European Heart Journal Advance Access originally published online on July 28, 2006
European Heart Journal 2006 27(18):2152-2153; doi:10.1093/eurheartj/ehl171
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Sudden cardiac death due to hypertrophic cardiomyopathy can be reduced by pre-participation cardiovascular screening in young athletes
1 Sint Maartenskliniek location Sports Medical Centre Papendal, Arnhem, The Netherlands
2 Heart Centre Radboud University Hospital, Nijmegen, The Netherlands
* *Corresponding author. Tel: +31 26 48 34 440; fax: +31 26 36 37 649. E-mail address: nicole.panhuyzen{at}planet.nl
This editorial refers to ‘Evidence for efficacy of the Italian national pre-participation screening program for identification of hypertrophic cardiomyopathy in competitive athletes’
by A. Pelliccia et al., on page 2196
Sudden death of a young well-trained athlete has a high impact on everyone involved and raises concern in the medical community. It is hardly acceptable that a ‘healthy’ individual dies suddenly during athletic activity. And the question that always arises is whether this athlete could have been identified by pre-participation cardiovascular screening and this sudden cardiac death could have been prevented. The first documented ‘marathon runner’ dying suddenly within an hour after physical exertion while announcing the Greek victory (‘Niké’) over the Persians was Pheidippides in 490 BC. More recently, the death of well-trained athletes has focused interest on pre-participation cardiovascular screening. The European Association for Cardiovascular Prevention and Rehabilitation (EACPR) Section Sports Cardiology of the European Society of Cardiology proposed an ‘European protocol’ for pre-participation cardiovascular screening to prevent sudden cardiac death in young athletes, which is supported by the International Olympic Committee (IOC).1,2 In this common ‘European protocol’, pre-participation cardiovascular screening is performed every year or every other year according to the Italian strategy consisting of personal and family history, physical examination, and 12-lead resting ECG. The EACPR Section Sports Cardiology has started to implement this protocol in European countries. Also the FIFA (Fédération Internationale de Football Association) performed pre-participation cardiovascular screening in all soccer players participating at the world championship in Germany 2006.3
Sudden cardiac death in young athletes (12–35 years) is usually caused by inherited or congenital cardiac disorders. These disorders are silent or concealed until they are manifested by syncope or sudden cardiac death in relation to physical exertion. In the United States, one of the leading causes of sudden cardiac death in athletes is hypertrophic cardiomyopathy (HCM). In a registry consisting of 385 athletes with sudden cardiac death, Maron4 reported that 102 young athletes (26.4%) died because of HCM. Corrado et al.5 described pre-participation cardiovascular screening (including 12-lead resting ECG) in 33.735 young athletes during 17 years in the Veneto region of Italy. There were 22 athletes with clinically proven HCM. In this same period, a large control non-athlete population was observed. A total of 269 young individuals died suddenly during follow-up: 49 athletes (18%) and 220 non-athletes (82%). In 17 young individuals (6.3%), the cause of death was HCM: one athlete (2%) and 16 non-athletes (7.3%).1,5 But the leading cause of sudden cardiac death in this series was arrhythmogenic right ventricular cardiomyopathy, which was seen in 11 athletes (22.4%) compared with 18 non-athletes (8.2%) during these 17 years. The incidence of sudden cardiac death in young athletes was 2.1/100.000, which is nowadays even declining below the incidence of sudden cardiac death in non-athletes probably because of the systematic pre-participation cardiovascular screening system in Italy according to law. Should then not all young individuals (athletes and non-athletes) be screened for HCM? The costs are probably too high for such mass screening, and not all countries can afford such a screening program and the work load. Why then is HCM still the leading cause of sudden cardiac death in young athletes in the United States? Pre-participation cardiovascular screening is performed in high school and college athletes with personal and family history and physical examination, but without 12-lead resting ECG. The 12-lead resting ECG is very useful to identify the ‘concealed HCM’ in a young athlete.1,5,6 HCM is a genetic disease with a mutant gene variation. HCM develops during adolescence where there is rapid hypertrophy during fast growth and development of the human body.7 The typical ECG changes in HCM are left bundle branch block, or high voltages indicating left ventricular (LV) hypertrophy with prominent septal Q-waves in the lateral leads, or giant negative T-waves in the precordial leads.
In this issue, Pelliccia8 has given a clear message. For 9 years, he examined 4450 young top-level athletes who were judged eligible for competition in Italy with additional routine transthoracic echocardiography. In only four athletes HCM was suspected by so-called ‘gray zone’ septal thickening (12–13 mm), and in three of them ECG changes suspect of HCM were seen. After 8 years of follow-up HCM was confirmed in one of them. Septal thickening was 15 mm, and cavity size was reduced. Cardiac MRI and gene analysis confirmed the diagnosis of HCM. The second athlete developed ventricular tachycardia with systolic anterior motion and myocardial bridging, suspect of HCM. In these two athletes HCM was always suspected, but could only be confirmed after 8 years. In the other two athletes HCM was unlikely after 8–12 years follow-up. ECG changes during follow-up were unfortunately not given in this article. Was HCM then probably induced by many years of high level of physical training? There is no evidence to answer this question. The authors conclude that transthoracic echocardiography should not be routinely used in pre-participation cardiovascular screening, and that 12-lead resting ECG in addition to history and physical examination can identify HCM. However, in this study there were only two black athletes. Both of them showed normal echocardiographic findings. In a Minneapolis registry in the United States, 1986 young athletes were identified with HCM, of these 158 were African Americans (8%). In this same registry, 286 young athletes had sudden cardiac death, 156 white Americans (55%) and 120 African Americans (42%). HCM was the cause of death in 102 of these athletes (36%): 42 white Americans (41%) and 56 African Americans (55%). The authors of this registry conclude that HCM is a common cause of sudden cardiac death in young unidentified African American athletes.9 This underscoring of clinical recognition justifies 12-lead resting ECG in pre-participation cardiovascular screening in young athletes. Routine use of transthoracic echocardiography in black athletes is justified, when they are seen for their first pre-participation cardiovascular screening. Routine use of echocardiography raises the costs of pre-participation cardiovascular screening in all European countries. However, reserving routine use of echocardiography in top-level athletes at least once before they are grown up into adulthood can be helpful, because most ECG's in athletes, and especially in black athletes, are abnormal and it is unclear if and when HCM develops before the adulthood. Routine echocardiography every (other) year in all athletes is not necessary as has been demonstrated before by the same author.6 Family screening in inherited electrical cardiac diseases and in HCM is technically possible. Recommendations on future screening by gene analysis in an athlete suspected of HCM, other electrical cardiac diseases, or even in athletes coming for pre-participation cardiovascular screening will be a great step forward.
Conflict of interest: none declared.
Footnotes
The opinions expressed in this article are not necessarily those of the Editors of the European Heart Journal or of the European Society of Cardiology.
doi:10.1093/eurheartj/ehl137 
References
- Corrado D, Pelliccia A, Bjornstad HH, Vanhees L, Biffi A, Borjesson M, Panhuyzen-Goedkoop NM, Deligiannis A, Solberg E, Dugmore D, Mellwig KP, Assanelli D, Delise P, van-Buuren F, Anastasakis A, Heidbuchel H, Hoffmann E, Fagard R, Priori S, Basso C, Arbustini E, Blomstrom-Lundqvist C, McKenna WJ, Thiene G. (2005) Cardiovascular pre-participation screening of young competitive athletes for prevention of sudden death: proposal for a common European protocol. Eur Heart J 26:516–524.
[Abstract/Free Full Text] - International Olympic Committee. Sudden cardiovascular death in sport. Lausanne, Switzerland. http//www.olympic.org/uk/news/Olympic_news/newsletter_full_story_ukasp?id=1182 Lausanne recommendations adopted 9–10 December 2004.
- FIFA. Prevention the priority for FIFA medical division. http://fifaworldcup.yahoo.com/06/en/050625/1/4ul.html (25 June 2005).
- Maron BJ. (2003) Sudden death in young athletes. N Engl J Med 349:1064–1075.
[Free Full Text] - Corrado D, Basso C, Schiavon M, Thiene G. (1998) Screening for hypertrophic cardiomyopathy in young athletes. N Engl J Med 339:364–369.
[Abstract/Free Full Text] - Pelliccia A and Maron BJ. (1995) Pre-participation cardiovascular evaluation of the competitive athlete: perspectives from the 30-year Italian experience. Am J Cardiol 75:827–829.[CrossRef][Web of Science][Medline]
- Gilette PC and Garson A Jr. (1992) Sudden death in the pediatric population. Circulation 85:(Suppl. I), I64–I69.
- Pelliccia A, Di Paolo FM, Corrado D, Buccolieri C, Quatrinni FM, Pisicchio C, Spataro A, Biffi A, Granata M, Maron BJ. (2006) Evidence for efficacy of the Italian national pre-participation screening programme for identification of hypertrophic cardiomyopathy in competitive athletes. Eur Heart J 27:2196–2200 First published on July 10, 2006, doi:10.1093/eurheartj/ehl137.
[Abstract/Free Full Text] - Maron BJ, Carney KP, Lever HM, Lewis JF, Barac I, Casey SA, Sherrid MV. (2003) Relationship of race to sudden cardiac death in competitive athletes with hypertrophic cardiomyopathy. J Am Coll Cardiol 41:974–980.
[Abstract/Free Full Text]
CiteULike 
Connotea 
Del.icio.us What's this?
Related articles in EHJ:
- Evidence for efficacy of the Italian national pre-participation screening programme for identification of hypertrophic cardiomyopathy in competitive athletes
- Antonio Pelliccia, Fernando M. Di Paolo, Domenico Corrado, Cosimo Buccolieri, Filippo M. Quattrini, Cataldo Pisicchio, Antonio Spataro, Alessandro Biffi, Maristella Granata, and Barry J. Maron
EHJ 2006 27: 2196-2200.[Abstract] [FREE Full Text]
This article has been cited by other articles:
|
|
 |
|
  B Bessem, F P Groot, and W Nieuwland The Lausanne recommendations: a Dutch experience Br. J. Sports Med., September 1, 2009; 43(9): 708 - 715. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. J. McLeod, M. J. Ackerman, R. A. Nishimura, A. J. Tajik, B. J. Gersh, and S. R. Ommen Outcome of patients with hypertrophic cardiomyopathy and a normal electrocardiogram. J. Am. Coll. Cardiol., July 14, 2009; 54(3): 229 - 233. [Abstract] [Full Text] [PDF]
|
|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||