Cardiac symptoms before sudden cardiac death caused by coronary artery disease: a nationwide study among young Danish people. Heart 2013; 99:938–943. 28. Sadjadieh G, Jabbari R, Risgaard B, Olesen MS, Haunsø S, Tfelt-Hansen J, Winkel BG: Nationwide (Denmark) study of symptoms preceding sudden death due to arrhythmogenic right ventricular cardiomyopathy. Am J Cardiol 2014; 113:1250–1254. 29. Hansen CM, Wissenberg M, Weeke P, Ruwald MH, Lamberts M, Lippert F, Gislason GH, Nielsen SL, Køber L, Torp-Pedersen C, Folke F: Automated External Defibrillators Inaccessible to More than Half of Nearby Cardiac Arrests in Public Locations during Evening, Nighttime and Weekends. Circulation 2013; . 30. Wissenberg M, Lippert FK, Folke F, et al.: Association of national initiatives to improve cardiac arrest management with rates of bystander intervention and patient survival after outof-hospital cardiac arrest. JAMA 2013; 310:1377–1384. 31. Maron BJ, Winkel BG, Tfelt-Hansen J: Perspectives on cardiovascular screening. JAMA 2015; 313:31–32.
8
PII: DOI: Reference:
S1547-5271(16)30087-X http://dx.doi.org/10.1016/j.hrthm.2016.03.043 HRTHM6674
To appear in: Heart Rhythm Received date: 11 January 2016 Cite this article as: Bjarke Risgaard, Jacob Tfelt-Hansen and Bo Gregers Winkel, Sports-Related Sudden Cardiac Death: How to Prove an Effect of PreParticipation Screening?, Heart Rhythm, http://dx.doi.org/10.1016/j.hrthm.2016.03.043 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Sports-related sudden cardiac death: How to prove an effect of pre-participation screening?
Bjarke Risgaard, MD, PhD; Jacob Tfelt-Hansen, MD, DMSc; Bo Gregers Winkel, MD, PhD Affiliations: The Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark;
Author contributions: Bjarke Risgaard designed and drafted the manuscript. Jacob Tfelt-Hansen and Bo Gregers Winkel designed and revised the manuscript critically.
Conflicts of interest: None to declare
Running Title: Sports-related sudden cardiac death
Word count: 1452
Corresponding author: Corresponding author: Bjarke Risgaard, MD, PHD. Address for correspondence: The Heart Centre, Copenhagen University Hospital, Rigshospitalet, Afnist 2143, Blegdamsvej 9, 2100 Copenhagen Ø. Tel: (+45) 35 45 35 45 Email: [email protected]
1
Viewpoint: Sports-related sudden cardiac deaths (SrSCD) are tragic events that occur in individuals who are believed to be in good health prior to death. These deaths are highly visible in the public health debate due to significant media attention. The reported annual incidence rate of SrSCD remains low at 0.93 and 0.70 per 100,000 participants in Minnesota high school athletes1,2; 0.87 in the Veneto region3; and 0.61 for the entire United States4. However, recently published data from the United States suggest that the incidence rate of SCD in athletes in the National Collegiate Athletic Association is higher (1.86 per 100,000 athlete-years)5. Whether we should implement mandatory pre-participation screening programs to lower the burden of SrSCD among competitive athletes is hotly debated. Pre-participation screening programs have been mandated in several places. In the United States, screening consists of a history and a physical examination, whereas in Italy and Israel, the pre-participation screening programs additionally include a 12-lead electrocardiogram (ECG)1,6–8. Data that favor pre-participation screening mainly come from the Veneto region in Italy3. Corrado and colleagues3 showed a declining incidence rate of SrSCD from 4.19 (1979-1981) to 0.87 (19932004) per 100,000 athletes following the implementation of these programs in Italy. Today, the incidence rate of SrSCD in Italy is lower than the incidence rate of SCD in the general population3. Several other studies, however, have challenged these findings by suggesting that pre-participation screening is of limited value2,4,9–12. The American Heart Association argues against routine ECG screening of all competitive athletes and highlights the many obstacles they may introduce 13–15. The low specificity and sensitivity of an ECG for detecting the diseases in question, together with the associated economic challenges, has led to a discussion of how best to bridge current knowledge with practice16. In addition, it has been argued that mandatory pre-participation screening of young competitive athletes also may introduce certain ethical dilemmas6,17. Despite the level of evidence, pre-participation screening has been implemented on very different levels throughout the world, and it has been used most extensively in Italy and Israel3,9. In Denmark, the Danish Society of Cardiology has decided to not recommend mandatory screening of competitive athletes, which makes the country suitable for investigating the incidence rate of SrSCD in a pre-screening era18. Among competitive athletes 12-35 years of age, we previously reported incidence rates of SrSCD of 1.21 (95% CI 0.73-2.01) (2000-2006) and 0.47 (95% CI 0.101.14)(2007-2009) per 100,000 athlete person-years11,10 corresponding to 1-2 cases of SrSCD per 2
year in Denmark in the 12-35-year age group. Young non-competitive athletes had a similarly risk to that of competitive athletes (incidence rates of 0.43 vs. 0.47), and competitive athletes 36-49 years of age had a much higher risk than competitive athletes 12-35 years of age (incidence rates of 6.64 vs. 0.47 per 100,000 athlete person-years)10. Most importantly, we have shown that despite the lack of mandatory screening programs in Denmark, the incidence rate of SrSCD is nearly 3 times lower than that of SCD in the general population19–21. As described above, the current literature highlights uncertainty with regard to the efficacy and cost-effectiveness of screening and also questions whether these screening programs should include ECGs. Importantly, no solid evidence from large randomized trials has been published due to low event rates and ethical considerations. Hence, the question remains whether we can show that screening all competitive athletes is beneficial with regard to reducing mortality. Is a gold standard prospective randomized controlled trial feasible? The calculation for a randomized controlled trial would be as follows. The highest incidence rate of SrSCD among competitive athletes in the 12-35-year age group in Denmark (where screening is not performed routinely) was 1.21 per 100,000 person-years. Theoretically, this means that among 100 athletes followed for one year, we would expect 0.00121 (0.00121%) cases of SrSCD. The lowest incidence rate of SrSCD in a screened population (without ECG) is reported to be 0.61 per 100,000 person-years4. This corresponds to 0.00061 (0.00061%) deaths among 100 athletes followed for one year, and if we assume that we will never eliminate all cases of SrSCD, this may be regarded as the lowest possible incidence rate. As the incidence rates are low, so are the absolute numbers. In Denmark, Italy and within the state of Minnesota, the numbers of SCD among the young has been estimated to be 1-2 deaths, 2 deaths and <1 death per year, respectively1,10,11. According to an internet-based sample size calculator, the numbers of individuals required, assuming an 80% chance of detecting a change in the endpoint, is nearly 8 million athletes22. However, using the incidence rate of SCD in the pre-screening era in Italy (4.19/100,000), the number of athletes required would be reduced (588,066 athletes). Nevertheless, due to the large numbers of athletes, it is very doubtful that such a study will be performed. Another question is whether the screening of young competitive athletes with or without ECGs fulfills the general criteria for a screening program. In 1968, the World Health Organization published a guideline with 10 criteria, referred to as the Wilson criteria, that outline the principles of screening (table 1)23. In 2009, a National Screening Committee in the UK published new criteria for
3
appraising the viability, effectiveness and appropriateness of a screening program24. The key message was that screening should be performed only if the disease in question is an important health problem. Furthermore, the epidemiology should be adequately understood and should have an identifiable risk factor. The screening test should be simple, safe, precise, and validated. In addition, it should be acceptable to the population. Finally, there should be an available intervention or treatment. The UK National Screening Committee24 also argues that the evidence for the entire program should come from high-quality randomized controlled trials that address efficacy in reducing mortality. There should be evidence that the complete screening program is ethical and acceptable and that the beneficial effects outweigh any physical or psychological harm. It is emphasized that the costs should be economical and that all other options for managing the disease should have been considered24. In our opinion, most of the criteria described above for implementing a screening program are not met with regards to SrSCD among young athletes. First, we do not believe that SrSCD constitutes a significant public health issue, as we have shown that SrSCD is rare even in an unscreened population, while SCD is much more common in the general population10,11. Second, although screening with ECG may be acceptable to the public (no adverse effects), sensitivity, specificity and interobserver variability are poor25. Third, the high numbers of false positive and false negative results, as well as the negative effects of sports restriction (both psychological and health), should be considered. Fourth, screening that is restricted to a subset of individuals with the lowest risk (competitive athletes age 12-35 years) is questionable from an ethical perspective. Fifth, costs of targeted screening with ECG have been shown to be high compared with the potential beneficial effect26. Finally, sixth, no randomized clinical controlled trial has been or is likely to be performed. Thus, overall, we believe that pre-participation screening of young athletes is questionable, and we particularly believe that the additional use of ECG in its current form is not justified. Other options for managing SrSCD should be considered. Athletes and coaches should be thoroughly educated with regard to which symptoms warrant a clinical evaluation. Cardiac symptoms prior to SCD or SrSCD are common, and identification of these patients may be an alternative approach to identifying patients at risk10,21,27,28. As secondary prevention, automated external defibrillators (AEDs) should be widely distributed and accessible in strategic areas29 to increase the chance of survival in cases of out-of-hospital cardiac arrest. Education of the general population in bystander
4
cardiopulmonary resuscitation should be performed and continued. This has proven very beneficial in Denmark, where survival rates have increased30. Several other governmental and private health initiatives have also been implemented over the past 2 decades in Denmark to address the prevention of sudden death. These programs have included large populations instead of targeting only those engaged in competitive sports. As a consequence, for example, prevention of suicide and motor vehicle accidents has been associated with saving 220 lives per year in young people aged 15-25 years31, whereas only 1-2 athletes die each year10,11. Pre-participation screening programs are currently in place to various extents in many countries. To eliminate established programs seems virtually impossible. It is our belief that other strategies should be considered to prevent SrSCD and to reduce the burden of SCD in the general population. Based on the Danish experience and the Wilson criteria, we believe that pre-participation screening programs with or without ECGs should not currently be recommended.
Tables Table 1. The key principles of screening according to the Wilson criteria*. Principles of screening 1) The condition should be an important health problem. 2) There should be a treatment for the condition. 3) Facilities for diagnosis and treatment should be available 4) There should be a latent stage of the disease 5) There should be a test or examination for the condition. 6) The test should be acceptable to the population 7) The natural history of the disease should be adequately understood 8) There should be an agreed policy on whom to treat. 9) The total cost of finding a case should be economically balanced in relation to medical expenditure as a whole 10) Case-finding should be a continuous process, not just a "once and for all" project *Wilson JMG, Jungner G. Principles and practice of screening for disease. Public Health Paper Number 34. Geneva: WHO, 1968 (available at http://whqlibdoc.who.int/php/WHO_PHP_34.pdf) 23
5
References 1.
Maron BJ, Haas TS, Doerer JJ, Thompson PD, Hodges JS: Comparison of U.S. and Italian experiences with sudden cardiac deaths in young competitive athletes and implications for preparticipation screening strategies. Am J Cardiol 2009; 104:276–280.
2.
Maron BJ, Haas TS, Ahluwalia A, Rutten-Ramos SC: Incidence of cardiovascular sudden deaths in Minnesota high school athletes. Heart Rhythm 2012; .
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: The Journal of the American Medical Association 2006; 296:1593–1601.
4.
Maron BJ, Doerer JJ, Haas TS, Tierney DM, Mueller FO: Sudden Deaths in Young Competitive Athletes. Circulation 2009; 119:1085–1092.
5.
Harmon KG, Asif IM, Maleszewski JJ, Owens DS, Prutkin JM, Salerno JC, Zigman ML, Ellenbogen R, Rao AL, Ackerman MJ, Drezner JA: Incidence, Cause, and Comparative Frequency of Sudden Cardiac Death in National Collegiate Athletic Association Athletes: A Decade in Review. Circulation 2015; 132:10–19.
6.
Maron BJ: Diversity of views from Europe on national preparticipation screening for competitive athletes. Heart Rhythm 2010; 7:1372–1373.
7.
Thiene G, Corrado D, Schiavon M, Basso C: Screening of competitive athletes to prevent sudden death: implement programmes now. Heart 2013; 99:304–306.
8.
Lawless CE: Minnesota high school athletes 1993-2012: evidence that American screening strategies and sideline preparedness are associated with very low rates of sudden cardiac deaths. J Am Coll Cardiol 2013; 62:1302–1303.
9.
Steinvil A, Chundadze T, Zeltser D, Rogowski O, Halkin A, Galily Y, Perluk H, Viskin S: Mandatory electrocardiographic screening of athletes to reduce their risk for sudden death proven fact or wishful thinking? J Am Coll Cardiol 2011; 57:1291–1296.
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