Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Preamble, Principles, and General Considerations

AHA/ACC SCIENTIFIC STATEMENT

Eligibility and Disqualiﬁcation

Recommendations for Competitive Athletes

With Cardio vascular Abnormalities:

Preamble, Principles, and

General Considerations

A Scientiﬁc Statement From the American Heart Association and American College of Cardiology

Barry J. Maron, MD, FACC, Co-Chair* Douglas P. Zipes, MD, FAHA, MACC,

Co-Chair*

Richard J. Kovacs, MD, FAHA, FACC,

Co-Chair*

This document addresses medical issues related to

trained athletes with cardiovascular abnormaliti es.

The objective is to present, in a readily useable

format, consensus recommendations and guidelines

principally addressing criteria for eligibility and

disqualiﬁcation from organized competitive sports for

the purpose of ensuring the health and safety of

young athletes. Recognizing certain medical risks

imposed on athletes with cardiovascular disease, it

is our aspiration that the recommendat ions that

constitute this document will serve as a useful guide

to the practicing community for clinical decision

making. The ultimate goal is prevention of sudden

death in the young, althoug h it is als o import ant n ot

to unfairly o r unnecessarily remove people from a

healthy athletic lifest yle or competitive sports (that

may be physiologically and psychologically inter-

twined with goo d quality of life and med ical well-

being) becaus e of fear of lit igation. It is our goal that

the recommendations in this document, together

with sound clinical j udgment, will lead to a healthier,

safer playing ﬁeld for young competitive athletes.

*On behalf of the American Heart Association Electrocardiography and

Arrhythmias Committee of the Council on Clinical Cardiology, Council on

Cardiovascular Disease in the Young, Council on Cardiovascular and

Stroke Nursing, Council on Functional Genomics and Translational

Biology, and the American College of Cardiology.

The American Heart Association and the American College of Car-

diology make every effort to avoid any actual or potential conﬂicts of

interest that may arise as a result of an outside relationship or a

personal, professional, or business interes t of a member of the writi ng

panel. Speciﬁcally, all members of the writing group are required to

complete and submit a Disclosure Questionnaire showing all such

relationships that might be perceived as real or pote ntial conﬂicts of

interest. The Task Force reports for these proc eedings are available

online at www.onlinejacc.org (J Am Coll Cardiol 2015;XX:000–000;

000–000; 000–000; 000–000; 000–000; 000–000; 000–000; 000–000;

000–000; 000–000; 000–000; 000–000; 000–000; 000–000; and

000–000).

This statement was approved by the American Heart Association

Science Advisory and Coordinating Committee on June 24, 2015, and

the American Heart Association Executive Committee on July 22, 2015,

and by the American College of Cardiology Board of Trustees and

Executive Committee on June 3, 2015.

The American College of Cardiology requests that this document be

cited as follows: Maron BJ, Zipes DP, Kovacs RJ; on behalf of the American

Heart Association Electrocardiography and Arrhythmias Committee of the

Council on Clinical Cardiology, Council on Cardiovascular Disease in the

Young, Council on Cardiovascular and Stroke Nursing, Council on Func-

tional Genomics and Translational Biology, and the American College of

Cardiology. Eligibility and disqualiﬁcation recommendations for

competitive athletes with cardiovascular abnormalities: preamble, prin-

ciples, and general considerations: a scientiﬁc statement from the

American Heart Association and American College of Cardiology. J Am

Coll Cardiol 2015;xx:–.

This article has been copublished in Circulation.

Copies: This document is available on the World Wide Web sites of the

American Heart Association (http://my.americanheart.org) and the

American College of Cardiology (www.acc.org). For copies of this docu-

ment, please contact Elsevier Inc. Reprint Department via fax (212-633-

3820) or e-mail ([email protected]).

Permissions: Multiple copies, modiﬁcation, alteration, enhancement,

and/or distribution of this document are not permitted without the ex-

press permission of the American College of Cardiology. Requests may be

completed online via the Elsevier site (http://www.elsevier.com/about/

policies/author-agreement/obtaining-permission).

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY VOL. - ,NO.- ,2015

ª 2015 BY THE AMERICAN HEART ASSOCIATION, INC. AND

THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION

ISSN 0735-1097/$36.00

http://dx.doi.org/10.1016/j.jacc.2015.09.032

PUBLISHED BY ELSEVIER INC.

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HISTORICAL CONTEXT

There have been 3 prior d ocuments, all sponsored by

the American College of Cardiology (ACC) (1–3),that

addressed eligibili ty and disqualiﬁcation criteria for com-

petitive athletes with cardiovascular diseases: Bethesda

Conferences 16 (1985), 26 (1994), and 36 (2005), published

and used over a 30-year period . Each of the 3 initiatives

(and the present American Heart Association (AHA)/ACC

scientiﬁc statement) were driven by the tenet that young

trained athletes with underlying cardiovascular abn or-

malities are likely at some increase in risk for sudden

cardiac death (usually on the athletic ﬁeld) compared to

nonathletes or competitive athletes without cardiovas-

cular disease (4–8).

All 3 Bethesda Conferences and the present derived

AHA/ACC document provide expert consensus recom-

mendations. These insights use 1) the experience a nd

expertise of the panelists (i.e., individual and collective

judgments, using the “art of medicine”) and 2) available

scientiﬁc ev idence that estimates the medical risk in

athletes with underlying acquired, genetic, and congen-

ital heart abnormali ties imposed by the unique lifestyle of

engagement in competitive sports.

These insig hts c an be applied to decision making f or

temporary or permanent disqualiﬁcati on versus eligibility

of athletes with probable or conclusive evidence of car-

diovascular d isease; however, the scientiﬁcdatasup-

porting many of the recommendations in this document

are u navoidably limited, as ev idenced by the frequent

assignment of a Level of Evidence C. Nevertheless, each

of the 3 prior Bethesda Conferences has served the prac-

ticing community well, o ffering clinicians a consensus

reference document that is potentially helpful in re-

solving predictably difﬁcult clinical dilemmas. It is our

expectation that the present conservative AHA/ACC

scientiﬁc statement will foll ow in that tradition. The ﬁnal

document was approved by all participants and assigned

outside reviewers.

IMPETUS FOR THE PRESENT DOCUMENT

There are a number of factors that support the decision to

update t he 36th Bethesda Co nference here (3).First,

sudden cardia c deaths in young healt hy athletes remain

tragic and counterin tuitive even ts, subject to persi stently

high public visibility, emotion, and media scrutiny, with

potential legal liability considerations. Therefore, a s trong

impetus remains to identify high-risk athletes to reduce

their exposure to sudden death risk. Indeed, there is

an ever-expanding population of competitive athletes,

including those participating in new and emerging orga-

nized sports. Second, cardiovascular medicine changes

rapidly. As evid ence of t his, in the almost 10 years since

publication of the 36th Bethesda Conference (3),new

conditions associated with sudden death in the young

have been recognized, and knowledge of the responsible

diseases a nd inh erent ris ks of sudden card iac death in the

young has evolved (4–8).Asaresult,someselectedareas

of the 36th Bethesda C onference may have become

obsolete, and novel issues not previously addressed, have

emerged. Third, an increasing number of adults with

congenital heart disease and cardiomyopa thies are now

being recognized (often with surgical pallia tion or

correction) who wish to engage in competitive athletics

and require contemporary recommendations. In addition ,

the increasing penetration into cardiovascular practice of

implantable devices (e.g., pacema kers and cardioverter-

deﬁbrillators) has created greater numbers of physically

active young people with genetic heart diseases who have

had devices implanted and wh o may aspire to participa-

tion in co mpetitive athletics. Recently, th ere ha s been

greater deployment of automatic external de ﬁbri llators at

athletic events in recognition of sudden death risk in

young athl etes. Finally, the practicing cardiovascular

community deserves and expects th e most up-to-date

information on which to ma ke important cl inical de-

cisions regarding eligibility versus disqualiﬁcation of

competitive athletes.

DEFINITIONS

As in the 3 B ethesda C onferences (1–3),thebasicdeﬁni-

tion of a competitive athlete remains unchanged: 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 require s so me fo rm of systematic

(and usually intense) training. Therefore, organized

competitive sport s are rega rded as a distinctive a ctivity

and lifestyle. An important principle concerns whether

competitive athletes with either known or unsuspected

cardiovascular disease can be expected to properly judge

when it is prudent to terminate physical exertion. Indeed,

the unique pressures of organized sports do not allow

athletes to exert strict individual control over their level

of exertion or reliably discern when cardiac-related

symptomsorwarningsignsoccurthatshoulddictate

termination of the activity.

Furthermore, it is emphas ized tha t these AHA /ACC

recommendations should not be regarded as a general

overriding injunction against all forms of exercise.

Notably, this document is concerned only with organized

and sanctioned competitive sports participation, such as

most commonly found in middle school, high school, and

college (1–3), and not with pure ly recreat ional physica l

activities (9). The panel recognizes and strongly supports

the well-documented health beneﬁts of exercise, with

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regular physical activities encouraged for those people

who have been removed from organized competitive

athletics, or who elect to participate in a wide range of

recreational sporting activities.

Although the Bethesda Conferences and the present

document are largely focused on student-athletes of high

school and college age (primarily 12 to 25 years old), the

panel recognizes the need to also be more expansive with

regard to age of the athletes, that is, that participation in

competitive athletics now increasingly begins earlier in a

variety of youth sports before high school and continues

beyond college. This consideration is also sub stantiated

by the realization that inherited arrhythmia syndromes

can impact very young people and that patients with

genetic, congenital, or acquired heart diseases now

engage in competitive athletic s at more advanced ages.

However, because systematic preparticipation screening

in the U nited State s does n ot usually begin before high

school (4), recognition of cardiovascular disease in such

younger athletes is unpredictable.

CAUSES OF SUDDEN DEATH IN ATHLETES

The cardiovascular causes of sudden death in yo ung

athletes have been well documented in forensic data-

bases (5–9). These deaths occur in both s exes (although

more commonly in males, by 9:1); in minorities, promi-

nently including African-A mericans and in a wide range

of individual and team sports. In the United States,

among people <35 years old, genetic heart diseases

predominate, with hypertrophic cardiomyopathy being

the most c ommon, accounting for at least one-third of

the mortality in autopsy-based a thlete study populati ons

(5–7). Congenital coronary anomalies (usually those of

wrong sinus origin) are second in frequency, occurring in

z15% to 20% of cases. Other less common diseases, each

responsible for z5% or fewer of t hese sudden d eaths,

include myocarditis, aortic valve stenosis, aorti c dissec-

tion/rupture (including cases of the Marfan phenotype),

atherosclerotic c oronary a rtery disease, ion channelo-

pathies, and arrhythmogenic right ventricular cardio-

myopathy. In addition, commotio cordis (i.e., sudden

deathcausedbyblunt,nonpenetratingchestblows,

associated with structurally normal hearts) is more

common as a cause of sudden death in young athletes

than many of the aforementioned structural cardiovas-

cular disease s (10).

Regional variations in the causes of sudden death

may exist (6–9).Notableamongthese,arrhythmogenic

right ventricular cardiomyopathy has been reported as

the most common cause of sudden death in young

athletes based on reports from the Veneto region of Italy

(8), whereas this disease is a much less frequent cause

of sudden death in U.S. athletes (6).Inmostathletes,

sudden death occur s in the setting of ventric ular ﬁbril-

lation, with the notable excepti on of aortic dilation that

leads to dissection and rupture. For older athletes (>35

years of age), atherosclerotic coronary artery disease is

the predominant cause of sudden death (7),butthis

occurs less frequently in younger participants .

HOW TO US E T HE DOCU MENT

Of the 15 Task Forces that make up this document, 9 are

disease (or multidisease) related. As b efore (1–3),speciﬁc

recommendations for s ports eligibility or temporary or

permanen t disqu aliﬁcation to reduce sudden death risk

areformulatedaroundtheclassiﬁc ation of sport s (Task

Force 1), which incorporates the principle that training

and competition demands may vary considerably among

competitive s ports (often within sports as well), that the

intensity of conditioning may exceed that of compet ition,

and t hat different levels of physical activity are likely

to impact underlying (and unsuspected) cardiovascular

diseases unpredictably and in different ways. Further-

more, it is difﬁcult to accurately grade or take into

account exercise intensity in v arious sports because of a

variety of factors, particularly moti vational attitudes.

Finally, as was the practice in prior Bethesda Conferences

16, 26, and 36 (1–3), the panel advises that sports partici-

pation recommendations or decisions be based on prob-

able or conﬁr med diagnostic evidence for cardiovascular

disease and not include diagno ses that are ambiguous ,

possible, or borderl ine.

The other 6 Task Forces deal with a variety of relevant

topics and issues surrounding the risks of the athletic

ﬁeld. These include strategies for preparticipation

screening (Task Force 2), use of the automatic external

deﬁbrillator on th e athletic ﬁeld (Tas k Force 12), the

impact of dietary supplements and performance-

enhancing substances (Ta sk Force 11), commotio cordis

as an acknowledged new risk o f sudden death during

sports (Task Force 13), and medical-legal perspectives

(Task Force 15). However, we should underscore t hat it is

not possibl e to foresee an d include in this do cument

every conceivable cardiova scular abnormality or clinical

situation relevant to athletes. Eligibilit y and disqualiﬁ-

cation decisions i n those particular situations would

be mad e on a ca se-by-case basis with indi vidual clinical

judgment. Each of the 15 Task Forces is cited indepen-

dently as a publication in Pub Med.

WHO SHOULD USE THIS DOCUMENT?

These recommendations are designed primarily for

cardiologists, internists, pediatricians, family medicine

physicians, and other practitioners (including team phy-

sicians) charg ed with decision-making responsibil ities

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related to the eligibil ity and disqua liﬁcation of those

competitive athletes with cardiovascular d isease.

Although this do cument essentially focuses on

disqualiﬁcation standards for trained competitive ath-

letes, particularly those in organized sanctioned pro-

grams, we also recognize that the principles espoused

herein may be, if appropriate, useful when translated to

physically active people in other circumstances, for

example, in occupations such as police ofﬁcers, ﬁre-

ﬁg hters, and pilots (11) ,aswellastoparticipantsin

certain recreational sports activities. In this regard, it

should be underscored that many people independently

choose to engage in recreational physical activities that

may in fact involve high-intensity vigorous training at

the same level of some competitive athletes. Therefore,

the use of thi s document for decision making will

require certain judgments a nd extrapolatio ns to account

for perceived differences in activity between trained

competitive athletes in organized sports and some other

physically active people. Hence, it may be possible to

selectively apply the principles contained in this docu-

ment to certain sporting activities that do not meet our

precise deﬁnition of “competitive.” Nevertheless, exces-

sive and unnecessary exercise restrictions for such people

with heart disease could potentially create physical and

psychological burdens (particularly in young children)

and are discouraged (9).

If the underlying medical considerations are similar to

high school- and college-aged athletes, the recommen-

dations in this document could be used to guide decisions

relevant to professional athletes with cardiovascular

abnormalities. However, professional athletes represent a

very small and unique subset of all competitive athletes

compared with the millions of student-athlete partici-

pants and are generally highly compensated adults with

employment contracts (12).

ASSESSMENT OF RISKS

Young people participating in competitive sports with

cardiovascular abno rmalities have limited co ntrol when

exposed to extreme and unpredictable environmental

conditions (a ssociated with alterations in blood volume,

hydration, and e lectrolytes), as a result of the unwavering

demands of sport. These circumstances can enhance the

risk for potenti ally lethal arrhythmias and sudden death,

given underlying cardiovascular disease. For many ath-

letes, removal from the lifestyle of athletic training and

competition will r educe this risk for sudden death or dis-

ease progression, even in the absence of established risk

factors related to their disease (13). However, appropriate

sports disqualiﬁcation is only one component of risk

reduction, and each of the relevant cardiovascular dis-

easesisattachedtoitsowntreatmentalgorithms,which

can include prophylactic implantation of a cardioverter-

deﬁbrillator should sudden d eath risk be judged unac-

ceptably high (14–16 ).

The present recommendations, formulated with

respect to allowable levels of sports activity, can be

regarded a s generally conservative. Certainly, this is a

prudent posture when the available evidence is limited in

many decision-mak ing areas. In this regard, the panel

acknowledges that the available data support the princi-

ple that participation in high-intensity sports is associ-

ated with an increased relativ e risk of sudden dea th in the

setting of some cardiovascular diseases (6–17).Onthe

other hand, this likelih ood canno t be det ermined wi th

certainty for each patient/athlete, and in fact may be low

in certai n peo ple. However, at present, add itional risk-

stratifying tools are not available to independently (and

more precisely) guide many of these difﬁcult medical

decisions in athletes, particularly for disea ses such as

hypertrophic cardiomyopathy (18).

Thus, it is possible that the recommendations of this

consensus panel (as with the 3 previous Bethesda Con-

ferences) (1–3) will occa sionally cause some athletes to

be unnecessarily withdraw n from competition. This is,

of course, unfortunate, because athletes derive consid-

erable self-assurance, conﬁdence, physical well-being,

andevenonoccasionﬁnancial security from these

activities. Nevertheless, the increased sudden death risk

associated with intense sports is a controllable v ariable

(by disqualiﬁcation from such sports), and we believe

the devastating impact on families a nd communities of

even infrequent sudden death events in this young

population underscores the wisdom of our conservative

recommendations.

In practice, indiv idual athletes ma y be encouraged

to change their competitive sport involvement from a

prohibited high-intensity activity to a mor e permissible

low-intensity one (i.e., usually to class IA). However, the

strategy of changing the position in whi ch an athlete

competes (e.g., from running back to place kicker in

football, or to goalie in hockey or so ccer) may be difﬁcult

to accomplish in practical terms and therefore should be

advised only if the training obligations outside of game

situations can be controlled and modiﬁed adequa tely.

RELATION OF AHA/ACC GUIDELINES

TO 36TH BE THESDA CONFERENCE

The present AHA/ACC recommendations are intended

to update (and are derived from) the prior Bethesda

Conferences (1–3).Forthemostpart,thespeciﬁcrecom-

mendations are similar or id entical to those i n the report

on the 36th Bethesda Conference (3). However, selected

recommend ations of the p resent A HA/ACC doc ument

do in fact deviate f rom those of th e 36th Bethesda

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Conference, becoming less restrictive as certain data

and observations have emerged since 2005. Nevertheless,

numerous “ gray areas” persist, for which the ass essment

of safe versus nonsafe sports participation continues to

be uncertain from a medical and scientiﬁc perspective,

with absolute certainty difﬁcult to achieve for many

cardiovascular issues. Thi s may result in differences of

opinion a mong physicia ns rega rding the exercise of

clinical judgment in individual cases. Thus, in making

certain eligibili ty or disqualiﬁcation decisions, some

physicians may rely on the more liberal guidelines in

portions of the present document, whereas others may

take a more conserv ative appro ach by adopting the more

restrictive recommendations from the 36th Bethesda

Conference.

It is also important to underscore that the recommen-

dations in this AHA/A CC document are not intended to

establish absolute mandates or the general medical (and

legal) standard of care applicable to all cases. These rec-

ommendations do not (and cannot) absolutely and arbi-

trarily replace individual cli nical judgm ent and informed

medical reasoning.

The panel recognizes that some practitioners,

depending on their perception of risk for speciﬁcindi-

vidual pat ients, may choos e to prudently deviate from the

published recommendations in selected clinical situa-

tions. Therefore, fully informed athletes with certai n

conditions may continue to engage in competitive sports

in concert with recommendations made by their physician

and athletic organization (i.e., high school or college).

Individual athletes in the past have ta ken this option to

continue or return to play, and we anticipate this w ill

occur in the future. There will always be tolerance in the

system for some ﬂexibility and individual responsibility

and choice, after the prevalent uncertainties have been

acknowledged.

As with all g uidelines, which cannot be r egarded as

rigid dictum, the speciﬁc medical clearance or disqualiﬁ-

cation recommendation in a particular case is ultimately

the responsib ility of the managing phys ician with medi-

cally relevant knowledge of the individual athlete-

patient. Although neither the 36th Bethesda Conference

or the present AHA/ACC recommendations ar bitrarily

establish the st andard of care, these documents never-

theless do provide the framework for good medical prac-

tice (19).

It is important to recognize that protection of the ath-

lete’s health and avoidance of any unreasonable risks for

sudden death during competitive a thletics should be

considered a priority in exercising individual clinical

judgment and making medical recommendations

regarding sports participation with a c ardiovascular ab-

normality. The level of importance that the athlete

personally attaches to enga gement in competit ive sports

should not be a deciding factor in formulati ng eligibility

recommendations.

Clinicians s hould also recognize that medi cal eli gibility

versus disqualiﬁcation decisions have become increas-

ingly complex . Als o, th ese decisions may be fraught with

potential legal liability ris ks. Therefore, it is unwis e to b e

unduly inﬂuenced by the l ibertarian (free wil l) desires of

athletes (with an importa nt cardiova scular abnormalit y)

willing to assume medically unreas onable risks to

participate in a sport, nor by the manag ing clinician’s

personal willingness to comply with the desires of the

individual athlete-patient. Finally, it i s important to

recognize that third-party interests (e.g., on behalf of

high schools, colleges, or professional clubs) unavoidably

contribute to the complexity in the decision-making

process, but these should not outweigh the paramount

concern for the athlete’s health and safety when making

medical eligibility recommendations.

Writing Group Disclosures

Writing Group

Member Employmen t

Research

Grant

Other Research

Support

Speakers Bureau/

Honoraria

Expert

Witness

Ownership

Interest

Consultant/

Advisory Board Other

Barry J. Maron Minneapolis Heart

Institute Foundation

None None None None None None None

Douglas P. Zipes Indiana University None None None None None None None

Richard J. Kovacs Indiana University None None None None None None None

This table represents the relationships of writing group members that may be perceived as actual or reasonably perceived conﬂicts of interest as reported on the Disclosure Ques-

tionnaire, which all members of the writing group are required to complete and submit. A relationship is considered to be “signiﬁcant” if (a) the person receives $10,000 or more during

any 12-month period, or 5% or more of the person’s gross income; or (b) the person owns 5% or more of the voting stock or share of the entity, or owns $10,000 or more of the fair

market value of the entity. A relationship is considered to be “modest” if it is less than “signiﬁcant” under the preceding deﬁnition.

DISCLOSURES

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Med. 2010;362:917–27. http://dx.doi.org/10.1056/

NEJMra0910111.

11. Maron BJ, Barry JA, Poole RS. Pilots, hypertrophic

cardiomyopathy, and issues of aviation and public

safety. Am J Cardiol. 2004;93:441–4. http://dx.doi.

org/10.1016/j.amjcard.2003.10.038.

12. Harris KM, Sponsel A, Hutter AM Jr., Maron BJ.

Brief communication: cardiovascular screening prac-

tices of major North American professional sports

teams. Ann Intern Med. 2006;145:507–11.

13. Maron BJ. C ontemporary insights and strategies

for risk stratiﬁcation and prevention of sudden

death in hypertrophic cardiomyopathy [published

correction appears in Circulation. 2010;122:e7].

Circulation. 2010;121:445–56. htt p://dx.doi.org/

10.1161/CIRCULATIONAHA.109.878579.

14. Maron BJ, Spirito P, Ackerman MJ, Casey SA,

Semsarian C, Estes NA 3rd, Shannon KM, Ashley EA,

Day SM, Pacileo G, Formisano F, Devoto E,

Anastasakis A, Bos JM, Woo A, Autore C, Pass RH,

Boriani G, Garberich RF, Almquist AK, Russell MW,

Boni L, Berger S, Maron MS, Link MS. Prevention of

sudden cardiac death with implantable cardioverter-

deﬁbrillators in children and adolescents with hyper-

trophic cardiomyopathy. J Am Coll Cardiol. 2013;61:

1527–35. http://dx.doi.org/10.1016/j.jacc.2013.01.037.

15. Maron BJ, Spirito P, Shen WK, Haas TS,

Formisano F, Link MS, Epstein AE, Almquist AK,

Daubert JP, Lawrenz T, Boriani G, Estes NA 3rd,

Favale S, Piccininno M, Winters SL, Santini M,

Betocchi S, Arribas F, Sherrid MV, Buja G, Semsarian C,

Bruzzi P. Implantable cardioverter-deﬁbrillators and

prevention of sudden cardiac death in hypertrophic

cardiomyopathy [published correction appears in

JAMA. 2007;298:1516]. JAMA. 2007;298:405–12.

http://dx.doi.org/10.1001/jama.298.4.405.

16. Bhonsale A, James CA, Tichnell C, Murray B,

Gagarin D, Philips B, Dalal D, Tedford R, Russell SD,

Abraham T, Tandri H, Judge DP, Calkins H. Incidence

and predictors of implantable cardioverter-deﬁbrillator

therapy in patients with arrhythmogenic right

ventricular dysplasia/cardiomyopathy undergoing im-

plantable cardioverter-deﬁbrillator implantation for

primary prevention. J Am Coll Cardiol. 2011;58:

1485–96. http://dx.doi.org/10.1016/j.jacc.2011.06.043.

17. 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.

18. Maron BJ, Ommen SR, Semsarian C, Spirito P,

Olivotto I, Maron MS. Hypertrophic cardiomyopathy:

present and future, with translation into contemporary

cardiovascular medicine [published correction appears

in J Am Coll Cardiol. 2014;64:1188]. J Am Coll Cardiol.

2014;64:83–99. http://dx.doi.org/10.1016/j.jacc.2014.

05.003.

19. Maron BJ, Mitten MJ, Quandt EF, Zipes DP.

Competitive athletes with cardiovascular disease: the

case of Nicholas Knapp. N Engl J Med. 1998;339:1632–5.

http://dx.doi.org/10.1056/NEJM199811263392211.

KEY WORDS ACC/AHA Scientiﬁc Statements,

athletes, cardiovascular abnormalities, genetics,

hypertrophic cardiomyopathy, sudden death

Reviewer Disclosures

Reviewer Employment

Research

Grant

Other Research

Support

Speakers Bureau/

Honoraria

Expert

Witness

Ownership

Interest

Consultant/

Advisory Board Other

Robert C. Hendel University of Miami None None None None None None None

Adolph M. Hutter, Jr Massachusetts

General Hospital

None None None None None None None

James L. Januzzi, Jr Massachusetts

General Hospital

None None None None None None None

Wojciech Krol Medical University of

Warsaw (Poland)

None None None None None None None

Geetha Raghuveer Children’s Mercy

Hospital, Kansas

City, MO

None None None None None None None

Barbara S. Wiggins Medical University of

South Carolina

None None None None None None None

This table represents the relationships of reviewers that may be perceived as actual or reasonably perceived conﬂicts of interest as reported on the Disclosure Questionnaire, which all

reviewers are required to complete and submit. A relationship is considered to be “signiﬁcant” if (a) the person receives $10,000 or more during any 12-month period, or 5% or more of

the person’s gross income; or (b) the person owns 5% or more of the voting stock or share of the entity, or owns $10,000 or more of the fair market value of the entity. A relationship

is considered to be “modest” if it is less than “signiﬁcant” under the preceding deﬁnition.

Maron et al.

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APPENDIX

Task Forces and Authors

Preamble, Principles, and General Considerations Barry J. Maron, MD, FACC, Co-Chair; Douglas P. Zipes, MD, FAHA, MACC, Co-Chair;

Richard J. Kovacs, MD, FAHA, FACC, Co-Chair

Task Force 1: Classiﬁcation of Sport: Dynamic, Static and Impact Benjamin D. Levine, MD, FAHA, FACC, Chair; Aaron L. Baggish, MD, FACC;

Richard J. Kovacs, MD, FAHA, FACC; Mark S. Link, MD, FACC; Martin S. Maron, MD,

FACC; Jere H. Mitchell, MD, FACC

Task Force 2: Preparticipation Screening for Cardiovascular Disease

in Competitive Athletes

Barry J. Maron, MD, FACC, Chair; Benjamin D. Levine, MD, FAHA, FACC;

Reginald L. Washington, MD, FAHA; Aaron L. Baggish, MD, FACC; Richard J. Kovacs,

MD, FAHA, FACC; Martin S. Maron, MD, FACC

Task Force 3: Hypertrophic Cardiomyopathy, Arrhythmogenic Right

Ventricular Cardiomyopathy and Other Cardiomyopathies,

and Myocarditis

Barry J. Maron, MD, FACC, Chair; James E. Udelson, MD, FAHA, FACC; Robert O. Bonow,

MD, MS, FAHA, MACC; Rick Nishimura, MD, FAHA, MACC; Michael J. Ackerman, MD,

PhD, FACC; N.A. Mark Estes III, MD, FACC; Leslie T. Cooper, Jr, MD, FAHA, FACC;

Mark S. Link, MD, FACC; Martin S. Maron, MD, FACC

Task Force 4: Congenital Heart Disease George F. Van Hare, MD, FACC, Chair; Michael J. Ackerman, MD, PhD, FACC;

Juli-anne K. Evangelista, DNP, APRN, CPNP-AC, FACC; Richard J. Kovacs, MD, FAHA,

FACC; Robert J. Myerburg, MD, FACC; Keri M. Shafer, MD; Carole A. Warnes, MD,

FACC; Reginald L. Washington, MD, FAHA

Task Force 5: Valvular Heart Disease Robert O. Bonow, MD, MS, FAHA, MACC, Chair; Rick Nishimura, MD, FAHA, MACC;

Paul D. Thompson, MD, FAHA, FACC; James E. Udelson, MD, FAHA, FACC

Task Force 6: Hypertension Henry R. Black, MD, FAHA, Chair; Domenic Sica, MD; Keith Ferdinand, MD, FAHA, FACC;

William B. White, MD

Task Force 7: Aortic Diseases, Including Marfan Syndrome Alan C. Braverman, MD, FACC, Chair; Kevin M. Harris, MD, FACC; Richard J. Kovacs, MD,

FAHA, FACC; Barry J. Maron, MD, FACC

Task Force 8: Coronary Artery Disease Paul D. Thompson, MD, FAHA, FACC, Chair; Robert J. Myerburg, MD, FACC;

Benjamin D. Levine, MD, FAHA, FACC; James E. Udelson, MD, FAHA, FACC;

Richard J. Kovacs, MD, FAHA, FACC

Task Force 9: Arrhythmias and Conduction Defects Douglas P. Zipes, MD, FAHA, MACC, Chair; Mark S. Link, MD, FACC; Michael J. Ackerman,

MD, PhD, FACC; Richard J. Kovacs, MD, FAHA, FACC; Robert J. Myerburg, MD, FACC;

N.A. Mark Estes III, MD, FACC

Task Force 10: The Cardiac Channelopathies Michael J. Ackerman, MD, PhD, FACC, Chair; Douglas P. Zipes, MD, FAHA, MACC;

Richard J. Kovacs, MD, FAHA, FACC; Barry J. Maron, MD, FACC

Task Force 11: Drugs and Performance Enhancing Substances N.A. Mark Estes III, MD, FACC, Chair; Richard J. Kovacs, MD, FAHA, FACC;

Aaron L. Baggish, MD, FACC; Robert J. Myerburg, MD, FACC

Task Force 12: Emergency Action Plans, Resuscitation, CPR, and AEDs Mark S. Link, MD, FACC, Chair; Robert J. Myerburg, MD, FACC; N.A. Mark Estes III, MD,

FACC

Task Force 13: Commotio Cordis Mark S. Link, MD, FACC, Chair; N.A. Mark Estes III, MD, FACC; Barry J. Maron, MD, FACC

Task Force 14: Sickle Cell Trait Barry J. Maron, MD, FACC, Chair; Kevin M. Harris, MD, FACC; Paul D. Thompson, MD,

FAHA, FACC; E. Randy Eichner, MD; Martin H. Steinberg, MD

Task Force 15: Legal Aspects of Medical Eligibility and Disqualiﬁcation

Recommendations

Matthew J. Mitten, JD, Chair; Douglas P. Zipes, MD, FAHA, MACC; Barry J. Maron, MD,

FACC; William J. Bryant, JD

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AHA/ACC SCIENTIFIC STATEMENT

Eligibility and Disqualiﬁcation

Recommendations for Competitive Athletes

With Cardio vascular Abnormalities:

Task Force 1: Classiﬁcation of Sports:

Dynamic, Static, and Impact

A Scientiﬁc Statement From the American Heart Association and American College of Cardiology

Benjamin D. Levine, MD, FAHA,

FACC, Chair*

Aaron L. Baggish, MD, FACC*

Richard J. Kovacs, MD, FAHA, FACC*

Mark S. Link, MD, FACC*

Martin S. Maron, MD, FACC*

Jere H. Mitchell, MD, FACC*

The “cl assiﬁcation of sports” section has been a part

of each iteration of the recommendations for partici-

pation in sp orts and p rovides a framework by which

athletes with heart disease can b e prescribed or pro-

scribed speciﬁc sports for participation (1–3).Forthe

36th Bethesda Conference, an earlier version of the

Figure was constructed that characterized sports by

their strength component, expressed as the relative

intensity of static muscl e contractions (percentage

of a maximal voluntary contraction), and their

endurance component, reﬂected by the relative in-

tensity of dynamic exercise (regular contraction of

large muscle groups) or percentage of maximal aero-

bic power (

2max

) (3).Therationaleforaclassiﬁca-

tion scheme applicable to the competitive athlete

with cardiac disease is based on the well-described

hemodynamics of ea ch different type of exercise

(static v ersus dynamic) (3,4),aswellastheapparent

cardiac adaptation of athletes who compete in these

sports (5),whichreﬂects the chro nic load on t he

*On behalf of the American Heart Association Electrocardiography and

Arrhythmias Committee of the Council on Clinical Cardiology, Council on

Cardiovascular Disease in the Young, Council on Cardiovascular and

Stroke Nursing, Council on Functional Genomics and Translational

Biology, and the American College of Cardiology.

The American Heart Association and the American College of Cardi-

ology make every effort to avoid any actual or potential conﬂicts of

interest that may arise as a result of an outside relationship or a

personal, professional, or business interest of a member of the writing

panel. Speciﬁcally, all members of the writing group are required to

complete and submit a Disclosure Questionnaire showing all such re-

lationships that might be perceived as real or potential conﬂicts of

interest. The Preamble and other Task Force reports for these pro-

ceedings are available online at www.onlinejacc.org (J Am Coll Cardiol

2015;XX:000–000; 000–000; 000–000; 000–000; 000–000; 000–000;

000–000; 000–000; 000–000; 000–000; 000–000; 000–000; 000–000;

000–000; and 000–000).

This statement was approved by the American Heart Association

Science Advisory and Coordinating Committee on June 24, 2015, and

the American Heart Association Executive Committee on July 22, 2015,

and by the American College of Cardiology Board of Trustees and

Executive Committee on June 3, 2015.

The American College of Cardiology requests that this document be cited

as follows: Levine BD; Baggish AL, Kovacs RJ, Link MS, Maron MS, Mitchell

JH; on behalf of the American Heart Association Electrocardiography and

Arrhythmias Committee of the Council on Clinical Cardiology, Council on

Cardiovascular Disease in the Young, Council on Cardiovascular and Stroke

Nursing, Council on Functional Genomics and Translational Biology, and

the American College of Cardiology. Eligibility and disqualiﬁcation rec-

ommendations for competitive athletes with cardiovascular abnormalities:

Task Force 1: classiﬁcation of sports: dynamic, static, and impact: a scien-

tiﬁc statement from the American Heart Association and American College

of Cardiology. J Am Coll Cardiol 2015;xx:–.

This article has been copublished in Circulation.

Copies: This document is available on the World Wide Web sites of the

American Heart Association (http://my.americanheart.org) and the

American College of Cardiology (www.acc.org). For copies of this docu-

ment, please contact Elsevier Inc. Reprint Department via fax (212-633-

3820) or e-mail ([email protected]).

Permissions: Multiple copies, modiﬁcation, alteration, enhancement,

and/or distribution of this document are not permitted without the ex-

press permission of the American College of Cardiology. Requests may be

completed online via the Elsevier site (http://www.elsevier.com/about/

policies/author-agreement/obtaining-permission).

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY VOL. - ,NO.- ,2015

ª 2015 BY THE AMERICAN HEART ASSOCIATION, INC. AND

THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION

ISSN 0735-1097/$36.00

http://dx.doi.org/10.1016/j.jacc.2015.09.033

PUBLISHED BY ELSEVIER INC.

PGL 5.4.0 DTD  JAC21831_proof  9 October 2015  10:43 am  ce

cardiovascular system. The underlying principle is that

speciﬁc cardiovas cular condition s may be more or les s

susceptible to complications (primarily ischemia, heart

failure, or vascular compromise) ba sed on unique c har-

acteristics of each lesion and the load placed on the heart

during athletic competition.

Static contractions stimulate mechanical and metabolic

afferents in skeletal muscle, w hich leads to large, sus-

tained changes in blood pressure via the exercise pressor

reﬂex (6–8). The larger the muscle mass involved, the

greater the intensity of contraction, and the greater the

rise in bl ood pressure (9); incorporation of a Valsalva

maneuver during contractions will acutely and tran-

siently increase transmural arterial pressure markedly

in blood vessels outside of the chest, although left ven-

tricular (LV) afterload does not appe ar to increase

(10) because of a balanced rise in intracardiac and

intrathoracic pressure inside t he chest. Dynamic exercise

increases t he demand for bloo d ﬂow and cardiac output

in proporti on to the metabolic d emand (

): for every

1 L/min increase in oxygen uptake, there is a n ob ligate

requirement for a 5 to 6 L/min increase in cardiac output

(4,11) as a function of the Fick equa tion. This increase is

independent of age, sex, or ﬁtness (4,12,13).

Both dynamic and static exercise result in an increase

in myocardial oxygen demand: heart rate, wall tension

(before and after the contraction, which determines pre-

load and afterload), and contractile state of the LV (14).

During high-intensity dynamic exercise, there is a large

increase in heart r ate and an increase in stroke v olume

that is achieved by both an increase in end-diasto lic vol-

ume (Frank-Starling mechanism) (15) and a decrease in

end-systolic volume (increased contractile state); for

athletes, the most important factor is the increase in

print & web 4C=FPO

FIGURE Classiﬁcation of Sports

This classiﬁcation is based on peak static and dynamic components achieved during competition; however, higher values may be reached during training.

The increasing dynamic component is deﬁned in terms of the estimated percentage of maximal oxygen uptake (

2max

) achieved and results in an increasing

cardiac output. The increasing static component is related to the estimated percentage of maximal voluntary contraction reached and results in an increasing

blood pressure load. The lowest total cardiovascular demands (cardiac output and blood pressure) are shown in the palest color, with increasing dynamic

load depicted by increasing blue intensity and increasing static load by increasing red intensity. Note the graded transition between categories, which

should be individualized on the basis of player position and style of play. *Danger of bodily collision (see Table for more detail on collision risk).

†Increased risk if syncope occurs. Modiﬁed from Mitchell et al. (3) with permission. Copyright ª 2005, Journal of the American College of Cardiology.

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end-diastolic volume (16). In high-intensity stat ic e xer-

cise, a smaller increase occurs in heart rate, and little

change occurs in end-diastolic and end-systolic volumes

of the LV; however, arterial pressure and contractile state

of the ventricle are increased. Thus, d ynamic exercise

primarily causes a volume load on the LV, whereas static

exercise causes a pressure load. Virtually all sports

require a combination of bo th types of effort, although

when both are high, such as in rowing sports, the rise in

blood pressure may be dramatic (17),andthecardiac

adaptation is among t he most prominent of all sports (18).

CLASSIFICATION OF SPORTS

On the basis of these considerations, the following matrix

was developed (Figure). This Figure has been modiﬁed

only slightly from the initial derivation published in the

36th Bethesda Conference, mostly to emphasize a more

graded increase in effort/cardiovascular load between

categories as opposed to a hard, discrete distinction.

Each sp ort is categori zed by the l evel of intens ity

(low, medium, high) of dynamic or static exercise

generally required to perform that sport during compe-

tition. It a lso recognizes those sports that pose a signif-

icant risk because of b odily collis ion, eit her because of

the probabilit y of h ard impact between competitors or

between a competitor and an object, projectile, or the

ground, as well as t he degree of risk to the athlete or

others if a sudden syncopal event occurs. Thus, in terms

of their dynamic and static demands, sports can be

classiﬁed as IIIC (high static, high dynamic), IIB (mod-

erate static, moderate dynamic), IA (low static, low

dynamic), and so forth. For example, an ath lete with a

cardiovascular abnormality that would preclude a spo rt

that produces a high pressure load on the LV may be

advised to avoid sports classiﬁed as IIIA, IIIB, and IIIC.

It should be emphasized that in terms of the classiﬁ-

cation of sports ma trix pre sented in the Figure,cardio-

vascular abnormali ties designated as compatible with a

high level of intensity in any particular category also

(by deﬁnition) permit participati on in levels of lesser

intensity. For example, if class IC sports are appropriate

(low static/high dynamic), then so are classes IA and IB

(low static/ low and modera te dynamic) . Sports in eac h

category are listed in al phabetical order to make them

easier to ﬁnd.

Although this scheme has been very useful in guiding

practitioners and allowing recommendations for sports

participation, there are a number of key limitations that

must be acknowledged to use this approach to guide

recommendations for individ ual athletes:

The scheme as d escribed is simplistic and is only a

rough guide. It must be acknowledged that within each

sport, different position players may have quite

different cardiovascular loads, for example, wide

receiver or offens ive lineman in American footb all,

goalie versus midﬁelders or forwards in soccer, 50 m

versus 400 m distances in swimming, and short-track

versus long track speed skating. This differential load

mayevenbemanifestatthelowest-intensitysports

such as yoga, which also can be practiced at much

higher intensities. Therefore, practitioners should be

prepared to individualize t he classiﬁcation scheme

based on individual athletes and how they play their

speciﬁcsportandposition.

Even within individual sports, the cardio vascular load

may be quite different at different times during the

competition. As such, it is recommended that the

highest level achieved during competition be used for

exercise prescription, even if this level is achieved

relatively infrequently.

The types and intensities of exercise required for

training may be different from those achieved during a

competition. Therefore, cardiovascular loads experi-

enced during t raining, includ ing high-intensity inter-

val efforts, and during a game must be considered.

These guidelines are intended for competitive sports

and their required training regimen but may no t

apply to p articipa tion in spor ts at a recreationa l

level. Mo reover, many higher-class activities (such as

cycling and running) can be performed b y patients

with cardiovascular disease after they have received

counseling about intensity restriction and com-

petition avoidance as pa rt of healthy secondar y

prevention.

Environmental conditions may alter the cardiovascu-

lar load f or a giv en sport substantially. Increas ing

altitude alters ox ygen a vailability and acutely in-

creases the hea rt rate and cardiac output f or any

given a bsolut e wor k rate (19). In patients with un-

derlying coronary heart disease, it may also reduce

the myocardial workload requi red to cause ischemia

(20) and increase the risk of sudden d eath (21),

although even short-term acclimatization appears to

reduce this risk signiﬁcantly (21).Heatisalsoasub-

stantial stressor; because humans th ermoregulate by

sending blood to the skin, a large extra amount of

cardiac output is required to maintain body temper-

ature (22), and this could increase the dynamic clas-

siﬁcation of some sports (especially “hot yog a”). For

patients with limited capability to augment cardiac

output, thermal stress may be particularly problem-

atic (23).Thepsychologicalandemotionaldemands

of sports, particularly during h igh-stakes co mpeti-

tions, are also relevant and may increase heart rate

substantially and unpredictably.

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THE EFFECT OF IMPACT AND CONSIDERATIONS

FOR ANTICOAGULATION

Athletes with cardiovascula r disease who are taking

anticoagulant drugs (vitamin K antagonists, direct

thrombin or factor Xa inhibitors) must also consider the

risk for impact during practice or competition. An impact

that occurs while taking anticoagulation medication i n-

creases the risk of severe injury, especially for intra-

cranial hemorrhage. Human-human or human-object

impacts occur in many sports. Indeed, there are some

sports in which impact is a key component of the game,

such as Am erican footba ll an d ic e hoc key. Converse ly,

there are some sports in which impact is extremely

unlikely to occur, such as golf or track and ﬁeld. For

other sports, the risk and occurrence of impact are

related t o the age and competiti veness of the athletes.

In thes e sports, suc h as basketba ll and soccer, th e older

the person and the more competitive the play, the more

likely t hese p eople will undergo impa cts. The Table

divides sports according to the age of the athlete and

the r elative risk f or impact.

Intracranial hemorrhage ri sk is possibly best ascer-

tained by concussion incidence in sports; however,

concussion incidences are certainly an underrepresenta-

tion of severe head injuries. Many head injuries do not

result in concussion but nevertheless could put the per-

son at a higher risk of intracranial bleeds i f the person has

been undergoing treatment with an anticoagulant agent.

In high school athletes, concussion incidence is highest in

American football (z23/10,000 exposures), followed by

ice hockey, lacrosse, soccer, basketball, and wrestling

(24,25). Concussion risk is much higher in c ompetition

than in practice, with most concussions occurring as a

result of player-player contact (70% of the concussions) or

player-surface contact (17%) (24,25). Severe injuries not

limitedtoheadinjury(deﬁned as injuri es that resulted

in >21 lost days of sports participation) show a similar

frequency distribution, with American football being

most common (z20/10,000 exposures) (26).

Recommendations

1. The risk of bleeding with athletes receiving vitamin K

antagonists or direct thrombin or factor X a inhibitors

is increased in sports in which impacts may occur, and

athlet es should be cautioned to a void these sp orts

(Class IIb; Level of Evidence C).

2. Athletes taking vitamin K antagonists or direct

thrombin or factor Xa inhibitors should not participate

in sports with impact expected, because the risk of

intracranial hemorrhage is i ncreased (Class III; Level

of Evidence C).

TABLE

Sports According to Risk of Impact and

Educational Background

Junior H igh Sch ool High Sch ool/College

Impact expected American football

Ice hockey

Lacrosse

Wrestling

Karate/judo

Fencing

Boxing

American football

Soccer

Ice hockey

Lacrosse

Basketball

Wrestling

Karate/judo

Downhill skiing

Squash

Fencing

Boxing

Impact may occur Soccer

Basketball

Field hockey

Downhill skiing

Equestrian

Squash

Cycling

Field hockey

Equestrian

Cycling

Baseball/softball

Gymnastics

Figure skating

Impact not expected Baseball/softball

Cricket

Golf

Riﬂery

Gymnastics

Volleyball

Swimming

Track and ﬁeld

Tennis

Figure skating

Cross-country skiing

Rowing

Sailing

Archery

Weightlifting

Badminton

Cricket

Golf

Riﬂery

Volleyball

Swimming

Track and ﬁeld

Tennis

Cross-country skiing

Rowing

Sailing

Archery

Weightlifting

Badminton

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DISCLOSURES

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8. Raven PB. Neural c ontrol of the circulati on

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11. Rowell LB. Neural control of muscle blood ﬂow:

importance during dynamic exercise. Clin Exp Phar-

macol Physiol. 1997;24:117–25.

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1433–5.

13. McGuire DK, Levine BD, Williamson JW, Snell PG,

Blomqvist CG, Saltin B, Mitchell JH. A 30-year follow-

up of the Dallas Bedrest and Training Study, I: effect of

age on the cardiovascular response to exercise. Circu-

lation. 2001;104:1350–7.

14. Mitchell JH, Hefner LL, Monroe RG. Performance of

the left ventricle. Am J Med. 1972;53:481–94.

15. Levine BD, Lane LD, Buckey JC, Friedman DB,

Blomqvist CG. Left ventricular pressure-volume and

Frank-Starling relations in endurance athletes: impli-

cations for orthostatic tolerance and exercise perfor-

mance. Circulation. 1991;84:1016–23.

16. Levine BD. Vo

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do we still need to know? J Physiol. 2008;586:25–34.

http://dx.doi.org/10.1113/jphysiol.2007.147629.

17. Clifford PS, Hanel B, Secher NH. Arterial blood

pressure response to rowing. Med Sci Sports Exerc.

1994;26:715–9.

18. Pelliccia A, Maron BJ, Spataro A, Proschan MA,

Spirito P. The upper limit of physiologic cardiac

hypertrophy in highly trained elite athletes. N Engl J

Med. 1991;324:295–301. http://dx.doi.org/10.1056/

NEJM199101313240504.

19. Bärtsch P, Saltin B, Dvorak J, Federation Inter-

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Reviewer Disclosures

Reviewer Employment

Research

Grant

Other

Research

Support

Speakers

Bureau/

Honoraria

Expert

Witness

Ownership

Interest

Consultant/

Advisory

Board Other

Michael S. Emery Greenville Health System None None None None None None None

Michael J. Joyner Mayo Clinic and Foundation,

Rochester

None None None None None None None

Matthew V. Park NorthWest Children’s Heart Care,

Pediatrix Medical Group

None None None None None None None

This table represents the relationships of reviewers that may be perceived as actual or reasonably perceived conﬂicts of interest as reported on the Disclosure Questionnaire, which all

reviewers are required to complete and submit. A relationship is considered to be “signiﬁcant” if (a) the person receives $10,000 or more during any 12-month period, or 5% or more of

the person’s gross income; or (b) the person owns 5% or more of the voting stock or share of the entity, or owns $10,000 or more of the fair market value of the entity. A relationship

is considered to be “modest” if it is less than “signiﬁcant” under the preceding deﬁnition.

Writing Group Disclosures

Writing Group

Member Employment

Research

Grant

Other

Research

Support

Speakers

Bureau/

Honoraria

Expert

Witness

Ownership

Interest

Consultant/

Advisory

Board Other

Benjamin D. Levine University of Texas Southwestern Medical

Center; Texas Health Presbyterian

Hospital Dallas Institute for Exercise

and Environmental Medicine

None None None None None None None

Aaron L. Baggish Massachusetts General Hospital None None None None None None None

Richard J. Kovacs Indiana University None None None None None None None

Mark S. Link Tufts University None None None None None None None

Martin S. Maron Tufts Medical Center None None None None None None None

Jere H. Mitchell University of Texas Southwestern

Medical Center

None None None None None None None

This table represents the relationships of writing group members that may be perceived as actual or reasonably perceived conﬂicts of interest as reported on the Disclosure Ques-

tionnaire, which all members of the writing group are required to complete and submit. A relationship is considered to be “signiﬁcant” if (a) the person receives $10,000 or more during

any 12-month period, or 5% or more of the person’s gross income; or (b) the person owns 5% or more of the voting stock or share of the entity, or owns $10,000 or more of the fair

market value of the entity. A relationship is considered to be “modest” if it is less than “signiﬁcant” under the preceding deﬁnition.

JACC VOL. - ,NO.- ,2015

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Med Sci Sports. 2008;18 suppl 1:96–9. http://dx.doi.

org/10.1111/j.1600-0838.2008.00837.x.

20. Levine BD, Zuckerman JH, deFilippi CR. Effect

of high-altitude exposure in the elderly: the Tenth

Mountain Division study. Circulation. 1997;96:1224–32.

21. Lo MY, Daniels JD, Levine BD, Burtscher M.

Sleeping altitude and sudden cardiac death. Am Heart

J. 2013;166:71–5. http://dx.doi.org/10.1016/j.ahj.2013.

04.003.

22. Crandall CG, González-Alonso J. Cardiovascular

function in the heat-stressed human. Acta Physiol

(Oxf). 2010;199:407–23. http://dx.doi.org/10.1111/

j.1748-1716.2010.02119.x.

23. Arbab-Zadeh A, Crandall CG, Levine BD. Thermo-

regulation in patients with cardiac disease. J Cardiopulm

Rehabil. 2002;22:38–9.

24. MararM,McIlvainNM,FieldsSK,ComstockRD.

Epidemiology of concussions among United States

high school athletes in 20 sport s. Am J Spor ts

Med. 2012;40:747–55. http://dx.doi.org/10.1177/

0363546511435626.

25. Harmon KG, Drezner J, Gammons M, Guskiewicz K,

Halstead M, Herring S, Kutcher J, Pana A, Putukian M,

Roberts W, American Medical Society for Sports Med-

icine. American Medical Society for Sports Medicine

position statement: concussion in sport. Clin J Sport

Med. 2013;23:1–18. http://dx.doi.org/10.1097/JSM.

0b013e31827f5f93.

26. Darrow CJ, Collins CL, Yard EE, Comstock RD.

Epidemiology of severe injuries among United

States high school athletes: 2005-2007. Am J

Sports Med. 2009;37:1798–805. http://dx.doi.org/

10.1177/0363546509333015.

KEY WORDS ACC/AHA Scientiﬁc Statements,

athletes, blood pressure, cardiac output,

cardiovascular abnormalities, classiﬁcation,

oxygen saturation, s port

Levine et al.

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AHA/ACC SCIENTIFIC STATEMENT

Eligibility and Disqualiﬁcation

Recommendations for Competitive Athletes

With Cardio vascular Abnormalities:

Task Force 2: Preparticipation Screening

for Cardio vascular Disease in

Competitive Athletes

A Scientiﬁc Statement From the American Heart Association and American College of Cardiology

Barry J. Maron, MD, FACC, Chair* Benjamin D. Levine , MD, FAHA, FACC*

Reginald L. Washington, MD, FAHA*

Aaron L. Baggish, MD, FACC*

Richard J. Kovacs, MD, FAHA, FACC*

Martin S. Maron, MD, FACC*

The central purpose of preparticipation screening

of trained competitive athletes is to identify or

raise suspicion of those cardiovascular abnorma lities

and diseases that are potentially responsible for

sudden unexpected death on the athletic ﬁeld (1–14).

When such athletes are recognized, they are exposed

to eligibil ity and disqualiﬁcation decisions that

become the responsibility of the practicing physician

(4,15–17) and are a subject of this document. There is

general (although not universal) (12) agreement with

the principle that screening to detect important

diseases and potentially prevent sudden death is

justiﬁed and potentially beneﬁcial (1–3,5–9,1 8).

There are many pathways and strategies by which

competitive athletes with c ardiovascular diseas e may

be recognized: 1) comprehensive evaluation by a

*On behalf of the American Heart Associ ation Electrocard iograph y

and Arrhythmias Committee of the Council on Clinical Cardio-

logy, C ouncil on Cardiovascular Disease in the Young, Council on

Cardiovascular and Stroke Nu rsing, Council on Functional

Genomics and Translational Biology, and the American College of

Cardiology.

The American Heart Association and the American College of

Cardiology make every effort to avoid any actual or potential conﬂicts of

interest that may arise as a result of an outside relationship or a per-

sonal, professional, or business interest of a member of the writing

panel. Speciﬁcally, all members of the writing group are required to

complete and submit a Disclosure Questionnaire showing all such re-

lationships that might be perceived as real or potential conﬂicts of in-

terest. The Preamble and other Task Force reports for these proceedings

are available online at www.onlinejacc.org (J Am Coll Cardiol

2015;xx:000–000; 000–000; 000–000; 000–000; 000–000; 000–000;

000–000; 000–000; 000–000; 000–000; 000–000; 000–000; 000–000;

000–000; and 000–000).

This statement was approved by the American Heart Association

Science Advisory and Coordinating Committee on June 24, 2015, and the

American Heart Association Executive Committee on July 22, 2015, and by

the American College of Cardiology Board of Trustees and Executive

Committee on June 3, 2015.

The American College of Cardiology requests that this document be

cited as follows: Maron BJ, Levine BD, Washington RL, Baggish AL,

Kovacs RJ, Maron MS; on behalf of the American Heart Association

Electrocardiography and Arrhythmias Committee of the Council on Clin-

ical Cardiology, Council on Cardiovascular Disease in the Young, Council

on Cardiovascular and Stroke Nursing, Council on Functional Genomics

and Translational Biology, and the American College of Cardiology.

Eligibility and disqualiﬁcation recommendations for competitive athletes

with cardiovascular abnormalities: Task Force 2: preparticipation

screening for cardiovascular disease in competitive athletes: a scientiﬁc

statement from the American Heart Association and American College of

Cardiology. J Am Coll Cardiol 2015;xx:–.

This article has been copublished in Circulation.

Copies: This document is available on the World Wide Web sites of the

American Heart Association (http://my.americanheart.org) and the

American College of Cardiology (www.acc.org). For copies of this docu-

ment, please contact Elsevier Inc. Reprint Department via fax (212-633-

3820) or e-mail ([email protected]).

Permissions: Multiple copies, modiﬁcation, alteration, enhancement,

and/or distribution of this document are not permitted without the ex-

press permission of the American College of Cardiology. Requests may be

completed online via the Elsevier site (http://www.elsevier.com/about/

policies/author-agreement/obtaining-permission).

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY VOL. - ,NO.- ,2015

ª 2015 BY THE AMERICAN HEART ASSOCIATION, INC. AND

THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION

ISSN 0735-1097/$36.00

http://dx.doi.org/10.1016/j.jacc.2015.09.034

PUBLISHED BY ELSEVIER INC.

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primary care physician; 2) systematic screening of fam-

ilies with known genetic diseases after diagnosis in a

relative; 3) incidental and fortuitous ﬁndings on clinical

examination o r imaging , detected during evaluation for

another medical problem; 4) systematic screening of large

populations, such as high school and college-aged ath-

letes, for the purpose of d etermining el igibility for

competitive sports, with or without diagnostic testing;

and 5) symptoms associated or unassociated with sports.

It is likely that a large number (or even most) athletes

with cardiovas cular disease c ome to clinica l attention

based on the circumstances described in items 1 through 3,

rather than with forma l preparticipation screening.

GENERAL CONSIDERATIONS

Currently, broad-based cardiovascular screening is prac-

ticed systematica lly in athletes at all levels of performance

(not conﬁned to the elite) in only 3 countries: in the United

States, with personal/family history and physical exami-

nation (but without ECGs) (1–3,19,20),andinbothItaly

(4–6,9) and Israel (7), with 12-lead ECGs in addition to his-

tory and physical examination. I n many European coun-

tries, screening of athletes is largely limited to those

performing at the elite level (e.g., in international,

Olympic, or professional sports) (21).Thepotentialbeneﬁt

of such initiatives is the identiﬁcation of a small number of

people with potentially letha l genetic or congenital car-

diovascular diseases (e.g., hypertrophic cardiomyopathy)

so that 1) they may be withdrawn from competitive sports

to decrease their personal risk and generally make the

athletic ﬁeld a safer environment, and 2) in the process,

some high-risk people may be recognized who may be

candidates for disease-modifying medical or surgical

intervention, or for prevention of sudden death with

implantable deﬁbrillators. In 1973, the Japanese School

Health Law mandated cardiovascular screening with

modiﬁed ECG and history/physical examina tion for thou-

sandsofchildrenintheﬁrst, seventh, and tenth grades

(22,23). Few disease-related data have emerged from this

initiative, althoug h a variety of generally minor cardio-

vascular abno rmalities or arrhythmias (unassociated with

underlying organic heart disease) were identiﬁed in only

2% to 3% of children (23).

DEBATE AND CONTROVERSY

Within the context of these potential beneﬁts, there has

nevertheless been substantial discussion surrounding the

most appropriate and efﬁcacious strategy for screening,

including national federally sponsored and mandated car-

diovascular screening. For example, Italian investigators

have intensely promoted screening with a routine 12-lead

ECG (as well as history and physical examination) based

on a unique >30-year program mandated by Italian law and

supported by sports medicine p hysicians dedicated full-

time to the program (4–6,9).Since1997,Israelhasmain-

tained a similar mandatory ECG-based initiative and

national sports law (7).For>50 years, it has been

customary practice i n t he United States to routinely screen

high school and college-a ged athletes wi th history and

physical exam ination ( but without noninvasive testing)

(1–3,19,20). In contrast, Denmark has pointedly rej ected

systematic screening for cardiovascular disease in both

athletes and any other segment of the population as being

unjustiﬁe d given the low event rate (12,13). Other than

Japan (22,23), no country has systematically attempted

broad-based cardiovascular screening in general healthy

populations (not limited to athletes), with or without ECGs.

UNIVERSAL SCREENING: ECGs VERSUS

HISTORY AND PHYSICAL EXAMINATION

Preparticipat ion screeni ng for cardio vascular di sease wi th

personal/family history and physical examinati on has

been the customary practice for all high sc hool and

college-aged competitive athletes in the United Sta tes for

decades, independent of their performance level. This

processisguidedbythe14-pointhistoryandphysical

examination elements proposed by the America n Heart

Association (AHA) (1).TheAHArecommendations

acknowledge that athletes and others with underlying

(but undiagnosed) cardiovascular abnormalities may well

manifest cli nical warning signs (e.g., chest pain, excessive

exertional dyspnea, or syncope) identiﬁable by c areful

and systematic history. Because most diseases respon-

sible for sudden death in the young are genetic/familial, a

thorough family history may raise suspicion of the dis-

order. An organic heart murmur can alert the examining

physician to valvular or other abno rmalities, including

left ventricular outﬂow tract obstruction.

A controversy persists as to whether an ECG (in

addition to history and physical examination) is a supe-

rior strategy to history/physical examination alone for

detecting potentially lethal cardiov ascular diseas e,

particularly when taki ng into account the important

issues of false-negative and false-positive results, as well

as cost and re source availability (1).Indeed,studies

comparing these 2 strategies have failed to demonstrate a

mortality beneﬁt for ECG screening (18).

The debate between those who strongly promote

routineECGsandthoseopposedtoECGsasaroutine

screening tool is not fully resolved as yet, al though a

substantial literature consisting largely of ed itorials and

viewpoint commentaries is accumulating rapidly . Never-

theless,severalpointsareindisputable.First,the12-lead

ECG, although a mainstay of hospital-based cardiovascu-

lar practice for decades, is an unproven diagnostic tool for

reliable detection of cardio vascular disease in generally

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healthy populations (1). Second, outcome data on athlete

screening and mortalit y have been driven primarily by

only 1 d atabase, from the Veneto region of Italy (9% of the

national population) as part of its long-term screening

program (6,9). This ambitious Italia n initiative has been

shown to be successful in identifying some at-risk ath-

letes with p otentially lethal cardiovascular diseas e (pri-

marily right ventricular cardiomyopathy, which appears

to be endemic in this area of Ita ly), resulting in their

mandatory withdrawal from sports. In addition, a sharp

decrease in mortality r ate over a 30-year period wa s

demonstrated, which these investigat ors attributed to

incorporation o f the 12 -lead ECG into the screening pro-

gram in the early 1980s .

Third, th e Ita lian data showing that ECG screening re-

duces mortality in athletes have yet to be replicated

elsewhere, and evidence from the United States (18) and

Israel (7) appears to dispute or diminish the value of the

ECG in reducing athlete mortality. For example, contem-

porary mortality rates in US athletes from Minnesota,

where screening is limited to history a nd physical exam-

ination, do not differ from those in the V eneto region of

Italy, where the ECG is used routinely (18);furthermore,

athlete mortality rates in Israel were not different before

and after legislation for mandatory ECGs (7).Thefactthat

it has been difﬁcult to consistently show a reduction in

athlete mortality directly attributable to routine ECGs is

an observation that may be driven by t he generally low

event rates in competitive athletes with cardiovascular

disease (1–3,6,10,11,18,24–26).

RELEVANCE OF SUDDEN DEATH INCIDENCE

TO SCREENING

Indeed, the low frequency with which sudden deaths occur

in the competitive athlete population negati vely impa cts

the justiﬁcation for broad-based screening in large pop-

ulations of young people, as well as the weight that can be

afforded to this issue as a public health problem. In this

regard, there is now overwhelming evidence that these

events are relatively uncommon, albeit exceedingly tragic

in each case. Most data place these cardiovascular sudden

deaths in the rang e of approximately 1 in 80,000 to 1 in

200 000 participants per year, much less common in rela-

tive t erms than mo tor v ehicle accidents (by 5,000-fold),

suicide, drugs, homicide, or cancer in the same age group

and similar in frequency to that of fatal lightning stri kes

(1,11,25). In a college ( National Collegi ate Athletic Asso ci-

ation) at hlete p opulation, drugs and suicide combined

accounted for a similar number of deaths as conﬁrmed

cardiac disease (24),althoughanon–forensic-based

analysis reported a higher incidence fo r sudden death (27).

Notably, the absolute number of sudden deaths

attributable to docume nted cardiovascular disease in

competitive athletes is small in popula tions for w hich

forensic data are reported. For example, the 33-year US

Sudden Death in Athletes Registry has reported a

maximum of 75 such d eaths in any given year nationally

(10), and the Veneto database reports 55 sudden deaths in

26 years, or only z2peryear(6). In other populations, the

average number of conﬁrmed cardiovascular deaths

annually is much less, for instan ce, <1inMinnesotahigh

school athlet es (11) or z4 in college (Natio nal Collegia te

Athletic Association) athletes (24). Notably, false-negative

screening results are a major concern, in which the system

fails to identify the c ardiac diseases for which it is in fact

established. Indeed, a substantial proportion of athletes

(z30% to 40%) may di e sudde nly of cardiovascul ar ab-

normalities that would not necessarily be reliably detec-

tedbyscreeningevenwithECGs(1,11,24,25).

UNIVERSAL ECG SCREENING

On3occasions(1996,2007,and2014),AHAconsensus

expert panels evaluated and decided not to support

mandatory national athlete screening in the United States,

particularly with routine use of ECGs (1–3). Ind eed, sudden

cardiovascular deaths in athlet es are rare (albeit trag ic)

events, insufﬁcient in number to be judged as a major

public health problem or to justify a cha nge in national

healthcare policy. The most frequently cited ob stacles to

mandatory national screening of trained athletes are as

follows: 1) the large number of ath letes to be screened

nationally on an annual basis (i.e., z10 to 12 million); 2)

the low incidence of events (1,8,10,11 ,18,24–26);3)the

substantial number of e xpected false-negativ e and false-

positive results, in the range of 5% to 20% depending on

the speciﬁc ECG criteria used (1– 3,28–32);4)cost-efﬁcacy

considerations, that is, the extensive resources and ex-

penses required versus few events in absolute numbers;

5) liability issues that unavo idably impact physicians wit h

the sole responsibility to disqualify athlet es from compe-

tition and enforce that decision; 6) the lack of resources or

physicians dedicated to performing examinations and

interpreting ECGs, in contrast to the long-standing

sports medicine program in Italy (4–6,9);7)theinﬂu-

ence of observer variability , technical considerations, and

the impact of ethnicity/race on the i nterpretation of

ECGs, which is particularly important for multicultural

athlete populations such as in the United States; 8)

the need for repetitive (i.e., annual) ECG screening during

adolescence, given the possibility o f developing pheno-

typic evidence of cardiomyopathies during this time

period or later (33); 9) the logistical challenges and costs

related to second-tier conﬁrmatory screening with imag-

ing and other testing, should primary evaluations raise

the suspicion of cardiac disease; and 10) recognition

that even with testing, screening cannot be expected to

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identify all athletes with importan t cardiovascular ab-

normalities, and a signiﬁcant fa lse-negati ve rate may

occur (34).

NONUNIVERSAL SCREENING FOR ATHLETES

Screening programs on a smaller, no nnational b asis have

been implemented in some high schools, colleges, and local

communities that use E CGs (or echocardiograms) with

varying expertise, quality control, and resul ts for identi-

fying important cardiac disease. Consistent ly, the AHA has

not opposed ECG-based screening initiatives (often per-

formed by volunteers) in smaller venues; however, for s uch

screening initia tives, the AHA has prudently advised

adequate quality control with due consideration for the

prominent limitations of the process (including false-

negative and false-positive test results), so that the risks

and beneﬁtscanbeunderstoodandareacceptabletoall

participants, communities, and organizati ons (1–3).

There are certa in known and anticipated limitations in

the use of ECGs in population screening, incl uding but not

limited to false-positive and false-negative test results,

technical and interpretation issues, “gray zone” ambig-

uous diagnoses, and cost and logistics involved in arran-

ging second-tier diagnostic testing, all of which promote

anxiety, uncertainty, and legal considerations (1,12,25,34).

SCREENING AND RACE

Sudden deaths attributable to cardiovascular disease have

been reported in athletes of both sexes and a variety of

races, although they are much less common in females (by

1:9) (10,14). Preparticipation screening is warranted with

the same frequency and criteria, independent of sex and

across racial lines. In particular, although hypertrophic

cardiomyopathy unrecognized during life is a frequent

cause of sudden death in African-Americans o n the athletic

ﬁeld and a major impetus for screening in the black com-

munity (1,14,35), there is no ev idence to justify different or

separate scr eening strategies based on race. However, it is

becoming increasingly apparent that ethnic/racia l differ-

encesinECGpatternsmaysigniﬁ cantly impact the deﬁni-

tion of normality (30,36–39) and therefore potentially the

outcome of the screening process for minorities.

ETHICAL CONSIDERATIONS:

WHO SHOULD BE SCREENE D?

Unfortunately, often overlooked in the ECG screening

debate is the potentially troublesome ethical dilemma

created by conﬁning (or proposing to limit) screening for

potentially lethal diseases to those who choose engage-

ment in competitive sports, while in the process

excluding those who are not athletes. The degree to which

people engaged in competitive athletics are at greater risk

(given unsuspected underlying heart disease) is not

completely resolved. It is likely that the absolute number

of sudden deaths is highest in nonathletes because that

segment of the population is much larger in size. The AHA

maintains the position (1) that theoretically there is no

compelling reason to conﬁne screening for cardiovascular

disease to young competitive athletes, and exclude

non-athletes.

Recommendations

The guidelines presented herearethoseoftheAHA/

American Colleg e of Cardiology 2 014 initiative (1).

1. It is recom mended that the AHA’s 14-point screening

guidelines and those of other societies, such as the

American Academy of Pediatrics’ Preparticipation

Physical Evaluation, be used by examiners as part

of a comprehensive history taking and physical ex-

amination to detect or raise suspicion of genetic/

congenital cardiovascular abnormalities (Class I;

Level of Evidence C).

2. It is recommended that standardization of the ques-

tionnaire forms used as guides for examiner s of high

school and college athletes in the United States be

pursued (Class I; Level of Evidence C).

3. Screening with 12-lead ECGs (or echocardiograms) in

association with comprehensive history-taking and

physical examination to identify or raise suspicion of

genetic/congenital and other cardiovascular abnor-

malities may be considered in relatively small cohorts

of young healthy people 12 to 25 years of age, not

necessarily limited to competitive athletes (e.g., in

high schools, coll eges/universities or local commu-

nities). Close physician involvement and sufﬁcient

quality control is mandatory. If undertaken, such

initiati ves should r ecogni ze the known an d antic i-

pated limitations of the 12-lead ECG as a population

screening test, including the expected frequency of

false-positive and false-negative test results, as well

as the cost required to support these initiatives over

time (Class IIb; Level of Evidence C).

4. Mandatory and universal mass screening with 12-lead

ECGs in large general populations of young healthy

people 12 to 25 years of age (including on a national

basis in the United States) to identify genetic/

congenital and other cardiovascular abnormalities is

not recommended for athletes and nonathletes alike

(Class III, no evidence of beneﬁt; Level of Evidence C).

5. Consideration for large-scale, general population,

and universal cardiovascular screening in the age

group 12 to 25 years with history taking and physical

examination alone is not recommended (including

on a national basis in the United States) (Class III,

no evidence of beneﬁt; Level of Evidence C).

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DISCLOSURES

REFERENCES

1. Maron BJ , Friedman R A, Kligﬁeld P, Levine BD,

Viskin S, Chaitman BR, Okin P, Saul JP, Salberg L, Van

Hare GF, Soliman EZ, Chen J, Matherene P, Bolling SF,

Mittten MJ, Caplan A, Balady GJ, Thompson PS, on

behalf of the American Heart Association Council on

Clinical Cardiology, Advocacy Coordinating Commit-

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Writing Group Disclosures

Writing Group Member Employment

Research

Grant

Other

Research

Support

Speakers

Bureau/

Honoraria

Expert

Witness

Ownership

Interest

Consultant/

Advisory

Board Other

Barry J. Maron Minneapolis Heart Institute

Foundation

None None None None None None None

Aaron L. Baggish Massachusetts General Hospital,

Harvard Medical School

None None None None None None None

Richard J. Kovacs Indiana University None None None None None None None

Benjamin D. Levine University of Texas Southwestern

Medical Center

None None None Texas

Legislature*

None None None

Martin S. Maron Tufts Medical Center None None None None None None None

Reginald L. Washington Rocky Mountain Hospital for

Children

None None None None None None None

This table represents the relationships of writing group members that may be perceived as actual or reasonably perceived conﬂicts of interest as reported on the Disclosure Ques-

tionnaire, which all members of the writing group are required to complete and submit. A relationship is considered to be “signiﬁcant” if (a) the person receives $10,000 or more during

any 12-month period, or 5% or more of the person’s gross income; or (b) the person owns 5% or more of the voting stock or share of the entity, or owns $10,000 or more of the fair

market value of the entity. A relationship is considered to be “modest” if it is less than “signiﬁcant” under the preceding deﬁnition.

*Modest.

Reviewer Disclosures

Reviewer Employment

Research

Grant

Other

Research

Support

Speakers

Bureau/

Honoraria

Expert

Witness

Ownership

Interest

Consultant/

Advisory

Board Other

Robert M. Campbell Children’s Healthcare

of Atlanta

None None None None None None None

Luciana D.N. Janot

De Matos

Hospital Israelita Albert

Einstein (Brazil)

None None None None None None None

Christine E. Lawless Self-employed, Sports

Cardiology Consultants

Bryan Health

Foundation†

None None None None SCAA* None

This table represents the relationships of reviewers that may be perceived as actual or reasonably perceived conﬂicts of interest as reported on the Disclosure Questionnaire, which all

reviewers are required to complete and submit. A relationship is considered to be “signiﬁcant” if (a) the person receives $10,000 or more during any 12-month period, or 5% or more of

the person’s gross income; or (b) the person owns 5% or more of the voting stock or share of the entity, or owns $10,000 or more of the fair market value of the entity. A relationship

is considered to be “modest” if it is less than “signiﬁcant” under the preceding deﬁnition.

*Modest.

†Signiﬁcant.

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Competitive Athletes: Preparticipation Screening

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KEY WORDS ACC/AHA Scientiﬁc Statements,

athletes, cardiovascular abnormalities,

competitive athletes, preparticipation screening,

sudden death

Maron et al.

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AHA/ACC SCIENTIFIC STATEMENT

Eligibility and Disqualiﬁcation

Recommendations for Competitive

Athletes With Cardiovascular Abnormalities:

Task Force 3: Hypertrophic Cardiomyopathy,

Arrhythmogenic Right Ventricular

Cardiomyopath y and Other

Cardiomyopathies, and Myocarditis

A Scientiﬁc Statement From the American Heart Association and American College of Cardiology

Barry J. Maron, MD, FACC, Chair*

James E. Udelson, MD, FAHA, FACC*

Robert O. Bonow, MD, MS,

FAHA, MACC*

Rick A. Nishimura, MD,

FAHA, MACC*

Michael J. Ackerman, MD, P

HD*

N.A. Mark Estes III, MD, FACC*

Leslie T. Cooper J

R, MD, FAHA,

FACC*

Mark S. Link, MD, FACC*

Martin S. Maron, MD, FACC*

Hypertrophic cardiomyopathy (HCM) (1,2) is a ma jor

focus of this document given that it is the single most

common cause of sudden death in young competitive

athletes in the United States, responsible for at least

one-third o f t hese events (3).

HYPERTROPHIC CARDIOMYOPATHY

HCM is the most frequent nontraumatic cause of

sudden death in the young (1,2) and a common ge-

netic heart disease, occurring in at least 1 in 500

*On behalf of the American Heart Association Electrocardiography and

Arrhythmias Committee of the Council on Clinical Cardiology, Council on

Cardiovascular Disease in the Young, Council on Cardiovascular and

Stroke Nursing, Council on Functional Genomics and Translational

Biology, and the American College of Cardiology.

The American Heart Association and the American College of Cardiol-

ogy make every effort to avoid any actual or potential conﬂicts of interest

that may arise as a result of an outside relationship or a personal, pro-

fessional, or business interest of a member of the writing panel. Specif-

ically, all members of the writing group are required to complete and

submit a Disclosure Questionnaire showing all such relationships that

might be perceived as real or potential conﬂicts of interest. The Preamble

and other Task Force reports for these proceedings are available online at

www.onlinejacc.org (J Am Coll Cardiol 2015;XX:000–000; 000–000; 000–

000; 000 –000; 000–000; 000–000; 000–000; 000–000; 000–000; 000–

000; 000–000; 000–000; 000–000; 000–000; and 000–000).

This statement was approved by the American Heart Associati on

Science Advisory and Coordinating Committee on June 24, 2015, and

the Ameri can Heart Association Executive Committee on July 22, 2015,

and by the American College of Cardiology Board of Trustees and

Executive Committee on June 3, 2015.

The American College of Cardiology requests that this document be

cited as follows: Maron BJ, Udelson JE, Bonow RO, Nishimura RA,

Ackerman MJ, Estes NAM 3rd, Cooper LT Jr, Link MS, Maron MS; on

behalf of the American Heart Association Electrocardiography and

Arrhythmias Committee of the Council on Clinical Cardiology, Council

on Cardiovascular Disease in the Young, Council on Cardiovascular

and Stroke Nursing, Council on Functional Genomics and Translational

Biology, and the American College of Cardiology. Eligibility and

disqualiﬁcation recommendations for competitive athletes with cardio-

vascular abnormalities: Task Force 3: hypertrophic cardiomyopathy,

arrhythmogenic right ventricular cardiomyopathy and other cardiomy-

opathies, and myocarditis: a scientiﬁc statement from the American

Heart Association and American College of Cardiology. J Am Coll Cardiol

2015;xx:–.

This article has been copublished in Circulation.

Copies: This document is available on the World Wide Web sites of the

American Heart Association (my.americanheart.org) and the American

College of Cardiology (www.acc.org). For copies of this document, please

contact Elsevier Inc. Reprint Department via fax (212-633-3820) or e-mail

([email protected]).

Permissions: Multiple copies, modiﬁcation, alteration, enhancement,

and/or distribution of this document are not permitted without the ex-

press permission of the American College of Cardiology. Requests may be

completed online via the Elsevier site (http://www.elsevier.com/about/

policies/author-agreement/obtaining-permission).

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY VOL. - ,NO.- ,2015

ª 2015 BY THE AMERICAN HEART ASSOCIATION, INC. AND

THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION

ISSN 0735-1097/$36.00

http://dx.doi.org/10.1016/j.jacc.2015.09.035

PUBLISHED BY ELSEVIER INC.

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people in the general population (4). HCM is a clinically

and genetically heterogenous disease, associated with

>1,500 mutations in $11 major genes (and a variety of

other susceptibility genes with lesser evidence for path-

ogenicity), encoding proteins of the cardiac sarcomere,

adjacent Z disk, and calcium handling (5).

AlthoughHCMisassociatedwithsubstantialdiversity

in morphological expression (6), clinical diagnosis usua lly

occurs with recognition of the characteristic disease

phenotype, that is, left ventricular (LV) hypertrophy

without chamber dilatation in the absence of another

cardiacorsystemicdiseasecapableofproducingthe

magnitude of hypertrophy evident (1,6). Neither systolic

anterior motion of the mitral valve, hyperdynamic LV

function, or identiﬁca tion of pathoge nic sarcome re mu-

tations is obligatory for the clinical diagnosis of HCM (2).

Atrial ﬁbrilla tion is a common cause of mo rbidity in HCM,

occurring in z20% of patients, although usually after

30 years of age (1,2). Notably, the clinical presenta tion

and course are diverse, with unexpected sudden death in

the young the most visible disease co mplication.

SUDDEN DEATH RISK

AmajorimpetusinHCMhasbeentheidentiﬁcation of

those patients at increased risk for sudden death. Indeed, a

risk-stratiﬁcation algorithm has been largely effective in

identifying those people at highest risk who are eligible

for primary prevention of sudden death with an implant-

able cardioverter-deﬁbrillator (ICD) (7–10), thereby mark-

edly reducing HCM-related mortality to 0.5% per year (7).

Sudden death events are attributable to potentia lly lethal

ventricular tachyarrhythmias (ventricular tachycardia/

ventricular ﬁbrillation) and usually occur in the presence

of $1 the major risk markers (appropriate ICD interven-

tions of 4% per year in patients implanted for primary

prevention) (7–10). Some HCM patients may nevertheless

die suddenly in the absence of all conventional risk

factors (0.6% per year in non-ICD populations) (7).

Indeed, in the presence of underlying (and often

unsuspected) HCM, participation in high-intensity com-

petitive sports may itself promote ventricular tachy-

cardia/ventricular ﬁbrillation and act as a potent (yet

modiﬁable) independent risk factor, even in the absence

of conventional risk markers intrinsic to the disease pro-

cess (3,7,11,12). Notably, the underlying electrophysiolog-

ical substrat e in HCM is unpredictable (1,2,7–10) and

potentially subject to instability by interaction with phys-

iological stresses inherent in athletic training and compe-

tition, including alternations in hydration, blood volume,

and electrolytes, as well as the catecholamine s urge.

Given these principles, it is dif ﬁc ult to apply conve ntional

risk-stratiﬁcation strategies to make reliable eligibility de-

cisions speciﬁcally for aspiring competitive athletes with

HCM. The estimation of risk level b ased on phenotypic

expression (e.g., speciﬁc LV wall thickness or LV outﬂow

tract gradient) or other aspects of the clinical proﬁle is a

highly problematic endeavor. Such c onsiderations are

inﬂuenced by the morphological diversity of HCM and the

unpredictable instability of the myocardial substrate, as

well as the additive risk created by intense training and

competition in susceptible pa tients with HCM (3).There-

fore, in HCM, the mos t common cause of sudden death in

young athletes (1–3), engagement in intense competitive

sports is itself an acknowledged modiﬁable risk factor (1–3).

These observations necessitate conservative and

prudent recommendations regarding sports eligibili ty

applied in a homogeneous fashion across the broad HCM

disease spectrum. This may unavoida bly result in rec-

ommendations for disqualiﬁcation in some athletes with

HCM probably at low risk and unlikely to ever experience

sudden death, who could potentially compete an d train

safely. Notably, the present disqualiﬁcation/eligibili ty

guidelines for competitive athletes with HCM do not

differ measurably from those previously stated in the 36 th

Bethesda C onferen ce (11), because alternative new data or

insights have n ot emerge d sufﬁcient to substantially

alter the recommendations.

On the other hand, the present American Heart Asso-

ciation/American College of Cardiology recommenda tions

do not strictly exclude in absolute terms fully informed

athletes from participating in competitive athletic pro-

gramsaslongassuchadecisionisultimatelymade

in concert with their physician and third-party interests

(e.g., high schools and coll eges). Although this expert

consensus report serves as a prudent guideline regarding

sports eligi bility or disqualiﬁcation, there will always b e

tolerance in the system for some degree of ﬂexibility,

individual res ponsibility, and choice in making these

decisions for individual student athlete-patients.

Genotype Positive–Phenotype Negative

An increasing number of HCM f amily members are recog-

nizedwithdocumentedpathogenic(disease-causing)

sarcomere mutations, but in the absence of a clinical

HCM phenotype (i.e., LV hypertrophy) (5,13) .Suchpatients

have been identiﬁed at a broad range of ages, although they

are most commonly adoles cents and young adults, and

some wish to engage in competitive sports.

Spontaneous conversion to LV hypertrophy in this

subset appears to occur most often in adolescence be-

tween 12 and 20 years of age (1,13) but has also been

observed in midlife and beyond (14,15). Nevertheless, such

changes are unpredictable, and some genetically affected

people will probably never develop the HCM phenotype.

Spontaneous morphological conversions are not usually

accompanie d by cardiac symptoms, disea se p rogression,

or events (1,2,13–15). However, o nce LV hypertrophy

Maron et al.

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evolves, that person m ay theore tically be subject t o an

unstable HCM electrophysiological substrate.

With negative or am biguous gen etic test result s, poten-

tially affected relatives can nevertheless be suspected

clinically by t he prese nce of several echocardi ographic

or cardiovascular magnetic resonance (CMR) ﬁndings in

the nonhypertro phied myocardium, that is, blood-ﬁlled

crypts, elongated mitral valve leaﬂets, diastolic dysfunc-

tion, and my ocardial sc arring (5,16–19). At present, the risk

for sudden death in gene-positive–phenotype-negative

family members appears to be extremely low and likely

no different from the risk in the general population of

thesameagewithoutheartdisease(5,20). CMR imaging

is also an important consideration in family members who

are gene positive a nd judged to be phenotype negative

based on echocardiography, because areas of segmental

LV hypertrophy may be detected only by CMR, particularly

in the anterolateral free wall and apex (6,21).

Recommendations

1. Participation i n competitive athletics f or asymptom-

atic, genotype-positive HCM patients without evidence

of LV hypertrophy by 2-dimensional echocardiography

and CMR is reasonable, particularly in the absence of

a family history of HCM-related sudden death (Class

IIa; Level of Evidence C).

2. Athletes with a probable or unequivocal clinical

expression and diagnosis of HCM (ie, with the disease

phenotype of LV hypertrophy) should not participate

in most competitive sports, with the exception of those

of low intensity (class IA s ports) (see “ Classiﬁcation

of Sport” [22]). This recommendation is i ndependent

of age, sex, magnitude of LV hypertrophy, particular

sarcomere mutation, presence or absence of LV

outﬂow obstruction (at rest or with physiological ex-

ercise), absence of prior cardiac symptoms, presence

or absence of late gadolinium enhancement (ﬁbrosis)

on CMR, and whether major interventions such as

surgical myectomy or alcohol ablation have been

performed previously (Class III; Level of Evidence C).

3. Pharmacological agents ( e.g.,

-blockers) to control

cardiac-related symptoms or ventricular tachyar-

rhythmia s sho uld n ot b e adm inister ed fo r t he sole

purpose of permitting participation in high-intensity

sports. Notably, such drugs may also be inconsistent

with maximal physical performance in most sports

(Class III; Level of Evidence C).

4. Prophylactic ICDs should not be placed in athlete-

patients with HCM for the sole or primary purpose

of permitting participat ion in h igh-inten sity spo rts

competition because of the possibility of device-related

complications. ICD indications for competitive athletes

with HCM should not differ fr om those in nonathlete

patients with HCM (Class III; Level of Evidence B).

Other recommendations for sports participation in

patients with HCM and ICDs can be found in the

Task Force 9 report on “Arrhythmias and C onducti on

Defects” (23).

LV NONCO MPACTION

LV noncompaction (LVNC) is an uncommon and recently

recognized cardiac disease with spora dic or familial

occurrence (24). I ts t rue incidence a nd p revalenc e a re

notknown,inpartbecauseofdifﬁcult y in mak ing the

diagnosis and lack of agreement on criteria, as well as its

heterogeneous clinical spectrum a nd usual requirement

of CMR for rel iable d iagnosis. Furthermore, its clinica l

presentation and implications differ with respect to

genetic pathogene sis, race/ethnic origin, presence in is o-

lation or in association with other d iseases, or depen-

ding on the presence or absence of right ventricular

involvement (25).

The natural history of LVNC remains incompletely

resolved because of its relatively recent recognition wit h

a short available follow-up period (26– 34). The clinical

expression of LVNC is va riable, ev en within families: with

or without symptom s, heart failure, a trial and ventricu lar

arrhythmias or preexcitatory pathways, thromboembolic

events, or sudden death (35). While LVNC patients with

heart failure and systolic dysfunction, thromboembolic

events, and sudden cardiac death have been reported

(26,31,33,34), many uncomplicated cases are less likely

to be recognized or appear in the literature (34).Risk

for adverse consequences, including mortality, presently

appear to be largely as sociated wit h LV systolic dysfunc-

tion or ventricular tachy arrhythmias (34).

Few competitive athletes with LVNC h ave been

reported clinically, and therefore, the consequences of

LVNC in this speciﬁc population are unknown. Further-

more, to date, forensic registries of sudden deaths in

young athl etes do not inc lude LVNC as a c ause (3),

although the d iagnosis may still be widely underappre-

ciated in the rou tine m edical examiner autopsy setting.

Therefore, given the lack of long-term follow-up studies

and other obstacles, it is not yet possible to reliably apply

risk-stratiﬁcation strategies to new patients (or athletes)

with LVNC. This is not unlike t he situation with other

uncommon myocardial disea ses for which few dat a con-

cerning sudden death risk during competitive sports are

available (e.g., dilat ed cardiomyopathy [DCM] or inﬁltra-

tive diseases). Therefore, the complete natural history

of noncompacted ventricular myocardium remains

unresolve d.

A variety of inheritance patterns have been reported

(ie, autosomal dominant, autosomal recessive, and X-

linked) (24,27). Mutations in genes encoding sarcomeric

proteins, which previously have been implicated in the

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pathogenesis of HCM and DCM, have also been identiﬁed

in patients with isolated LVNC (24,27). These observ a-

tions suggest that LVNC shares genetic overlap with other

cardiomyopathies, a nd indeed, some individual patients

have been reported with morphological feat ures consis-

tent with both HCM and LVNC (32).

LVNC is thou ght t o b e caused by the intrauterine arrest

of the compaction process of the primordial embryonic

myocardium. Diagnosis is considered in the presence of

a 2-layered LV chamber that consists of noncompacted

trabeculations with intertrabecular recesses layered on

top of the typical compacted myocardium, with or with-

out systolic dys function. The trabecula ted layer is pre-

dominantly conﬁnedtothedistalandmidportionsof

the LV cha mber, sparing the base. Currently, there are

no universally accepted criteria or guidelines for the

morphological dia gnosis of LVNC, altho ugh a ra tio of

noncompacted to compacted myocardium >2.1:1 at end

systole (echocardiography) or >2.3:1 in end diastole

(CMR) have been proposed (30,32,36). It is uncertain how

athletic training may a lter those deﬁnitions (28,29) or

the frequency of LVNC-appearing morphology in a

normal athlete population. CMR is generally superior to

echocardiography for identiﬁcation of re gions of n on-

compacted myocardium and for more deﬁnitive diagnosis

of LVNC.

Recommendations

1. Until more clinical information is available, partici-

pation in competitive sports may be considered for

asymptomatic patients with a diagnosis of LVNC and

normal systolic function , wit hout i mportan t vent ric-

ular tachyarrhythmias on ambulatory monitoring

or exercise test ing, and speciﬁcally with no prior

history of unexplained syn cope (Class IIb; Level of

Evidence C).

2. Athletes with an unequivocal diagnosis of LVNC and

impaired systolic function or important atrial or ven-

tricular tachyarrhythmias on ambulatory monitoring

or exercise testing (or with a history of syncope)

should not participate in competitive sports, with the

possible exception of low-intensity c lass 1A sports,

at leas t unt il mor e clin ical information i s avail abl e

(Class III; Level of Evidence C).

OTH ER M YO CA RD IA L DI SE AS ES

A number of other uncommon diseases of the myocar-

dium deserve consideration as potential causes of sudden

death in athletes. These include D CM (attributable to

a variety of causes, including genetic), primary non-

hypertrophied restrictive cardiomyopathy, and systemic

inﬁltrative diseases with secondary cardiac involvement,

such as sarcoidosi s. F ew data are available at present

regarding the relative risks of athletic training and

competition in athletes with these myocardial diseases.

It is important to differentiate physiolo gical LV

enlargement caused b y systematic training from patho-

logical DCM. Long-term aerobic athletic training can lead

to cardiac morphological changes, including increased LV

cavity dimension and calculated mass. Increased cavity

size can produce a higher stroke volume, and thus, the

ejection fraction at rest may be in the low-normal to

mildly reduced range. Up to 15% of trained at hletes will

have substant ial enlargem ent of the LV c avity, with end-

diastolic dimensions up to 70 mm in men and 66 mm in

women (37,38). Ejection fraction in trained athletes has

been shown to be as low as 45% (37).Whethernewer

imaging techniques such as myocardial Doppler tissue

imaging, strain imaging, or contras t-CMR scanni ng can

differentiate patients with borderline LV enlargement and

low-normal or mild ly reduced e jection fraction from DCM

is unresolved.

It is unclear wh ether as ymptomatic pat ients with DCM

are at risk for sudden death during competitive athletics,

because ventricular tachyarrhythmias are most common

in patients with more advanced disease, that is, with

cardiac symptoms and lower ejection fraction.

Recommendations

1. Symptomatic athletes with D CM, primary non-

hypertrophied restrictive cardiomyopathy, and inﬁl-

trative cardiac myopathies should not participate in

most competitive sports, with the possible excep tion

of low-intensity (class 1A sports) in selected cases, at

least until more information is available (Class III;

Level of Evidence C).

MYOCARDITIS

General Considerations

Myocarditis commonly presents with disproportionate

dyspnea on exertion, chest pain, and arrhythmias. It can

also present as an acute myocardial infarction–like syn-

drome with sudden death in the presence of normal

epicardial coronary arteries (39– 44).Thecontribution

of myocarditis to ca rdiovascular sudden death varies

signiﬁcantly with age, causing cardiovascular sudden

death in z2% of infants, 5% of children, and 4% to 7.5% of

athletes (3,40) . H igher rates of myocarditis are occasion-

ally reported in postmortem studies from general pop-

ulations younger than 35 to 40 years of age (41).Most

cardiovascular sudden deaths attributable to myocarditis

occur in males (42), and in som e ca ses, myocar ditis results

in sudden death without antecedent s ymptoms or

macroscopic cardiac abnormalities (40,42,43).

The data linking myocarditis to sudden death are strong

and include autopsy studies a nd experimental myocarditis

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models. For example, strenuous physical exertion was

associated with sudden death i n a cohort of U.S. military

recruits, with the most frequent underlying cause being

myocarditis (44). Case series of s udden death in athletes

have established myocarditis as a signiﬁcant risk in this

speciﬁcgroup(3). In a murine model of coxsackie B3

myocarditis, 60 minutes of swimming daily increased viral

titers, w orsened car diomyopathy, and increased t he like-

lihood of death (45). In a chronic autoimmune myocarditis

model, humeral and cellular immunity directed against

heart tissues increased with treadmill exercise (46). Unlike

heart failure, the risk of sudden death caused by myocar-

ditis d oes n ot appear to correlate with the severity of

myocardial inﬂammation (40). Sudden death has been

observed occasiona lly after myo pericarditis in association

with normal LV function (47–49).

The pathogenesis of myocarditis consists of 3 over-

lapping phases: acute injury, often caused by a virus; the

host innate and acquired immunologic response; and

ﬁnally, recovery or a transition to scar and DCM. There is

rarely a clear distinction between these phases clinically.

The initial injury may cause an acute D CM with contractile

impairment mediated by cytokines generated by the local

inﬂammatory process. Several months later, the same

dilated ventricle may have p oor contractility caus ed by

diffuse sca r, with little or no inﬂammation. The transition

from acute myocarditis to chronic DCM probably occurs

over months, with substantial individual variability (50).

In clinical practice, my ocarditis is often suspected but

infrequently conﬁrmed b y endomyocardial biopsy, which

creates a need for noninvasive diag nostic criteria to guide

recommendations for athletic participa tion. For the pur-

poses of this document, probable acute myo carditis is

diagnosed when both of the following criteria are met:

1. A clinical syndrome that includes acute heart failure,

angina-type chestpain, or myopericarditisof <3months’

duration.

2. An otherwise unexplained elevation i n serum troponin;

electrocardiographic features of cardiac ischemia;

otherwise unexp lained high-degree AV block or

arrhythmias; wall motion abnormalities; pericardial

effusion on echocardiography or CMR imaging. Addi-

tional CMR ﬁndings that sugg est myocarditi s in the

acute clinical setting include characterist ic alteration s

in tissue signal on T2- or T1-weighted images and the

presence of late gadolinium enhancement (LGE).

CMR features t hat may be used to diagnose probable

myocarditis include a regional increase in water con-

tent visible on T2-weighted images, an increase in regional

contrast-enhanc ed T1-weighted epic ardial or m id-

myocardial sign al obtained within a few min utes of the

gadolinium bolus (“hyperemia” or “early-enhancement”

sequences), and epicardial or midmyocardial LGE (51).A

regional an d rev ersible increase in wall thickness that in-

dicates myocardial edema is a supportive ﬁnding of acute

myocarditis . My ocardial ﬁbrosis, the late sequelae o f

myocarditis characteristic of DCM, may be indistinguish-

able from active myocarditis on LGE sequences.

The sens itivity of CMR f or my ocarditis also decrea ses a

few weeks after the initial illness (51).

Although a cute m yocarditis is asso ciated wi th the

characte ristic ﬁndings of myocardial injury described in

the diagnostic criteri a above, there is no sensitive o r

speciﬁc test that can determine when the inﬂammatory

process ends. DCM associated with acute myocarditis

often resolves over 6 to 12 months . Athletes in whom the

ﬁnd ings of acute inﬂammation have resolved may s till

have a risk of a rrhythmias related to the resultant

myocardial scar. The presence of LGE may convey a

heightened risk for arrhythmias (52).Theintervalbe-

tween initial assessment and retesting before resumption

of sports will vary dependi ng on the severi ty of th e i nitial

illness. A reasona ble minimum interva l for retesting

based on experimenta l model s is 3 to 6 month s. Th e rec-

ommendations presented here recognize these gaps in

knowledge and the need for additional clinical research

to reﬁne risk stratiﬁcation for sudden death after acute

myocarditis .

A diagnosis of myocarditis by biopsy is usually not

required to guide clinical management, but a b iopsy may

be considered in select cases according to current pro-

fessional society recommendations from the American

Heart A ssociation , Ame rican College of Cardiology, and

Europea n Society of Card iology (53).Conﬁrmation of

myocarditis by endomyocardial biopsy c reates a deﬁni-

tive d iagnosis.

Recommendations

1. Before returning to competitive sports, athletes who

initially present with an acute clinical syndrome

consistent with myocarditis should undergo a resting

echocar diogram , 2 4-hour Holt er m onit oring, and an

exercise ECG no less than 3 to 6 months after the

initial illn ess (Class I; Level o f Evidence C).

2. It is reasonable that athletes resume training and

competition if all of the following criteria are met

(Class IIa; Level of Evidence C):

a. Ventricular systolic function has returned to the

normal range.

b. Serum markers of myocardial injury, inﬂammation,

and h eart failure have normalized.

c. Clinically relevant arrhythmias such as frequent

or complex repetitive forms of ventricular or sup-

raventricular ectopic activity are absent on Holter

monitor and graded exercise ECGs.

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At present, it is unresolved whether resolution of

myocarditis-related LGE should be required to permit

return to competitive sports.

3. Athletes with probable or deﬁnite myocarditis should

not participate in competitive sports while active

inﬂammation is present. This recommendation is

independent of age, gender, and LV function (Class III;

Level of Evidence C).

ARRHYTHMOGENIC RIGHT VENTRICULAR

CARDIOMYOPATHY

Arrhythmogenic right ventric ular cardiomyopathy (ARVC)

is a cause of sudden d eath in young people a nd athletes,

particularly in the northeas tern (Veneto) regio n of Italy

(54), but is seemingly less common in the United States

(3). ARVC is characterized by a broad phenotypic spec-

trum and characteristically b y loss of myocytes in the

right ventricul ar myocardium, with fatty or ﬁbrofatty

replacement, which results in segmental or diffuse wall

thinning, but there is a lso frequent involvement of the LV

and an association with myocarditis (55). Genetics studies

have demonstra ted tha t ARV C is a d esmosomal cardio-

myopathy that results from genetically defective cell-

adhesion proteins such as plakoglobin, plakophilin-2,

desmoplakin, desmocollin-2, and desmoglein-2 (56,57).

Clinical diagnosis can be challenging but relies largely on

familial occurrence, left b undle-branch pattern ventricular

tachyarrhythmias, ECG ﬁndings of T-wave inversion in

precordial leads V

through V

, and epsilon waves, as well

as right ventricular dilatio n or segmental wal l motion ab-

normalities, a neurysm formation, or fatty deposition in the

right ventricular wall identiﬁed w ith CMR imaging if sub-

stantial and unequivocal (or by biopsy tissue analysis).

Diagnostic criteria for ARVC have b een revised and upda-

ted and now include quantitative variables (58).

These criteria include global or regional struc tural

dysfunction, as documented by echocardiography or

CMR, biopsy abnormal ities, ECG repolariza tion or depo-

larization ab normalities, arrhythmias, and family history.

Each of these criteria is separated into major and minor

criteria based on the severity of the ﬁnding. Patients meet

an ARVC diagnosis if they possess 2 major, or 1 major and

2 minor, or 4 minor criteria. Borderline patients are those

with 1 major and 1 min or criterion or 3 m inor criteri a.

Patients with possible ARVC ha ve 1 major criterion or 2

minor criteria. Athletes with borderline or possible ARVC,

as well as those who are genoty pe positi ve–phenotype

negative, should receive continued follow-up, because

ARVC may progress phenotypically, and become more

clinically apparent with time.

There is evidence in the experimental murine model

that exercise increases the penetrance and arrhythmic

risk in mutational carriers of ARVC (59). More recently,

these data have been conﬁrmed in genetically positive

patients (60), which is particularly relev ant to the athlete,

raising concern not only with regard to competitive sports

but also regarding participation in moderate to extreme

recreational physical activities.

Ventricular tachyarrhythmias and sudden death in

ARVC commonly occur during exertion, including

competitive sports (55,60,61), and frequent endurance

exercise increases the risk for ventricular tachycardia/

ventricular ﬁbrillation and heart fa ilure (60). However,

risk factors for sudden cardiac death in ARVC are not

as well deﬁned a s in H CM (1,2,7,8).Thereisgeneral

agreement that a pri or history of s udden cardiac death,

sustained ventricular tachycardia, or syncope represent

themostimportantprogno stic factors and deﬁne man y

high-risk patients who are most appropriatel y treated

with a primary prevention ICD (62–64).

Recommendations

1. Athletes with a deﬁnite diagnosis of ARVC should not

participate in most competitive sports, with the

possible exception of low-intensity class 1A sports

(Class III; Level of Evidence C).

2. Athletes with a borderline diagnosis of ARVC should

not participate in most competitive sports, with the

possible exception of low-intensity class 1A sports

(Class III; Level of Evidence C).

3. Athletes with a possible diagnosis of ARVC should not

participate in most competitive sports, with the

possible exception of low-intensity class 1A sports

(Class III; Level of Evidence C).

4. Prophylactic ICD placement in athlete-patients with

ARVC for the sole or primary purpose of permitting

participation i n high-intensity sports competition is

not recommended because of the possibility of device-

related complications (Class III; Level of Evidence C).

Otherrecommendationsforsports participation in pa-

tients with ARVC and ICDs can be found in t he Task Force 9

report on “Ar rhythmia s and Cond uction D efects” (23).

PERICARDITIS

The causes of pericarditis/myopericardit is are varied and

are either infectious or noninfectious. The natural history

is incompletely resolved, althoug h long-term prognosis

is generall y favorable. The diagnosi s o f acute pericard itis

is typically based on clinical criteria: chest pain, pericar-

dial rub, ST-segment elevation, or new/worsening peri-

cardial effusion. This syndrome may be considered part

of the clinical spect rum of myocarditis. Recurrences are

asigniﬁcant consideration, and follow-up surveil-

lance w ith echocardiography or CMR is recommended to

exclude pericardia l thickening or restriction consistent

with restrictive pericarditis (50).

Maron et al.

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Recommendations

1. Athletes with pericarditis, regardless of its patho-

genesis, should not participate in competitive

sports during the acute phase. Such athletes can

return to full activity when there is complete

absence of evidence for active disease, including

effusion by echocardiography, and when serum

markers of inﬂammation have normalized. For

pericarditis associated with evidence of myocardial

involvement, eligibility should also be based on the

course of myocarditis. Chronic pericardial disease

that results in c onstriction disqualiﬁes the person

from all competitive sports (Class III; Level of

Evidence C).

Writing Group Disclosures

Writing Group

Member Employment Research Grant

Other

Research

Support

Speakers

Bureau/

Honoraria

Expert

Witness

Ownership

Interest

Consultant/Advisory

Board Other

Barry J. Maron Minneapolis

Heart Institute

Foundation

None None None None None None None

Michael J. Ackerman Mayo Clinic NIH (R01 grants)† None None None None Boston Scientiﬁc*; Gilead

Sciences*; Medtronic*;

St. Jude Medical*

Transgenomic†

Robert O. Bonow Northwestern

University

None None None None None None None

Leslie T. Cooper, Jr Mayo Clinic None None None None None None None

N.A. Mark Estes III Tufts Medical Center None None None None None Medtronic*; St. Jude

Medical†; Boston

Scientiﬁc†

None

Mark S. Link Tufts Medical Center None None None None None None None

Martin S. Maron Tufts Medical Center None None None None None None None

Rick A. Nishimura Mayo Clinic None None None None None None None

James E. Udelson Tufts Medical Center Gilead (DSMB member

for a clinical trial)*

None None None None None None

This table represents the relationships of writing group members that may be perceived as actual or reasonably perceived conﬂicts of interest as reported on the Disclosure Ques-

tionnaire, which all members of the writing group are required to complete and submit. A relationship is considered to be “signiﬁcant” if (a) the person receives $10,000 or more during

any 12-month period, or 5% or more of the person’s gross income; or (b) the person owns 5% or more of the voting stock or share of the entity, or owns $10,000 or more of the fair

market value of the entity. A relationship is considered to be “modest” if it is less than “signiﬁcant” under the preceding deﬁnition.

*Modest.

†Signiﬁcant.

Reviewer Disclosures

Reviewer Employment

Research

Grant

Other Research

Support

Speakers Bureau/

Honoraria

Expert

Witness

Ownership

Interest

Consultant/

Advisory Bo ard Other

Linda J. Addonizio Columbia University None None None None None None None

Eugene DePasquale University of California,

Los Angeles

None None None None None None None

Michelle A. Grenier University of Mississippi None None None None None None None

This table represents the relationships of reviewers that may be perceived as actual or reasonably perceived conﬂicts of interest as reported on the Disclosure Questionnaire, which all

reviewers are required to complete and submit. A relationship is considered to be “signiﬁcant” if (a) the person receives $10,000 or more during any 12-month period, or 5% or more of

the person’s gross income; or (b) the person owns 5% or more of the voting stock or share of the entity, or owns $10,000 or more of the fair market value of the entity. A relationship

is considered to be “modest” if it is less than “signiﬁcant” under the preceding deﬁnition.

DISCLOSURES

JACC VOL. - ,NO.- ,2015

Maron et al.

- ,2015:- – -

Competitive Athletes: Hypertrophic Cardiomyopathy, ARVC, and Myocarditis

PGL 5.4.0 DTD  JAC21833_proof  15 October 2015  4:15 am  ce

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KEY WORDS ACC/AHA Scientiﬁc Statements,

athletes, arrhythmogenic ri ght ve ntricular

cardiomyopathy, cardiomyopathy, cardiovascular

abnormalities, cardiovascular magnetic

resonance, hypertrophic cardiomyopathy,

myocarditis, su dden death

Maron et al.

JACC VOL. - ,NO.- ,2015

Competitive Athletes: Hypertrophic Cardiomyopathy, ARVC, and Myocarditis

- ,2015:- – -

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AHA/ACC SCIENTIFIC STATEMENT

Eligibility and Disqualiﬁcation

Recommendations for Competitive Athletes

With Cardio vascular Abnormalities:

Task Force 4: Congenital Heart Disease

A Scientiﬁc Statement From the American Heart Association and American College of Cardiology

George F. Van Hare, MD, FACC,

Chair*

Michael J. Ackerman, MD, P

HD,

FACC*

Juli-anne K. Evangelista, DNP,

APRN, CPNP-AC, FACC*

Richard J. Kovacs, MD, FAHA, FACC*

Robert J. Myerburg, MD, FACC*

Keri M. Shafer, MD*

Carole A. Warnes, MD, FACC*

Reginald L. Washington, MD, FAHA*

Congenital heart disease (CHD) is the most common

form of serious birth defect, occurring in 8 per 1,000

live b irths (1). The past several decades have seen

dramatic improvements in survival with palliativ e or

corrective heart surgery, such that there are now more

adult patients than pediatric patients alive with CHD.

Although restriction from competitive athletics may

well be indicat ed f or s ome, th e great majority of

patients can and should engage in some form of

physical activity and should avoid a sedentary life-

style. Clinicians should encourage their patients

to engage in healthy physical activities, bearing in

mind speciﬁc features in some p atients, s uch as

residual obstruction, pulmonary vascular disease,

low systemic ventricular function, and preexisting

arrhythmias in the presence of implanted cardiac

rhythm dev ices such as pacemakers and i m-

plantable cardioverter-deﬁbrillators. In addition, the

physiological effects of athletic activities at high

altitude should be considered for patients with

elevated pulmonary vascular resistance. These issues

are covered elsewhere in this document. Fortunately,