- Email: [email protected]
Comparison of the Frequency of Sudden Cardiovascular Deaths in Young Competitive Athletes Versus Nonathletes: Should We Really Screen Only Athletes?
Accepted Manuscript Comparison of the Frequency of Sudden Cardiovascular Deaths in Young Competitive Athletes vs. Non-athletes: should we really screen only Athletes? Barry J. Maron, MD, Tammy S. Haas, RN, Emily R. Duncanson, MD, Ross F. Garberich, MS, Andrew M. Baker, MD, Shannon Mackey-Bojack, MD PII:
S0002-9149(16)30148-5
DOI:
10.1016/j.amjcard.2016.01.026
Reference:
AJC 21672
To appear in:
The American Journal of Cardiology
Received Date: 20 November 2015 Revised Date:
13 January 2016
Accepted Date: 18 January 2016
Please cite this article as: Maron BJ, Haas TS, Duncanson ER, Garberich RF, Baker AM, MackeyBojack S, Comparison of the Frequency of Sudden Cardiovascular Deaths in Young Competitive Athletes vs. Non-athletes: should we really screen only Athletes?, The American Journal of Cardiology (2016), doi: 10.1016/j.amjcard.2016.01.026. 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 proof before it is published in its final 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.
ACCEPTED MANUSCRIPT
COMPARISON OF THE FREQUENCY OF SUDDEN CARDIOVASCULAR DEATHS
RI PT
IN YOUNG COMPETITIVE ATHLETES VS. NON-ATHLETES:
SC
SHOULD WE REALLY SCREEN ONLY ATHLETES?
Barry J. Maron, MDa, Tammy S. Haas, RNa, Emily R. Duncanson, MDb,
M AN U
Ross F. Garberich, MSa, Andrew M. Baker, MDc, Shannon Mackey-Bojack, MDb
a
Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation,
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Minneapolis, MN; b
Jesse E. Edwards Registry of Cardiovascular Disease, Nasseff Heart Center, St. Paul, MN; Hennepin County Medical Examiner's Office, Minneapolis, MN
EP
c
AC C
The authors report no conflicts of interest. Address for Correspondence: Barry J. Maron, MD Minneapolis Heart Institute Foundation 920 East 28th Street, Suite 620 Minneapolis, MN 55407 612-863-3996/Fax: 612-863-3875 [email protected]
Word count: 2009 1
ACCEPTED MANUSCRIPT
ABSTRACT The issue of sudden death in young athletes and consideration for the most practical and optimal strategy to identify those genetic and/or congenital heart diseases
RI PT
responsible for these tragic events continues to be debated. However, proponents of broad-based and mandatory national preparticipation screening, including with 12-lead ECGs have confined the focus to a relatively small segment of the youthful population
SC
who choose to engage in competitive athletic programs at the high school, college and elite-professional level. Therefore, lost in this discussion of preparticipation screening of
M AN U
athletes is that the larger population of young people not involved in competitive sports (and, therefore, a priori, excluded from systematic screening) who nevertheless may die suddenly of the same cardiovascular diseases as athletes. To substantiate this hypothesis, we accessed the forensic Hennepin County, Minnesota registry in which
TE D
cardiovascular sudden deaths were 8-fold more common in non-athletes (n=24) than athletes (n=3), and 3-fold more frequent in terms of incidence. The most common diseases responsible for sudden death were hypertrophic cardiomyopathy (n=6) and
EP
arrhythmogenic right ventricular cardiomyopathy (n=4). These data raise ethical considerations inherent in limiting systematic screening for unsuspected genetic and/or
AC C
congenital heart disease to competitive athletes.
Key Words:
Sudden death Athletes Screening
2
ACCEPTED MANUSCRIPT
Introduction Screening healthy general populations for cardiovascular disease has become a topic of considerable interest, triggered by the high visibility afforded sudden deaths in
RI PT
young competitive athletes (1-5). Attention directed toward these often highly visible and public events may have created the misconception that sudden deaths in high school and college-aged individuals occur predominantly (or even exclusively) in
SC
student-athletes (1-5).
A debate has emerged concerning the most effective strategy for identifying the
M AN U
unsuspected genetic and/or congenital diseases responsible for these tragic events (1,2, 6-8). Should systematic screening be confined to those who choose to engage in competitive sports, or should non-athletes (who can harbour the same potentially lethal cardiovascular diseases as athletes) be included in this process? (7) We have
TE D
addressed this highly relevant issue for the ongoing preparticipation screening conversation by interrogating a large forensic database to assess the frequency of genetic and/or congenital cardiovascular diseases causing sudden death in trained
EP
athletes vs. young people not engaged in organized sports programs. METHODS
AC C
We accessed the case records of the Medical Examiner of Hennepin County, the
largest of the 87 Minnesota counties, representing the Minneapolis metropolitan area. Hennepin County includes 1,198,778 residents (23% of the state); all sudden deaths <40 years of age undergo complete autopsy and toxicologic studies. The database was assessed to identify naturally occurring sudden cardiovascular deaths, age 14-23 years, 2000 to 2014. In addition to the Medical Examiner evaluation, gross and
3
ACCEPTED MANUSCRIPT
histopathologic cardiac examinations were conducted by expert cardiovascular pathologists at the Jesse E. Edwards Registry (Saint Paul, MN)(ERD;AMB;SM-B). A competitive student-athlete in organized high school and college sports
RI PT
programs was defined as: One who participates in an organized team or individual sport that requires regular competition against others as a central component, places a high premium on excellence and achievement and requires some form of systematic
SC
(and usually intense) training (9).
To calculate the relative incidence of competitive athlete vs. non-athlete events,
M AN U
we constructed the size of at-risk populations within Hennepin County, 2000 to 2014, from publically available data (10). Specifically, this included total student enrollment in those high schools (n = 131) and colleges (n = 4) with athletic programs that practice some measure of preparticipation screening for athletes, usually history and physical
TE D
examination (1). In Hennepin County, athlete participation rates for individual colleges and high schools were estimated by utilizing combined data from the National Center for Educational Statistics and the Minnesota State High School League Sponsored Activity
EP
Participation Survey.”
RESULTS
AC C
During the 15-year study period, 39 cases of sudden death with virtually
instantaneous collapse, (2,3), were identified. Twelve were excluded due to confounding toxicology results or a known history of congenital heart disease. Therefore, 27 sudden deaths due to a variety of cardiovascular diseases constitute the final study group (Table 1). Ages were 14 to 23 years; 22 (81%) were males. Sixteen decedents were white, 9 were black, and one each were Hispanic or Asian.
4
ACCEPTED MANUSCRIPT
Of the 27 deaths, only 3 occurred in individuals who were or had been participants in formal competitive athletic programs in high school or college (1-4): a 22-year-old college football player with hypertrophic cardiomyopathy (HC); a 19-year-
RI PT
old high school wrestler with a structurally normal heart; and a 17-year-old high school basketball player with an anomalous origin of the left main coronary artery. Of these 3, sudden death occurred while sedentary in 2 and during intense physical exertion in one.
SC
The other 24 young people, without a history of systematic engagement in (or disqualification from) organized competitive sports programs, died while sedentary or
M AN U
during mild exertion consistent with normal daily activities, including while in the home. Therefore, while physical activity can promote arrhythmia-related sudden death in the presence of underlying cardiovascular disease (1-4), only one of the 27 deaths in this study (3.7%) was associated with intense exertion.
TE D
Overall, structural cardiac abnormalities were identified in 21 of the 27 cases, commonly including: 6 with autopsy findings consistent with HC (3-5), 4 with arrhythmogenic right ventricular cardiomyopathy (ARVC) (2), 5 with either
EP
atherosclerotic coronary artery disease, thrombotic coronary occlusion or nonatherosclerotic coronary abnormality, 2 with ruptured ascending aorta, 2 with
AC C
anomalous left main coronary artery and 1 each with myocarditis and hypertensive heart disease (Table 1). Six other deaths were judged at autopsy to be associated with structurally normal hearts based on heart weight standards (11) and LV wall thickness <15mm.
The numerical ratio of sudden deaths in non-athletes to sudden deaths in competitive athletes is 24:3 or 8:1. Incidence of 24 non-athlete sudden deaths among
5
ACCEPTED MANUSCRIPT
946,889 person-years was 1:39,454, substantially exceeding by 3-fold that for the 3 sudden deaths in competitive athletes among 361,841 person-years (1:120,614;
DISCUSSION
RI PT
p=0.07) (Figure 1).
The ongoing debate concerning preparticipation cardiovascular screening for competitive athletes (history and physical examination with or without 12-lead ECGs)
SC
(1,2,8,11,12) to date has failed to address a fundamental issue – i.e., the frequency of sudden deaths occurring in young people in the general population who are not
M AN U
engaged in competitive sports. Indeed, an ethical dilemma has emerged in this regard because non-athletes may die suddenly of the same genetic and/or congenital cardiovascular diseases as do athletes, but by convention are customarily excluded from the opportunity to have these otherwise unsuspected conditions identified through
TE D
systematic screening (6,7).
The present data show, in a defined forensic population, that sudden deaths due to cardiovascular disease in young non-athletes numerically exceeded those in
EP
competitive athletes 8-fold, as well as by 3-fold with respect to incidence, and were most commonly due to HC, ARVC and atherosclerotic coronary artery disease or
AC C
coronary anomalies. Furthermore, almost 50% of the non-athlete deaths were due to genetic and/or congenital heart diseases that probably would have been identifiable by some form of cardiovascular screening currently available only to competitive athletes (1). These comparative data are also consistent with cardiovascular death rates in U.S. high school students that overall are 2.7-fold higher than that specifically for athletes (13).
6
ACCEPTED MANUSCRIPT
We cannot exclude the possibility that standard history and physical examination preparticipation athlete screening during the study period may have inadvertently affected the prevalence gap between sudden death in athletes and non-athletes we
RI PT
have reported, i.e., athletes may have been identified and disqualified from competitive sports with reduction in risk, who therefore do not appear in our forensic tabulation. However, data on identification of heart disease and disqualification through
SC
preparticipation screening of high school and college athletes are not available for
Hennepin County, MN or elsewhere. Nevertheless, given the rarity of many of the
M AN U
congenital and/or genetic diseases known to cause sudden death in the youthful general population, and likelihood of detection by clinical screening with history and physical examination (1), we do not believe that the theoretic identification of athletes with cardiovascular disease by preparticipation screening could have significantly
TE D
influenced our mortality data.
In conclusion, cardiovascular-related sudden deaths occur no more frequently (and probably less commonly) in competitive athletes compared to non-athletes. This
EP
observation underscores a major ethical limitation inherent in confining cardiovascular screening to trained athletes engaged in competitive sports programs, as is the current
AC C
practice in the U.S. and elsewhere.
7
ACCEPTED MANUSCRIPT
LEGEND Figure 1.
Incidence of sudden deaths (SDs) in high school and college students
comparing competitive athletes and non-athletes, 2000 to 2014, in Hennepin County,
AC C
EP
TE D
M AN U
SC
SDs due to genetic and/or congenital heart disease (i.e., 3:1).
RI PT
Minnesota. Non-athletes greatly exceed athletes in person-years and the number of
8
ACCEPTED MANUSCRIPT
1.
Maron BJ, Friedman RA, Kligfield P, Levine BD, Viskin S, Chaitman BR, Okin PM, Saul JP, Salberg L, Van Hare GF, Soliman EZ, Chen J, Matherne GP, Bolling SF, Mitten MJ, Caplan A, Balady GJ, Thompson PD. Assessment of the
RI PT
12-lead electrocardiogram as a screening test for detection of cardiovascular disease in general healthy populations of young people (12-25 years of age). Circulation. 2014;130:1303–34; J Am Coll Cardiol 2014;64:1479–1514.
Corrado D, Basso C, Pavei A, Michieli P, Schiavon M, Thiene G. Trends in
SC
2.
sudden cardiovascular death in young competitive athletes after implementation
3.
M AN U
of a preparticipation screening program. JAMA 2006;296:1593–601. Maron BJ, Doerer JJ, Haas TS, Tierney DM, Mueller FO. Sudden deaths in young competitive athletes: analysis of 1866 deaths in the United States, 19802006. Circulation 2009;119:1085–1092.
Maron BJ, Carney KP, Lever HM, Lewis JF, Barac I, Casey SA, Sherrid MV.
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4.
Relationship of race to sudden cardiac death in competitive athletes with hypertrophic cardiomyopathy. J Am Coll Cardiol 2003;41:974-980. Maron BJ. Sudden death in young athletes. N Engl J Med 2003;349:1064-1075.
6.
Maron BJ, Winkel BG, Tfelt-Hansen J. Perspectives on cardiovascular
EP
5.
7.
AC C
screening. JAMA 2015;313:31-32.
Maron BJ, Friedman RA, Caplan A. Ethics of preparticipation cardiovascular
screening for athletes. Nat Rev Cardiol 2015;12:375-378.
8.
Corrado D, Pelliccia A, Bjornstad HH, Vanhees L, Biffi A, Borjesson M,
Panhuyzen-Goedkoop N, Deligiannis A, Solberg E, Dugmore D, Mellwig KP, Assanelli D, Delise P, van-Buuren F, Anastasakis A, Heidbuchel H, Hoffmann E,
9
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Fagard R, Priori SG, Basso C, Arbustini E, Blomstrom-lundqvist C, McKenna WJ, Thiene G. Cardiovascular pre-participation screening of young competitive athletes for prevention of sudden death: proposal for a common European
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protocol. Consensus statement of the study group of sport cardiology of the working group of cardiac rehabilitation and exercise physiology and the working
Cardiology. Eur Heart J 2005;26:516-524. 9.
SC
group of myocardial and pericardial diseases of the European Society of
Maron BJ, Zipes DP, Kovacs RJ. Eligibility and disqualification recommendations
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for competitive athletes with cardiovascular abnormalities: preamble, principles and general considerations: a scientific statement from the American Heart Association and American College of Cardiology. J Am Coll Cardiol 2015;66:2343-2349. Circulation 2015;132:e256-261.
Minnesota Department of Education. Data Reports and Analytics-Student.
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10.
Available at http://w20.education.state.mn.us/MDEAnalytics/Data.jsp. Accessed February 16, 2015.
Kitzman DW, Scholz DG, Hagen PT, Ilstrup DM, Edwards WD. Age-related
EP
11.
chanes in normal human hearts during the first 10 decades of life. Part II
AC C
(Maturity): A quantitative anatomic study of 765 specimens from subjects 20 to 99 years old. Mayo Clin Proc 1988;137-146.
12.
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.
10
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Rutten-Ramos S, Garberich RF, Haas TS, Maron BJ. Cardiovascular deaths in student-athletes and in the general population: are trained athletes really at
EP
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SC
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increased risk? Circulation 2013;128:A18424.
AC C
13.
11
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Table 1. Sudden Deaths in 27 Young People in Hennepin County, Minnesota
B B W
HC; 510g; LV: 19 mm Anomalous origin of LCA from right sinus Structurally normal heart: 448 g
ATHLETES 25 26 27
22 M 17 M 19 M
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12
Circumstances of death After coaching Sedentary (bed) Sedentary (home) Home Sedentary (home) After bath Driving Sedentary (home) Sedentary (bed) Sedentary (bed) Sedentary (bed) Sedentary (home) Home Standing at bus stop Bathroom after practice Sedentary Carrying luggage Bedroom Bedroom Bedroom Bedroom Sedentary (at work) Sedentary (bed) Bathroom
SC
Diagnosis CAD - LAD 75% CAD - proximal LAD 75% CAD - LAD 75% (thrombus) RCA thrombus with posterior LV infarct Fibromuscular dysplasia HC; 405g/LV: 18 mm* HC; 520g/LV: 19 mm HC; 440g/LV: 17 mm* HC; 530g/LV: 18 mm Probable HC; 400 g; LV: 16mm Morbid obesity; 640g/LV: 17 mm† ARVC ARVC ARVC ARVC Anomalous origin of LCA from right sinus Structurally normal heart: 346 g Structurally normal heart: 266 g Structurally normal heart: 450 g Structurally normal heart: 375 g Structurally normal heart: 340 g Ruptured aorta (dissection) Ruptured aorta (coarctation) Acute myocarditis
M AN U
Race W B B B W W W H W B W W W B W B W W W A W W W W
EP
Age/Gender 22 M 21 M 20 M 23F 21 F 21 F 19 M 23 M 23 M 23 M 20 M 14 M 23 M 22 M 21 M 15 F 19 M 23 F 23 M 22 M 21 M 23 M 22 M 19 M
AC C
No. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24
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NON-ATHLETES
Sedentary (dormitory) Playing basketball In bed
ACCEPTED MANUSCRIPT
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Abbreviations: A = Asian; ARVC = arrhythmogenic right ventricular cardiomyopathy; B = black; CAD = coronary artery disease; F = female; g = grams (for heart weight); H = Hispanic; HC = hypertrophic cardiomyopathy; LAD = left anterior descending; LCA = left coronary artery; LV = left ventricular (for maximum LV wall thickness); M = male; RCA = right coronary artery; W = white
AC C
EP
TE D
M AN U
SC
Symbols: * histopathology shows myocyte disarray † 373 pounds; 66 inches Ŧ increased cardiac weight of uncertain etiology
13
ACCEPTED MANUSCRIPT
3 SDs/15 y (0.2/y)
361,841 (28%)
SC
Athletes
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Figure 1
EP
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946,889 (72%)
AC C
NonAthletes
M AN U
1 : 120,614
3:1 24 SDs/15 y (1.7/y)
1 : 39,454
S0002-9149(16)30148-5
DOI:
10.1016/j.amjcard.2016.01.026
Reference:
AJC 21672
To appear in:
The American Journal of Cardiology
Received Date: 20 November 2015 Revised Date:
13 January 2016
Accepted Date: 18 January 2016
Please cite this article as: Maron BJ, Haas TS, Duncanson ER, Garberich RF, Baker AM, MackeyBojack S, Comparison of the Frequency of Sudden Cardiovascular Deaths in Young Competitive Athletes vs. Non-athletes: should we really screen only Athletes?, The American Journal of Cardiology (2016), doi: 10.1016/j.amjcard.2016.01.026. 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 proof before it is published in its final 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.
ACCEPTED MANUSCRIPT
COMPARISON OF THE FREQUENCY OF SUDDEN CARDIOVASCULAR DEATHS
RI PT
IN YOUNG COMPETITIVE ATHLETES VS. NON-ATHLETES:
SC
SHOULD WE REALLY SCREEN ONLY ATHLETES?
Barry J. Maron, MDa, Tammy S. Haas, RNa, Emily R. Duncanson, MDb,
M AN U
Ross F. Garberich, MSa, Andrew M. Baker, MDc, Shannon Mackey-Bojack, MDb
a
Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation,
TE D
Minneapolis, MN; b
Jesse E. Edwards Registry of Cardiovascular Disease, Nasseff Heart Center, St. Paul, MN; Hennepin County Medical Examiner's Office, Minneapolis, MN
EP
c
AC C
The authors report no conflicts of interest. Address for Correspondence: Barry J. Maron, MD Minneapolis Heart Institute Foundation 920 East 28th Street, Suite 620 Minneapolis, MN 55407 612-863-3996/Fax: 612-863-3875 [email protected]
Word count: 2009 1
ACCEPTED MANUSCRIPT
ABSTRACT The issue of sudden death in young athletes and consideration for the most practical and optimal strategy to identify those genetic and/or congenital heart diseases
RI PT
responsible for these tragic events continues to be debated. However, proponents of broad-based and mandatory national preparticipation screening, including with 12-lead ECGs have confined the focus to a relatively small segment of the youthful population
SC
who choose to engage in competitive athletic programs at the high school, college and elite-professional level. Therefore, lost in this discussion of preparticipation screening of
M AN U
athletes is that the larger population of young people not involved in competitive sports (and, therefore, a priori, excluded from systematic screening) who nevertheless may die suddenly of the same cardiovascular diseases as athletes. To substantiate this hypothesis, we accessed the forensic Hennepin County, Minnesota registry in which
TE D
cardiovascular sudden deaths were 8-fold more common in non-athletes (n=24) than athletes (n=3), and 3-fold more frequent in terms of incidence. The most common diseases responsible for sudden death were hypertrophic cardiomyopathy (n=6) and
EP
arrhythmogenic right ventricular cardiomyopathy (n=4). These data raise ethical considerations inherent in limiting systematic screening for unsuspected genetic and/or
AC C
congenital heart disease to competitive athletes.
Key Words:
Sudden death Athletes Screening
2
ACCEPTED MANUSCRIPT
Introduction Screening healthy general populations for cardiovascular disease has become a topic of considerable interest, triggered by the high visibility afforded sudden deaths in
RI PT
young competitive athletes (1-5). Attention directed toward these often highly visible and public events may have created the misconception that sudden deaths in high school and college-aged individuals occur predominantly (or even exclusively) in
SC
student-athletes (1-5).
A debate has emerged concerning the most effective strategy for identifying the
M AN U
unsuspected genetic and/or congenital diseases responsible for these tragic events (1,2, 6-8). Should systematic screening be confined to those who choose to engage in competitive sports, or should non-athletes (who can harbour the same potentially lethal cardiovascular diseases as athletes) be included in this process? (7) We have
TE D
addressed this highly relevant issue for the ongoing preparticipation screening conversation by interrogating a large forensic database to assess the frequency of genetic and/or congenital cardiovascular diseases causing sudden death in trained
EP
athletes vs. young people not engaged in organized sports programs. METHODS
AC C
We accessed the case records of the Medical Examiner of Hennepin County, the
largest of the 87 Minnesota counties, representing the Minneapolis metropolitan area. Hennepin County includes 1,198,778 residents (23% of the state); all sudden deaths <40 years of age undergo complete autopsy and toxicologic studies. The database was assessed to identify naturally occurring sudden cardiovascular deaths, age 14-23 years, 2000 to 2014. In addition to the Medical Examiner evaluation, gross and
3
ACCEPTED MANUSCRIPT
histopathologic cardiac examinations were conducted by expert cardiovascular pathologists at the Jesse E. Edwards Registry (Saint Paul, MN)(ERD;AMB;SM-B). A competitive student-athlete in organized high school and college sports
RI PT
programs was defined as: One who participates in an organized team or individual sport that requires regular competition against others as a central component, places a high premium on excellence and achievement and requires some form of systematic
SC
(and usually intense) training (9).
To calculate the relative incidence of competitive athlete vs. non-athlete events,
M AN U
we constructed the size of at-risk populations within Hennepin County, 2000 to 2014, from publically available data (10). Specifically, this included total student enrollment in those high schools (n = 131) and colleges (n = 4) with athletic programs that practice some measure of preparticipation screening for athletes, usually history and physical
TE D
examination (1). In Hennepin County, athlete participation rates for individual colleges and high schools were estimated by utilizing combined data from the National Center for Educational Statistics and the Minnesota State High School League Sponsored Activity
EP
Participation Survey.”
RESULTS
AC C
During the 15-year study period, 39 cases of sudden death with virtually
instantaneous collapse, (2,3), were identified. Twelve were excluded due to confounding toxicology results or a known history of congenital heart disease. Therefore, 27 sudden deaths due to a variety of cardiovascular diseases constitute the final study group (Table 1). Ages were 14 to 23 years; 22 (81%) were males. Sixteen decedents were white, 9 were black, and one each were Hispanic or Asian.
4
ACCEPTED MANUSCRIPT
Of the 27 deaths, only 3 occurred in individuals who were or had been participants in formal competitive athletic programs in high school or college (1-4): a 22-year-old college football player with hypertrophic cardiomyopathy (HC); a 19-year-
RI PT
old high school wrestler with a structurally normal heart; and a 17-year-old high school basketball player with an anomalous origin of the left main coronary artery. Of these 3, sudden death occurred while sedentary in 2 and during intense physical exertion in one.
SC
The other 24 young people, without a history of systematic engagement in (or disqualification from) organized competitive sports programs, died while sedentary or
M AN U
during mild exertion consistent with normal daily activities, including while in the home. Therefore, while physical activity can promote arrhythmia-related sudden death in the presence of underlying cardiovascular disease (1-4), only one of the 27 deaths in this study (3.7%) was associated with intense exertion.
TE D
Overall, structural cardiac abnormalities were identified in 21 of the 27 cases, commonly including: 6 with autopsy findings consistent with HC (3-5), 4 with arrhythmogenic right ventricular cardiomyopathy (ARVC) (2), 5 with either
EP
atherosclerotic coronary artery disease, thrombotic coronary occlusion or nonatherosclerotic coronary abnormality, 2 with ruptured ascending aorta, 2 with
AC C
anomalous left main coronary artery and 1 each with myocarditis and hypertensive heart disease (Table 1). Six other deaths were judged at autopsy to be associated with structurally normal hearts based on heart weight standards (11) and LV wall thickness <15mm.
The numerical ratio of sudden deaths in non-athletes to sudden deaths in competitive athletes is 24:3 or 8:1. Incidence of 24 non-athlete sudden deaths among
5
ACCEPTED MANUSCRIPT
946,889 person-years was 1:39,454, substantially exceeding by 3-fold that for the 3 sudden deaths in competitive athletes among 361,841 person-years (1:120,614;
DISCUSSION
RI PT
p=0.07) (Figure 1).
The ongoing debate concerning preparticipation cardiovascular screening for competitive athletes (history and physical examination with or without 12-lead ECGs)
SC
(1,2,8,11,12) to date has failed to address a fundamental issue – i.e., the frequency of sudden deaths occurring in young people in the general population who are not
M AN U
engaged in competitive sports. Indeed, an ethical dilemma has emerged in this regard because non-athletes may die suddenly of the same genetic and/or congenital cardiovascular diseases as do athletes, but by convention are customarily excluded from the opportunity to have these otherwise unsuspected conditions identified through
TE D
systematic screening (6,7).
The present data show, in a defined forensic population, that sudden deaths due to cardiovascular disease in young non-athletes numerically exceeded those in
EP
competitive athletes 8-fold, as well as by 3-fold with respect to incidence, and were most commonly due to HC, ARVC and atherosclerotic coronary artery disease or
AC C
coronary anomalies. Furthermore, almost 50% of the non-athlete deaths were due to genetic and/or congenital heart diseases that probably would have been identifiable by some form of cardiovascular screening currently available only to competitive athletes (1). These comparative data are also consistent with cardiovascular death rates in U.S. high school students that overall are 2.7-fold higher than that specifically for athletes (13).
6
ACCEPTED MANUSCRIPT
We cannot exclude the possibility that standard history and physical examination preparticipation athlete screening during the study period may have inadvertently affected the prevalence gap between sudden death in athletes and non-athletes we
RI PT
have reported, i.e., athletes may have been identified and disqualified from competitive sports with reduction in risk, who therefore do not appear in our forensic tabulation. However, data on identification of heart disease and disqualification through
SC
preparticipation screening of high school and college athletes are not available for
Hennepin County, MN or elsewhere. Nevertheless, given the rarity of many of the
M AN U
congenital and/or genetic diseases known to cause sudden death in the youthful general population, and likelihood of detection by clinical screening with history and physical examination (1), we do not believe that the theoretic identification of athletes with cardiovascular disease by preparticipation screening could have significantly
TE D
influenced our mortality data.
In conclusion, cardiovascular-related sudden deaths occur no more frequently (and probably less commonly) in competitive athletes compared to non-athletes. This
EP
observation underscores a major ethical limitation inherent in confining cardiovascular screening to trained athletes engaged in competitive sports programs, as is the current
AC C
practice in the U.S. and elsewhere.
7
ACCEPTED MANUSCRIPT
LEGEND Figure 1.
Incidence of sudden deaths (SDs) in high school and college students
comparing competitive athletes and non-athletes, 2000 to 2014, in Hennepin County,
AC C
EP
TE D
M AN U
SC
SDs due to genetic and/or congenital heart disease (i.e., 3:1).
RI PT
Minnesota. Non-athletes greatly exceed athletes in person-years and the number of
8
ACCEPTED MANUSCRIPT
1.
Maron BJ, Friedman RA, Kligfield P, Levine BD, Viskin S, Chaitman BR, Okin PM, Saul JP, Salberg L, Van Hare GF, Soliman EZ, Chen J, Matherne GP, Bolling SF, Mitten MJ, Caplan A, Balady GJ, Thompson PD. Assessment of the
RI PT
12-lead electrocardiogram as a screening test for detection of cardiovascular disease in general healthy populations of young people (12-25 years of age). Circulation. 2014;130:1303–34; J Am Coll Cardiol 2014;64:1479–1514.
Corrado D, Basso C, Pavei A, Michieli P, Schiavon M, Thiene G. Trends in
SC
2.
sudden cardiovascular death in young competitive athletes after implementation
3.
M AN U
of a preparticipation screening program. JAMA 2006;296:1593–601. Maron BJ, Doerer JJ, Haas TS, Tierney DM, Mueller FO. Sudden deaths in young competitive athletes: analysis of 1866 deaths in the United States, 19802006. Circulation 2009;119:1085–1092.
Maron BJ, Carney KP, Lever HM, Lewis JF, Barac I, Casey SA, Sherrid MV.
TE D
4.
Relationship of race to sudden cardiac death in competitive athletes with hypertrophic cardiomyopathy. J Am Coll Cardiol 2003;41:974-980. Maron BJ. Sudden death in young athletes. N Engl J Med 2003;349:1064-1075.
6.
Maron BJ, Winkel BG, Tfelt-Hansen J. Perspectives on cardiovascular
EP
5.
7.
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screening. JAMA 2015;313:31-32.
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Rutten-Ramos S, Garberich RF, Haas TS, Maron BJ. Cardiovascular deaths in student-athletes and in the general population: are trained athletes really at
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increased risk? Circulation 2013;128:A18424.
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Table 1. Sudden Deaths in 27 Young People in Hennepin County, Minnesota
B B W
HC; 510g; LV: 19 mm Anomalous origin of LCA from right sinus Structurally normal heart: 448 g
ATHLETES 25 26 27
22 M 17 M 19 M
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Circumstances of death After coaching Sedentary (bed) Sedentary (home) Home Sedentary (home) After bath Driving Sedentary (home) Sedentary (bed) Sedentary (bed) Sedentary (bed) Sedentary (home) Home Standing at bus stop Bathroom after practice Sedentary Carrying luggage Bedroom Bedroom Bedroom Bedroom Sedentary (at work) Sedentary (bed) Bathroom
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Diagnosis CAD - LAD 75% CAD - proximal LAD 75% CAD - LAD 75% (thrombus) RCA thrombus with posterior LV infarct Fibromuscular dysplasia HC; 405g/LV: 18 mm* HC; 520g/LV: 19 mm HC; 440g/LV: 17 mm* HC; 530g/LV: 18 mm Probable HC; 400 g; LV: 16mm Morbid obesity; 640g/LV: 17 mm† ARVC ARVC ARVC ARVC Anomalous origin of LCA from right sinus Structurally normal heart: 346 g Structurally normal heart: 266 g Structurally normal heart: 450 g Structurally normal heart: 375 g Structurally normal heart: 340 g Ruptured aorta (dissection) Ruptured aorta (coarctation) Acute myocarditis
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Race W B B B W W W H W B W W W B W B W W W A W W W W
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Age/Gender 22 M 21 M 20 M 23F 21 F 21 F 19 M 23 M 23 M 23 M 20 M 14 M 23 M 22 M 21 M 15 F 19 M 23 F 23 M 22 M 21 M 23 M 22 M 19 M
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No. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24
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NON-ATHLETES
Sedentary (dormitory) Playing basketball In bed
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Abbreviations: A = Asian; ARVC = arrhythmogenic right ventricular cardiomyopathy; B = black; CAD = coronary artery disease; F = female; g = grams (for heart weight); H = Hispanic; HC = hypertrophic cardiomyopathy; LAD = left anterior descending; LCA = left coronary artery; LV = left ventricular (for maximum LV wall thickness); M = male; RCA = right coronary artery; W = white
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Symbols: * histopathology shows myocyte disarray † 373 pounds; 66 inches Ŧ increased cardiac weight of uncertain etiology
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3 SDs/15 y (0.2/y)
361,841 (28%)
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Athletes
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Figure 1
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946,889 (72%)
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NonAthletes
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1 : 120,614
3:1 24 SDs/15 y (1.7/y)
1 : 39,454