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Analysis

Harms and benefits of screening young people to prevent sudden cardiac death

BMJ 2016; 353 doi: https://doi.org/10.1136/bmj.i1156 (Published 20 April 2016) Cite this as: BMJ 2016;353:i1156

Rapid Response:

Italian Preparticipation Screening for Prevention of Sudden Cardiac Death: it Works!

We read with attention the review paper by Van Brabandt et al. (1), questioning the utility of pre-participation screening for prevention of sudden cardiac death (SCD) during sports activity. The paper is not a balanced “pro and cons” analysis; rather, it appears as a direct attack to the Italian screening program and to the Italian researchers that provided scientific evidence that cardiovascular evaluation of athletes is a life-saving strategy based on the >30 years experience in the Veneto Region, North-East Italy (2-6).

The paper is signed by laypersons with no experience in the field of sports cardiology, who provided a divorced from reality personal viewpoint. Surprisingly, the analysts omitted the “real world” opinion of thousands international physicians daily involved in athletes evaluation, who overwhelmingly favoured the need of pre-participation screening (7) (Figure 1).

The Authors started by mistakenly reporting that the Italian screening of young competitive athletes has been mandatory since 1970 (or 1979). Instead, screening has been in practice by law in Italy since 1982 (8), which is the correct starting time for evaluating its impact on mortality. This initial inaccuracy jeopardizes the entire “analysis”.

The inaccuracy is confirmed by their statement that usefulness of ECG screening is confined to the detection of athletes with Wolff-Parkinson-White syndrome, which clearly demonstrates the ignorance that the strength of ECG screening is the identification of inherited cardiomyopathies and channelopathies, which are ECG detectable causes of SCD. In this regard, it is amazing that the review did not mention our fundamental study proving the efficacy of pre-participation ECG screening for pre-symptomatic identification of hypertrophic cardiomyopathy (Figure 2), which is the leading cause of SCD in the athlete worldwide (2,9,10).

It is not surprising that the economic model developed by the Stanford University to demonstrate that screening is cost-effective for prevention of SCD in the athlete necessarily relied on the Italian data on mortality trends, being the only available (11). Considering that 30-40% of conditions at risk of SCD in the young are genetically transmissible, the aim of the screening is not only to safeguard the athlete’s life, but also the relatives’ lives by cascade screening, including genetic analysis.

We are fully aware that some diseases at risk, mostly premature coronary atherosclerosis and congenital coronary anomalies, may escape identification at screening (5,6,12,13) and we agree that secondary prevention, by dissemination of external defibrillators in playgrounds and training for cardiopulmonary resuscitation, should be recommended as well (14-16).

The most misleading assertion by Van Brabandt et al. (1) is that our study does not provide evidence that screening was the cause of the 90% mortality reduction in competitive athletes (4), because there was no unscreened control population. Although a randomized study design was not feasible because screening is compulsory by law, the study was actually a comparison of incidence rates for SCD in screened athletes versus unscreened non-athletes aged 12–35 years, before and during this screening programme. Systematic pre-participation evaluation, coupled with sports restriction, resulted in a decline in deaths among screened athletes from 3.6 per 100 000 person-years in the 2 years before screening implementation to 0.4 per 100 000 person-years two decades later. In contrast, there was no change in deaths among unscreened non-athletes. The strong cause–effect relationship between ECG screening and the substantial reduction of SCD is supported by several results: (i) there was a coincident timing between decline of SCD in young competitive athletes and screening implementation; (ii) most of the reduced incidence of SCD was due to fewer deaths from cardiomyopathies and it was accompanied by a concomitant increase in the proportion of competitive athletes who were identified affected by these diseases and disqualified from competition at the Center for Sports Medicine; and iii) the incidence of SCD did not change among the unscreened non-athletic population of the same age range. Although additional factors—environmental, socio-economic, or medical/surgical—may have contributed to mortality reduction over the time, such factors are expected to impact mortality similarly in screened young athletes and unscreened young non-athletes, and hence cannot explain the declining trend.

The database of SCD in the Veneto region of Italy is unique in so far as cases are collected according to a prospective study design with systematic investigation of all young people (≤35 years), including competitive athletes, who die suddenly and undergo a standardized pathologic investigation of the heart by a team of cardiovascular pathologists. The heart specimens (Figure 3), as well as the clinical records of all SCD victims since 1979, are stored at the Registry of Cardiovascular Pathology, University of Padova, giving the chance to go back and review the case (6,12). This reflects the indisputable reliability of our data on causes and trends of SCD in young people and athletes. Reports on SCD from other countries were mostly based on retrospective analysis of data from public media reports and insurance claims, which unavoidably led to an inaccuracy with mortality underestimation.

Finally, we were astonished at reading the strong “accuse” that we have refused to share the updated findings (1,17). Our published data on the efficacy of pre-participation screening were referring to the time interval 1982-2004 (4). As anticipated in private correspondence, in 2015 we completed another 10 years interval with follow-up of screened athletes. The results were not ready at the time of the request because we rigorously investigate each SCD victim both clinically and pathologically, according to a standardized protocol which implies in selected cases the study of the conduction system and molecular autopsy. This protocol represents the basis for the Guidelines for Autopsy Investigation of SCD by the Association for European Cardiovascular Pathology (18).

Our strict policy is to provide and publish only conclusive results. Moreover, we firmly believe that accurate collection and analysis of data are vital requisites for an unbiased scientific research (19). Definite results on the 2005-2014 decade of SCD monitoring are now available and will be submitted for publication in a peer-reviewed journal, that is the only way to disseminate original data: we can anticipate that they confirm the trend of decreasing mortality in screened athletes.

The pre-participation screening represents a major achievement of the Italian National Health System and an application of our Bill, where health and life are fundamental rights to be warranted. 1.8% of young to be engaged in sports are disqualified (2), because they are found affected by life-threatening cardiac conditions. By no way it should be considered discriminatory, on the opposite it is an ethical, deontological attitude to safe life.

REFERENCES
1. Van Brabandt H, Desomer A, Gerkens S, Neyt M. Preparticipation screening for the prevention of sudden cardiac death in young non-professional athletes: harms outweigh benefits. BMJ 2016;352:i1156.
2. Corrado D, Basso C, Schiavon M, Thiene G. Screening for hypertrophic cardiomyopathy in young athletes. N Engl J Med 1998;339:364–9
3. Corrado D, Basso C, Rizzoli G, Schiavon M, Thiene G. Does sports activity enhance the risk of sudden death in adolescents and young adults? J Am Coll Cardiol 2003;42:1959-63
4. 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 pre-participation screening program. JAMA. 2006;296:1593-601.
5. Thiene G, Carturan E, Corrado D, Basso C. Prevention of sudden cardiac death in the young and in athletes: dream or reality? Cardiovasc Pathol. 2010;19:207-17
6. Thiene G: Sudden Cardiac Death and Cardiovascular Pathology: from Anatomic Theater to Double Helix Am J Cardiol 2014;114:1930-1936
7. Colbert JA. Clinical decisions. Cardiac screening before participation in sports--polling results. N Engl J Med. 2014;370:e16
8. Decree of the Italian Ministry of Health, February 18, 1982. Norme per la tutela sanitaria dell'attività sportiva agonistica (rules concerning the medical protection of athletic activity). Gazzetta Ufficiale March 5, 1982:63.
9. Maron BJ, Doerer JJ, Haas TS, Tierney DM, Mueller FO. Sudden deaths in young competitive athletes: analysis of 1866 deaths in the United States, 1980-2006. Circulation. 2009;119:1085-92.
10. Myerburg RJ, Vetter VL. Electrocardiograms should be included in preparticipation screening of athletes. Circulation. 2007;116:2616-26
11. Wheeler MT, Heidenreich PA, Froelicher VF, Hlatky MA, Ashley EA. Cost-effectiveness of preparticipation screening for prevention of sudden cardiac death in young athletes. Ann Intern Med 2010;152:276-286
12. Thiene G, Corrado D, Basso C. Sudden Cardiac Death in the Young and Athletes. Text Atlas of Pathology and Clinical Correlates. Springer Verlag, Milan 2016
13. Basso C, Maron BJ, Corrado D, Thiene G. Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes. J Am Coll Cardiol. 2000;35:1493-501
14. Corrado D, Drezner J, Basso C, Pelliccia A, Thiene G. Strategies for the prevention of sudden cardiac death during sports. Eur J Cardiovasc Prev Rehabil. 2011;18:197-208.
15. Semsarian C, Ingles J. Preventing sudden cardiac death in athletes. BMJ. 2016;353:i1270
16. d'Amati G, De Caterina R, Basso C. Sudden cardiac death in an Italian competitive athlete: Pre-participation screening and cardiovascular emergency care are both essential. Int J Cardiol. 2016 Mar 1;206:84-6
17. Cohen D. Data on benefits of screening for sudden cardiac death are withheld. BMJ. 2016 Apr 20;353:i2208
18. Basso C, Burke M, Fornes P, Gallagher PJ, de Gouveia RH, Sheppard M, Thiene G, van der Wal A; Association for European Cardiovascular Pathology. Guidelines for autopsy investigation of sudden cardiac death. Virchows Arch 2008;452:11-8
19. Solberg EE, Borjesson M, Sharma S, Papadakis M, Wilhelm M, Drezner JA, Harmon KG, Alonso JM, Heidbuchel H, Dugmore D, Panhuyzen-Goedkoop NM, Mellwig KP, Carre F, Rasmusen H, Niebauer J, Behr ER, Thiene G, Sheppard MN, Basso C, Corrado D; Sport Cardiology Section of the EACPR of the ESC. Sudden cardiac arrest in sports - need for uniform registration: A Position Paper from the Sport Cardiology Section of the European Association for Cardiovascular Prevention and Rehabilitation. Eur J Prev Cardiol. 2016;23:657-67.

Figure 1
Response to 2013 online poll by the New England Journal of Medicine on the need of screening before participation in sports.
The website of the Journal received 1266 votes from 86 countries. Overall, only 18% opposed cardiac screening of young athletes before participation in sports; among the remaining, 24% favoured screening with a history and physical examination only, and 58% favoured screening with a history, physical examination, and ECG. The figure shows the online polling results for voters from the USA as compared with voters from Italy. The vast majority of voters favoured screening before participation in sports, either in the USA (80%) or in Italy (88%). Screening with ECG was recommended by 66% of the voters from Italy versus 45% of those from the USA (from Colbert JA. Clinical decisions. Cardiac screening before participation in sports — Polling Results. N Engl J Med 2014; 370:e16)

Figure 2
Italian athlete screening program disqualifications. A) Among 33 735 athletes screened, 621 (1.8%) were disqualified for cardiovascular (CV) diseases. B) Among the 22 patients with a diagnosis of HCM, 18 (82%) had an abnormal ECG, whereas only 5 (23%) had a positive family history (FH) or cardiac murmur. Thus, in the absence of ECG the majority would have been considered eligible and exposed to the risk of SCD. In the follow-up, none of the 22 disqualified HCM athletes suffered SCD. (from Myerburg RJ, Vetter VL. Electrocardiograms should be included in pre-participation screening of athletes. Circulation. 2007;116:2616-26)

Figure 3
Registry of SCD, Cardiovascular Pathology Unit, at the University of Padova, Italy.
The heart specimens of all young people including athletes, who have died suddenly since 1979, are stored and available for re-examination, together with clinical records.

Competing interests: No competing interests

17 June 2016
Gaetano Thiene
Full Professor
Domenico Corrado, Cristina Basso, Maurizio Schiavon, Gaetano Thiene
Department of Cardiac, Thoracic and Vascular Sciences, University of Padua-I
via Gabelli, 61 35121 Padova