36th bethesda eligibility recommendations for competitive athletes with cardiovascular abnormalities

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Journal of the American College of Cardiology Vol. 45, No. 8, 2005© 2005 by the American College of Cardiology Foundation ISSN 0735-1097/05/$30.00P

6th Bethesda Conferenceligibility Recommendations forompetitive Athletes With Cardiovascular Abnormalities

ublished by Elsevier Inc. doi:10.1016/j.jacc.2005.02.002

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ETHESDA CONFERENCE REPORT

6th Bethesda Conference:ligibility Recommendations forompetitive Athletes With Cardiovascular Abnormalities

arry J. Maron, MD, FACC, Conference Co-Chairouglas P. Zipes, MD, MACC, Conference Co-Chair

This Conference, sponsored by the American College of Cardiology Foundation, was heldat Galerie 1, New Orleans Marriott, New Orleans, Louisiana, on November 6, 2004.Please refer to the appendix of each Task Force report for author disclosure information.


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forth in d do noollege ofs availab ollege ont may r by wriServices, 20814-16ies, mod n of this

ICHAEL J. ACKERMAN, MD, PHD,FACC

Pediatric CardiologistMayo Clinic/Mayo FoundationDepartment of Pediatric and Adolescent

Medicine - E9200 First Street SWRochester, MN 55905-0001

ARY JOHN BALADY, MD, FACCDirector, Preventive CardiologyProfessor of MedicineBoston University Medical CenterDivision of Cardiology88 E. Newton StreetBoston, MA 02118-2308

OBERT O. BONOW, MD, FACCGoldberg Distinguished ProfessorChief, Division of CardiologyNorthwestern UniversityFeinberg School of Medicine201 East Huron Street, Suite 10-240Chicago, IL 60611-2957

ERNARD R. CHAITMAN, MD, FACCDirector, Cardiovascular ResearchSt. Louis University School of MedicineDivision of Cardiology1034 S. Brentwood Blvd., Suite 1550St. Louis, MO 63117

ELVIN D. CHEITLIN, MD, MACCEmeritus Professor of MedicineUniversity of California San FranciscoSan Francisco General Hospital224 Castenada AvenueSan Francisco, CA 94116-1445

The recommendations setposition of the American C

Copies: This document iSingle copies of this documeof Cardiology, Educational

Permissions: Multiple cop
without the express [email protected].
Participants/AuthorsUTHER T. CLARK, MD, FACCChief, Division of Cardiovascular

MedicineState University of New YorkDownstate Medical Center85-42 Midland ParkwayJamaica, NY 11432-2222

ICHAEL H. CRAWFORD, MD,FACC

Professor of MedicineChief of Clinical CardiologyUniversity of California San Francisco

Medical Center505 Parnassus AvenueCardiology Box 0124San Francisco, CA 94143-0124

AMELA S. DOUGLAS, MD, FACCUrsula Geller Professor of Research in

Cardiovascular DiseaseChief, Division of Cardiovascular

MedicineDuke UniversityDUMC 3943 Duke North 7451Erwin RoadDurham, NC 27710

AVID J. DRISCOLL, MD, FACCMayo Clinic, East 9200 First Street SWRochester, MN 55905-0001

. A. MARK ESTES, III, MD, FACCProfessor of MedicineTufts University School of Medicine750 Washington StreetBoston, MA 02111

this report are those of the Conference participants anCardiology Foundation.

le on the World Wide Web site of the American Cbe purchased for $10.00 by calling 1-800-253-4636 o9111 Old Georgetown Road, Bethesda, Marylandification, alteration, enhancement, and/or distributio
of the American College of Cardiology Foundation.
OHN M. FONTAINE, MD, FACCMain Clinical Campus of the

Medicine College of Pennsylvania3300 Henry AvenuePhiladelphia, PA 19129

ELTON M. GERSONY, MD, FACCProfessor of PediatricsNew York Presbyterian HospitalChildren’s Hospital of New York3959 Broadway 2-NorthNew York, NY 10032-1537

AMUEL S. GIDDING, MD, FACCDupont Hospital for ChildrenNemours Cardiac Center1600 Rockland RoadWilmington, DE 19803

HOMAS P. GRAHAM, JR, MD, FACCDirector, Pediatric CardiologyVanderbilt Medical Center1161 21st Avenue South, D2220 MCNNashville, TN 37232-2572

UGUSTUS O. GRANT, MBCHB, PHD,FACC

Duke University Medical CenterBox 3504Durham, NC 27702-3504

ILLIAM L. HASKELL, PHDProfessor730 Welch RoadPalo Alto, CA 94304-1506

t necessarily reflect the official

f Cardiology (www.acc.org).ting to the American College99.document are not permitted
Please direct requests to
http://www.acc.org

mailto:[email protected]

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1314 Maron and Zipes JACC Vol. 45, No. 8, 200536th Bethesda Conference Participants April 19, 2005:1313–5

ARK A. HLATKY, MD, FACCProfessor of Health Research & Policy,

and of MedicineStanford University School of MedicineHRP Redwood Building, Room 150Palo Alto, CA 94305-5405

ORMAN M. KAPLAN, MDUniversity of TexasSouthwestern Medical Center at Dallas5323 Harry Hines Blvd.Dallas, TX 75235

OBERT A. KLONER, MD, PHD, FACCProfessor of Medicine, Keck School of

MedicineUniversity of Southern CaliforniaDirector of ResearchGood Samaritan Hospital Heart Institute1225 Wilshire Blvd.Los Angeles, CA 90017-2308

ENJAMIN D. LEVINE, MDDirector, Institute for Exercise and

Environmental MedicineS. Finley Ewing Jr. Chair for Wellness of

Presbyterian Hospital of DallasHarry S. Moss Heart Chair for

Cardiovascular ResearchProfessor of MedicineUniversity of Texas Southwestern Medical

Center of Dallas5323 Harry Hines Blvd.Dallas, TX 75390-9034

ARK S. LINK, MD, FACCTufts-New England Medical Center750 Washington StreetBox 197Boston, MA 02111

ARRY J. MARON, MD, FACCDirector, Hypertrophic Cardiomyopathy

CenterMinneapolis Heart Institute Foundation920 E. 28th Street, Suite 60Minneapolis, MN 55407-3984

ERE H. MITCHELL, MD, FACCMoss Heart CenterUniversity of TexasSouthwestern Medical Center5323 Harry Hines Blvd.
Dallas, TX 75390-7208
ATTHEW J. MITTEN, JDAssociate DeanProfessor of LawDirector, NALIMarquette University Law School1103 W. Wisconsin AvenueMilwaukee, WI 53233

OBERT J. MYERBURG, MD, FACCDirector, Division of CardiologyUniversity of Miami School of MedicineJackson Memorial HospitalDivision of Cardiology D-39PO Box 016960Miami, FL 33101-6960

ANE W. NEWBURGER, MD, MPH,FACC

Associate Cardiologist-in-ChiefChildren’s Hospital BostonDepartment of Cardiology300 Longwood AvenueBoston, MA 02115-5724

ICK A. NISHIMURA, MD, FACCProfessor of MedicineMayo Clinic, Division of Cardiovascular

Disease200 First Street SWGonda 5-468Rochester, MN 55905-0001

RIAN OLSHANSKY, MD, FACCDirector, Cardiac ElectrophysiologyUniversity of Iowa200 Hawkins Drive, 4426A JCPIowa City, IA 52242

HOMAS G. PICKERING, MD, DPHIL

Director, Integrative and BehavioralCardiology Program

Columbia University Health ServicesBehavioral Cardiovascular Health and

HypertensionPH 9 Room 946622 W. 168th StreetNew York, NY 10032

EED E. PYERITZ, MD, FACCUniversity of Pennsylvania Medical CenterChief of the Division of Medical Genetics538 Maloney Building36th and Spruce StreetsPhiladelphia, PA 19104

LBERT ROCCHINI, MDUniversity of MichiganBox 0204
Ann Arbor, MI 48109-0204
ETER SNELL, PHDMoss Heart CenterUniversity of Texas Southwestern Medical

Center5323 Harry Hines Blvd.Dallas, TX 75390-7208

AUL D. THOMPSON, MD, FACCDirector, Preventive Cardiology/

Cholesterol Management CenterHartford HospitalDivision of Cardiology80 Seymour StreetHartford, CT 06102-8000

EFFREY A. TOWBIN, MD, FACCProfessor and Chief, Pediatric CardiologyBaylor College of MedicineTexas Children’s Hospital6621 Fannin Street, MC 19345-CHouston, TX 77030

AMES E. UDELSON, MD, FACCDivision of CardiologyUniversity of California, Irvine4244 Jackson StreetSan Diego, CA 92103

TEVEN P. VAN CAMP, MD, FACC4244 Jackdaw StreetSan Diego, CA 92103

EORGE F. VAN HARE, MD, FACCAssociate Professor of PediatricsStanford UniversityPediatric Cardiology750 Welch Road, Suite 305Palo Alto, CA 94304

ENU VIRMANI, MD, FACCChair, Department of Cardiovascular

PathologyArmed Forces Institute of PathologyBuilding 54, Room 200514th Street and Alaska Ave. NWWashington, DC 20306-6000

ACKSON T. WRIGHT, JR, MD, PHDUniversity Hospitals of Cleveland11100 Euclid AvenueCleveland, OH 44106

OUGLAS P. ZIPES, MD, MACCDistinguished Professor of MedicinePharmacology and ToxicologyDirector, Division of CardiologyIndiana University School of MedicineDirector, Krannert Institute of Cardiology1800 North Capitol, Suite E475
Indianapolis, IN 46202

1315JACC Vol. 45, No. 8, 2005 Maron and ZipesApril 19, 2005:1313–5 36th Bethesda Conference Participants

Participants/ReviewersJOHN H. McANULTY, JR, MD, FACCLAWRENCE RINK, MD

Task Force on Clinical Expert Consensus DocumentsROBERT A. VOGEL, MD, FACC, CHAIR

JONATHAN ABRAMS, MD, FACCJEFFREY L. ANDERSON, MD, FACCERIC R. BATES, MD, FACCMARK J. EISENBERG, MD, MPH, FACCCINDY L. GRINES, MD, FACCMARK A. HLATKY, MD, FACCSANJIV KAUL, MD, FACCROBERT C. LICHTENBERG, MDJONATHAN R. LINDNER, MD, FACCGERALD M. POHOST, MD, FACCRICHARD S. SCHOFIELD, MD, FACCSAMUEL J. SHUBROOKS, JR, MD, FACCCYNTHIA M. TRACY, MD, FACC

StaffCHRISTINE W. MCENTEE, CHIEF EXECUTIVE OFFICER

DAWN R. PHOUBANDITH, MSW, ASSOCIATE DIRECTOR,CLINICAL POLICY AND DOCUMENTS

LISA BRADFIELD, SPECIALIST, CLINICAL POLICY AND DOCUMENTS

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reamble .................................................................................1317

ntroduction: Eligibility Recommendations forompetitive Athletes With Cardiovascular Abnormalities—eneral Considerations.........................................................1318Impetus for the Revision ...................................................1318Definitions .........................................................................1318Prevalence...........................................................................1318Conference Design and Format ........................................1319Recommendations for Disqualification and Eligibility .....1319Special Considerations .......................................................1320References ..........................................................................1321Appendix 1.........................................................................1321

ask Force 1: Preparticipation Screening and Diagnosisf Cardiovascular Disease in Athletes .................................1322

Preparticipation Screening .................................................1322Diagnostic Testing Strategies ............................................1323Athlete’s Heart and Cardiovascular Disease .....................1324References ..........................................................................1325Appendix 1.........................................................................1326

ask Force 2: Congenital Heart Disease ............................1326General Considerations .....................................................1326Types of Congenital Defects .............................................1326References ..........................................................................1333Appendix 1.........................................................................1333

ask Force 3: Valvular Heart Disease.................................1334General Considerations .....................................................1334Mitral Stenosis ...................................................................1334Mitral Regurgitation ..........................................................1335Aortic Stenosis ...................................................................1336Aortic Regurgitation ..........................................................1337Bicuspid Aortic Valves With Aortic Root Dilatation .........1338Tricuspid Regurgitation.....................................................1338Tricuspid Stenosis..............................................................1338Multivalvular Disease.........................................................1338Prosthetic Heart Valves .....................................................1338Valve Repair or Percutaneous Mitral Balloon

Valvotomy.....................................................................1339References ..........................................................................1339Appendix 1.........................................................................1340

ask Force 4: HCM and Other Cardiomyopathies, Mitralalve Prolapse, Myocarditis, and Marfan Syndrome .........1340

Hypertrophic Cardiomyopathy ..........................................1340Mitral Valve Prolapse (MVP) ...........................................1341Myocarditis ........................................................................1342Marfan Syndrome ..............................................................1342Ehlers-Danlos Syndrome...................................................1343Arrhythmogenic Right Ventricular Cardiomyopathy

(ARVC) ........................................................................1343Other Myocardial Diseases................................................1343Pericarditis..........................................................................1344References ..........................................................................1344
Appendix 1.........................................................................1345
ask Force 5: Systemic Hypertension .................................1346General Considerations .....................................................1346References ..........................................................................1347Appendix 1.........................................................................1348

ask Force 6: Coronary Artery Disease ..............................1348Atherosclerotic Coronary Artery Disease..........................1348Coronary Artery Vasospasm..............................................1351CAD in Cardiac Transplant Recipients............................1351Myocardial Bridging ..........................................................1352References ..........................................................................1352Appendix 1.........................................................................1353

ask Force 7: Arrhythmias ...................................................1354General Considerations .....................................................1354Syncope ..............................................................................1355Types of Arrhythmias ........................................................1356References ..........................................................................1362Appendix 1.........................................................................1363

ask Force 8: Classification of Sports.................................1364Response and Adaptation to Exercise ...............................1364Athlete’s Heart...................................................................1365Classification of Sports ......................................................1365Limitations of Classification..............................................1366References ..........................................................................1367Appendix 1.........................................................................1367

ask Force 9: Drugs and Performance-Enhancingubstances ..............................................................................1368

General Considerations .....................................................1368References ..........................................................................1369Appendix 1.........................................................................1369

ask Force 10: Automated External Defibrillators ............1369General Considerations of Cardiac Arrest Risk

Among Athletes ...........................................................1369Role of Automated External Defibrillators in

Response to Cardiac Arrest..........................................1370AEDs at Sites of Training and Competition ...................1370References ..........................................................................1370Appendix 1.........................................................................1371

ask Force 11: Commotio Cordis ......................................1371General Considerations .....................................................1371References ..........................................................................1373Appendix 1.........................................................................1373

ask Force 12: Legal Aspects of the 36th Bethesdaonference Recommendations ............................................1373

General Considerations .....................................................1373References ..........................................................................1375
Appendix 1.........................................................................1375

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6TH BETHESDA CONFERENCE

reamble


obert A. Vogel, MD, FACC, Chair, ACCF Task Force on Clinical Expert Consensus Documents

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his 36th Bethesda Conference report is the result of aonsensus conference held on November 6, 2004, in Newrleans, Louisiana. Consensus conferences are designed to

acilitate consideration of significant and timely issues re-arding the practice of cardiovascular medicine and mattersffecting patient care, research, and training for whichbsolute or hard data are incomplete. Appropriate organi-ations other than the American College of Cardiologyoundation (ACCF) are invited to send representatives toarticipate in the process, as appropriate to each topic.hese may include physicians and health care specialists asell as representatives from industry, government, founda-

ions, and the public sector. A report is published toighlight recommendations, along with dissenting opinion,

f appropriate. The recommendations set forth in this reportre those of the conference participants only and do not

The Task Force on Clinical Expert Consensus recog-izes the importance of maintaining high ethical stan-ards and avoiding conflicts of interests. Because theevelopment of this conference report depends on thenowledge and experience of its volunteers, many ofhom have relationships with industry, the policy forriting committee members’ relationships must be real-

stic, workable, and implemented in a way that protectshe integrity of the process while allowing an open andonest exchange of information. Therefore, the Taskorce facilitates a process for collection of disclosuretatements of all relationships of conference participantshat might be perceived as real or potential conflicts ofnterest relevant to the conference activity and publisheshis information with the conference report. Please refero the appendix of each Task Force report for author

ecessarily reflect the official position of the ACCF. disclosure information.

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6TH BETHESDA CONFERENCE

ntroduction: Eligibilityecommendations for Competitive Athletes Withardiovascular Abnormalities—General Considerations

arry J. Maron, MD, FACC, Co-Chair


ouglas P. Zipes, MD, MACC, Co-Chair

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he focus of this 36th Bethesda Conference is the trainedthlete with an identified cardiovascular abnormality. Theoal is to formally develop prudent consensus recommen-ations regarding the eligibility of such individuals forompetition in organized sports, and to present theseonsiderations in a readily useble format for clinicians. Thisocument constitutes an update of the 16th (in 1985) (1)nd more recent 26th (in 1994) (2) Bethesda Conferences.nce again, we have attempted to ascertain by the consen-

us of an expert panel which cardiovascular abnormalitiesand with what degree of severity) place the competitivethlete at increased risk for sudden and unexpected death orisease progression so as to justify medical recommenda-ions against participation in all or certain competitiveports, for the purpose of reducing that risk.

MPETUS FOR THE REVISION

here are several reasons to offer this revision of the 26thethesda Conference (2). Over the past decade, substantialdvances have taken place in the diagnosis and management ofvariety of genetic and acquired cardiovascular diseases, in the

ecognition of the causes of athletic field deaths (3–5), and inthical and legal issues that impact medical decision making3–7). Also, sudden cardiac deaths in competitive athletesontinue to be highly visible, compelling, and emotional eventsith significant liability considerations (3,6,7). These catastro-hes are frequently subjected to intense public scrutiny largelyecause of their occurrence in young and otherwise healthyppearing individuals, including elite participants in collegiatend professional sports (3,6,7). Such sudden deaths are knowno occur in athletes of both genders (although much moreommonly in men by 9:1), minorities, at a wide range of ages,nd in a broad spectrum of sports (most commonly basketballnd football in the U.S. and soccer in Europe) (3–5). All ofhese considerations, as well as the legal decision in Knapp vs.orthwestern, which placed the Bethesda Conference recom-endations for the eligibility of athletes with cardiovascular

bnormalities as a precedent (6), have led to the necessity forhis revised document.

EFINITIONS

s before, we define the competitive athlete as one who
articipates in an organized team or individual sport that d
equires regular competition against others as a centralomponent, places a high premium on excellence andchievement, and requires some form of systematic (andsually intense) training. Therefore, organized competitiveports are regarded as a distinctive activity and lifestyle. Anmportant component of a competitive sports activity con-erns whether athletes are able to properly judge when it isrudent to terminate physical exertion. For example, thenique pressures of organized sports do not allow athletes toemonstrate strict control over their level of exertion oreliably discern when cardiac-related symptoms or warningigns arise. However, the panel recognizes that this defini-ion is most easily applied to high school, college, androfessional sports. Clinicians may want to use individual

udgment in defining competitive forms of physical activityor participants in many youth sports activities, particularlyhose for children less than age 12 years.

Athletes may be regarded as competitive in many sport-ng disciplines—at almost any age or level of participation,ncluding involvement in high school, college, professional,nd master’s sports (8). The recommendations in thisocument do not apply to (and are not specifically designedor) non-competitive recreational sports activities; suchppropriate guidelines appear elsewhere (9). Nevertheless,e also recognize that some practitioners will choose to

xtrapolate or translate the recommendations for competi-ive athletes selectively to some recreational sports, and toon-athletes with occupations that require vigorous physicalxertion (e.g., firefighters or emergency medical techni-ians), or to cardiac rehabilitation programs.

Furthermore, it is emphasized that these Bethesda Con-erence recommendations should not be regarded as annjunction against physical activity in general; indeed, theanel recognizes the well-documented health benefits ofxercise. In particular, regular recreational physical activitieshould be encouraged. Excessive and unnecessary restric-ions could potentially create physical and psychologicalurdens (particularly in young children).

REVALENCE

he prevalence of cardiovascular disease in the youngthletic population is low. Furthermore, although the pre-ise risk of sudden cardiac death in athletes with underlying
isease is unresolved, it is also undoubtedly low (3,10).

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1319JACC Vol. 45, No. 8, 2005 Maron and ZipesApril 19, 2005:1318–21 Introduction

ndeed, the number of athletes who die of cardiovascular orelated causes each year in the U.S. is probably less than 300,ompared with the large number of athletes participating in

broad spectrum of organized sports (about 10 to 15illion) of all ages in the U.S.If sudden death in athletes is a relatively uncommon

vent, why is it regarded as a substantive medical issue? Thiselates largely to the generally held perception that compet-tive athletes represent the healthiest and most dynamic

embers of society, in whom cardiovascular sudden deathsecome symbolic and riveting and strike to the core of ourensibilities. Indeed, these events are counterintuitive, andhe visibility of such catastrophes is often enhanced by theirortrayal in the news media as public events rather thanersonal and family tragedies (3,6,7). For elite athletes whoften achieve celebrity status, the economic stakes may beigh, making medical decision making even more difficult3,6,7). For all these reasons, the sudden deaths of athletesave had enormous impact on the public consciousness andttitudes of the medical profession.

ONFERENCE DESIGN AND FORMAT

primary foundation for this 36th Bethesda Conference ishe generally accepted tenet that young trained athletes withnderlying cardiovascular abnormalities are at increased riskor sudden cardiac death (usually on the athletic field) (3–5),n comparison to non-athletes with cardiovascular diseaserelative risk 2.5, in one study) (11), largely by virtue ofxposure to the unique physiologic and psychologic stressesf intense training and competition. Indeed, physical activ-ty during training or competition appears to trigger the vast

ajority of sudden deaths in athletes (3–5). That trainedthletes may occasionally die suddenly unrelated to exerciser significant physical activity does not negate this principle.Task Force reports 2 to 7 of this conference document offer

pecific recommendations for the eligibility and temporary orermanent disqualification of trained athletes with cardiovas-ular abnormalities and structural diseases previously impli-ated in sudden cardiac death (or disease progression). Theost common of these conditions in young athletes (less than

5 years of age) are hypertrophic cardiomyopathy (HCM),ongenital coronary artery anomalies of wrong sinus origin,yocarditis, Marfan syndrome (with aortic dissection), and

rrhythmogenic right ventricular cardiomyopathy (predomi-antly in Italy) (3–5,11) (Table 1).Although many of these diseases have been generally

egarded as rare, it now appears that HCM is relativelyommon (1:500 in the general population) (12). In contrast,therosclerotic coronary artery disease is by far the mostommon cause of sudden death in older athletes, andelatively uncommon in the young (3,13). We have arbitrarilyxcluded from consideration other particularly rare potentialauses of sudden death, such as heat stroke, bronchial asthma,erebral hemorrhage, and hematologic disorders.

Other sections of this document address related areas s

uch as preparticipation screening and diagnostic strategies,se of illicit drugs and dietary supplements, ethical andedical-legal considerations for sports disqualification, asell as sudden death due to blunt non-penetrating chestlows in the absence of heart disease (i.e., commotio cordis)14,15).

ECOMMENDATIONS FORISQUALIFICATION AND ELIGIBILITY

lthough recommendations of the 36th Bethesda Confer-nce do not reflect policy of the American College ofardiology Foundation, they are well considered views of a

roup of experts convened to address the medical risksmposed by competition on an athlete with a cardiovascularbnormality. The present consensus recommendations wereecessarily based largely on individual and collective judg-ents and the experience of the panel, as well as on the

vailable pertinent scientific data. Indeed, in the process oformulating the recommendations, the panel participants,ho are cardiovascular authorities most comfortable with aigh level of precision, were often required to confront areas

n which there was a paucity of hard evidence and utilize theart of medicine” in designing recommendations.

It is the premise of the expert panel that firm recommen-ations for temporary or permanent sports disqualificatione confined to individual athletes with probable or conclu-

able 1. Causes of Sudden Death in 387 Young Athletes*

CauseNo. of

Athletes Percent

ypertrophic cardiomyopathy 102 26.4ommotio cordis 77 19.9oronary artery anomalies 53 13.7eft ventricular hypertrophy ofindeterminate causation†

29 7.5

yocarditis 20 5.2uptured aortic aneurysm (Marfansyndrome)

12 3.1

rrhythmogenic right ventricularcardiomyopathy

11 2.8

unneled (bridged) coronary artery‡ 11 2.8ortic valve stenosis 10 2.6therosclerotic coronary artery disease 10 2.6ilated cardiomyopathy 9 2.3yxomatous mitral valve degeneration 9 2.3sthma (or other pulmonary condition) 8 2.1eat stroke 6 1.6rug abuse 4 1.0ther cardiovascular cause 4 1.0ong QT syndrome§ 3 0.8ardiac sarcoidosis 3 0.8rauma causing structural cardiac injury 3 0.8uptured cerebral artery 3 0.8

Data are from the registry of the Minneapolis Heart Institute Foundation (3).Findings at autopsy were suggestive of HCM but were insufficient to be diagnostic.Tunneled coronary artery was deemed the cause of death in the absence of any otherardiac abnormality. §The long QT syndrome was documented on clinical evaluation.ource: Reproduced from Maron B.J. (3) with permission of the Massachusettsedical Society.

ive evidence of disease rather than those with only border-

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1320 Maron and Zipes JACC Vol. 45, No. 8, 2005Introduction April 19, 2005:1318–21

ine findings or the presumption of a diagnosis. In this way,nnecessary restrictions from sports and the stigma of aardiac diagnosis in healthy individuals may be minimized.

e do recognize, however, that such an approach will inevi-ably permit an occasional athlete to participate who mighttherwise be at some risk. Nevertheless, the level of importancehe individual athlete personally attaches to continuing oresuming competitive sports is not regarded as a primaryeterminant in formulating eligibility recommendations.The recognition by panel members that all competitive

ports do not necessarily involve identical types or intensityf exercise is reflected in the Task Force 8: Classification ofports. Training demands vary considerably even within theame sport and the intensity of conditioning regimens oftenxceed that of competition itself. However, it is oftenifficult to accurately grade such differences in exercise

ntensity owing to a variety of factors, including differingotivational attitudes and training demands. The demands

f competitive sports may place athletes with certain car-iovascular abnormalities in extreme, unusual, and unpre-ictable environmental conditions (associated with alter-tions in blood volume, hydration, and electrolytes), overhich they have limited control. These circumstances could

nhance the risk for potentially lethal arrhythmias andudden death and unavoidably distort the reliability ofndividualized and prospective risk stratification. Con-ersely, it is suspected that for many athletes the removalrom their lifestyle of athletic training and competition willeduce risk for sudden death or disease progression.

The recommendations in this report should also beiewed in perspective. Appropriate sports disqualification isnly one component for potentially reducing risk, and eachelevant cardiovascular disease has its own treatment algo-ithms, which can include selective implantation of aardioverter-defibrillator in high-risk patients (16).

The present recommendations formulated with respect tollowable levels of sports activity can be regarded as gener-lly conservative. Certainly, this is a prudent posture tossume when the amount of available hard data and evi-ence is limited in many decision-making areas, as may behe case in portions of the 36th Bethesda Conferenceocument. The panel acknowledges that while availableata support the principle that competition in sports isssociated with an increased relative risk for sudden death inhe setting of known cardiovascular disease (11), the abso-ute risk cannot be determined with certainty in an individ-al patient/athlete, and in fact may be low in certainndividuals. However, at present, additional risk-stratifyingools are not available to independently (and more precisely)uide many of these difficult medical decisions. Thus, it isossible that the recommendations of this consensus panelill occasionally cause some athletes to be withdrawn from

ompetition unnecessarily. This is, of course, unfortunateecause athletes derive considerable self-assurance, confi-ence, physical well-being, and, even on occasion, financial
ecurity from these activities. Nonetheless, the increased risk i
f sudden death associated with intense athletic participa-ion is a controllable risk factor, and the devastating impactf even infrequent sudden deaths in this young populationnderscores the wisdom of the conservative nature of theseecommendations. Indeed, various cultures harbor differingocietal views on the individual rights and prerogatives ofthletes to persist in their chosen lifestyle, independent ofhe potential risks involved. In practice, consideration maye given in individual athletes to changing their competitiveport from a prohibited high-intensity activity to a permis-ible low-intensity one (i.e., usually to class IA). However,hanging the position in which an athlete competes (e.g.,rom running-back to place-kicker in football) to accom-lish the same end within high-intensity team sports mayrove difficult in practical terms.Consequently, the 36th Bethesda Conference report is

resented here in the context of measured and prudentecommendations—intended neither to be overly permissiveor restrictive—and which should not be regarded as anbsolutely rigid dictum. Indeed, the managing physicianith particular knowledge regarding a given athlete’s car-iovascular abnormality, psychological response to compe-ition, and other medically relevant factors may choose todopt somewhat different recommendations in selectedndividuals.

PECIAL CONSIDERATIONS

hree considerations relevant to much of the documenteserve special consideration. First, medications, such aseta-blockers commonly used to treat a variety of cardiaciseases including systemic hypertension, HCM, long QTyndrome, and Marfan syndrome, are likely to inhibiterformance in trained competitive athletes (7). The use ofuch drugs in athletes cannot be regarded as either a meansf affording safety and specific protection against arrhyth-ias, nor as a primary means for retaining eligibility in

igorous competitive sports. Furthermore, use of beta-lockers is specifically contraindicated in some sports.Second, the availability of a free-standing automatic

xternal defibrillator at a sporting event should not beonsidered either as absolute protection against a suddeneath event, a prospectively designed treatment strategy forthletes with known cardiovascular disease, or justificationor participation in competitive sports that otherwise woulde restricted owing to underlying cardiac abnormalities andhe risk of life-threatening ventricular tachyarrhythmias.

Third, with the increased employment of the implantableardioverter-defibrillator (ICD) it is inevitable that increas-ng numbers of high-risk athletes with defibrillators willome to recognition. Although differences of opinion existnd little direct evidence is available, the panel asserts thathe presence of an ICD (whether for primary or secondaryrevention of sudden death) should disqualify athletes fromost competitive sports (with the exception of low-

ntensity, class IA), including those that potentially involve

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1321JACC Vol. 45, No. 8, 2005 Maron and ZipesApril 19, 2005:1318–21 Introduction

odily trauma. The presence of an implantable device inigh-risk patients with cardiovascular disease should not beegarded as protective therapy and therefore a justificationor permitting participation in competitive sports thatould otherwise be restricted. This conservative but prudentosture is justified on the basis of the uncertainties associ-ted with ICDs during intense competitive sports, includinghe possibility that the device will not perform effectively ateak exercise, the likelihood of a sinus tachycardia-triggerednappropriate shock or an appropriate discharge, and theisk for physical injury to the athlete or other competitors ashe result of an ICD shock. Also, pacemaker-dependentthletes should not participate in most competitive sportshat potentially involve bodily trauma.

NTRODUCTION REFERENCES

1. Mitchell JH, Maron BJ, Epstein SE. 16th Bethesda Conference:cardiovascular abnormalities in the athlete: recommendations regard-ing eligibility for competition. J Am Coll Cardiol 1985;6:1186–232.

2. Maron BJ, Mitchell JH. 26th Bethesda Conference: recommendationsfor determining eligibility for competition in athletes with cardiovas-cular abnormalities. J Am Coll Cardiol 1994;24:845–99.

3. Maron BJ. Sudden death in young athletes. N Engl J Med 2003;349:1064–75.

4. Maron BJ, Shirani J, Poliac LC, Mathenge R, Roberts WC, MuellerFO. Sudden death in young competitive athletes: clinical, demo-graphic, and pathological profiles. JAMA 1996;276:199–204.

5. Maron BJ, Carney KP, Lever HM, et al. Relationship of race to

6. Maron BJ, Mitten MJ, Quandt EF, Zipes DP. Competitive athleteswith cardiovascular disease—the case of Nicholas Knapp. N EnglJ Med 1998;339:1632–5.

7. Maron BJ. Sudden death in young athletes: lessons from the HankGathers affair. N Engl J Med 1993;329:55–7.

8. Maron BJ, Araújo CG, Thompson PD, et al. Recommendations forpreparticipation screening and the assessment of cardiovascular diseasein Masters athletes: an advisory for healthcare professionals from theworking groups of the World Heart Federation, the InternationalFederation of Sports Medicine, and the American Heart AssociationCommittee on Exercise, Cardiac Rehabilitation, and Prevention.Circulation 2001;103:327–34.

9. Maron BJ, Chaitman BR, Ackerman MJ, et al. AHA scientificstatement: recommendations for physical activity and recreationalsports participation for young patients with genetic cardiovasculardiseases. Circulation 2004;109:2807–16.

0. Maron BJ, Gohman TE, Aeppli D. Prevalence of sudden cardiacdeath during competitive sports activities in Minnesota high schoolathletes. J Am Coll Cardiol 1998;32:1881–4.

1. Corrado D, Basso C, Rizzoli G, Schiavon M, Thiene G. Does sportsactivity enhance the risk of sudden death in adolescents and youngadults? J Am Coll Cardiol 2003;42:1959–63.

2. Maron BJ. Hypertrophic cardiomyopathy: an important global disease.Am J Med 2004;116:63–5.

3. Burke AP, Farb A, Virmani R, Goodin J, Smialek JE. Sports-relatedand non-sports-related sudden cardiac death in young adults. AmHeart J 1991;121:568–75.

4. Maron BJ, Gohman TE, Kyle SB, Estes NA III, Link MS. Clinicalprofile and spectrum of commotio cordis. JAMA 2002;287:1142–6.

5. Maron BJ, Poliac L, Kaplan JA, Mueller FO. Blunt impact to thechest leading to sudden death from cardiac arrest during sportsactivities. N Engl J Med 1995;333:337–42.

6. Maron BJ, Shen W-K, Link MS, et al. Efficacy of implantablecardioverter-defibrillators for the prevention of sudden death in pa-tients with hypertrophic cardiomyopathy. N Engl J Med 2000;342:
sudden cardiac death in competitive athletes with hypertrophic car-
diomyopathy. J Am Coll Cardiol 2003;41:974–80. 365–73.

ppendix 1. Author Relationships With Industry and Others

Name Consultant Research Grant Scientific Advisory Board Stock Holder Expert Witness Testimony

r. Barry J. Maron None ● Medtronic None None ● 1996, Defense, Knappvs. Northwestern

r. Douglas P. Zipes ● Cardiofocus● Janssen

● Medtronic ● Medtronic ● MVMD ● 1996, Defense, Knappvs. Northwestern

● Medtronic

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ASK FORCES

ask Force 1: Preparticipation Screening andiagnosis of Cardiovascular Disease in Athletes

arry J. Maron, MD, FACC, Chairamela S. Douglas, MD, FACC, Thomas P. Graham, MD, FACC, Rick A. Nishimura, MD, FACC,

ublished by Elsevier Inc.

aul D. Thompson, MD, FACC

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he present consensus panel recommendations of the 36thethesda Conference for eligibility and disqualification ofompetitive athletes are predicated on the prior diagnosis ofardiovascular abnormalities. However, the methodology byhich these diseases are identified (including preparticipa-

ion screening) and how athletes come to evaluation forompetitive eligibility, may involve several scenarios. First,thletes may be referred for assessment of clinical symptomsr signs. Second, fortuitous recognition may occur inoutine clinical practice, triggered by findings on history andhysical examination, such as a heart murmur. Third, youngthletes may be suspected of having cardiovascular diseasey virtue of formalized large population screening examina-ions that are customary before participation in competitivethletics (1).

REPARTICIPATION SCREENING

ndeed, the ultimate objective of preparticipation screeningarried out in general populations of trained athletes is theecognition of “silent” cardiovascular abnormalities that canrogress or cause sudden cardiac death. Such screeningfforts have the capability of raising the clinical suspicion ofeveral cardiovascular diseases—usually by virtue of a hearturmur, regarded to be of potential clinical significance,

ardiac symptoms (e.g., exertional chest pain, dispropor-ionate dyspnea, or impairment in consciousness), or aamily history of heart disease or sudden unexpected death.

owever, a major obstacle to implementation of large-scalecreening in the U.S. is the substantial number of youngthletes eligible for evaluation (about 10 to 12 million) andhe rarity of the cardiac diseases capable of causing suddeneath in this population (estimated prevalence, less than orqual to 0.3%) (2).

Customary screening strategies for U.S. high school andollege athletes is confined to history-taking and physicalxamination, generally acknowledged to be limited in itsower to consistently identify important cardiovascularbnormalities. In one retrospective study, only 3% of trainedthletes who died suddenly of heart disease (and had beenxposed to preparticipation screening) were suspected ofarboring cardiovascular disease on the basis of history andhysical examination—and none had been disqualified fromompetition (3). Although most trained athletes with occult
ardiovascular disease are asymptomatic, the prior history of r
xertional-related syncope in a young athlete unavoidablyaises the consideration of a number of diseases known toause sudden cardiac death, including hypertrophic cardio-yopathy (HCM) and ion-channel disorders, but in par-

icular should also heighten the level of clinical suspicion forongenital coronary anomalies of wrong sinus origin (4).

Furthermore, the quality of cardiovascular screening for.S. high school and college athletes, particularly the designf approved questionnaires, has come under scrutiny regard-ng inadequacies (5,6) when measured against American

eart Association (AHA) recommendations (1) (Table 1).egislation in several states allows health care workers withastly different levels of training and expertise (includinghiropractors and naturopathic clinicians) to conductreparticipation sports examinations, often under subopti-al conditions. Improvement in this screening process,

ncluding the training level of examiners, would undoubt-dly result in a greater number of athletes identified withreviously unsuspected but clinically relevant cardiovascularbnormalities. Indeed, development and dissemination of atandardized and uniform national preparticipation historynd physical examination form for medical screening in alligh schools and colleges (which incorporates the AHAecommendations) would be the most practical approach forchieving this goal.

Certainly, the diagnosis of genetic diseases, such asCM, arrhythmogenic right ventricular cardiomyopathy

ARVC), long QT and Brugada syndromes, and othernherited arrhythmia syndromes in asymptomatic patientsas now taken on even greater relevance. This is because

ndividuals judged to be at sufficiently high-risk may beligible for primary prevention of sudden cardiac death withn implantable cardioverter-defibrillator (7,8).

Conversely, in Italy, for the past 25 years, a formalational preparticipation screening and medical clearancerogram has been mandated for young competitive athletesn organized sports programs (9,10). The Italian system isnique by virtue of requiring annual evaluations that rou-inely include a 12-lead electrocardiogram (ECG) as well as

history and physical examination; the ECG itself hasroven most useful in the identification of many previouslyndiagnosed athletes with HCM (10). However, suchcreening efforts may be complicated by the substantialroportion of false-positive test results that potentially
epresent a major burden to athletes, their families, and the

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1323JACC Vol. 45, No. 8, 2005 Maron et al.April 19, 2005:1322–6 Task Force 1: Preparticipation Screening and Diagnosis

esting facilities. Obstacles in the U.S. to implementingbligatory government-sponsored national screening in-luding ECGs or echocardiograms are the particularly largeopulation of athletes to screen, major cost-benefit consid-rations, and the recognition that it is impossible to abso-utely eliminate the risks associated with competitive sports1,2). Nevertheless, some volunteer-based small-scalecreening programs using portable echocardiograms to ex-mine high-school athletes on the field for HCM havemerged.

Systematic preparticipation cardiovascular screening, pri-arily to exclude atherosclerotic coronary artery disease in

lder athletes, is not customary practice. Such persons areargely participants in individual athletic activities such asoad and marathon racing, or in a variety of other organizedaster’s sports (11).

IAGNOSTIC TESTING STRATEGIES

hen a cardiovascular abnormality is initially suspected (byormal screening or otherwise), the diagnostic strategyhould focus on the systematic exclusion of those conditionsnown to cause sudden death in young athletes; thesepproaches include echocardiography, ECG, history, andhysical examination. Additional noninvasive (and invasive)esting with cardiac magnetic resonance imaging (CMR),xercise testing, ambulatory Holter ECG recording, im-lanted loop recorder, tilt table examination, or electro-hysiologic testing with programmed stimulation can beonsidered in selected patients. Diagnostic myocardial bi-psies are used only selectively in athletes suspected clini-ally of myocarditis.

Despite considerable assembled data regarding DNA-ased diagnosis over the past decade, identification ofenetic cardiovascular diseases such as HCM, long QTyndrome, and other ion-channel disorders, ARVC, and

arfan syndrome continues to be made through clinical

able 1. AHA Consensus Panel Recommendations forreparticipation Athletic Screening (1)

Family History1. Premature sudden cardiac death2. Heart disease in surviving relatives less than 50 years old

Personal History3. Heart murmur4. Systemic hypertension5. Fatigue6. Syncope/near-syncope7. Excessive/unexplained exertional dyspnea8. Exertional chest pain

Physical Examination9. Heart murmur (supine/standing*)

10. Femoral arterial pulses (to exclude coarctation of aorta)11. Stigmata of Marfan syndrome12. Brachial blood pressure measurement (sitting)

In particular, to identify heart murmur consistent with dynamic obstruction to leftentricular outflow. From Maron BJ, et al. Circulation 1996;94:850–6, reprinted withermission of the American Heart Association.

esting in the vast majority of cases, and this will remain so n

n the foreseeable future. At present, genetic testing is notasily available on a routine clinical basis for most geneticeart diseases, or for application to large athletic popula-ions given the expensive and complex methodologies in-olved and the genetic heterogeneity characteristic of theseiseases (12).chocardiography. Two-dimensional echocardiography is

he principal diagnostic imaging modality for clinical iden-ification of HCM by demonstrating otherwise unexplainednd usually asymmetric left ventricular (LV) wall thickening12,13). In this regard, a maximal LV end-diastolic wallhickness of 15 mm or more (or on occasion, 13 or 14 mm)s the absolute dimension generally accepted for the clinicaliagnosis of HCM in an adult athlete (two or more standardeviations from the mean relative to body surface area;-score of two or more in children) (12,13); however, anypecific LV wall thickness (including normal) is theoreticallyompatible with the presence of a mutant HCM gene12,14). Echocardiography would also be expected to detectnd define other specific and relevant congenital structuralbnormalities associated with sudden death or disease pro-ression in young athletes such as valvular heart diseasee.g., mitral valve prolapse and aortic valve stenosis), aorticoot dilatation and mitral valve prolapse in Marfan orelated syndromes, and LV dysfunction and/or enlargementevident in myocarditis and dilated cardiomyopathy). Suchiagnostic testing requires interpretation by physiciansrained in echocardiography, but cannot guarantee fullecognition of all relevant lesions, and some importantiseases may escape detection despite expert screeningethodology. For example, the HCM phenotype may not

e evident when echocardiography is performed in there-hypertrophic phase (i.e., a patient less than 14 yearsf age) (12). Annual serial echocardiography is recom-ended in HCM family members throughout adoles-

ence (12,14).lectrocardiography. The 12-lead ECG may be of use in

he diagnosis of cardiovascular disease in young athletes,nd has been promoted as a practical and cost-effectivetrategic alternative to routine echocardiography foropulation-based preparticipation screening. For example,he ECG is abnormal in up to 75% to 95% of patients with

CM, and often before the appearance of hypertrophy12). The ECG will also identify many individuals with theong QT, Brugada, and other inherited syndromes associ-ted with ventricular arrhythmias. It raises the suspicion ofyocarditis by premature ventricular complexes and ST-T

bnormalities, or ARVC by T-wave inversion in leads V1hrough V3 and low amplitude potentials (epsilon waves)1,2). Of note, however, a not inconsequential proportion ofenetically affected family members with long QT syn-rome may not express QT interval prolongation, and ECGbnormalities are usually absent in random recordings fromatients with congenital coronary artery anomalies (4).ther tests. In those cases in which the echocardiogram is

ormal or borderline for LV hypertrophy, but a suspicion

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1324 Maron et al. JACC Vol. 45, No. 8, 2005Task Force 1: Preparticipation Screening and Diagnosis April 19, 2005:1322–6

or HCM persists (often due to an abnormal 12-lead ECG),MR may be useful in clarifying wall thickness or detecting

egmental areas of hypertrophy in selected regions of the LVhamber which may be more difficult to image reliably withonventional echocardiography, such as anterolateral freeall or apex (15,16).Definitive identification of congenital coronary artery

nomalies of wrong sinus origin usually requires sophisti-ated laboratory imaging, including multi-slice computedomography or coronary arteriography. However, in youngthletes it is possible to raise the suspicion of these malfor-ations with transthoracic or transesophageal echocardiog-

aphy or CMR imaging. Often, ARVC cannot be diag-osed reliably with echocardiography, and CMR is probablyhe most useful noninvasive test for identifying the struc-ural abnormalities in this condition (i.e., right ventricularnlargement, wall motion abnormalities, adipose tissue re-lacement within the wall, and aneurysm formation); how-ver, CMR is not an entirely sensitive or specific diagnosticodality in ARVC (17).

THLETE’S HEART AND CARDIOVASCULAR DISEASE

ystematic training in endurance or isometric sports mayrigger physiologic adaptations and structural cardiac re-odeling, including increased LV wall thickness, enlarged

entricular and atrial cavity dimensions, and calculatedardiac mass, in the presence of normal systolic and diastolicunction (i.e., athlete’s heart) (18). The magnitude ofhysiologic hypertrophy may also vary according to thearticular type of sports training. Other adaptations toraining include a variety of abnormal 12-lead ECG pat-

igure 1. Gray area of overlap between athlete’s heart and cardiomyorrhythmogenic right ventricular cardiomyopathy (ARVC). ECG � e003;349:1064–75, with permission of Massachusetts Medical Society (2)

erns in about 40% of elite athletes, which not infrequently m

imic those of cardiac disease (i.e., increased R- or S-waveoltages, Q waves, and repolarization abnormalities) (19).requent and/or complex ventricular tachyarrhythmias onmbulatory (Holter) ECG are not uncommonly found inthletes and can also mimic certain cardiac diseases, includ-ng myocarditis (20).

Clinical distinctions between physiologic athlete’s heartnd pathologic conditions (18–23) have critical implicationsor trained athletes, because cardiovascular abnormalitiesay trigger disqualification from competitive sports to

educe the risk of sudden death or disease progression. Anver-diagnosis may lead to unnecessary restrictions, depriv-ng athletes of the psychological, social, or possibly (in somelite athletes) economic benefits of sports (2).

Morphologic adaptations of athlete’s heart can closelyesemble certain cardiovascular diseases and lead to a dif-erential diagnosis with HCM, dilated cardiomyopathy, andRVC (2) (Fig. 1). Such clinical dilemmas not infrequently

rise when cardiac dimensions fall outside clinically ac-epted partition values. For example, 2% of highly traineddult male athletes show relatively mild increases in LV wallhickness (13 to 15 mm) and 15% have LV cavity enlarge-ent greater than or equal to 60 mm (2,21,22); both fall

nto a borderline and inconclusive “gray zone” for whichxtreme expressions of benign athlete’s heart and mildorphologic forms of cardiomyopathy overlap (2,22,23).

ndeed, the two most common clinical scenarios encoun-ered that unavoidably generate ambiguous diagnoses inrained athletes are: 1) differentiating HCM from athlete’seart in athletes with an LV wall thickness of 13 to 15 mm,on-dilated and normally contractile LV, and absence of

ies, including myocarditis, hypertrophic cardiomyopathy (HCM), andcardiogram; LV � left ventricular. From Maron BJ, N Engl J Med

pathlectro.

itral valve systolic anterior motion; and 2) differentiating

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1325JACC Vol. 45, No. 8, 2005 Maron et al.April 19, 2005:1322–6 Task Force 1: Preparticipation Screening and Diagnosis

arly presentation of dilated cardiomyopathy from athlete’seart with LV end-diastolic cavity dimension 60 mm orore with low-normal LV function (i.e., ejection fraction of

0% to 55%).Such cases with diagnostic uncertainty are not uncom-on and may be resolved in many athletes by a number of

ndependent noninvasive clinical parameters, including theesponse of cardiac mass to short periods of deconditioning,r assessment of diastolic filling (22) (Fig. 2). Clarification

igure 2. Chart showing criteria used to favor or distinguish hypertrophicardiomyopathy (HCM) from athlete’s heart when maximal left ventricularLV) wall thickness is within shaded gray zone of overlap (i.e., 13 to5 mm), consistent with both diagnoses. *Assumed to be the non-bstructive form of HCM (under resting conditions) in this discussionecause the presence of substantial mitral valve systolic anterior motionould confirm the diagnosis of HCM in the athlete. †May involve a varietyf abnormalities, including heterogeneous distribution of left ventricularypertrophy (LVH) in which asymmetry is prominent and adjacent regionsay show greatly different thicknesses with sharp transitions evident

etween segments, as well as unusual patterns in which the anteriorentricular septum is spared from the hypertrophic process and LVhickening may be in posterior portion of septum or anterolateral orosterior free wall or apex. ‡Assessed with cardiopulmonary (metabolic)xercise testing. 2 � decreased; ECG � electrocardiogram; LA � lefttrial. From Maron BJ, et al. Circulation 1995;91:1596–601, reprintedith permission of the American Heart Association (22).

f such diagnostic ambiguities may also be achieved with

MR imaging, genotyping, and serial acquisition of clinicalnd morphologic evidence over time.

doi:10.1016/j.jacc.2005.02.007

ASK FORCE 1 REFERENCES

1. Maron BJ, Thompson PD, Puffer JC, et al. Cardiovascular prepartici-pation screening of competitive athletes: a statement for healthprofessionals from the Sudden Death Committee (clinical cardiology)and Congenital Cardiac Defects Committee (cardiovascular disease inthe young), American Heart Association. Circulation 1996;94:850–6.



4. Basso C, Maron BJ, Corrado D, Thiene G. Clinical profile ofcongenital coronary artery anomalies with origin from the wrong aorticsinus leading to sudden death in young competitive athletes. J Am CollCardiol 2000;35:1493–501.

5. Glover DW, Maron BJ. Profile of preparticipation cardiovascularscreening for high school athletes. JAMA 1998;279:1817–9.

6. Pfister GC, Puffer JC, Maron BJ. Preparticipation cardiovascularscreening for U.S. collegiate student-athletes. JAMA 2000;283:1597–9.

7. Maron BJ, Shen WK, Link MS, et al. Efficacy of implantablecardioverter-defibrillators for the prevention of sudden death in pa-tients with hypertrophic cardiomyopathy. N Engl J Med 2000;342:365–73.

8. Corrado D, Leoni L, Link MS, et al. Implantable cardioverter-defibrillator therapy for prevention of sudden death in patients witharrhythmogenic right ventricular cardiomyopathy/dysplasia. Circula-tion 2003;108:3084–91.

9. Pelliccia A, Maron BJ. Preparticipation cardiovascular evaluation ofthe competitive athlete: perspectives from the 30-year Italian experi-ence. Am J Cardiol 1995;75:827–9.

0. Corrado D, Basso C, Schiavon M, Thiene G. Screening for hyper-trophic cardiomyopathy in young athletes. N Engl J Med 1998;339:364–9.

1. Maron BJ, Araújo CG, Thompson PD, et al. Recommendations forpreparticipation screening and the assessment of cardiovascular diseasein Master’s athletes: an advisory for healthcare professionals from theworking groups of the World Heart Federation, the InternationalFederation of Sports Medicine, and the American Heart AssociationCommittee on Exercise, Cardiac Rehabilitation, and Prevention.Circulation 2001;103:327–34.

2. Maron BJ, McKenna WJ, Danielson GK, et al. American College ofCardiology/European Society of Cardiology clinical expert consensusdocument on hypertrophic cardiomyopathy: a report of the AmericanCollege of Cardiology Foundation Task Force on Clinical ExpertConsensus Documents and the European Society of CardiologyCommittee for Practice Guidelines. J Am Coll Cardiol 2003;42:1687–713.

3. Klues HG, Schiffers A, Maron BJ. Phenotypic spectrum and patternsof left ventricular hypertrophy in hypertrophic cardiomyopathy: mor-phologic observations and significance as assessed by two-dimensionalechocardiography in 600 patients. J Am Coll Cardiol 1995;26:1699–708.

4. Maron BJ, Seidman JG, Seidman CE. Proposal for contemporaryscreening strategies in families with hypertrophic cardiomyopathy.J Am Coll Cardiol 2004;44:2125–32.

5. Rickers C, Wilke NM, Jerosch-Herold M, et al. Utility of cardiacmagnetic resonance imaging in the diagnosis of hypertrophic cardio-myopathy. Circulation 2005. In press.

6. Moon JCC, Fisher NG, McKenna WJ, Pennell DJ. Detection ofapical hypertrophic cardiomyopathy by cardiovascular magnetic reso-nance in patients with non-diagnostic echocardiography. Heart 2004;90:645–49.

7. Tandri H, Calkins H, Nasir K, et al. Magnetic resonance imagingfindings in patients meeting task force criteria for arrhythmogenic
right ventricular dysplasia. J Cardiovasc Electrophysiol 2003; 14:476–82.

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1326 Graham, Jr. et al. JACC Vol. 45, No. 8, 2005Task Force 2: Congenital Heart Disease April 19, 2005:1326–33

8. Pluim BM, Zwinderman AH, van der Laarse A, van der Wall EE.The athlete’s heart: a meta-analysis of cardiac structure and function.Circulation 2000;101:336–44.

9. Pelliccia A, Maron BJ, Culasso F, et al. Clinical significance ofabnormal electrocardiographic patterns in trained athletes. Circulation2000;102:278–84.

0. Biffi A, Pelliccia A, Verdile L, et al. Long-term clinical significance offrequent and complex ventricular tachyarrhythmias in trained athletes.

ane W. Newburger, MD, MPH, FACC, Albert Rocchin

emodynamic status with time.

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1. Pelliccia A, Maron BJ, Spataro A, Proschan MA, Spirito P. The upperlimit of physiologic cardiac hypertrophy in highly trained elite athletes.N Engl J Med 1991;324:295–301.

2. Maron BJ, Pelliccia A, Spirito P. Cardiac disease in young trainedathletes: insights into methods for distinguishing athlete’s heart fromstructural heart disease, with particular emphasis on hypertrophiccardiomyopathy. Circulation 1995;91:1596–601.

3. Pelliccia A, Culasso F, Di Paolo FM, Maron BJ. Physiologic left ventricular
J Am Coll Cardiol 2002;40:446–52. cavity dilatation in elite athletes. Ann Intern Med 1999;130:23–31.

Name Consultant Research Grant Speaker’s Bureau Stock Holder Expert Witness Testimony

r. Barry J. Maron None ● Medtronic None None ● 1996, Defense, Northwestern vs. Knapp

r. Pamela S. Douglas None None None None None

r. Rick A. Nishimura None None None None None

r. Thomas P. Graham None None None None None

r. Paul D. Thompson ● Astra Zeneca● Bristol Myers

● Astra Zeneca● Merck

● Astra Zeneca● Merck

● Pfizer● Schering

● 2005, Plaintiff, Sudden death in collegeathlete

Squibb ● Pfizer● Schering


● 2004, Defense, Stress test/recreationalsports

● 2002, Sudden death, World Gym● 2002, Plaintiff, Sudden death, truck driver● 1998, Plaintiff, Sudden death, recreational

sports

ask Force 2: Congenital Heart Diseasehomas P. Graham, JR, MD, FACC, Chairavid J. Driscoll, MD, FACC, Welton M. Gersony, MD, FACC,

i, MD, Jeffrey A. Towbin, MD, FACC

ENERAL CONSIDERATIONS

he most common congenital heart lesions that have beenssociated with sudden death during sports participation areypertrophic cardiomyopathy, coronary artery anomalies,arfan syndrome, and aortic valve disease (1–3). Less

ommon lesions include complex defects, such as transpo-ition and single ventricle, and those with associated pul-onary vascular disease.The recommendations presented are intended to provide

road guidelines for patients with congenital heart defects4). When questions about the safety of sports participationrise, there is no substitute for a comprehensive evaluationy a knowledgeable and experienced physician. Exerciseesting can be useful, particularly if symptoms, the electro-ardiogram (ECG), and blood pressure are monitoreduring conditions that simulate the sport in question.rrhythmias discussed in this Task Force are usually iden-

ified by exercise testing or some form of long-term moni-oring (including ambulatory Holter and event recording).erial evaluations may be required because of changing

YPES OF CONGENITAL DEFECTS

trial septal defect (ASD)— untreated. Most childrenith ASD are asymptomatic, and closure is usually carriedut before they are active in competitive sports. An ECGnd echocardiogram are required for evaluation. Small atrialefects are characterized by minimal or no right ventricularolume overload, moderate or large defects have significantolume overload but pulmonary hypertension is unusual.

ecommendations:

. Athletes with small defects, normal right heart vol-ume, and no pulmonary hypertension can participatein all sports.

. Athletes with a large ASD and normal pulmonaryartery pressure can participate in all competitive sports.

. Athletes with an ASD and mild pulmonary hyperten-sion can participate in low-intensity competitivesports (class IA). Patients with associated pulmonaryvascular obstructive disease who have cyanosis and alarge right-to-left shunt cannot participate in com-
petitive sports.

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1327JACC Vol. 45, No. 8, 2005 Graham, Jr. et al.April 19, 2005:1326–33 Task Force 2: Congenital Heart Disease

. Athletes with ASD and symptomatic atrial or ven-tricular tachyarrhythmia or moderate-to-severe mi-tral regurgitation should follow the recommenda-tions in Task Force 1: Preparticipation Screeningand Diagnosis of Cardiovascular Disease in Athletes,and Task Force 6: Coronary Artery Disease,respectively.

SD— closed at operation or by interventional catheter-zation. The ASDs usually are completely closed by oper-tion or device insertion. When closure is performed inhildhood there is little or no residual right ventricularnlargement. Supraventricular arrhythmias can occur afterlosure and are more common when the defect is repairedater in life (5–7). Evaluation should include an estimate ofardiac performance, pulmonary vascular resistance andight ventricular size, and a search for conduction or rhythmisturbances. A chest radiograph, ECG, and echocardio-ram usually are needed. Patients with preoperative pulmo-ary hypertension, a right-to-left shunt, or both, beforeperation require postoperative assessment of pulmonaryrtery pressure with Doppler echocardiography or cardiacatheterization.

ecommendations:

. Three to six months after the operation or interven-tion, patients can participate in all sports unless thefollowing are present: 1) evidence of pulmonaryhypertension; 2) symptomatic atrial or ventriculartachyarrhythmias or second- or third-degree heartblock; and 3) evidence of myocardial dysfunction.

. Patients with any of these abnormalities should havean exercise evaluation and an individualized exerciseprescription with respect to competitive sports. Forrelated recommendations on competitive sports par-ticipation, see the section entitled Elevated Pulmo-nary Resistance and Ventricular Dysfunction AfterCardiac Surgery in this Task Force report, as well asTask Force 7: Arrhythmias.

entricular septal defect (VSD)— untreated. The VSDsre categorized as small, moderate, or large. If physicalxamination and echocardiography indicate normal heartize and normal pulmonary artery pressure in patients withsuspected small VSD, further evaluation is not required.A patient with a VSD that does not fit into the small

efect category may require further investigation. Cardiacatheterization might be required.

A moderate-sized defect with low pulmonary vascular resis-ance will have a pulmonary/systemic flow ratio of about 1.5 to.9. A large defect with low or mildly increased pulmonaryascular resistance is defined as a pulmonary/systemic flowatio greater than or equal to 2 and pulmonary resistance lesshan 3 U/m2. Large right-to-left shunts are discussed in the
ection entitled Elevated Pulmonary Resistance.
ecommendations:

. Athletes with a VSD and normal pulmonary arterypressure can participate in all sports.

. Athletes with a large VSD who do not have markedelevation of pulmonary resistance are candidates forrepair, and full participation in all sports wouldnormally occur after a successful VSD closure.

SD— closed at operation or by interventional catheter-zation. Successful repair is characterized by the absence ofymptoms, absence of significant shunt, cardiomegaly, orrrhythmias, and the presence of normal pulmonary arteryressure. Minimal diagnostic evaluation after surgery beforearticipation in sports includes a chest radiograph, ECG,nd echocardiogram. Patients with residual left or rightentricular enlargement, myocardial dysfunction, or pulmo-ary hypertension may require an exercise test or cardiacatheterization before a decision for participation in sportsan be made.

ecommendations:

. At three to six months after repair, asymptomaticathletes with no defect or only a small residual defectcan participate in all competitive sports if they haveno evidence of pulmonary artery hypertension, ven-tricular or atrial tachyarrhythmia, or myocardial dys-function.

. Athletes with symptomatic atrial or ventriculartachyarrhythmias or second- or third-degree atrio-ventricular (AV) block should follow the recommen-dations in Task Force 7: Arrhythmias. Athletes withmild-to-moderate pulmonary hypertension or ven-tricular dysfunction should follow the recommenda-tions in the section entitled Elevated PulmonaryResistance or Ventricular Dysfunction After CardiacSurgery.

. Athletes with persistent, severe pulmonary hyperten-sion cannot participate in competitive sports (seesection entitled Elevated Pulmonary Resistance).

atent ductus arteriosus (PDA)— untreated. The patientith a small PDA has a characteristic murmur, absence of

ymptoms, and normal left heart chamber dimensions.hose with a larger patent ductus have cardiomegaly andidened pulse pressure. There may be evidence of pulmo-ary hypertension. Minimal diagnostic studies generally

nclude echocardiography.

ecommendations:

. Athletes with a small PDA and normal left heartchamber dimension can participate in all competitivesports.

. Athletes with a moderate or large PDA, causing leftventricular (LV) enlargement, should undergo surgi-cal or interventional catheterization closure before
unrestricted competition.

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. For athletes with a moderate or large PDA, severepulmonary hypertension, and cyanosis, see the sec-tion entitled Elevated Pulmonary Resistance.

DA, closed at operation or by interventional catheter-zation. A successful result is characterized by the absencef symptoms, a normal cardiac examination, and normalcho/Doppler study.

ecommendations:

. Three months after PDA closure, patients with nosymptoms, with normal cardiac examination, andwith no evidence of pulmonary hypertension or LVenlargement can participate in all competitive sports.

. For athletes with residual pulmonary artery hyperten-sion see the section entitled Elevated PulmonaryResistance.

ulmonary valve stenosis (PS)— untreated. Mild stenosiss characterized by a systolic ejection murmur, variablejection click, and a normal ECG. A Doppler peak in-tantaneous gradient less than 40 mm Hg usually indicatesild stenosis, 40 to 60 mm Hg moderate stenosis, and

reater than 60 mm Hg severe stenosis. Most patients withgradient 50 mm Hg or greater undergo balloon

alvuloplasty.

ecommendations:

. Athletes with a peak systolic gradient less than 40mm Hg and normal right ventricular function canparticipate in all competitive sports if no symptomsare present. Annual re-evaluation is recommended.

. Athletes with a peak systolic gradient greater than 40mm Hg can participate in low-intensity competitivesports (classes IA and IB). Patients in this categoryusually are referred for balloon valvuloplasty or op-erative valvotomy before sports participation.

S—treated by operation or balloon valvuloplasty. Ad-quate relief of PS is characterized by the absence ormprovement in symptoms, an improved physical examina-ion, and Doppler study showing mild or no residualradient and mild or no pulmonary valvular regurgitation.

ecommendations:

. Athletes with no or only residual mild PS and normalventricular function without symptoms can partici-pate in all competitive sports. Participation in sports

Table 1. Definitions of Mild, Moderate, and S

Peak-to-Peak SystolicGradient (Catherization)

Mild AS less than 30 mm HgModerate AS 30 to 50 mm HgSevere AS greater than 50 mm Hg

*Gradients obtained from apical window usually most predic

can begin two to four weeks after balloon valvulo-

plasty. After operation, an interval of approximatelythree months is suggested before resuming sportsparticipation.

. Athletes with a persistent peak systolic gradient greaterthan 40 mm Hg should follow the same recommenda-tions as those for patients before treatment.

. Athletes with severe pulmonary incompetence charac-terized by a marked right ventricular enlargement canparticipate in class IA and IB competitive sports.

ortic valve stenosis (AS)— untreated. This section dis-usses congenital valvular AS in young patients; AS indults is addressed in Task Force 3. Differentiation betweenild and either moderate or severe AS is accomplished by

hysical examination, ECG, and Doppler echocardiogra-hy. The distinction between moderate or severe stenosis isore difficult and may require cardiac catheterization in rare

ituations when clinical examination, ECG, echocardiogra-hy, and/or other data are discrepant. Patients with aistory of fatigue, light-headedness, dizziness, syncope,hest pain, or pallor on exercise deserve a full evaluation,hich may include cardiac catheterization and exercise

esting. Because AS may progress, periodic re-evaluation iseeded. Sudden death is more likely to occur in patientsith severe LV hypertrophy, exertional syncope, chest painr dyspnea and a LV strain pattern on the ECG. Betweenpproximately 20% and 80% of sudden deaths in patientsith severe AS have been found to occur on physical

xertion (2,8).For the purpose of these recommendations, Table 1

ummarizes the definitions of mild moderate and severe AS.t should be emphasized that virtually all patients classifieds moderate or severe AS would be expected to have leftentricular hypertrophy by echocardiography.

ecommendations:

. Athletes with mild AS can participate in all compet-itive sports if they have a normal ECG, normalexercise tolerance, and no history of exercise-relatedchest pain, syncope, or atrial or ventricular tachyar-rhythmia associated with symptoms.

. Athletes with moderate AS can participate in lowstatic/low-to-moderate dynamic, and moderate static/low-to-moderate dynamic (classes IA, IB, and IIA)competitive sports if the following conditions are met:• Mild or no LV hypertrophy by echocardiography

Aortic Stenosis

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• Normal exercise test without evidence of myocar-dial ischemia or atrial or ventricular tachyarrhyth-mia and with normal exercise duration and bloodpressure response. Those athletes with supraven-tricular tachycardia or multiple or complex ven-tricular tachyarrhythmias at rest or with exercisecan participate only in low-intensity competitivesports, classes IA and IB.

• Absence of symptoms, as defined in the precedingtext.

. Athletes with severe AS should not participate incompetitive sports.

The criteria in this section also apply to athletes withiscrete (membranous) subaortic stenosis and supravalvularortic stenosis.ortic stenosis—treated by operation or balloon valvu-

oplasty. After operation, a variable degree of residualtenosis or regurgitation, or both, can be present. Re-valuation by physical examination, ECG, and echocardi-graphy is necessary for reassessment. In addition, exercisetress testing or catheterization, or both, can be required foratients whose physiologic and anatomic severity cannottherwise be determined.

ecommendations:

. Athletes with residual mild, moderate, or severestenosis should follow the same recommendations aspreviously defined for untreated patients.

. Athletes with moderate to severe aortic regurgitationshould follow the recommendations in Task Force 3:Valvular Heart Disease.

. Because of the propensity for recurrence of LVoutflow obstruction in discrete subaortic stenosispostoperatively, these patients require continuedfollow-up and re-evaluation annually for exerciserecommendations. This recommendation also ap-plies to all other forms of fixed AS.

oarctation of the aorta— untreated. This abnormality isharacterized by obstruction usually present in the juxtaduc-al or juxtaligamental area. Severity is assessed by the armnd leg pressure gradient, physical examination, exerciseesting, echocardiographic/Doppler studies, and cardiacagnetic resonance (CMR) studies. Virtually all patients,

xcept those with mild coarctation, will undergo eitherurgical repair or balloon dilation/stenting.

ecommendations:

. Athletes with mild coarctation and the absence oflarge collateral vessels or significant aortic root dila-tion (z-score 3.0 or less) (score 3.0 � 3 standarddeviations from the mean for patient size), with anormal exercise test and a small pressure gradient atrest (usually 20 mm Hg or less between upper and
lower limbs), and a peak systolic blood pressure 230 t
mm Hg or less with exercise can engage in allcompetitive sports.

. Athletes with a systolic arm/leg gradient more than20 mm Hg or exercise-induced hypertension with asystolic blood pressure more than 230 mm Hg canengage in only low-intensity competitive sports (classIA) until treated.

oarctation of the aorta—treated by surgery or balloonngioplasty. The majority of patients will have coarctationepair or balloon arterioplasty performed during childhood.fter repair, abnormalities can persist, such as a mild

esidual gradient, ventricular hypertrophy, systemic hyper-ension, and residual obstruction evident on exercise (9,10).efore a decision for participation, minimal diagnostic

tudies, including a chest radiograph, ECG, exercise testing,nd echocardiographic evaluation of LV function and aorticnatomy, should be done. Magnetic resonance imaging cane useful to document residual anatomical abnormalities,ortic dilation, or aneurysm formation.

ecommendations:

. Participation in sports, three or more months aftersurgical or balloon angioplasty for coarctation of theaorta, is permitted for athletes with a 20 mm Hg orless arm/leg blood pressure gradient at rest and anormal peak systolic blood pressure during rest andexercise.

. During the first postoperative year, athletes shouldrefrain from high-intensity static exercise (classesIIIA, IIIB, and IIIC) and sports that pose the dangerof bodily collision.

. After three months, if patients continue to be asymp-tomatic, with normal blood pressure at rest andexercise, all sports are permissible except those with alarge static component (particularly, classes IIIA,IIIB, and IIIC).

. For athletes with evidence of significant aortic dila-tion, wall thinning, or aneurysm formation, partici-pation should be restricted to low-intensity compet-itive sports (classes IA and IB).

levated pulmonary resistance with congenital heartisease. Patients who have pulmonary vascular disease andongenital heart disease are at risk for sudden death duringports activity. As pulmonary vascular obstruction progresses,hese patients develop cyanosis at rest and intense cyanosis withxercise. Although most of these patients self-limit theirctivity, they should not participate in competitive sports.

Patients who have suspected elevated pulmonary arteryressure after operation or interventional catheterization forhunt lesions should be evaluated by echocardiographynd/or cardiac catheterization before engaging in competi-
ive athletics.

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ecommendations:

. If pulmonary artery peak systolic pressure is 30 mmHg or less, athletes can participate in all sports.

. If pulmonary artery pressure is more than 30 mm Hg,a full evaluation and individual exercise prescriptionare required for athletic participation.

entricular dysfunction after cardiac surgery. Leftnd/or right ventricular dysfunction can occur afterurgical treatment of both simple and complex congenitaleart diseases and affect exercise performance. Periodicssessment of ventricular function is required for partic-pation in sports because ventricular function may dete-iorate over time.

ecommendations:

. For full participation, normal or near-normal ven-tricular function is required (ejection fraction 50% ormore).

. Athletes with mildly depressed ventricular function(ejection fraction 40% to 50%) should participate inlow-intensity static competitive sports only (classesIA, IB, and IC).

. Athletes with moderately to severely depressed ven-tricular function (ejection fraction less than 40%)should not participate in competitive sports.

yanotic congenital cardiac disease— unoperated. Cya-otic congenital heart disease is associated with exercise

ntolerance and progressive hypoxemia with increasing ef-ort. Patients are unlikely to engage in competitive sportsecause of their own self-limiting activity. There are rareatients with cyanotic congenital heart disease (such asulmonary stenosis or mildly elevated pulmonary vascularesistance plus atrial or ventricular defects) who reachdolescence or even adult life with mild cyanosis at rest andhortness of breath only with exercise. These patients mayxperience a profound increase in cyanosis during sportsarticipation.

ecommendation:

. Patients with untreated cyanotic heart disease canusually participate in low-intensity competitivesports of only class IA.

ostoperative palliated cyanotic congenital heart disease.alliative surgical intervention can be performed to increaseulmonary blood flow in patients with decreased flow or to

imit blood flow in those with excessive flow. Often theseatients have significant relief of symptoms at rest, butrterial desaturation during exercise frequently persists.

ecommendation:

. Patients can participate in low-intensity competitivesports (class IA), provided that the following criteria
are met: g
• Arterial saturation remains above approximately80%

• Tachyarrhythmias associated with symptoms ofimpaired consciousness not present

• There is not moderate/severe ventricular dys-function.

ostoperative tetralogy of Fallot (T/F). The current treat-ent for T/F is early repair, and patients have varying degrees

f pulmonary stenosis and mild/moderate pulmonary insuffi-iency. Diagnostic evaluation usually includes physical exami-ation, chest radiograph, echocardiography, CMR, ambula-ory ECG monitoring, and exercise testing. Cardiacatheterization and/or exercise testing may be required foromplete evaluation in selected patients, particularly those withignificant cardiomegaly and/or symptoms. Patients with im-ortant residual abnormalities, such as a significant left-to-ight shunt, right ventricular hypertension, moderate-to-severeulmonary regurgitation, or right ventricular dysfunction, wholso have a history of syncope and/or arrhythmia, may be at riskor sudden death (11).

ecommendations:

. Athletes with an excellent repair should be allowed toparticipate in all sports, providing that the followingcriteria are met:• Normal or near-normal right heart pressure• No or only mild right ventricular volume overload• No evidence of a significant residual shunt• No atrial or ventricular tachyarrhythmia abnor-

mality on ambulatory ECG monitoring or exercisetesting

. Patients with marked pulmonary regurgitation andright ventricular volume overload, residual right ven-tricular hypertension (peak systolic right ventricularpressure greater than or equal to 50% systemic pres-sure), or atrial or ventricular tachyarrhythmias,should participate in low-intensity competitive sportsonly (class IA).

ransposition of the great arteries (TGA)—post-perative Mustard or Senning operation. Patients whoave had atrial repair of TGA can have significant hemo-ynamic abnormalities including impaired systemic venouseturn, abnormal right ventricular (systemic ventricular)unction, pulmonary stenosis or pulmonary hypertension,bnormalities of pulmonary venous return, tricuspid insuf-ciency, and significant atrial or ventricular arrhythmias12). After atrial repair, the right ventricle is subjected toystemic pressure, and because of the intrinsic properties ofhe right ventricle its reserve is believed to be less than thatf the left ventricle. The consequences of hypertrophy andilation in a trained athlete after an otherwise excellent atrialepair of transposition are unknown. Evaluation beforeraining and competition in moderate- and low-intensityports should include history and examination, chest radio-
raph, ECG, echocardiogram, CMR, ambulatory ECG

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onitoring, and exercise testing. For patients in whom datare unclear with regard to hemodynamic abnormalities orentricular function, cardiac catheterization may beecessary.

ecommendations:

. Selected patients can engage in low and moderatestatic/low dynamic competitive sports (classes IA andIIA) provided there is:• Mild or no cardiac chamber enlargement on chest

radiograph, echocardiography, or CMR• No history of atrial flutter, supraventricular tachy-

cardia, or ventricular tachyarrhythmia• No history of syncope or other cardiac symptoms• A normal exercise test defined as normal duration,

workload, heart rate, ECG, and blood pressureresponse for age and gender.

. Patients not in this category require an individual-ized exercise prescription.

ostoperative arterial switch for TGA. A significant co-ort of patients have had arterial switch repair of TGA andre now old enough to engage in competitive sports. Theseatients have a low prevalence of ventricular dysfunction,rrhythmia with symptoms, and hemodynamic sequelaewith the possible exception of pulmonary artery or anasto-otic stenosis and neoaortic root dilation). Only limited

xercise data are available.

ecommendations:

. Athletes with normal ventricular function, normalexercise test, and no atrial or ventricular tachyar-rhythmias can participate in all sports.

. Athletes with more than mild hemodynamic abnor-malities or ventricular dysfunction can participate inlow and moderate static/low dynamic competitivesports (classes IA, IB, IC, and IIA), provided thattheir exercise test is normal.

ongenitally corrected transposition of the great arteriesCCTGA). Usually, CCTGA is associated with otherongenital malformations of the heart, such as ventriculareptal defect, pulmonary stenosis, and systemic AV valvebnormalities, which may dictate the level of participationn competitive sports. These patients are at risk for devel-pment of supraventricular tachycardia and spontaneousV block.

ecommendations:

. Asymptomatic patients with CCTGA without othercardiac abnormalities may be eligible for participa-tion in class IA and IIA sports if there is no systemicventricle enlargement, no evidence of atrial or ven-tricular tachyarrhythmia on ambulatory ECG moni-toring or exercise testing or normal exercise tests(including normal maximum oxygen consumption for
age and gender).
. Periodic re-evaluation is important to detect devel-opment of arrhythmias and deterioration of systemic(right) ventricular function and systemic (tricuspid)AV valve regurgitation. In particular, sports with alarge static component (classes IIIA, IIIB, IIIC)such as power weightlifting are not recommended.

ostoperative Fontan operation. The Fontan operation isharacterized by systemic venous return bypassing the rightentricle. The operation is used for the long-term palliationf patients with tricuspid atresia or other complex types ofingle ventricle. Although many patients improve clinicallyfter the Fontan operation, they usually have limited exer-ise capacity and reduced cardiac output at rest and duringxercise (13). Postoperative arrhythmias have been associ-ted with significant morbidity and mortality. Diagnosticvaluation before sports participation should include a chestadiograph, ECG, echocardiography or CMR, and exerciseesting with oxygen saturations.

ecommendations:

. Athletes can participate in low-intensity competitivesports (class IA).

. Athletes can engage in class IB sports if they havenormal ventricular function and oxygen saturation.

bstein’s anomaly. A great deal of variability exists in theeverity of this malformation, which is characterized byariable degrees of tricuspid regurgitation and right-heartnlargement caused by a malformed and displaced tricuspidalve. Cyanosis may be present due to atrial right-to-lefthunting. Even mild cases may be associated with importantrrhythmias. Severe cases can be associated with physicalisability and increased risk for sudden death with exercise.

ecommendations:

. Athletes with a mild expression of Ebstein’s anomalywithout cyanosis, with normal right ventricular size,and with no evidence of atrial or ventricular tachyar-rhythmias can participate in all sports.

. Athletes with tricuspid regurgitation of moderateseverity can participate in low-intensity competitivesports (class IA) if there is no evidence of arrhythmiaon ambulatory ECG Holter monitoring other thanisolated premature contractions.

. Athletes with severe Ebstein’s anomaly are precludedfrom all sports participation. However, after surgicalrepair, low-intensity competitive sports (class IA) canbe permitted if tricuspid regurgitation is absent ormild, cardiac chamber size on chest radiograph or byechocardiography is not substantially increased, andsymptomatic atrial or ventricular tachyarrhythmiasare not present on ambulatory ECG monitoring andexercise test. Selected athletes with an excellenthemodynamic result after repair may be permitted
additional participation on an individual basis.

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ongenital coronary artery anomalies. Congenital coro-ary anomalies of wrong sinus origin are the second mostommon cardiovascular cause of sudden death in youngthletes (1). The most common of these malformations isnomalous origin of the left main coronary artery from thenterior (right) sinus of Valsalva, with an acute angled bendoursing between the pulmonary trunk and the anteriorspect of the aorta (14,15). Rare cases of anomalous originf the right coronary artery from the left coronary sinus,ongenitally hypoplastic coronary arteries, and anomalousrigin of the left main coronary artery from the pulmonaryrunk have also been associated with sudden cardiac deathuring exercise (1). Identification of these anomalies during

ife can be difficult because patients often do not experiencearning symptoms, and rest and exercise ECGs are usuallyormal. Coronary anomalies should be considered in ath-

etes with exertional syncope or symptomatic ventricularrrhythmia and should be investigated using appropriatetudies such as echocardiography, CMR, or ultrafast com-uted tomography imaging. Coronary arteriography is in-icated if other studies are not diagnostic. Surgery is usuallyerformed when the diagnosis is made (16).

ecommendations:

. Detection of coronary anomalies of wrong sinusorigin in which a coronary artery passes betweengreat arteries should result in exclusion from allparticipation in competitive sports.

. Participation in all sports three months after success-ful operation would be permitted for an athletewithout ischemia, ventricular or tachyarrhythmia, ordysfunction during maximal exercise testing.

. Athletes with previous myocardial infarction (MI)should follow the appropriate recommendations inTask Force 6: Coronary Artery Disease.

discussion of coronary artery myocardial bridging appearsn Task Force 6: Coronary Artery Disease.

awasaki disease. Kawasaki disease, an acute, self-limitedasculitis of unknown etiology, is now the most commonause of acquired heart disease in children in the U.S. (17).oronary artery aneurysms develop in approximately 20% ofntreated children and in 4% of those treated with high-ose intravenous gammaglobulin in the acute phase (18).oronary aneurysms, together with progressive coronary

rtery stenosis, can lead to ischemic heart disease, MI, orudden death (19). Because coronary artery morphologyvolves over time, the risk of exercise for the individualatient may change. Patients without coronary arteryhanges on echocardiography at any stage of the illnessppear to have risk for ventricular tachyarrhythmias andudden death similar to that of the normal population in therst 20 years after illness onset (19). When aneurysmsegress to normal lumen diameter, structural and functional
oronary abnormalities persist (20). Arteries with persistent
neurysmal morphology may develop stenoses or occlusion,ncreasing the risk of myocardial ischemia (21). The riskssociated with competitive sports in individuals who havead Kawasaki disease depends upon the degree of coronaryrtery involvement. Of note, because of the general cardio-ascular benefits of exercise, all patients with Kawasakiisease should avoid a sedentary lifestyle.

ecommendations:

. Patients with no coronary artery abnormalities ortransient coronary artery ectasia resolving during theconvalescent phase of the disease are encouraged toparticipate in all sports after six to eight weeks.

. Patients with regressed aneurysms can participate inall competitive sports if they have no evidence ofexercise-induced ischemia by stress testing with myo-cardial perfusion imaging.

. Patients with isolated small- to medium-sized aneu-rysms in one or more coronary arteries and judged tobe at low risk for ischemic complications (normal leftventricular function, absence of exercise-inducedischemia or arrhythmia) may participate in low tomoderate static and dynamic competitive sports(classes IA, IB, IIA, and IIB). Stress testing withevaluation of myocardial perfusion should be re-peated at one- to two-year intervals to monitorischemia and guide further recommendations aboutsports competition.

. Patients with one or more large coronary aneurysmsor multiple (segmented) or complex aneurysms withor without obstruction to coronary flow may partici-pate in class IA and IIA sports if they have noevidence of reversible ischemia on stress testing,normal LV function, and absence of exercise-inducedarrhythmia. Stress testing with evaluation of myocar-dial perfusion should be repeated at one-year inter-vals to monitor ischemia and guide further recom-mendations about sports competition.

. Athletes with recent MI or revascularization shouldavoid competitive sports until their recovery is com-plete— usually six to eight weeks. Those with normalLV ejection fraction, exercise tolerance, absence ofreversible ischemia or myocardial perfusion testing,and absence of exercise-induced arrhythmias canparticipate in class IA and IB sports. Those with leftventricular ejection fraction less than 40%, exerciseintolerance, or exercise-induced ventricular tachyar-rhythmias should not participate in competitivesports.

. Patients with coronary lesions who are taking anti-coagulants and/or antiplatelet drugs (aspirin, clopi-dogrel) should not participate in sports that pose adanger of high speed collision.

doi:10.1016/j.jacc.2005.02.009

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ASK FORCE 2 REFERENCES1. Maron BJ. Sudden death in young athletes. N Engl J Med 2003;349:

1064–75.2. Driscoll DJ, Edwards WD. Sudden unexpected death in children and

adolescents. J Am Coll Cardiol 1985;5:118B–21B.3. Garson AJ, McNamara DG. Sudden death in a pediatric cardiology

population, 1958 to 1983: relation to prior arrhythmias. J Am CollCardiol 1985;5:134B–7B.

4. Mitchell JH, Maron BJ, Epstein SE. 16th Bethesda conference:cardiovascular abnormalities in the athlete: recommendations regard-ing eligibility for competition: October 3–5, 1984. J Am Coll Cardiol1985;6:1186–232.

5. Sealy WC, Fanner JC, Young WG J, Brown IW J. Atrial dysrhythmiaand atrial secundum defects. J Thorac Cardiovasc Surg 1969;57:245–50.

6. Vetter VL, Horowitz LN. Electrophysiologic residua and sequelae ofsurgery for congenital heart defects. Am J Cardiol 1982;50:588–604.

7. Bink-Boelkens MT, Velvis H, van der Heide JJ, Eygelaar A, Hard-jowijono RA. Dysrhythmias after atrial surgery in children. AmHeart J 1983;106:125–30.

8. Doyle EF, Arumugham P, Lara E, Rutkowski MR, Kiely B. Suddendeath in young patients with congenital aortic stenosis. Pediatrics1974;53:481–9.

9. Pelech AN, Kartodihardjo W, Balfe JA, Balfe JW, Olley PM, LeenenFH. Exercise in children before and after coarctectomy: hemodynamic,echocardiographic, and biochemical assessment. Am Heart J 1986;112:1263–70.

0. Freed MD, Rocchini A, Rosenthal A, Nadas AS, Castaneda AR.Exercise-induced hypertension after surgical repair of coarctation ofthe aorta. Am J Cardiol 1979;43:253–8.

1. Garson AJ, Gillette PC, Gutgesell HP, McNamara DG. Stress-induced ventricular arrhythmia after repair of tetralogy of Fallot. Am J

2. Graham TP Jr. Hemodynamic residua and sequelae following intra-atrial repair of transposition of the great arteries: a review. PediatrCardiol 1982;2:203–13.

3. Driscoll DJ. Exercise responses in functional single ventricle beforeand after Fontan operation. Prog Pediatr Cardiol 1993;2:44–9.

4. Basso C, Maron BJ, Corrado D, Thiene G. Clinical profile ofcongenital coronary artery anomalies with origin from the wrong aorticsinus leading to sudden death in young competitive athletes. J Am CollCardiol 2000;35:1493–501.

5. Davis JA, Cecchin F, Jones TK, Portman MA. Major coronary arteryanomalies in a pediatric population: incidence and clinical importance.J Am Coll Cardiol 2001;37:593–7.

6. Romp RL, Herlong JR, Landolfo CK, et al. Outcome of unroofingprocedure for repair of anomalous aortic origin of left or right coronaryartery. Ann Thorac Surg 2003;76:589–95.

7. Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treat-ment, and long-term management of Kawasaki disease: a statement forhealth professionals from the Committee on Rheumatic Fever, Endo-carditis and Kawasaki Disease, Council on Cardiovascular Disease inthe Young. Circulation 2004;110:2747–71.

8. Terai M, Shulman ST. Prevalence of coronary artery abnormalities inKawasaki disease is highly dependent on gamma globulin dose butindependent of salicylate dose. J Pediatr 1997;131:888–93.

9. Kato H, Sugimura T, Akagi T, et al. Long-term consequences ofKawasaki disease: a 10- to 21-year follow-up study of 594 patients.Circulation 1996;94:1379–85.

0. Tsuda E, Kamiya T, Kimura K, Ono Y, Echigo S. Coronary arterydilatation exceeding 4.0 mm during acute Kawasaki disease predicts ahigh probability of subsequent late intima-medial thickening. PediatrCardiol 2002;23:9–14.

1. Tsuda E, Kamiya T, Ono Y, Kimura K, Kurosaki K, Echigo S.Incidence of stenotic lesions predicted by acute phase changes incoronary arterial diameter during Kawasaki disease (epub ahead of

Cardiol 1980;46:1006–12. print). Pediatr Cardiol 2004;May 12.


Name Research Grant Expert Witness Testimony

r. David Driscoll None None

r. Welton M. Gersony None None

r. Thomas P. Graham None None

r. Jane W. Newburger ● Pfizer None● Philips None

r. Albert Rocchini None None

r. Jeffrey Towbin None None● 2005, Defense, Myocarditis, sudden death in child● 2004, Defense, Myocarditis, sudden death in child● 2004, Defense, Myocarditis, sudden death in child● 2002, Defense, Myocarditis, sudden death in child● 2000, Defense, Myocarditis, sudden death in child● 1996, Plaintiff, Myocarditis, sudden death in professional athlete● 1995, Plaintiff, Myocarditis, sudden death in professional athlete

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1334 Bonow et al. JACC Vol. 45, No. 8, 2005Task Force 3: Valvular Heart Disease April 19, 2005:1334–40

ask Force 3: Valvular Heart Diseaseobert O. Bonow, MD, FACC, Chair
elvin D. Cheitlin, MD, FACC, Michael H. Crawford, MD, FACC, Pamela S. Douglas, MD, FACC
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he following valvular disorders are considered here in theontext of competitive athletes: mitral stenosis (MS), mitralegurgitation (MR), aortic stenosis (AS), aortic regurgita-ion (AR), tricuspid regurgitation (TR), tricuspid stenosis,nd multivalvular disease. Recommendations are also pro-ided for patients who have undergone valve repair oreplacement.

The diagnosis of valvular heart disease usually can beade on the basis of the characteristic murmurs and

ssociated findings on physical examination. Estimation ofhe severity of valvular disease is more difficult. In athletes,here secondary gain in denying symptoms is likely, theistory may be unreliable. In truly asymptomatic patients,everity can usually be determined from the physical exam-nation and Doppler echocardiography. Additional testings often not necessary, but the electrocardiogram (ECG) andhest radiograph can also aid in assessing severity, andadionuclide angiography or cardiac magnetic resonance areelpful in assessing left ventricular (LV) function when thechocardiogram is of suboptimal quality.

Doppler echocardiography is the most important diag-ostic test for valvular heart disease and is generally reliable

n distinguishing the severity of the lesion and assessingemodynamics and ventricular compensation. Althoughery sensitive in identifying regurgitant jets, quantitationan be more difficult. Recent guidelines have providedecommendations for quantifying valvular regurgitation (1).nly rarely are cardiac catheterization and angiography

ecessary to determine the hemodynamic severity of valvularesions.

Valvular regurgitation is frequently detected in normalubjects, including those without murmurs. With pulsedoppler echocardiography, TR has been reported in 24% to

6% of normal subjects, MR in 10% to 40%, pulmonicegurgitation in 18% to 92%, and AR in 0% to 33% (2,3). Inthletes, the prevalence of valvular regurgitation detected byoppler is even higher, with at least one regurgitant jet

ound in greater than 90% of subjects and triple-valveegurgitation in 20% (4). The vast majority of these jetsepresents a trivial regurgitant volume and is of no clinicalignificance.

Although considerable information exists concerning theatural history, development of symptoms, importance ofV function, and indications for surgical or catheter-based

ntervention in patients with valvular heart disease (5), therere few data with regard to the rate of progression of thealvular disease (especially in those exercising vigorously) or
actors that influence the rate of progression. The rate of v
rogression of aortic stenosis is highly variable amongndividuals and difficult to predict (6). Chronic MR mayncrease in severity with time but is more likely to do so inatients who experience a new chordal rupture and flailitral leaflet (7). Little is known about the influence of

trenuous exertion on the progression of ventricular dys-unction, especially when that exertion is periodic in nature.

hen valvular disease coexists with another cardiovascularbnormality, such as arrhythmias or coronary artery diseaseCAD), recommendations with regard to eligibility forports should be based on the most restrictive of theseuidelines.

The present recommendations with regard to the permittedevel of athletic activity are offered only as guidelines. Physi-ians with knowledge of an individual athlete, including theeverity of the lesion and the physiological and psychologicalesponse to competition, may liberalize these recommenda-ions in selected instances. The recommendations are for thosethletes who are asymptomatic, as symptoms will preventigorous exercise in most subjects and, in most circumstances,ymptoms represent an indication for valve replacement orepair (5). In situations in which the history may be unreliablee.g., when secondary gain in denying symptoms is likely),xercise tolerance testing is useful in confirming normal effortolerance and suitability for the proposed athletic activity.

ITRAL STENOSIS

he etiology of MS is almost always rheumatic. Most patientsith significant MS will be sufficiently symptomatic thatarticipation in competitive sports is not an issue, but patientsith mild-to-moderate MS may be asymptomatic even with

trenuous exercise. Although MS rarely causes sudden death,xercise (with an increase in heart rate and cardiac output) canause sudden marked increases in pulmonary capillary andulmonary artery pressures, at times resulting in sudden acuteulmonary edema (8). Furthermore, the long-term effect ofepeated exertion-related increases in pulmonary artery wedgend pulmonary artery pressures on the lungs or right ventricles unknown. The effect of even periodic strenuous exercise onhe likelihood of developing atrial fibrillation is also not known.

hen atrial fibrillation occurs, even patients with mild MSust be anticoagulated. The aforementioned considerationsust be understood by the patient and the family in consid-

ring participation in strenuous competitive activity. Anotherroblem associated with MS is systemic embolization, occur-ing most commonly in the presence of atrial fibrillation, buthere is no evidence that this potential complication is pro-
oked by strenuous exercise.

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1335JACC Vol. 45, No. 8, 2005 Bonow et al.April 19, 2005:1334–40 Task Force 3: Valvular Heart Disease

valuation. Clues regarding the hemodynamic severity ofS may often be obtained from the history, physical

xamination, ECG, and chest radiograph, but accurateoninvasive assessment of severity requires two-dimensionalnd Doppler echocardiography (9). In patients with MS andinimal or no symptoms who wish to engage in competitive

ports, exercise stress testing should be performed to at leasthe level of activity approximating the exercise demands ofhe sport, particularly when there is a question as to theeverity of the MS. In addition, pulmonary artery systolicressure during exercise can be estimated noninvasively byoppler echocardiography and may be helpful in making a

ecision as to how much activity is safe, even if the severityf MS in an individual patient is estimated to be only mild5). If this is not practical or possible, then right heartatheterization with exercise can be performed to measurehe pulmonary capillary wedge pressure and pulmonaryrtery pressure.

Hemodynamic severity of MS can be categorized asollows: mild � mitral valve area greater than 1.5 cm2,xercise pulmonary artery wedge pressure less than or equalo 20 mm Hg, or rest pulmonary artery systolic pressure lesshan 35 mm Hg; moderate � mitral valve area 1.0 to 1.5m2, exercise pulmonary artery wedge pressure less than orqual to 25 mm Hg, or rest pulmonary artery systolicressure less than or equal to 50 mm Hg; severe � mitralalve area less than 1.0 cm2, exercise pulmonary arteryedge pressure greater than 25 mm Hg, or rest pulmonary

ystolic pressure greater than 50 mm Hg.

ecommendations:

. Athletes with mild MS (as previously defined) in sinusrhythm with peak pulmonary artery systolic pressureduring exercise less than 50 mm Hg can participate inall competitive sports. Athletes with mild MS in atrialfibrillation, or a history of atrial fibrillation, are dis-cussed in recommendation 4 below.

. Athletes with moderate MS (as previously defined),either in sinus rhythm or in atrial fibrillation, withpeak pulmonary artery systolic pressure during exer-cise less than 50 mm Hg can participate in low andmoderate static and low and moderate dynamic com-petitive sports (classes IA, IB, IIA, and IIB [seeTable 1 in Task Force Report 8: Classification ofSports]).

. Athletes with severe MS in either sinus rhythm oratrial fibrillation or those with peak pulmonary arterypressure greater than 50 mm Hg during exerciseshould not participate in any competitive sports.

. Patients with MS of any severity who are in atrialfibrillation or have a history of atrial fibrillation, andwho must receive anticoagulation therapy, should notengage in any competitive sports involving the riskfor bodily contact (see Task Force Report 8: Classi-
fication of Sports) or possible trauma. o
ITRAL REGURGITATION

itral regurgitation, unlike MS, has a variety of etiologies,he most common of which is mitral valve prolapse (myx-matous mitral valve). Other common causes are rheumaticeart disease, infective endocarditis, CAD, connective tissueiseases (such as Marfan syndrome), and dilated cardiomy-pathy. The recommendations outlined in this section areor patients with primary valvular MR rather than MRecondary to CAD or other conditions causing LV dilationsee Task Force 4: HCM and Other Cardiomyopathies,

itral Valve Prolapse, Myocarditis, and Marfan Syndrome,nd Task Force 6: Coronary Artery Disease).valuation. Mitral regurgitation can be detected by the

haracteristic physical findings and confirmed by Dopplerchocardiography. The severity of the MR is related to theagnitude of the regurgitant volume, which results in LV

ilation and increased left atrial pressure. The increased LViastolic volume enhances total LV stroke volume enough toccommodate the regurgitant volume and to maintain theorward stroke volume within normal limits. The lowmpedance presented by regurgitation into the left atriumnloads the left ventricle during ventricular systole, suchhat measures of LV pump function (e.g., ejection fraction)end to overestimate true myocardial performance (10).

The severity of chronic MR can be adequately judged byoninvasive techniques, principally two-dimensional andoppler echocardiography. The larger the jet area, and theider the jet at its origin above the valve, the more severe

he regurgitation. The entry of the jet into the atrialppendage or pulmonary vein or systolic reversal of the flown pulmonary veins are all indicators of severity. Various

easures of the severity of MR have been described (1,11).n some patients with eccentric jets or those impinging onhe atrial wall, the assessment may be difficult (1). Gener-lly, the LV diastolic volume reflects the severity of chronic

R. However, it should be noted that the upper limit of normalV size is increased in the healthy, highly trained athlete. In a

eries of elite athletes, echocardiographic LV end-diastolic di-ensions as high as 66 mm were recorded in women (mean, 48m) and up to 70 mm in men (mean, 55 mm) (12). LV

nd-diastolic dimensions greater than or equal to 55 mm werebserved in 45% of participants and greater than 60 mm in4% of participants. Therefore, assessment of LV enlargement inhighly trained athlete with known or suspected valvular heart

isease must take this issue into consideration. Hence, forurposes of this discussion, an LV end-diastolic dimensionreater than 60 mm is considered likely to represent theffects of LV volume overload due to valvular disease andot per se to physiologically based exercise training.Patients with chronic MR should be followed longitudi-

ally with serial echocardiograms (5). A decrease in ejectionraction and/or increase in end-systolic volume with time ishelpful marker of declining LV function and an indication
f having reached the limits of cardiac compensation.

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ffects of exercise. In general, exercise produces no signifi-ant change or a mild decrease in the regurgitant fractionecause of reduced systemic vascular resistance. However,atients with elevation of heart rate (increased systolic ejectionime per min) or blood pressure with exercise may manifestarked increases in regurgitant volume and pulmonary capil-

ary pressures. Hence, static exercise that increases arterialressure is potentially deleterious. Ejection fraction usuallyoes not change or decreases slightly with exercise, althoughhe ejection fraction response may be completely normal inoung, asymptomatic subjects.

The etiology of MR may be important in making recom-endations concerning heavy physical activity. In patients withR secondary to previous infective endocarditis or ruptured

hordae, the valve tissues theoretically could be further dam-ged or torn by marked sustained increases in LV systolicressure.

ecommendations:

. Athletes with mild to moderate MR who are in sinusrhythm with normal LV size and function and withnormal pulmonary artery pressures can participate inall competitive sports.

. Athletes with mild to moderate MR in sinus rhythmwith normal LV systolic function at rest and mild LVenlargement (compatible with that which may resultsolely from athletic training [less than 60 mm {12}])can participate in some low and moderate static andlow, moderate, and high dynamic competitive sports(classes IA, IB, 1C, IIA, IIB, and IIC).

. Athletes with severe MR and definite LV enlarge-ment (greater than or equal to 60 mm [12]), pulmo-nary hypertension, or any degree of LV systolicdysfunction at rest should not participate in anycompetitive sports.

. Patients in atrial fibrillation or a history of atrialfibrillation who are receiving long-term anticoagula-tion should not engage in sports involving any riskfor bodily contact (see Task Force 8: Classification ofSports) or danger of trauma.

ORTIC STENOSIS

he diagnosis of AS is established by the characteristichysical findings and two-dimensional and Doppler echo-ardiography. The three most common etiologies are: 1)heumatic, 2) congenital, and 3) calcific or degenerative.he majority of young adults with AS participating in

ompetitive athletics have congenital lesions.valuation. Continuous-wave Doppler echocardiography

an reliably estimate the severity of AS, especially in theresence of normal cardiac output, which is the case in thereat majority of those engaging in competitive sports (13).

Symptoms of dyspnea, syncope, or angina pectoris occurate in the course of AS (14), and the likelihood of sudden
eath increases significantly with the onset of symptoms.
ecause even transient symptoms are so important inarking the onset of increased risk of sudden death, the

hysician must be aware that dyspnea, near-syncope, andven syncope are likely to be unreported in competitivethletes. Although sudden death is more frequent in symp-omatic patients with severe AS, it may also occur inompletely asymptomatic patients (15). When doubt per-ists with regard to the severity of AS after Doppler study,r if a patient with mild-to-moderate AS has symptoms,ardiac catheterization should be performed. Sudden deaths rare with mild AS.

Athletes with a history of syncope, even with mild AS,hould be carefully evaluated by a cardiologist. This shouldnclude assessment of arrhythmias with exercise. Syncopehould be regarded as a possible surrogate for spontaneouslyborted sudden death and should be thoroughly investigatedsee Task Force 7: Arrhythmias).

Severity of AS measured by continuous-wave Dopplerchocardiography (or in those instances previously noted byardiac catheterization) is categorized as follows with re-pect to the calculated aortic valve area: mild � greater than.5 cm2; moderate � 1.0 to 1.5 cm2; and severe � less thanr equal to 1.0 cm2 (5). This translates roughly (assuminghat athletes have normal cardiac output) to the estimatedean aortic valve pressure gradient as follows: mild � less

han 25 mm Hg; moderate � 25 to 40 mm Hg; and severe �reater than 40 mm Hg (5).

Because AS is often progressive, periodic re-evaluation ateast yearly is necessary and should be performed by ahysician with expertise in cardiology. This reassessmentncludes physical examination and Doppler echocardiogra-hy, but may require cardiac catheterization in selectedatients as previously noted. In addition, Holter monitoringith intense exercise resembling competition is recom-ended to detect ventricular arrhythmias in patients withS who wish to participate in competitive athletics.In patients with AS, a markedly elevated cardiac output

r peripheral vascular resistance for sustained periods ofime could result in an exaggerated valvular gradient and aarked increase in LV systolic pressure. Given these pre-

autions, the following recommendations can be made.

ecommendations:

. Athletes with mild AS can participate in all compet-itive sports, but should undergo serial evaluations ofAS severity on at least an annual basis.

. Athletes with moderate AS can engage in low-intensitycompetitive sports (class IA). Selected athletes mayparticipate in low and moderate static or low andmoderate dynamic competitive sports (classes IA, IB,and IIA) if exercise tolerance testing to at least the levelof activity achieved in competition demonstrates satis-factory exercise capacity without symptoms, ST-segment depression or ventricular tachyarrhythmias,
and with a normal blood pressure response. Those

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athletes with supraventricular tachycardia or multiple orcomplex ventricular tachyarrhythmias at rest or withexercise can participate only in low-intensity competi-tive sports (class IA).

. Patients with severe AS or symptomatic patients withmoderate AS should not engage in any competitivesports.

ORTIC REGURGITATION

ortic regurgitation has multiple etiologies, as any diseaseffecting the aortic valve, annulus, or proximal ascendingorta can result in AR. The common etiologies are: 1)ongenital bicuspid aortic valve; 2) rheumatic heart disease;) infective endocarditis; and 4) aortic root diseases, includ-ng Marfan syndrome, ascending aortic aneurysm, aorticissection, systemic hypertension, and rheumatoid spondy-

itis. Aortic regurgitation increases LV diastolic volume andtroke volume, which may ultimately lead to LV systolicysfunction (10). In addition, myocardial oxygen supply/emand imbalance may develop because of the increasedall stress, LV hypertrophy, and reduced diastolic bloodressure (reduced coronary perfusion pressure).Patients with severe AR may remain asymptomatic and

thletic for many years, but angina pectoris, syncope, andentricular arrhythmias ultimately may appear. Suddeneath is rare among asymptomatic patients (less than 0.2%er year [5]) but can occur.valuation. The hemodynamic severity of AR can be

ssessed noninvasively by physical examination (the severityeing reflected by the degree of LV dilation and theeripheral signs of AR), chest radiography, and echocardi-graphy. As noted previously, the upper limit of normal LVnd-diastolic size is increased in the healthy, highly trainedthlete (12), and this may well affect assessment of LVnlargement in the setting of AR.

Because of the importance of assessing LV function andhe size of the aortic root and proximal ascending aorta inetermining the etiology of AR, with resulting implicationsor athletic participation, evaluation by echocardiography isssential. Doppler echocardiography is very sensitive inetecting any degree of AR. Similar to MR, the greatestifficulty arises in differentiating moderate and severe AR.ualitatively, the width of the regurgitant jet and the

roportion of the LV outflow tract occupied by the jet areelated to the severity of the AR, as is the slope velocityrofile of the diastolic jet. The regurgitant volume can alsoe measured quantitatively by Doppler methods (1).The LV function should be assessed serially by two-

imensional echocardiography (5). Radionuclide angiogra-hy or cardiac magnetic resonance may be helpful ifchocardiograms are of suboptimal quality. Exercise testingan be useful in assessing exercise capacity, especially inhose patients having nonspecific or mild symptoms, and its recommended that testing be performed to at least the
evel of exertion required by the proposed competitive sport.
olter monitoring with intense exercise resembling compe-ition is recommended to detect ventricular arrhythmias inatients who wish to participate in competitive athletics.ffects of exercise. With exercise, regurgitant volume de-

reases because of the decrease in peripheral vascular resis-ance that reduces diastolic blood pressure and the decreasen diastolic filling period that accompanies the increase ineart rate (16). Because of these changes in preload andfterload, the failure of the ejection fraction to increase withxercise is of uncertain significance, and there are insuffi-ient data with which to use this finding in formulatingecommendations regarding participation in competitivethletics. There are also no data to define whether severencreases in physical activity permanently affect the functionf the left ventricle.For purposes of the following recommendations, hemo-

ynamic severity of AR is graded as follows: mild � absento slight peripheral signs of AR, normal LV size; moderate �eripheral signs of AR with mild-to-moderate increases inV size with normal systolic function; and severe � periph-ral signs of AR with severe LV enlargement and/or LVystolic dysfunction.

ecommendations:

. Athletes with mild or moderate AR, but with LVend-diastolic size that is normal or only mildlyincreased, consistent with that which may resultsolely from athletic training (12), can participate inall competitive sports. In selected instances, athleteswith AR and moderate LV enlargement (60 to 65mm) can engage in low and moderate static and low,moderate, and high dynamic competitive sports(classes IA, IB, 1C, IIA, IIB, and IIC) if exercisetolerance testing to at least the level of activityachieved in competition demonstrates no symptomsor ventricular arrhythmias. Those with asymptomaticnonsustained ventricular tachycardia at rest or withexertion should participate in low-intensity compet-itive sports only (class IA) (see also Task Force 7:Arrhythmias).

. Athletes with severe AR and LV diastolic diametergreater than 65 mm as well as those with mild ormoderate AR and symptoms (regardless of LV di-mension) should not participate in any competitivesports.

. Those with AR and significant dilation of the prox-imal ascending aorta (greater than 45 mm) can en-gage only in low-intensity competitive sports (classIA). These criteria do not apply to athletes withMarfan syndrome and AR, in whom the risks ofaortic dissection and rupture are high, and any degreeof aortic dilatation would be sufficient to prohibitcompetitive athletics, as discussed in Task Force 4:HCM and Other Cardiomyopathies, Mitral Valve
Prolapse, Myocarditis, and Marfan Syndrome.

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ICUSPID AORTIC VALVESITH AORTIC ROOT DILATATION

here is growing awareness that many patients withicuspid aortic valves have disorders of vascular connec-ive tissue, involving loss of elastic tissue, that may resultn aortic root dilatation even in the absence of hemody-amically significant AS or AR (17,18). These patientsave a risk of aortic dissection; surgery to repair the aortaas been recommended for those patients with greatlynlarged aortic roots (5,18). Recommendations for ath-etic participation in patients with bicuspid valve diseasend associated aortic root dilatation are based on limitedata, but with the understanding that aortic dissectionan occur in some patients with aortic root diameters lesshan 50 mm. The recommendations that follow are foratients with bicuspid valves and associated aortic rootnlargement. If such patients also have significant AS orR, these recommendations should be considered in

oncert with those discussed in the present Task Forceor patients with AS or AR. The following recommen-ations do not pertain to patients with Marfan syndrome,hich are discussed in Task Force 4.

ecommendations:

. Patients with bicuspid aortic valves with no aorticroot dilatation (less than 40 mm or the equivalentaccording to body surface area in children and ado-lescents) and no significant AS or AR may participatein all competitive sports.

. Patients with bicuspid aortic valves and dilated aorticroots between 40 and 45 mm may participate in lowand moderate static or low and moderate dynamiccompetitive sports (classes IA, IB, IIA, and IIB), butshould avoid any sports in these categories thatinvolve the potential for bodily collision or trauma.

. Patients with bicuspid aortic valves and dilated aorticroots greater than 45 mm can participate in onlylow-intensity competitive sports (class IA).

RICUSPID REGURGITATION

ricuspid regurgitation is most often secondary to rightentricular (RV) dilation and failure due to pulmonary orV hypertension. Other etiologies that may affect athletes

nclude rheumatic heart disease, tricuspid valve prolapse,nfective endocarditis, congenital anomalies such asbstein’s anomaly, and the sequelae of surgery for congen-

tal heart disease leaving the patient with RV dilatation.ecommendations here with respect to athletes are for thoseith primary TR.Primary TR leads to RV volume overload with the risk of

ubsequent RV failure as well as increased systemic venousressure and its consequences. Severity of TR and estima-ion of right atrial and RV pressures can be determined
oninvasively from physical examination, chest radiograph, s
nd Doppler echocardiography. Occasionally, right-heartatheterization is necessary when these measures cannot beetermined noninvasively. There is no evidence that thethlete with isolated primary TR is placed in jeopardy byngaging in heavy physical exertion.

ecommendation:

. Athletes with primary TR, regardless of severity, withnormal RV function in the absence of right atrialpressure greater than 20 mm Hg or elevation of RVsystolic pressure can engage in all competitive sports.

RICUSPID STENOSIS

ricuspid stenosis is for the most part caused by rheumaticeart disease and is nearly always associated with MS. Inhat instance, the patient should be judged according to theeverity of the MS. Isolated tricuspid stenosis is rare. Suchatients should undergo exercise testing at least to the levelnticipated in their sport. If asymptomatic, athletes mayompete in all competitive activities.

ULTIVALVULAR DISEASE

ultivalvular disease occurs in the context of rheumaticeart disease, myxomatous valvular disease, and infectivendocarditis. The lesions can be diagnosed by physicalxamination and Doppler echocardiography. The relativeontribution of each of the lesions may be difficult to assessoninvasively, and cardiac catheterization and angiocardio-raphy are frequently necessary to resolve these distinctions.

ecommendation:

. Multiple lesions of moderate severity may have ad-ditive physiologic effects. In general, athletes withsignificant multiple valvular disease should not par-ticipate in any competitive sports.

ROSTHETIC HEART VALVES

everal general comments apply to all patients who havendergone valve replacement. First, although most patientsmprove after valve replacement and many become asymp-omatic, the long-term mortality after operation is greaterhan that of a normal population of similar age. Second, aransvalvular gradient of varying severity is present in mostatients after valve replacement (5,19). Third, althoughemodynamic variables at rest may be essentially normalfter valve replacement, many patients have an abnormalesponse to exercise (20). Finally, after valve replacementwith few exceptions) patients with mechanical prosthesesequire anticoagulant agents, and those with bioprostheticalves in sinus rhythm usually do not. In assessing thethlete’s capacity for physical activity, exercise stress testingo at least the level of activity performed in the competitive
port is valuable.

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ffects of exercise. There are insufficient data to determinehether vigorous repetitive exercise after valve replacementas any long-lasting effect on ventricular or prosthetic valveunction. The patient should be made aware of theseeficiencies in our knowledge before deciding whether toarticipate in competitive athletics. Because mechanical andost tissue valves have reduced effective valve areas, they

erform best at normal heart rates. Therefore, a sustainedeart rate greater than 120 beats/min might result inlevated valve gradients and cardiac outputs that are lesshan normally expected.

ecommendations:

. Athletes with a bioprosthetic mitral valve not takinganticoagulant agents and who have normal valvularfunction and normal or near-normal LV function canparticipate in low and moderate static and low andmoderate dynamic competitive sports (classes IA, IB,IIA, and IIB).

. Athletes with a mechanical or bioprosthetic aorticvalve, with normal valve function and with normalLV function, can engage in low and moderate staticand low and moderate dynamic competitive sports(classes IA, IB, and IIA). Athletes participating ingreater than low-intensity competitive sports (classIA) should undergo exercise testing to at least thelevel of activity achieved in competition to evaluateexercise tolerance and symptomatic and hemody-namic responses.

. Independent of other considerations, athletes with amechanical or bioprosthetic mitral valve or aorticvalve who are taking anticoagulant agents should notengage in sports involving the risk of bodily contact(see Task Force 8: Classification of Sports) or thedanger of trauma.

ALVE REPAIR ORERCUTANEOUS MITRAL BALLOON VALVOTOMY

everal general comments apply to athletes who havendergone valve repair or percutaneous mitral balloon mitralalvotomy (PMBV). The benefits of surgical mitral valveepair for MR appear to persist long term, but the effects oftrenuous exercise on repaired mitral valves have not beentudied systematically. After treatment for MS with PMBVr either closed or open surgical mitral commissurotomy,he patient should have no disability if there has been nonjury to the left ventricle or development of significant

R. After aortic valvuloplasty in young patients withongenital AS, there is the risk of subsequent endocarditis,R, or recurrent AS. Athletes should be evaluated for

ompetitive sports on the basis of their residual hemody-
amic state.
ecommendations:

. For patients with MS who have undergone successfulPMBV or surgical commissurotomy, recommenda-tions for participation in competitive sports shouldbe based on the residual severity of the MS or MR, asin patients without operation. Capacity to engage inphysical exercise should be evaluated with an exercisetolerance test at least to the level of anticipatedactivity. Patients with LV dysfunction should berestricted from athletic participation in the samecontext as those without operation.

. Athletes who have undergone mitral valve repair forMR should not engage in sports involving the risk orlikelihood of bodily contact (see Task Force 8: Clas-sification of Sports) or possible trauma, which mightdisrupt the repair. They can participate in low-intensity competitive sports (class IA) and, in se-lected athletes, in low and moderate static and lowand moderate dynamic competitive sports (classesIA, IB, and IIA).

doi:10.1016/j.jacc.2005.02.010


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2. Kostucki W, Vandenbossche JL, Friart A, Englert M. Pulsed Dopplerregurgitant flow patterns of normal valves. Am J Cardiol 1986;58:309–13.

3. Richards KL, Cannon SR, Crawford MH, Sorensen SG. Noninvasivediagnosis of aortic and mitral valve disease with pulsed-Dopplerspectral analysis. Am J Cardiol 1983;51:1122–7.

4. Douglas PS, Berman GO, O’Toole ML, Hiller WD, Reichek N.Prevalence of multivalvular regurgitation in athletes. Am J Cardiol1989;64:209–12.

5. Bonow RO, Carabello B, de Leon AC, et al. ACC/AHA guidelinesfor the management of patients with valvular heart disease: a report ofthe American College of Cardiology/American Heart AssociationTask Force on Practice Guidelines (Committee on Management ofPatients With Valvular Heart Disease). J Am Coll Cardiol 1998;32:1486–582.

6. Otto CM, Burwash IG, Legget ME, et al. Prospective study ofasymptomatic valvular aortic stenosis: clinical, echocardiographic, andexercise predictors of outcome. Circulation 1997;95:2262–70.

7. Enriquez-Sarano M, Basmadjian AJ, Rossi A, Bailey KR, Seward JB,Tajik AJ. Progression of mitral regurgitation: a prospective Dopplerechocardiographic study. J Am Coll Cardiol 1999;34:1137–44.

8. Rahimtoola SH, Durairaj A, Mehra A, Nuno I. Current evaluationand management of patients with mitral stenosis. Circulation 2002;106:1183–8.

9. Faletra F, Pezzano A J, Fusco R, et al. Measurement of mitral valve areain mitral stenosis: four echocardiographic methods compared with directmeasurement of anatomic orifices. J Am Coll Cardiol 1996;28:1190–7.

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5. Rosenhek R, Binder T, Porenta G, et al. Predictors of outcome insevere, asymptomatic aortic stenosis. N Engl J Med 2000;343:611–7.

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ppendix 1

he authors of this report declared they have no relation-
aortic cross-sectional area to height ratio and the risk of aortic ships with industry pertinent to this topic.
ask Force 4: HCM andther Cardiomyopathies, Mitral Valverolapse, Myocarditis, and Marfan Syndrome

arry J. Maron, MD, FACC, Chairichael J. Ackerman, MD, PHD, FACC, Rick A. Nishimura, MD, FACC, Reed E. Pyeritz, MD, PHD,

FACC

YPERTROPHIC CARDIOMYOPATHY

eneral considerations. Hypertrophic cardiomyopathyHCM) is a relatively common form of genetic heart disease0.2%; 1:500 in the general population) (1), and the mostommon cause of sudden unexpected cardiac death in youngeople, including competitive athletes (2). Sudden deathay occur at any age, but is most common in individuals 30

ears of age or less. At present, 12 mutant genes (mostncoding sarcomeric proteins) and over 400 specific muta-ions in these genes have been implicated in the pathogen-sis of clinically diagnosed HCM (3).

The disease is characterized by heterogeneous presen-ation and natural history in which the most consistentiagnostic feature demonstrated by echocardiography istherwise unexplained and usually asymmetric hypertro-hy associated with a non-dilated left ventricle (LV)3–5). Clinical diagnosis of HCM is made by recognitionf the disease phenotype with LV hypertrophy (3,4). Inhis regard, a maximal LV end-diastolic wall thickness of5 mm or more (or on occasion, 13 or 14 mm) is thebsolute dimension generally accepted for the clinicaliagnosis of HCM in an adult athlete (in children, 2 orore standard deviations from the mean relative to body

urface are; z-score of 2 or more); however, any LV wallhickness (including normal) is theoretically compatibleith the presence of a mutant HCM gene (3,4). Of note,

ndividuals of virtually any age (but usually less than 14ears old) harboring a HCM-causing mutant gene mayot manifest LV hypertrophy (3,4).

rom non-athletes to highly trained competitive athletes isenuous. This relates to the unstable electrophysiologicubstrate and propensity for potentially lethal ventricularachyarrhythmias in HCM, interacting with the physiologictresses inherent in athletic training and competition (i.e.,lterations in blood volume, hydration, and electrolytes).urthermore, no single clinical, morphologic, or electro-hysiologic factor has emerged as the reliable predictor ofisk in HCM (3,4). Therefore, because the panel could notrecisely stratify sudden death risk specifically for all athletesith HCM, the present recommendations for sports eligi-ility remain conservative and homogeneous for thosethletes within the diverse HCM clinical spectrum.

Given the inability to precisely stratify risk on clinicalrounds in individual young patients with HCM, a broadecommendation to exclude such individuals from compet-tive sports will, by definition, deny participation to somennecessarily. However, given the frequency with whichCM is associated with sudden death in young athletes (2),

nd recent data showing that athletic activity per se isssociated with higher risk in those with underlying cardio-ascular abnormalities (6), the present recommendations areiewed as prudent. That is, the goal is to encompass allreventable sudden deaths in young persons with HCM,hile acknowledging that other athletes who may not beestined for sudden death will also be subjected to the sameecommendations.reclinical diagnosis. With the availability of preclinicalenetic diagnosis, a relatively small number of youthfulamily members have been identified as affected by a

CM-causing mutant gene solely on the basis of laboratory

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1341JACC Vol. 45, No. 8, 2005 Maron et al.April 19, 2005:1340–5 Task Force 4: HCM, Other Cardiomyopathies, and Marfan

NA analysis, and in the absence of typical morphologicphenotypic) features of the disease (3,4). As family geneticcreening for HCM becomes more widespread, cliniciansay be increasingly faced with the dilemma of making

ecommendations regarding sports participation for subjectsho have only preclinical evidence of HCM (i.e., genotypeositive-phenotype negative). Nonetheless, it is likely thatost such individuals are destined to ultimately develop theCM phenotype with the attendant possibility of a poten-

ially unstable electrophysiologic milieu. Moreover, theCM phenotype (i.e., LV hypertrophy) may develop over

he course of several years (3,4) after initial evaluation, whenompetitive sports participation could still be an ongoingnd important lifestyle issue.

Based on these considerations, prudent recommendationsor athletes with preclinical HCM would include, on a 12-o 18-month basis, in addition to serial two-dimensionalchocardiography: 12-lead ECG and ambulatory Holterlectrocardiogram (ECG), and less frequently cardiac mag-etic resonance (CMR) imaging and exercise stress testingo a level similar to that expected in the sport underonsideration (for evaluation of exercise tolerance, bloodressure, and ventricular tachyarrhythmias). If all of thesearameters continue to be normal, then based on the level ofresent knowledge, restriction from competitive athleticctivities is not recommended. Such systematic follow-up ofhis subgroup is strongly recommended, particularly if theres a family history of HCM and sudden cardiac death.

Relevant to these considerations are the prior observa-ions that abnormalities on 12-lead ECG and preload-ndependent measures of diastolic dysfunction with tissue

oppler ultrasonography may precede the appearance ofV hypertrophy, providing clues to impeding developmentf wall thickening (7–9). Athletes with abnormal 12-leadCG and absence of LV hypertrophy on two-dimensional

chocardiogram (particularly if relatives in HCM families)hould be afforded a high index of suspicion for HCM andndergo CMR imaging to determine whether areas ofegmental hypertrophy undetected by echocardiography areresent in regions of the LV chamber such as anterolateralree wall or apex (7). However, 12-lead ECG abnormalitiesn family members without LV hypertrophy (particularly ifelatively minor or nonspecific) should not per se be re-arded as evidence of HCM.

ecommendations:

. Athletes with a probable or unequivocal clinical diag-nosis of HCM should be excluded from most com-petitive sports, with the possible exception of those oflow intensity (class IA). This recommendation isindependent of age, gender, and phenotypic appear-ance, and does not differ for those athletes with orwithout symptoms, LV outflow obstruction, or priortreatment with drugs or major interventions withsurgery, alcohol septal ablation, pacemaker, or im-
plantable defibrillator. (
. Although the clinical significance and natural historyof genotype positive-phenotype negative individualsremains unresolved, no compelling data are availableat present with which to preclude these patients fromcompetitive sports, particularly in the absence ofcardiac symptoms or a family history of sudden death.

Given the effectiveness of implantable cardioverter-efibrillators (ICDs) in preventing sudden death in HCM10), clinicians will increasingly be faced with decisionsegarding athletic participation for HCM patients withCDs. Although effective for sudden death prevention inbservational studies (10), the unique physiologic milieussociated with competitive athletic activities, includingntravascular volume and electrolyte disturbances, neurohor-

onal activity, and the potential for myocardial ischemiaake the absolute reliability of ICDs in such settings

npredictable. Furthermore, there is a possibility for devicealfunction and the risk for traumatic injury to the athlete-

atient (or other competitors) should the ICD dischargeither appropriately or inappropriately. Thus, the placementf an ICD in an HCM patient does not change theompetitive sports recommendations for this disease (asreviously noted), namely, that restriction from participa-ion in contact and most non-contact sports is advisable11); such individuals may engage only in low-intensityompetitive sports (class IA).

The presence of a free-standing automated externalefibrillator (AED) at sporting events should not be con-idered either absolute protection against sudden death, arospectively designed treatment strategy for known cardio-ascular disease, nor a justification for participation inompetitive sports in athletes with previously diagnosedCM. Athletes with HCM using drugs such as anabolic

teroids or energy stimulant drinks may in fact increase theirisk of arrhythmias, although definitive data are lacking.

ITRAL VALVE PROLAPSE (MVP)

itral valve prolapse (i.e., myxomatous degeneration) is ofarticular importance in the evaluation of athletes, given itselatively high prevalence in the general population (esti-ated, 2% to 3%) (12–14). The condition is defined by

chocardiography as systolic displacement of one or bothitral leaflets into the left atrium beyond the plane of theitral anulus in the parasternal long-axis view (13). These

atients may be identified by auscultation with a mid-ystolic click and/or murmur of mitral regurgitation. Addi-ionally, MVP is characterized by a mostly favorable prog-osis and low event rate (12–15). In general, the greatestisks for unfavorable clinical sequelae—which include severerogressive mitral regurgitation requiring valve surgery,nfective endocarditis, embolic events, atrial and ventricularachyarrhythmias, and sudden death appear to be associatedith substantial structural abnormality of the mitral valve

i.e., “classic” MVP) with diffuse leaflet thickening, elonga-

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ion, and redundancy, and in some cases ruptured chordaeendineae (12–15).

Sudden cardiac death due to isolated MVP is rare amongoung patients, particularly in relation to exercise and/or inrained athletes (12–16). Such events are probably not morerequent than in the general population and occur predom-nantly in patients older than 50 years with severe mitralegurgitation and/or systolic dysfunction (15).

Some individuals with MVP appear to be part of aonnective tissue phenotypic spectrum with tall, thin habi-us, thoracic cage deformity, and joint hypermobility (i.e.,

ASS phenotype), for which there is a risk, albeit low, forrogression to aortic dilatation or sudden death (17).

ecommendations:

. Athletes with MVP— but without any of the followingfeatures— can engage in all competitive sports:a. prior syncope, judged probably to be arrhythmo-

genic in originb. sustained or repetitive and nonsustained supraven-

tricular tachycardia or frequent and/or complexventricular tachyarrhythmias on ambulatory Holtermonitoring

c. severe mitral regurgitation assessed with color-flowimaging

d. LV systolic dysfunction (ejection fraction less than50%)

e. prior embolic eventf. family history of MVP-related sudden death

. Athletes with MVP and any of the aforementioneddisease features can participate in low-intensity com-petitive sports only (class IA).

Recommendations related to hemodynamic burden sec-ndary to moderate-severe mitral regurgitation (as assessedy physical examination and two-dimensional echocardio-ram and Doppler study) in athletes with MVP appear inask Force 3.

YOCARDITIS

yocarditis is an inflammatory disease of myocardium andcause of sudden death in young athletes (2,18–24). It is

sually of infectious etiology due to a variety of viral agents,ost commonly enterovirus (e.g., Coxsackie virus), adeno-

irus, or parvovirus in young people, but also by drugs andoxic agents such as cocaine (22–25). Myocarditis evolveshrough active, healing, and healed pathologic stages—haracterized progressively by inflammatory cell infiltrateseading to interstitial edema and focal myocyte necrosis andeplacement fibrosis (20)—which potentially create an elec-rically unstable substrate for development of ventricularachyarrhythmias (2,18,24). In some instances, viral myo-arditis can culminate in dilated cardiomyopathy with LVystolic dysfunction, presumably as a consequence of viral-ediated immunologic damage to the myocardium or

ytoskeletal disruption (22–25). t

Myocarditis can be diagnosed by established histopatho-ogic, histochemical, or molecular criteria (20,23–25), but ishallenging to identify clinically. Suspicion may be raised byhest pain, exertional dyspnea, fatigue, syncope, palpita-ions, ventricular tachyarrhythmias and conduction abnor-alities or by acute congestive heart failure associated withV dilatation and/or segmental systolic dysfunction, cardio-enic shock, or ST-T changes on ECG (22,24).

When clinical judgment suggests the presence of myo-arditis, an endomyocardial biopsy may clarify an otherwisembiguous clinical profile. Because of patchy distribution ofnflammatory cells, biopsies are often insensitive and fre-uently yield false-negative histologic results (20,22,24).owever, the diagnostic yield of histology can be enhanced

y molecular analysis with PCR amplification of the viralenome (23,25).

ecommendations:

. Athletes with probable or definite evidence of myo-carditis should be withdrawn from all competitivesports and undergo a prudent convalescent period ofabout six months following the onset of clinicalmanifestations.

. Athletes may return to training and competition afterthis period of time if:a. LV function, wall motion, and cardiac dimensions

return to normal (based on echocardiographic and/orradionuclide studies at rest and with exercise)

b. clinically relevant arrhythmias such as frequentand/or complex repetitive forms of ventricular orsupraventricular ectopic activity are absent on am-bulatory Holter monitoring and graded exercisetesting

c. serum markers of inflammation and heart failurehave normalized

d. the 12-lead ECG has normalized. Persistence ofrelatively minor ECG alterations such as someST-T changes are not, per se, the basis for restric-tion from competition.

ARFAN SYNDROME

arfan syndrome (and related disorders), caused by morehan 400 individual mutations in the gene encodingbrillin-1 (FBN1), is an autosomal dominant disorder ofonnective tissue with estimated prevalence of 1:5,000 to,10,000 in the general population (26–29). It is character-zed clinically by a diverse constellation of abnormalitiesariable in severity and involving primarily the ocular,keletal, and cardiovascular organ systems (28–30). Diag-osis is made according to the Gent nosology if majorriteria are present in two organ systems and a third isnvolved, or when there is a family history of Marfanyndrome (28,29). Skeletal abnormalities include arm span-o-height ratio greater than 1.05, tall stature, arachnodac-
yly, dolichostenomelia (long, thin limbs), hyperextensibility

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nd ligamentous laxity, scoliosis, and chest wall deformitypectus excavatum or carinatum), in addition to ectopiaentis (lens dislocation).

Cardiovascular manifestations that impact on naturalistory are: 1) progressive dilatation of the aortic root orescending aorta, which predisposes to dissection and rup-ure (30–33); and 2) MVP with associated mitral regurgi-ation or LV systolic dysfunction, which may occasionallyredispose to ventricular tachyarrhythmias and suddeneath (33). The risk for aortic rupture is usually linked toarked enlargement of the aorta (transverse dimension

reater than 50 mm), although dissection can occur with aormal (or near-normal) aortic root dimension (30–36) (A.ePaepe, personal communication, April 12, 2004).eightlifting has been specifically associated with aortic

issection in athletes with cystic medial necrosis (with orithout systemic hypertension or use of anabolic steroids)

35,36). The incidence of aortic dissection appears to haveecreased with earlier prophylactic surgical aortic rooteconstruction and beta-blocker therapy (30,32,34).

Ascending aortic root dilatation (and dissection) has alsoeen associated with congenital bicuspid aortic valve dis-roportionate to that attributable to abnormal valvularunction (37–41), as well as familial aortic aneurysm andissection, independent of Marfan syndrome (41–43).

ecommendations:

. Athletes with Marfan syndrome can participate in lowand moderate static/low dynamic competitive sports(classes IA and IIA) if they do not have one or more ofthe following:a. aortic root dilatation (i.e., transverse dimension 40

mm or greater in adults, or more than 2 standarddeviations from the mean for body surface area inchildren and adolescents; z-score of 2 or more)

b. moderate-to-severe mitral regurgitationc. family history of dissection or sudden death in a

Marfan relativeIt is recommended, however, that these athletes havean echocardiographic measurement of aortic root di-mension repeated every six months, for close surveil-lance of aortic enlargement.

. Athletes with unequivocal aortic root dilatation(transverse dimension 40 mm or greater in adults orgreater than 2 standard deviations beyond the meanfor body surface area in children and adolescents;z-score of 2 or more) (41,43), prior surgical aortic rootreconstruction, chronic dissection of aorta or otherartery, moderate-to-severe mitral regurgitation, orfamily history of dissection or sudden death canparticipate only in low-intensity competitive sports(class IA).

. Athletes with Marfan syndrome, familial aortic aneu-rysm or dissection, or congenital bicuspid aortic valvewith any degree of ascending aortic enlargement (as
defined in 1 and 2 above) also should not participate h
in sports that involve the potential for bodily colli-sion.

. Recommendations related to aortic regurgitation arethe same as those in Task Force 3.

These recommendations are offered independent ofhether beta-blockers are administered to mitigate aortic

oot enlargement.

HLERS-DANLOS SYNDROME

he vascular form of Ehlers-Danlos syndrome carries aubstantial risk of rupture of the aorta and its majorranches (28). This is a rare autosomal dominant disorder,aused by a defect in type III collagen, encoded by theOL3A1 gene. Patients have variable joint hypermobility,

usceptibility to bruising, difficult wound healing, and oftenrematurely aged appearance.

ecommendation:

. Individuals with the vascular form of Ehlers-Danlossyndrome should not engage in any competitiveathletic activity.

RRHYTHMOGENIC RIGHT VENTRICULARARDIOMYOPATHY (ARVC)

ited as a major cause of sudden death in young people andthletes (44–46), particularly in the northeastern (Veneto)egion of Italy (45) but seemingly less common in the U.S2,18), ARVC is characterized by a broad phenotypicpectrum and characteristically by loss of myocytes in theight ventricular myocardium with fatty or fibro-fatty re-lacement resulting in segmental or diffuse wall thinning. Its frequently associated with myocarditis (44,45,47). Clini-al diagnosis is challenging, but relies largely on familialccurrence, ventricular tachyarrhythmias (particularly ven-ricular tachycardia of right ventricular origin elicited byxercise), T-wave inversion in precordial leads V1 through3 and epsilon waves on ECG, or right ventricular dilata-

ion and/or segmental wall motion abnormalities, aneurysmormation, and fatty deposition in the right ventricular walldentified with echocardiography, multi-slice computed to-

ography, or cardiac magnetic resonance imaging.

ecommendation:

. Athletes with probable or definite diagnosis ofARVC should be excluded from most competitivesports, with the possible exception of those of lowintensity (class IA).

THER MYOCARDIAL DISEASES

number of other uncommon diseases of the myocardiumeserve consideration as potential causes of sudden death inthletes. These include dilated cardiomyopathy (due to aariety of etiologies including genetic); primary non-
ypertrophied restrictive cardiomyopathy, systemic infiltra-

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ive diseases with secondary cardiac involvement such asarcoidosis, and also isolated non-compaction of LV myo-ardium with or without systolic dysfunction (48,49). Fewata are presently available regarding the relative risks ofthletic training and competition in athletes with theforementioned myocardial diseases.

ecommendation:

. Until more information is available in this regard, itis most prudent to exclude athletes with these dis-eases from most competitive sports, with the possibleexception of those of low intensity (class IA) inselected cases.

ERICARDITIS

ecommendation:

. Athletes with pericarditis, regardless of etiology,should not participate in competitive sports duringthe acute phase. Such athletes can return to fullactivity when there is no longer evidence of activedisease, including effusion by echocardiography, andwhen serum markers of inflammation have normal-ized. For pericarditis associated with evidence ofmyocardial involvement, eligibility recommendationsshould also be based on the course of myocarditis.Chronic pericardial disease that results in constric-tion disqualifies one from all competitive sports.

doi:10.1016/j.jacc.2005.02.011


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Name Consultant Research GrantScientific

Advisory Board Stock Holder Expert Witness Testimony

r. Michael J. Ackerman ● CV Therapeutics None ● Genaissance ● Genaissance None● Genaissance

PharmaceuticalsPharmaceuticals Pharmaceuticals

● Medtronic● Pfizer

r. Barry J. Maron None ● Medtronic None None ● 1996, Defense, Northwesternvs. Knapp

r. Rick A. Nishimura None None None None None

r. Reed E. Pyeritz ● Genzyme ● Genzyme None None None

r. Jeffrey A. Towbin None None None None ● 2005, Defense, Myocarditis,sudden death in child

● 2004, Defense, Myocarditis,sudden death in child




● 1996, Plaintiff, Myocarditis,sudden death in professionalathlete

● 1995, Plaintiff, Myocarditis,sudden death in professionalathlete

r. James E. Udelson None None None None None

TNS

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1346 Kaplan et al. JACC Vol. 45, No. 8, 2005Task Force 5: Systemic Hypertension April 19, 2005:1346–8

ask Force 5: Systemic Hypertensionorman M. Kaplan, MD, Chair
amuel S. Gidding, MD, FACC, Thomas G. Pickering, MD, DPHIL, Jackson T. Wright, JR, MD, PHD
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ystemic hypertension is the most common cardiovascularondition observed in competitive athletes. The diagnosis ofypertension is based on the presence of blood pressureBP) persistently at or above certain levels as measured byoutine sphygmomanometry on at least two separate occa-ions. A level above 140/90 mm Hg is considered to beypertensive in people over age 18 years (1). In children anddolescents, hypertension is defined as average systolic oriastolic levels greater than or equal to the 95th percentileor gender, age, and height (2). In determining the level ofompetitive athletic activity that a hypertensive person mayssume, it is also important to ascertain the degree ofypertension-related target organ damage. Although hyper-ension may be associated with an increased risk for complexentricular arrhythmias and sudden death, this disease bytself has not been incriminated as a cause of sudden cardiaceath in young competitive athletes (3). For the generalopulation, increased levels of noncompetitive physical ac-ivity are regarded not only as beneficial by reducing BP (4)nd the incidence of hypertension (5), but also protect-ng against stroke (6). In view of the striking increase inbesity-induced hypertension among children and adoles-ents related in part to physical inactivity, all people shoulde encouraged to engage in regular exercise. Those who areypertensive derive protection from all-cause and cardiovas-ular mortality by maintaining higher levels of cardiorespi-atory fitness (7).ssessment of blood pressure. Blood pressure should be

ccurately measured in all individuals who wish to partici-ate in competitive athletics before they begin training.lood pressure should be measured by routine sphygmoma-ometry, using the guidelines listed in Table 1 (8). Therere often “white-coat” elevations induced by anxiety relatedo the examination, particularly in young people concernedbout the potential consequences of the examination.herefore, additional BP recordings outside the office

hould be obtained in those with elevated office readings,ither with readily available and inexpensive home self-ecorders or with less accessible and more expensive auto-atic ambulatory monitors.valuation. Those individuals with any degree of persis-

ent hypertension should have a thorough history andhysical examination and limited laboratory testing tovaluate secondary causes and to ascertain target organamage (1). If agents that may raise BP such as non-teroidal anti-inflammatory drugs (NSAIDs) are beingaken, additional measurements should be obtained after
hey have been discontinued. Laboratory testing for most a
ubjects with stage 1 hypertension (140 to 159 mm Hg/0 to 99 mm Hg) should include an automated blood chem-stry (glucose, creatinine, or the corresponding estimated glo-

erular filtration rate, electrolytes, lipid profile), hematocrit,rine analysis, and an electrocardiogram. If hypertension istage 2 (greater than or equal to 160/100 mm Hg), if results ofhe initial laboratory tests are abnormal, or if features suggestivef identifiable (secondary) causes are noted by history orhysical examination, the patient should be referred for addi-ional study (including echocardiography) and therapy.ffects of exercise on BP. As noted during stress testing,

ystolic BP rises during aerobic (dynamic) exercise. Bothystolic and diastolic pressures rise even more acutely and toigher levels during resistance (static or isometric) exercise.ecause strenuous aerobic or resistance exertion may pre-ipitate myocardial infarction and sudden death in suscep-ible, untrained individuals (9), those individuals who wisho engage in competitive athletics should increase exerciseevels gradually to avoid such cardiac catastrophes.

However, repetitive performance of both aerobic and resis-ance exercise lowers systolic and diastolic BP (4,10). Afterach 30-min period of aerobic exercise at 50% of maximalxygen uptake, the blood pressure remains lower for up to 24 h,ith an even greater reduction after 30 min of aerobic exercise

t 75% of maximal oxygen uptake (11). As a consequence, theisks of developing elevated BP (5) and of incurring a cardio-ascular consequence of hypertension (6,7) are less in thoseho maintain higher levels of physical activity. Some condi-

ioned athletes (particularly young men), with a slow heart ratend compensatory increase in stroke volume, have high systolicP that is considered “spurious” hypertension (12) but shouldevertheless be carefully monitored.In normotensive untrained subjects, an excessive rise in

ystolic BP to above 200 mm Hg during an exercise stressest is predictive of a greater likelihood of the developmentf persistent hypertension in the future and may be associ-ted with subtle systolic dysfunction (13) and an increasedisk of subsequent cardiovascular disease (14). Therefore,uch subjects should be advised to increase levels of physicalctivity gradually to moderate such excessive rises in pres-ure. Such rises should not restrict activity in those who areell conditioned. Because intensive resistive training may

educe arterial compliance with potential adverse conse-uences (15), such training should be limited in thosethletes with hypertension.ffects of blood pressure on exercise. Untreated hyper-

ension in athletes may be accompanied by some limitationn exercise performance (16). Before initiating drug therapy,
thletes should be strongly encouraged to adopt healthy

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ifestyle behavior and avoid tobacco in any form, excesslcohol, drugs of abuse (especially sympathomimetics suchs cocaine or ephedra), androgens, steroids, growth hor-one, NSAIDs, and excessive sodium intake. It should be

mphasized that the use of antihypertensive drugs mayurther limit exercise capacity, more so with beta-adrenergiceceptor blocking agents than with vasodilators (alpha-drenergic blocking agents, angiotensin-converting enzymenhibitors, angiotensin II-receptor blockers, or calciumhannel blockers). Indeed, high-intensity competitive ath-etes may find it very difficult to perform satisfactorily whilesing beta-blockers (17).

ecommendations:. Before individuals commence training for competi-

tive athletics, they should undergo careful assessmentof BP and those with initially high levels (above140/90 mm Hg) should have out-of-office measure-

able 1. Guidelines for Blood Pressure Measurement

ostureBlood pressure obtained in the seated position is recommended. The

subject should sit quietly for 5 min, with the back supported in achair, with feet on the floor, and the arm supported at the levelof the heart, before recording blood pressure.

ircumstancesNo caffeine during the hour preceding the reading.No smoking during the 30 min preceding the reading.A quiet, warm setting.

quipmentCuff size

The bladder should encircle and cover at least 80% of the length ofthe arm; if it does not, use a larger cuff. If bladder is too short,misleadingly high readings may result.

ManometerUse a mercury, recently calibrated aneroid, or validated electronic

device.echniqueNumber of readings

On each occasion, take at least two readings, separated by as muchtime as is practical. If readings vary by greater than 5 mm Hg,take additional readings until two consecutive readings are close.If the arm pressure is elevated, take the measurement in one leg(particularly in patients less than 30 years old).

Initially, take pressures in both arms; if the pressures differ, use thearm with the higher pressure.

If the initial values are elevated, obtain two other sets of readings atleast 1 week apart.

PerformanceInflate the bladder quickly to a pressure 20 mm Hg above the

systolic pressure, as recognized by the disappearance of the radialpulse.

Deflate the bladder 2 mm Hg per second.Record the Korotkoff phase I (appearance) and phase V

(disappearance).If the Korotkoff sounds are weak, have the patient raise the arm,

open and close the hand 5 to 10 times and then reinflate thebladder quickly.

ecordingsBlood pressure, patient position, and arm and cuff size.

rom Pickering TG, et al. Hypertension 2005;45:142–61, reprinted with permissionf the American Heart Association.

ments to exclude isolated office “white-coat” hyper-

tension. Those with pre-hypertension (120/80 mmHg up to 139/89 mm Hg) should be encouraged tomodify lifestyle but should not be restricted fromphysical activity. Those with sustained hypertensionshould have echocardiography. Left ventricular hy-pertrophy (LVH) beyond that seen with “athletes’heart” should limit participation until BP is normal-ized by appropriate drug therapy.

. The presence of stage 1 hypertension in the absence oftarget organ damage including LVH or concomitantheart disease should not limit the eligibility for anycompetitive sport. Once having begun a training pro-gram, the hypertensive athlete should have BP remea-sured every two to four months (or more frequently, ifindicated) to monitor the impact of exercise.

. Athletes with more severe hypertension (stage 2),even without evidence of target organ damage such asLVH, should be restricted, particularly from highstatic sports (classes IIIA to IIIC), until their hyper-tension is controlled by either lifestyle modificationor drug therapy.

. All drugs being taken must be registered with appro-priate governing bodies to obtain a therapeutic ex-emption.

. When hypertension coexists with another cardiovas-cular disease, eligibility for participation in compet-itive athletics is usually based on the type and severityof the associated condition.

doi:10.1016/j.jacc.2005.02.012


1. Chobanian AV, Bakris GL, Black HR, et al. The seventh report of theJoint National Committee on Prevention, Detection, Evaluation, andTreatment of High Blood Pressure: the JNC 7 report. JAMA2003;289:2560–72.

2. National High Blood Pressure Education Program Working Groupon High Blood Pressure in Children and Adolescents. The fourthreport on the diagnosis, evaluation, and treatment of high bloodpressure in children and adolescents. Pediatrics 2004;114 Suppl:555–76.


4. Whelton SP, Chin A, Xin X, He J. Effect of aerobic exercise on bloodpressure: a meta-analysis of randomized, controlled trials. Ann InternMed 2002;136:493–503.

5. Hu G, Barengo NC, Tuomilehto J, Lakka TA, Nissinen A, JousilahtiP. Relationship of physical activity and body mass index to the risk ofhypertension: a prospective study in Finland. Hypertension 2004;43:25–30.

6. Lee CD, Folsom AR, Blair SN. Physical activity and stroke risk: ameta-analysis. Stroke 2003;34:2475–81.

7. Church TS, Kampert JB, Gibbons LW, Barlow CE, Blair SN.Usefulness of cardiorespiratory fitness as a predictor of all-cause andcardiovascular disease mortality in men with systemic hypertension.Am J Cardiol 2001;88:651–6.

8. Pickering TG, Hall JE, Appel L J, et al. Recommendations for bloodpressure measurement in humans and animals: part 1: blood pressuremeasurement in humans: an AHA scientific statement from theCouncil on High Blood Pressure Research, Professional, and Publi-cation Subcommittee. Hypertension 2005;45:142–61.

9. Albert CM, Mittleman MA, Chae CU, Lee IM, Hennekens CH,
Manson JE. Triggering of sudden death from cardiac causes byvigorous exertion. N Engl J Med 2000;343:1355–61.

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1348 Thompson et al. JACC Vol. 45, No. 8, 2005Task Force 6: Coronary Artery Disease April 19, 2005:1348–53

0. Kelley GA, Kelley KS. Progressive resistance exercise and restingblood pressure: a meta-analysis of randomized controlled trials. Hy-pertension 2000;35:838–43.

1. Quinn TJ. Twenty-four hour, ambulatory blood pressure responsesfollowing acute exercise: impact of exercise intensity. J Hum Hypertens2000;14:547–53.

2. Mahmud A, Feely J. Spurious systolic hypertension of youth: fit youngmen with elastic arteries. Am J Hypertens 2003;16:229–32.

3. Mottram PM, Haluska B, Yuda S, Leano R, Marwick TH. Patientswith a hypertensive response to exercise have impaired systolic functionwithout diastolic dysfunction or left ventricular hypertrophy. J Am

enjamin D. Levine, MD, FACC, Robert J. Myerburg, M

xercise-related cardiac events in adults (8), variously defined as

o(tttiTetdTa

4. Kjeldsen SE, Mundal R, Sandvik L, Erikssen G, Thaulow E, ErikssenJ. Supine and exercise systolic blood pressure predict cardiovasculardeath in middle-aged men. J Hypertens 2001;19:1343–8.

5. Miyachi M, Kawano H, Sugawara J, et al. Unfavorable effects ofresistance training on central arterial compliance: a randomized inter-vention study. Circulation 2004;110:2858–63.

6. Missault L, Duprez D, de Buyzere M, de Backer G, Clement D.Decreased exercise capacity in mild essential hypertension: non-invasiveindicators of limiting factors. J Hum Hypertens 1992;6:151–5.

7. Vanhees L, Defoor JG, Schepers D, et al. Effect of bisoprolol andatenolol on endurance exercise capacity in healthy men. J Hypertens

Coll Cardiol 2004;43:848–53. 2000;18:35–43.


Name Research Grant Scientific Advisory Board Speakers’ Bureau Steering Committee

r. Samuel S. Gidding ● Astra Zeneca None None None

r. Norman M. Kaplan None None ● AstraZeneca None● Bayer● Norvartis Pharma AG● Pfizer● Servier

r. Thomas G. Pickering None None ● Boehringer Ingelheim None

r. Jackson T. Wright, Jr. ● Beechham ● Novartis Pharma AG ● Astra Zeneca ● Beechham● GlaxoSmithKline ● Pfizer ● Aventis Pharmaceuticals ● GlaxoSmithKline● Novartis Pharma AG ● Bayer ● Phoenix Pharmaceuticals● Phoenix Pharmaceuticals ● BMS ● Solvay/Unimed● Solvay/Unimed ● Beechham

● Eli Lilly & Co.● GlaxoSmithKline● Merck & Co.● Novartis Pharma AG● Pfizer● Phoenix Pharmaceuticals● Proctor & Gamble● Reliant Pharmaceutical● Solvay/Unimed

ask Force 6: Coronary Artery Diseaseaul D. Thompson, MD, FACC, Chairary J. Balady, MD, FACC, Bernard R. Chaitman, MD, FACC, Luther T. Clark, MD, FACC,

D, FACC

THEROSCLEROTIC CORONARY ARTERY DISEASEeneral considerations. Compelling evidence indicates

hat physical activity reduces cardiovascular events inealthy subjects and cardiac mortality in patients withiagnosed coronary artery disease (CAD) (1). Despite theseeneficial exercise effects, vigorous physical activity alsoransiently increases the risk of both acute myocardialnfarction (AMI) (2–4) and sudden cardiac death (SCD)5–7) with the greatest exercise risk among the mostabitually sedentary individuals (2,4,7).Atherosclerotic CAD is the most frequent cause of these

lder than 30, 35, or 40 years of age. Both plaque rupture9,10) and possibly plaque erosion (11) have been implicated ashe immediate cause of exercise-related events in adults, al-hough plaque rupture is more frequent. Several studies overhe last decade document that cardiac events frequently occurn coronary arteries that were not previously critically narrowed.his appears to be particularly true for exercise-related cardiac

vents because angiographic studies of exercise-related AMI inhe general population (4) and in sport participants (10)emonstrate less extensive CAD than in comparison subjects.his observation may reflect either selection bias for less severe

therosclerosis in those capable of exercising at high intensity

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r the ability of exercise to provoke events in individuals withess severe disease.

Prognosis for diagnosed CAD patients worsens with thextent of disease, left ventricular (LV) systolic dysfunction,nducible ischemia, and electrical instability. Both the recog-ition that acute cardiac events often occur at the site ofreviously mild coronary stenoses in the general populationnd the observation that victims of exercise-related CADvents have less extensive disease than do individuals sufferingon-exercise related events reduce the utility of standard CADisk assessment in evaluating older competitive athletes. Ado-escent and young adult athletes also may have variants in theiresting electrocardiograms (ECGs) that make the interpreta-ion of their resting and exercise ECGs difficult. In addition,arly CAD is increasingly identified by such imaging tech-iques as coronary artery calcification scoring, further compli-ating risk assessment because the presence of any coronaryrtery calcification indicates atherosclerotic disease. Finally,ince CAD primarily occurs in adult athletes, the age of thethlete at risk for CAD events complicates the decisionrocess. Older athletes are often engaged in individual com-etitive pursuits and may not require formal clearance forontinued competition. Preparticipation screening guidelinespecifically for participants in master’s sports have been devel-ped in association with the American Heart AssociationAHA) (12).

iagnosis. The diagnosis of atherosclerotic CAD is estab-ished in the presence of any of the following: 1) a history of

myocardial infarction (MI) confirmed by conventionaliagnostic criteria; 2) a history suggestive of angina pectorisith objective evidence of inducible ischemia; and 3) coro-ary atherosclerosis of any degree is demonstrated byoronary imaging studies such as catheter-based coronaryngiography, magnetic resonance angiography, or electroneam computerized tomography (EBCT).

ORONARY CALCIFICATION BY COMPUTED TOMOGRAPHY

CT). Since the last version of these guidelines, the wide-pread dissemination of noninvasive techniques such asBCT, or even more recently, multi-slice gated CT, hasarkedly increased the number of individuals, including

ompetitive athletes, who may be diagnosed with athero-clerotic CAD. Although exceedingly rare in young persons6% of men and 3% of women 20 to 29 years of age), theresence of coronary calcium increases substantially withge, such that for master’s athletes, age 40 to 49 years,pproximately 41% of men and 13% of women may haveeasurable coronary calcium (13). Among individuals age

0 to 59 years, 68% of men and 27% of women haveocumented coronary calcium (13). There is compellingvidence that the presence of any coronary calcium indicatesnderlying atherosclerosis (14) and that increasing coronaryalcium scores are associated with increased CAD risk (15).he coronary calcium score that warrants additional evalu-

tion in asymptomatic competitive athletes is unknown,
lthough scores of more than 100 (15) have been associated
ith increased risk for coronary events (16) in the generalopulation compared to patients with no coronary calcium.t is unknown whether the risk of coronary events duringntense exercise is increased in the presence of this or lessermounts of coronary calcium. Nevertheless, for the purposef the present document, athletes with coronary arteryalcification scores more than 100 should undergo the samevaluation as those with more clinically evident CAD.

ISK ASSESSMENT. A paucity of data exists in competitivethletes directly relating the presence and severity of CADo the risk of athletic participation. This requires that theseecommendations for athletes with CAD be based in partn observations obtained from non-athletes with CAD.evertheless, it is likely that risk is increased to some degreehenever coronary atherosclerosis is present. It is also likely

hat the risk of exercise-related events increases with thextent of disease, LV dysfunction, inducible ischemia, andlectrical instability, and that the risk increases with thentensity of the competitive sport and the intensity of thearticipant’s effort.

valuation.. Athletes with CAD diagnosed by any method including

coronary artery classification scoring more than 100,coronary angiography, evidence of inducible ischemia,or prior coronary event, and who are undergoing eval-uation for competitive athletics, should have their LVfunction assessed.

. These athletes should undergo maximal treadmill (orbicycle) exercise testing to assess their exercise capacityand the presence or absence of provocable myocardialischemia. Exercise testing should approximate as closelyas possible the cardiovascular and metabolic demands ofthe planned competitive event and its training regimen.Despite such simulation, graded exercise testing cannotreplicate the cardiovascular stress produced by the sud-den bursts of activity, the combination of high dynamicand static exercise, such as rowing, or the sustainedbouts of exercise required by athletic training andcompetition. Therefore, standard clinical exercise testsmay not be appropriate for the evaluation of athleteswith coronary heart disease.

ISK STRATIFICATION. Two levels of risk can be defined onhe basis of testing.

Mildly increased risk. Athletes with CAD diagnosed byny method are judged to be at mildly increased risk if theyemonstrate all of the following:

. Preserved LV systolic function at rest (i.e., ejectionfraction greater than 50%).

. Normal exercise tolerance for age, demonstrated duringtreadmill or cycle ergometer exercise testing: greaterthan 10 metabolic equivalents (METS), or greater than35 O2/kg-min if less than 50 years old; greater than 9METS, or greater than 31 ml O2/kg-min for 50 to 59
years old; greater than 8 METS, or greater than 28 ml

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O2/kg-min, if 60 to 69 years old; and greater than 7METS, or greater than 24 ml O2/kg-min, if greaterthan or equal to 70 years old. It should be noted thatyoung, highly competitive endurance athletes shouldhave maximal oxygen uptakes far in excess of rangesregarded as normal, which in fact may represent sub-stantial functional impairment in this population.

. Absence of exercise-induced ischemia and exercise-induced or post-exercise complex ventricular arrhyth-mias, including frequent premature ventricular contrac-tions (greater than 10% of beats/min), couplets, orventricular tachycardia.

. Absence of hemodynamically significant stenosis (generallyregarded as 50% or more luminal diameter narrowing) inany major coronary artery by coronary angiography.

. Successful myocardial revascularization by surgical orpercutaneous techniques if such revascularization wasperformed.

ubstantially increased risk. Athletes with CAD identified byoninvasive or invasive testing are judged to be at substan-ially increased risk if they demonstrate any of the following:

. Impaired LV systolic function at rest (i.e., ejectionfraction less than 50%).

. Evidence of exercise-induced myocardial ischemia orcomplex ventricular arrhythmias.

. Hemodynamically significant stenosis of a major coro-nary artery (generally regarded as 50% or more lumendiameter narrowing) if coronary angiography wasperformed.

The American College of Cardiology/AHA guidelinesn exercise testing note that it is not necessary to stopeta-blockers before routine exercise testing, although thisractice may reduce the diagnostic and prognostic value ofhe test (17). The decision whether or not to stop beta-locker therapy before exercise testing of athletes should beade on an individual basis. Stopping beta-blockers and

ther anti-ischemic mediations before testing may be usefulo more closely approximate the probable risk if the athleteither intentionally or unintentionally does not take theseedications before competition, or when certain athletic

egulatory bodies prohibit beta-blockers. If anti-ischemicedications are stopped, this should be done carefully to

void a potential hemodynamic rebound effect, which couldead to accelerated angina or hypertension.

Coronary arteriography is not required to determineligibility for competition in patients with known CAD,nd no evidence of inducible ischemia, but is recommendedn athletes with exercise-induced ischemia who choose toarticipate in sports against medical advice. Such studiesay identify coronary lesions that may be better managed by

ercutaneous or surgical myocardial revascularization pro-edures to relieve exercise-induced ischemia and potentiallyo reduce exercise-related risk.

The panel wishes to emphasize that the following rec-

mmendations are prepared as a guidelines for permittingarticipation in competitive sports. Restrictions in the follow-ng recommendations, therefore, should not be misinter-reted as an injunction against regular physical activity aspposed to athletic competition. Indeed, regular and recre-tional physical activity and moderate-intensity exerciseraining are recommended for patients with CAD for itseneral cardiovascular benefits (1).

ecommendations:

. Athletes in the mildly increased risk group canparticipate in low dynamic and low/moderate staticcompetitive sports (classes IA and IIA—see Fig. 1 inTask Force 8: Classification of Sports) but shouldavoid intensely competitive situations. We recognizethat selected athletes with mildly increased risk maybe permitted to compete in sports of higher levels ofintensity when their overall clinical profile suggestsvery low exercise risk. This is particularly true forathletes in whom the only indication that coronaryatherosclerosis is present is from an EBCT per-formed for screening purposes, and in which the totalcoronary calcium score is relatively low (i.e., less than15). Increasing amounts of coronary calcium, sugges-tive of increasing burden disease, should dictate amore cautious approach, particularly if the coronarycalcium score is more than 100. All athletes shouldunderstand that the risk of a cardiac event withexertion is probably increased once coronary athero-sclerosis of any severity is present. Athletes withmildly increased risk engaging in competitive sportsshould undergo re-evaluation of their risk stratifica-tion at least annually.

. Athletes in the substantially increased risk categoryshould generally be restricted to low-intensity com-petitive sports (class IA).

. Athletes should be informed of the nature of prodro-mal symptoms (such as chest, arm, jaw and shoulderdiscomfort, unusual dyspnea) and should be in-structed to cease their sports activity promptly and tocontact their physician if symptoms appear. Physi-cians should be aware that competitive athletes mayminimize symptoms that occur during exertion.

. Those with a recent MI or myocardial revasculariza-tion should cease their athletic training and compe-tition until recovery is deemed complete. This inter-val varies among patient groups, but depends on theseverity of the cardiovascular event and the extentand success of the revascularization procedure. Suchpatients may benefit from cardiac rehabilitation dur-ing the recovery period. No firm guidelines exist forhow long patients should avoid vigorous exercisetraining, but in general, patients post-stent place-ment for stable CAD symptoms should avoid vigor-ous exercise training for competition for approxi-
mately four weeks. Patients undergoing stent

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placement for unstable disease should wait at leastthis long. Following coronary bypass surgery, pa-tients should avoid vigorous training until theirincisions can tolerate vigorous activity. After recu-peration period, the risk and activity level should bedefined as in recommendations 1 and 2.

. All athletes with atherosclerotic CAD should havetheir atherosclerotic risk factors aggressively treatedas studies suggest that comprehensive risk reductionis likely to stabilize coronary lesions and may reducethe risk of exercise-related events.

It must be emphasized that even athletes identified aseing at mildly increased risk and permitted to participate inow dynamic and low/moderate static competitive sportsclasses IA and IIA) cannot be assured that such participa-ion will not increase the risk of cardiac events because it isrobable that any exercise transiently poses some increasedxercise risk once CAD is established.

ORONARY ARTERY VASOSPASM

oronary artery vasospasm classically presents as rest anginassociated with ST-segment elevation, but can be provokedy physical exertion on rare occasions (18). Vasospasm is anncommon cause of chest pain that is evident in 2% to 3%f patients presenting with chest pain undergoing coronaryngiography (19). Vasospasm is most frequently observed atoronary sites damaged by atherosclerosis (20), but a sub-tantial cohort may have angiographically normal coronaryrteries or minimal angiographic luminal narrowing (18,19).

vasospastic contribution to ischemia should be suspectedhen there is marked variation in the exercise threshold for

ngina (18), and when there is evidence of myocardialschemia with little or no coronary luminal narrowing.resently, no widely accepted noninvasive test exists forliciting and quantifying vasospastic angina in the setting ofonobstructive or mildly obstructive coronary arteries. Theccurrence of ST-segment elevation during exercise testingppears to correlate with the degree of disease activity (i.e.,hose with more frequent episodes of angina will more likelyave a positive test) (21). Provocative testing withrgonovine-related substances during coronary arteriogra-hy is rarely used (22), but remains the only test recom-ended in current practice guidelines (23). However, forced

yperventilation testing, particularly when combined withuclear perfusion imaging, may be a useful noninvasive testot requiring the administration of ergonovine (24). Theisk associated with participation in sports for athletes withoronary artery spasm is not known, but we recommend aautious approach to patients with documented coronaryasospasm until the risk of physical exertion for theseatients is better defined.

ecommendations:

. Athletes with CAD as previously defined and clinically
important coronary artery vasospasm should follow the
evaluation and risk stratification approach delineatedfor athletes with coronary atherosclerosis.

. Athletes with coronary vasospasm documented at restor with exercise and angiographically normal coronaryarteries or without evidence of arterial plaquing shouldbe restricted to low-intensity competitive sports (classIA). This restriction should be re-evaluated at leastannually because some patients with coronary vaso-spasm may experience spontaneous remission.

AD IN CARDIAC TRANSPLANT RECIPIENTS

rthotopic transplanted hearts develop an accelerated formf coronary vasculopathy, usually detected by serial coronaryngiography or intravascular ultrasound studies, that is aeading cause of death after the first post-transplant year25,26). The coronary disease is different from that seen inon-transplanted hearts with coronary atherosclerosis; theisease is diffuse and characterized by pronounced intimalhickening and involvement of the entire coronary tree.iscrete stenoses of epicardial arteries can coexist in some

nstances. Cardiac allografts are denervated, and althoughome recipients may develop a degree of sympathetic rein-ervation, acute coronary syndromes may present withtypical symptoms as opposed to angina (27). Noninvasiveesting for CAD is less sensitive in the transplant recipient;any patients do not achieve VO2max, and cardiac denerva-

ion can limit peak heart rate response and symptoms.rovocative myocardial perfusion imaging can fail to detect

schemia (25,27) although dobutamine echocardiographyas been shown to predict subsequent ischemic cardiacvents (26,28–31) after the first three to five years post-ransplant. In many cardiac transplant centers, a normaltress echocardiogram justifies postponement of annualoronary angiography (26,28–31). Coronary angiographyan also underestimate disease severity because of the diffuseature of the CAD process; intravascular ultrasound studies

ncrease the sensitivity (26).

valuation.. Cardiac transplant recipients participating in competi-

tive athletics should undergo yearly maximal exercisetesting with echocardiography using a protocol designedto simulate the cardiac and metabolic demands of thecompetitive event and its training.

. Additional evaluation, including such procedures ascoronary angiography and intravascular ultrasonography(IVUS) should be performed as directed by the trans-plant center and the transplant cardiologist. Coronaryangiography/IVUS should also be performed if theannual exercise test is abnormal and to evaluate unex-plained symptoms such as dyspnea or exertional fatigueas these may be the only symptoms of progressive
vascular disease.

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ecommendations:

. Because of the special issues involved with transplantpatient management, decisions as to the feasibility ofathletic competition for cardiac transplant recipientsshould be made in conjunction with the patient’stransplant cardiologist.

. Athletes with no coronary luminal narrowing, noexercise-induced ischemia, and with normal exercisetolerance for age (as previously defined ) can gener-ally participate in all competitive sports as appropri-ate for their exercise capacity.

. Athletes with coronary luminal narrowing should berisk stratified as outlined in the section entitledEvaluation with activity recommendations as indi-cated in the section entitled Atherosclerotic Coro-nary Artery Disease.

YOCARDIAL BRIDGING

yocardial bridging is a condition in which a segment ofajor epicardial coronary artery (most commonly the left

nterior descending) is tunneled within and completelyurrounded by LV myocardium. Myocardial bridging isound in approximately 30% of hearts examined at necropsy32), but is visualized during angiography in less than 5% ofatients probably because the thin bridges identified atecropsy cause little anatomic compression during systole32). Consequently, most tunneled epicardial coronary ar-eries appear to be of little clinical significance, but this

alformation has occasionally been associated withxercise-related sudden death (33,34) and exercise-inducedngina pectoris (35). Clinically significant myocardialridges have a long deeply-tunneled segment and aressociated with regional ischemia. Treatment options in-lude medical management with beta-adrenergic or calciumhannel-blocking agents, coronary stenting, and surgicalesection of the myocardial bridge.

However, coronary stenting has been associated withestenosis and periprocedural complications in 50% of cases32). Surgical resection in selected symptomatic patients haseen shown to reduce angina (35) and improve myocardiallood flow (36). Task Force 4: HCM and Other Cardio-yopathies, Mitral Valve Prolapse, Myocarditis, andarfan Syndrome provides recommendations for the man-

gement of myocardial bridging in patients with hypertro-hic cardiomyopathy (HCM).

ecommendations:

. Athletes with myocardial bridging of an epicardialcoronary artery and no evidence of myocardial isch-emia at rest or during exercise can participate in allcompetitive sports as appropriate for their exercisecapacity.

. Athletes with myocardial bridging of an epicardial
coronary artery and objective evidence of myocardial
ischemia or prior MI should be restricted to low-intensity competitive sports (class IA).

. Athletes with surgical resection of the myocardialbridge or stenting should be restricted to low-intensity sports for at least six months after theprocedure. Athletes who remain asymptomatic afterthe procedure should undergo exercise testing. Ifexercise tolerance is normal for age and gender, andthere is no evidence of exercise-induced ischemia, theathlete may participate in all competitive sports.

Finally, recommendations for other congenital coronaryrtery anomalies, including those originating from therong coronary sinus, are provided in Task Force 1:reparticipation Screening and Diagnosis of Cardiovascularisease in Athletes.

doi:10.1016/j.jacc.2005.02.013

ASK FORCE 6 REFERENCES1. Thompson PD, Buchner D, Pina IL, et al. Exercise and physical

activity in the prevention and treatment of atherosclerotic cardiovas-cular disease: a statement from the Council on Clinical Cardiology(Subcommittee on Exercise, Rehabilitation, and Prevention) and theCouncil on Nutrition, Physical Activity, and Metabolism (Subcom-mittee on Physical Activity). Circulation 2003;107:3109–16.

2. Mittleman MA, Maclure M, Tofler GH, Sherwood JB, Goldberg RJ,Muller JE. Triggering of acute myocardial infarction by heavy physicalexertion: protection against triggering by regular exertion. Determi-nants of Myocardial Infarction Onset Study Investigators. N EnglJ Med 1993;329:1677–83.

3. Willich SN, Lewis M, Lowel H, Arntz HR, Schubert F, Schroder R.Physical exertion as a trigger of acute myocardial infarction. Triggersand Mechanisms of Myocardial Infarction Study Group. N EnglJ Med 1993;329:1684–90.

4. Giri S, Thompson PD, Kiernan FJ, et al. Clinical and angiographiccharacteristics of exertion-related acute myocardial infarction. JAMA1999;282:1731–6.

5. Thompson PD, Funk EJ, Carleton RA, Sturner WQ. Incidence ofdeath during jogging in Rhode Island from 1975 through 1980.JAMA 1982;247:2535–8.

6. Siscovick DS, Weiss NS, Fletcher RH, Lasky T. The incidence ofprimary cardiac arrest during vigorous exercise. N Engl J Med1984;311:874–7.

7. Albert CM, Mittleman MA, Chae CU, Lee IM, Hennekens CH,Manson JE. Triggering of sudden death from cardiac causes byvigorous exertion. N Engl J Med 2000;343:1355–61.

8. Ragosta M, Crabtree J, Sturner WQ, Thompson PD. Death duringrecreational exercise in the state of Rhode Island. Med Sci SportsExerc 1984;16:339–42.

9. Black A, Black MM, Gensini G. Exertion and acute coronary arteryinjury. Angiology 1975;26:759–83.

0. Ciampricotti R, Deckers JW, Taverne R, el Gamal M, Relik-van WelyL, Pool J. Characteristics of conditioned and sedentary men with acutecoronary syndromes. Am J Cardiol 1994;73:219–22.

1. Burke AP, Farb A, Malcom GT, Liang Y, Smialek JE, Virmani R.Plaque rupture and sudden death related to exertion in men withcoronary artery disease. JAMA 1999;281:921–6.

2. Maron BJ, Araujo CG, Thompson PD, et al. Recommendations forpreparticipation screening and the assessment of cardiovascular diseasein masters athletes: an advisory for healthcare professionals from theworking groups of the World Heart Federation, the InternationalFederation of Sports Medicine, and the American Heart AssociationCommittee on Exercise, Cardiac Rehabilitation, and Prevention.Circulation 2001;103:327–34.

3. Cheng YJ, Church TS, Kimball TE, et al. Comparison of coronaryartery calcium detected by electron beam tomography in patients with
to those without symptomatic coronary heart disease. Am J Cardiol2003;92:498–503.

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4. Wexler L, Brundage B, Crouse J, et al. Coronary artery calcification:pathophysiology, epidemiology, imaging methods, and clinical impli-cations. A statement for health professionals from the American HeartAssociation Writing Group. Circulation 1996;94:1175–92.

5. O’Rourke RA, Brundage BH, Froelicher VF, et al. American Collegeof Cardiology/American Heart Association expert consensus docu-ment on electron-beam computed tomography for the diagnosis andprognosis of coronary artery disease. Circulation 2000;102:126–40.

6. Greenland P, LaBree L, Azen SP, Doherty TM, Detrano RC.Coronary artery calcium score combined with Framingham score forrisk prediction in asymptomatic individuals. JAMA 2004;291:210–5.

7. Gibbons RJ, Balady GJ, Bricker JT, et al. ACC/AHA 2002 guideline updatefor exercise testing: a report of the American College of Cardiology/AmericanHeart Association Task Force on Practice Guidelines (Committee onExercise Testing). Available at: www.acc.org/clinical/guidelines/exercise/dirIndex.htm. Accessed October 1, 2004.

8. Yasue H, Omote S, Takizawa A, Nagao M, Miwa K, Tanaka S.Circadian variation of exercise capacity in patients with Prinzmetal’svariant angina: role of exercise-induced coronary arterial spasm.Circulation 1979;59:938–48.

9. Mark DB, Califf RM, Morris KG, et al. Clinical characteristics andlong-term survival of patients with variant angina. Circulation 1984;69:880–8.

0. Gordon JB, Ganz P, Nabel EG, et al. Atherosclerosis influences thevasomotor response of epicardial coronary arteries to exercise. J ClinInvest 1989;83:1946–52.

1. Waters DD, Szlachcic J, Bourassa MG, Scholl JM, Theroux P.Exercise testing in patients with variant angina: results, correlationwith clinical and angiographic features and prognostic significance.Circulation 1982;65:265–74.

2. Hamilton KK, Pepine CJ. A renaissance of provocative testing forcoronary spasm? J Am Coll Cardiol 2000;35:1857–9.

3. Gibbons RJ, Abrams J, Chatterjee K, et al. ACC/AHA 2002 guidelineupdate for the management of patients with chronic stable angina—summary article: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines(Committee on the Management of Patients With Chronic StableAngina). Circulation 2003;107:149–58.

4. Shanoudy H, Raggi P, Gasperetti C, et al. Detection of coronaryvasospasm by posthyperventilation technetium-99m sestamibi single-

5. Miller LW, Schlant RC, Kobashigawa J, Kubo S, Renlund DG. 24thBethesda Conference: cardiac transplantation. Task force 5: compli-cations. J Am Coll Cardiol 1993;22:41–54.

6. Uretsky BF, Kormos RL, Zerbe TR et al. Cardiac events after hearttransplantation: incidence and predictive value of coronary arteriogra-phy. J Heart Lung Transplant 1992;11:S45–51.

7. Schroeder JS, Gao SZ, Hunt SA, Stinson EB. Accelerated graftcoronary artery disease: diagnosis and prevention. J Heart LungTransplant 1992;11:S258–65.

8. Spes CH, Klauss V, Mudra H, et al. Diagnostic and prognostic valueof serial dobutamine stress echocardiography for noninvasive assess-ment of cardiac allograft vasculopathy: a comparison with coronaryangiography and intravascular ultrasound. Circulation 1999;100:509–15.

9. Spes CH, Klauss V, Rieber J, et al. Functional and morphologicalfindings in heart transplant recipients with a normal coronary angio-gram: an analysis by dobutamine stress echocardiography, intracoro-nary Doppler and intravascular ultrasound. J Heart Lung Transplant1999;18:391–8.

0. Larsen RL, Applegate PM, Dyar DA, et al. Dobutamine stressechocardiography for assessing coronary artery disease after transplan-tation in children. J Am Coll Cardiol 1998;32:515–20.

1. Akosah KO, McDaniel S, Hanrahan JS, Mohanty PK. Dobutaminestress echocardiography early after heart transplantation predicts de-velopment of allograft coronary artery disease and outcome. J Am CollCardiol 1998;31:1607–14.

2. Mohlenkamp S, Hort W, Ge J, Erbel R. Update on myocardialbridging. Circulation 2002;106:2616–22.

3. Morales AR, Romanelli R, Tate LG, Boucek RJ, de Marchena E.Intramural left anterior descending coronary artery: significance of thedepth of the muscular tunnel. Hum Pathol 1993;24:693–701.


5. Betriu A, Tubau J, Sanz G, Magrina J, Navarro-Lopez F. Relief ofangina by periarterial muscle resection of myocardial bridges. AmHeart J 1980;100:223–6.

6. Hill RC, Chitwood WR Jr., Bashore TM, Sink JD, Cox JL, Wechsler
photon emission computed tomography imaging in patients withcoronary artery disease. Am J Cardiol 1998;81:573–7.
AS. Coronary flow and regional function before and after supraarterialmyotomy for myocardial bridging. Ann Thorac Surg 1981;31:176–81.


Name ConsultantResearch

GrantScientific

Advisory BoardSpeakers’

BureauStock

HolderExpert Witness

Testimony

r. Gary J. Balady None None None None None None

r. Bernard R. Chaitman ● CV Therapeutics ● Aventis ● Aventis ● Pfizer None None● Pfizer ● CV Therapeutics

r. Luther T. Clark None None None None None None

r. Benjamin D. Levine None None None None None None

r. Robert J. Myerburg ● Guidant● Procter & Gamble

None ● Procter & Gamble● Reliant

Pharmaceutical

None None ● 2000, Defense, Lewis vs.Mudge

● 2002, Defense, Weinervs. Vitello

● 2005, Defense, EphedraMulti-District Litigation

r. Paul D. Thompson ● Astra Zeneca● Bristol Myers

Squibb

● Astra Zeneca● Merck● Pfizer● Schering

None ● Astra Zeneca● Merck● Pfizer● Schering


● 2005, Plaintiff, Suddendeath in college athlete

● 2004, Defense, Stresstest/recreational sports

● 2002, Sudden death,World Gym

● 2002, Plaintiff, Suddendeath, truck driver

● 1998, Plaintiff, Suddendeath, recreational sports

http://www.acc.org/clinical/guidelines/exercise/dirIndex.htm

http://www.acc.org/clinical/guidelines/exercise/dirIndex.htm

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1354 Zipes et al. JACC Vol. 45, No. 8, 2005Task Force 7: Arrhythmias April 19, 2005:1354–63

ask Force 7: Arrhythmiasouglas P. Zipes, MD, MACC, Chairichael J. Ackerman, MD, PHD, FACC, N. A. Mark Estes III, MD, FACC,ugustus O. Grant, MB, CHB, PHD, FACC, Robert J. Myerburg, MD, FACC,
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uidelines for athletic participation are needed to reducehe risk for arrhythmia-related morbidity or mortality.

owever, it is often difficult to establish the importance ofcardiac rhythm disturbance in assessing an athlete’s

ligibility for competition. Few data exist that have beenbtained prospectively from well-designed, scientificallycceptable studies to determine whether a particular rhythmisturbance predisposes an athlete to sudden death or toymptoms, such as syncope or presyncope, that couldrecipitate severe injury. Sudden unexpected cardiac deathn the young is rare, estimated at less than 1% of thatbserved in adults. Nonetheless, a significant proportion ofhese deaths occur in relation to exercise (1). The deaths ofeveral prominent athletes have focused attention on ath-etes with known arrhythmias.

Arrhythmias commonly are evanescent, often disappear-ng unpredictably for long periods of time, in some casesears. If they recur when the athlete is not exercising, therrhythmia may not be noted or may not produce significantymptoms. The same arrhythmia may minimally affect aompetitive golfer but severely incapacitate other athletes,uch as cross-country skiers, performing at peak physicalffort. The athlete may not develop the arrhythmia duringach sporting event. Although the reasons for this are notnown, factors related to the autonomic nervous systemrobably play a very important role in determining whethern arrhythmia occurs and its rate and effect on hemody-amic responses and symptoms. Autonomic “tone” probablyaries greatly and perhaps unpredictably between and withinthletic events and from one athlete to another. Mentaltress during competition can produce important electro-hysiologic and hemodynamic changes that are probablyediated through the autonomic nervous system.It is important to understand the range of normal heart

ate and rhythm for the trained athlete. Heart rates of 25eats/min and sinus pauses lasting greater than 2 s may beound on 24-h Holter ambulatory electrocardiographicECG) recordings. Type I second-degree atrioventricularAV) block and single uniform premature ventricular com-lexes each may occur in approximately 40% of athletes.omplex ventricular arrhythmias (multiform premature

entricular complexes, couplets, nonsustained ventricularachycardia) are less common (2,3).

Many of our conclusions result from data obtained inon-athletes, from general perceptions, or experience and
rom a heavy input of “what seems reasonable.” Decision- a
aking based on this type of logic is often faulty but is theest available. Recommendations and guidance need to bealanced between an effort to avoid restricting activitynduly and the hope of reducing the risk of death and injuryue to a rhythm disturbance.Despite the lack of complete information, some firm

onclusions can be reached. Certain arrhythmias, such asentricular tachyarrhythmias, create symptoms and are dan-erous in and of themselves regardless of the clinicalituation in which they occur (4). These arrhythmias gen-rally are characterized by very rapid or very slow heart rateshat significantly compromise cardiac output, coronary orerebral blood flow, or maintenance of blood pressure. Suchrrhythmias may include atrial flutter or atrial fibrillationith uncontrolled ventricular rates of 200 to 300 beats/min,sually (but not exclusively) in athletes with Wolff-arkinson-White (WPW) syndrome, rapid sustained ven-

ricular tachycardias, and AV block, or sinus node diseaseith very slow ventricular rates. Certain persistent arrhyth-ias, such as chronic tachycardias, can worsen cardiac

unction by a process called “remodeling” (5). Other ar-hythmias, such as AV nodal re-entrant tachycardia, gener-lly well-tolerated in most people, may not produce impor-ant symptoms at rest but only during exercise in athletesith structural heart disease due, in part, to an increase in

he tachycardia rate. Arrhythmias that might otherwise bennocuous and no more than a nuisance might, underonditions of participation in certain sports involving bodyontact or high speed, place the athlete at risk of injury oreath because of transient impaired mental function causing

oss of physical control. For example, it is possible that anthlete with supraventricular tachycardia participating inotentially dangerous sports, such as diving, downhill ski-ng, or auto racing, may be at greater risk because ofizziness, near syncope (a feeling of impending loss ofonsciousness), or syncope than if he or she were playingasketball or baseball.The search for significant structural heart disease is an

mportant element in evaluating athletes with arrhythmiasrior to sports participation. Some athletes with coronaryrtery disease (CAD), hypertrophic cardiomyopathy, ar-hythmogenic right ventricular cardiomyopathy (ARVC),ortic stenosis, some inherited cardiac channelopathies likeongenital long QT syndrome (LQTS) (6), and other formsf congenital heart disease, including repaired congenitaleart disease, are probably at greater risk for cardiac arrest
nd sudden death during and, perhaps, just after exercise.

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1355JACC Vol. 45, No. 8, 2005 Zipes et al.April 19, 2005:1354–63 Task Force 7: Arrhythmias

his is probably true whether or not arrhythmias have beenecognized previously. In general, athletes with symptomsossibly related to cardiac arrhythmia, such as exertional- oruditory-triggered syncope, near syncope, and palpitations,hould be carefully evaluated (see Task Force 2: Congenitaleart Disease) before being permitted to participate in

ompetitive sports. A consideration of cardiac hemody-amic status is critical because right or left ventricularysfunction is an additional important predictor of arrhyth-ic death. The presence of a significant rhythm distur-

ance, such as a rapid supraventricular or ventricular tachy-rrhythmia in athletes with abnormal cardiac hemodynamictatus (from any cause), itself is definitely incompatible witharticipation in all competitive sports. However, it is im-ortant to emphasize that some disease states, such as ayocarditis, can produce arrhythmias that can be self-

imited, with subsequent full recovery.In general, all athletes with significant cardiac arrhyth-ias being considered for athletic activity should have a

areful cardiac examination, a 12-lead ECG, echocardio-ram, exercise test, and, in some, a long-term 24-h Holtermbulatory ECG recording, if possible during the specificype of exercise being considered. Arrhythmias, as discussedn the various Task Force documents, are usually identifiedy exercise testing or some form of long-term monitoringincluding ambulatory Holter and event recording). Ar-hythmias precipitated during the specific type of exerciseeing considered can be important because a conventionalxercise test may not replicate the specific clinical situationroduced by actively participating in the sport. In thisegard, exercise tests may need to be adapted specifically forhe athlete; that is, to begin exercise at peak energyxpenditure, as a sprinter in a race might, rather than withhe slow increase in workload commonly used in testingthletes with CAD. Resuscitation equipment and trainedersonnel may be needed on a standby basis.All athletes with an arrhythmia who are permitted to

ngage in athletics should be re-evaluated at 6 to 12 monthntervals after they are trained, to determine whether theonditioning process affected the arrhythmia. It should alsoe stressed that athletes with arrhythmias controlled byntiarrhythmic drugs may stop taking these drugs for aariety of reason and, therefore, compliance with recom-ended therapy, as well as evaluation for recurrence of

ymptoms, must be established periodically. Abuse withrugs like cocaine or ephedra can precipitate life-hreatening arrhythmias, and such considerations are anmportant part of the evaluation. Of note, the use of certainardioactive drugs, such as beta-adrenergic blocking agents,s banned in some competitive sports (see the Introduction).n addition, it is important to realize that the cat-cholamines released during exercise may counteract thealutary effects of some antiarrhythmic agents. For somerrhythmias, an ablation approach, usually with a catheternd usually with radiofrequency energy, to eliminate the
rrhythmia may be preferable to drug treatment. Other t
blation energies, such as cryoablation, or other approachesuch as surgery, can be used as indicated. After successfulblation of the arrhythmia, a return to athletics can beithin days for those in whom repeated attempts at tachy-

ardia induction during isoproterenol administration is un-uccessful, and in whom the tachycardia was easily inducedrior to ablation. For those in whom such provocativeesting is not performed, waiting two to four weeks seemsdvisable.

It may be difficult for team physicians and consultantsrom the local community to make objective decisions toestrict or proscribe sports for a competitive athlete. Bor-erline cases can be reviewed by non-biased experts for theurpose of helping make these decisions. Whether thatpproach can be practically implemented has not beenetermined. Alternatively, it has also been suggested that inuch borderline cases, both appropriate emergency equip-ent and medical professionals versed in the operation of

hat equipment be present at all practices and games.owever, there is considerable concern with regard to this

ractice because its safety and efficacy have not beenstablished. Indeed, the practical implementation and reli-bility of this approach to reverse potentially lethal arrhyth-ias in out-of-hospital settings for the competing athlete is

ighly questionable.

YNCOPE

nexplained syncope in an athlete is a potentially importantymptom that mandates a thorough evaluation. It may beue to a variety of causes, including cardiovascular disease;lternatively, it may not be associated with either structuraleart disease or a primary electrical disorder but rather toechanisms such as vasovagal syncope, which is a common

nding in highly trained athletes. Although vasovagal syn-ope may be compatible with continued participation in allompetitive sports, caution should be used in making thisiagnosis in highly trained athletes without first definitivelyxcluding underlying structural cardiovascular disease. Aardiac arrhythmia should be considered, particularly whenyncope occurs during or immediately after exercise.

A cause of syncope can be established in approximately0% of patients. Often, a careful history and physicalxamination will identify the etiology (7). When such anvaluation does not determine the cause, further testing inearch of an arrhythmia is indicated. Ambulatory ECGecordings are often unrevealing but nevertheless probablyorthwhile to obtain during the initial evaluation. Event or

oop recorders or implantable devices can be used to increasehe ECG sampling time. Tilt-table testing has been used tossess patients at risk for vasovagal syncope, but the lack ofpecificity of this test (particularly in endurance-trainedthletes in whom false-positive results can occur) requires aarticularly cautious interpretation of the results. Exerciseesting is useful and is optimally performed while recording
he ECG during the athletic activity in which the person

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articipates. Provocative catecholamine stress testing withither epinephrine, procainamide, or isoproterenol may beseful to unmask cases of concealed LQTS, Brugada syn-rome (BrS), or catecholaminergic polymorphic ventricularachycardia (CPVT) (8). Invasive electrophysiologic testings most likely to identify an arrhythmia responsible foryncope in those patients with structural heart disease or anbnormal ECG but can be considered in other athleteshen no other cause of the syncope has been identified,

emembering that the diagnostic yield of invasive electro-hysiologic testing is low in the absence of structural heartisease.

YPES OF ARRHYTHMIAS

isturbances of sinus node function. Sinus tachycardiand sinus bradycardia appropriate for the clinical situationre not considered abnormal, and no tests are necessary.inus arrhythmia and wandering pacemaker are generallyonsidered normal, and no tests are necessary unless therrhythmias result in inappropriately slow rates accompa-ied by symptoms. Sinus arrhythmia and sinus bradycardiare particularly common in the trained athlete.

Asymptomatic sinus pause or sinus arrest (less than 3 s) isrobably of no significance. Longer symptomatic pauses,inoatrial exit block, and sick sinus syndrome are consideredbnormal, and athletes should have a 12-lead, 24-h ECG,nd an exercise test. Mutations involving the cardiac sodiumhannel encoded by SCN5A has been demonstrated in someatients with progressive cardiac conduction disease andongenital sick sinus syndrome (9,10). In an occasionalthlete experiencing syncope or near syncope, an electro-hysiologic study may be indicated and reveal abnormalinus node function, but in general, invasive electrophysi-logic testing is not helpful. Echocardiography should beerformed to exclude structural heart disease; other tests tovaluate ventricular or valvular function may be indicated.

ecommendations:

. Athletes with a normal or structurally abnormal heartin whom the bradycardic rate is increased appropri-ately by physical activity can participate in all com-petitive sports consistent with the limitations im-posed by the structural heart disease. They should bereassessed periodically to determine that trainingdoes not aggravate the bradycardia.

. Athletes with syncope or near syncope should notparticipate in sports where the likelihood of even amomentary loss of consciousness may be hazardousuntil the cause has been determined and treated, ifnecessary.

. Athletes with symptoms such as impaired conscious-ness and fatigue clearly attributed to the arrhythmiasshould be treated and if asymptomatic for two to threemonths during treatment, they can participate in all
competitive sports after physician re-evaluation.
. Athletes with symptomatic tachycardia/bradycardiasyndrome or inappropriate sinus tachycardia shouldbe treated. If no structural heart disease and asymp-tomatic for two to three months, they can participatein all competitive sports.

. Athletes with pacemakers should not engage insports with a danger of bodily collision because suchtrauma may damage the pacemaker system. Thisrestriction should clearly exclude activities wheredirect blows to the chest are a part of the sport, suchas football, rugby, boxing, martial arts, hockey, andlacrosse. Protective padding for the device is advis-able for other sports such as soccer, basketball,baseball, and softball where trauma is possible butless likely.

remature atrial complexes. In the absence of evidence oftructural heart disease and in the absence of symptomsther than occasional palpitation, no evaluation other than12-lead ECG is necessary.

ecommendation:

. Athletes can participate in all competitive sports.

trial flutter (in the absence of WPW syndrome). In thebsence of an acute, limiting illness, sustained atrial flutter isn uncommon rhythm disturbance in athletes withouttructural heart disease. Therefore, an echocardiogramhould be performed to evaluate cardiovascular structure andunction. Because the potential for very rapid ventricularates exists if the atrial flutter conducts 1:1 to the ventricles,CG determination of the ventricular response during an

xercise test or athletic event during treatment is essential.or some patients with paroxysmal atrial flutter, inductionf the arrhythmia by electrical stimulation may be consid-red before the exercise test, recognizing the difficult logis-ics in doing this. A 12-lead ECG and, at times, long-term4-h ECG may be necessary. Asymptomatic athletes whoave transient episodes of atrial flutter lasting less than 10 shat do not increase in duration during exercise can partic-pate in all sports.

ecommendations:

. Athletes with atrial flutter in the absence of structuralheart disease who maintain a ventricular rate thatincreases and slows appropriately comparable to thatof a normal sinus response in relation to the level ofactivity, while receiving no therapy or therapy withAV nodal blocking drugs, can participate in class IAcompetitive sports with the warning that rapid 1:1conduction still may occur. However, full participa-tion in all competitive sports should not be allowedunless the athlete has been without atrial flutter fortwo to three months with or without drug treatment.Note that the use of beta-blockers is prohibited in
some competitive sports (see Introduction).

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. Athletes with structural heart disease who have atrialflutter can participate in class IA competitive sportsonly after two to four weeks have elapsed without anepisode of atrial flutter.

. Athletes without structural heart disease who haveelimination of the atrial flutter by an ablation tech-nique or surgery can participate in all competitivesports after two to four weeks without a recurrence,or in several days after an electrophysiologic studyshowing non-inducibility of the atrial flutter in thepresence of bi-directional isthmus block.

. Athletes in whom anticoagulation is deemed neces-sary cannot participate in competitive sports wherethe danger of bodily collision is present.

trial fibrillation (in the absence of WPW syndrome). At-ial fibrillation is far more common than atrial flutter anday be present intermittently or chronically (11,12). Eval-

ation should include a search for the cause, such ashyrotoxicosis. More often atrial fibrillation occurs in asso-iation with diseases such as CAD or hypertension. Evalu-tion includes determination of the ventricular responseuring athletic activity or an exercise test comparable to the

ntended athletic competition. For some patients witharoxysmal atrial fibrillation, electrical induction of atrialbrillation before the exercise test may be necessary, recog-izing the difficult logistics in doing this. A 12-lead ECG isecessary, and long-term 24-h ECG recordings and anchocardiogram are helpful in establishing the presence oftructural heart disease. Asymptomatic athletes who havepisodes of atrial fibrillation of 5 to 15 s that do not increasen duration during exercise can participate in all sports.

ecommendations:

. Athletes with asymptomatic atrial fibrillation in theabsence of structural heart disease who maintain aventricular rate that increases and slows appropri-ately and is comparable to that of a normal sinusresponse in relation to the level of activity, whilereceiving no therapy or therapy with AV nodal-blocking drugs, can participate in all competitivesports. Note that the use of beta-blockers is prohib-ited in some competitive sports.

. Athletes who have atrial fibrillation in the presenceof structural heart disease who maintain a ventricularrate comparable to that of an appropriate sinustachycardia during physical activity while receivingno therapy or therapy with AV nodal-blocking drugscan participate in sports consistent with the limita-tions of the structural heart disease.

. Athletes who require anticoagulation should notparticipate in sports with danger of bodily collision(13).

. Athletes without structural heart disease who haveelimination of atrial fibrillation by an ablation tech-
nique, including surgery, may participate in all com- c
petitive sports after four to six weeks without arecurrence or after an electrophysiologic study hasconfirmed non-inducibility.

inus node re-entry, inappropriate sinus tachycardia, andtrial tachycardia (in the absence of WPW syndrome).inus node re-entry, inappropriate sinus tachycardia, andtrial tachycardia should be evaluated as described for atrialutter. Asymptomatic athletes who have episodes of tachy-ardia of 5 to 10 s that do not increase in duration duringxercise can participate in all sports.

ecommendations:

. Athletes with sinus node re-entry, inappropriate si-nus tachycardia, or atrial tachycardia in the absenceof structural heart disease who maintain a ventricularrate that increases and slows appropriately and iscomparable to that of a normal sinus response inrelation to the level of activity, with or withouttherapy, can participate in all competitive sports.

. Athletes with underlying structural heart disease canparticipate only in competitive sports consistent withthe limitations of the heart disease.

. Athletes without structural heart disease who haveelimination of the atrial tachyarrhythmia by an ablationtechnique, including surgery, may participate in allcompetitive sports after two to four weeks without arecurrence or in several days after an electrophysiologicstudy showing non-inducibility.

V junctional escape beats/rhythm. Atrioventricularunctional escape beats and junctional rhythm are commonn athletes. The clinical approach and final recommenda-ions are the same as those given earlier for symptomaticthletes with disturbances of sinus node function.remature AV junctional complexes. If the athlete issymptomatic except for occasional episodes of palpitationshat do not suggest a sustained tachycardia, evaluation neednclude only a 12-lead ECG. In some athletes, a 24-h ECGecording (during athletic activity if possible), echocardio-ram, and an exercise test may be indicated.

ecommendations:

. Athletes with a structurally normal heart and anormal heart rate response to activity without evi-dence of a sustained tachycardia can participate in allcompetitive sports.

. Athletes with an abnormal heart, depending on thetype and extent of the heart disease, can participate incompetitive sports consistent with the limitations ofthe structural cardiac disease.

on-paroxysmal AV junctional tachycardia. A form ofunctional tachycardia, so-called junctional ectopic tachycar-ia, can be found in a permanent form in infants less thanix months old, whereas a transient form occurs mostly in
hildren but occasionally in adults (13). Most adults have a

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lower form of non-paroxysmal junctional tachycardia.valuation generally includes a 12-lead ECG, echocardio-ram, exercise test, and 24-h ECG recording during activ-ty. Invasive studies may be necessary for some symptomaticatients or for those with very rapid ventricular rates.

ecommendations:

. Athletes without structural heart disease or symp-toms who have a controlled ventricular rate thatincreases and slows appropriately and is comparableto that of a normal sinus response in relation to thelevel of activity, with or without therapy, can partic-ipate in all sports.

. Athletes who have no symptoms but who have struc-tural heart disease or incompletely controlled ven-tricular rates can engage in class IA competitivesports depending on the nature and extent of thestructural heart disease and the ventricular rate.

. Athletes with inappropriately rapid ventricular rates,with or without structural heart disease, should beconsidered for treatment to control the ventricularrate before participating in any sports. Athleteswhose tachycardia is controlled by therapy and veri-fied by appropriate testing can participate in allcompetitive sports consistent with their cardiacstatus.

upraventricular tachycardia. Atrioventricular nodal re-ntrant tachycardia and AV re-entry over a concealedccessory pathway (with only retrograde conduction) (4,14)re included in this category. If the diagnosis of theupraventricular tachycardia cannot be made with certainty,nd if other clinical therapeutic circumstances warrant it,nvasive electrophysiologic studies might be indicated. It ismportant to identify the rate response of the supraventric-lar tachycardia during exercise. If the exercise does notnduce the tachycardia, attempts to induce the supraventric-lar tachycardia (possibly with atrial or esophageal pacing)ay be useful, followed once again by an exercise test

erformed by the athlete when the supraventricular tachy-ardia has been initiated. The logistics to accomplish thisay be difficult. Asymptomatic athletes who have episodes

f supraventricular tachycardia of 5 to 10 s that do notncrease in duration during exercise can participate in allports.

ecommendations:

. Athletes without structural heart disease who areasymptomatic and have reproducible exercise-induced supraventricular tachycardia prevented bytherapy and verified by appropriate testing can par-ticipate in all competitive sports.

. Athletes who do not have exercise-induced supraven-tricular tachycardia but experience sporadic recur-rences should be treated. However, because of the
unpredictable nature of the tachycardia, end points p
for adequate therapy may be difficult to achieve; butonce established, these athletes can participate in allactivities consistent with their cardiac status. Asymp-tomatic athletes who have episodes of supraventric-ular tachycardia of 5 to 15 s that do not increase induration during exercise can participate in all sportsconsistent with their cardiac status.

. Athletes with syncope, near-syncope, or significantsymptoms secondary to arrhythmia or who havesignificant structural heart disease in addition to thearrhythmia should not participate in any competitivesports until they have been adequately treated andhave no recurrence for two to four weeks (4). At thattime they can participate in class IA competitivesports.

. For those athletes with no structural heart diseasewho have had successful catheter or surgical ablation,are asymptomatic, and have no inducible arrhythmiaon follow-up electrophysiologic testing, all competi-tive sports are permitted in several days. If noelectrophysiologic testing is done, full participationis permitted if no spontaneous recurrence of tachy-cardia for two to four weeks after ablation.

entricular pre-excitation (WPW syndrome). Requiredoninvasive tests include a 12-lead ECG, exercise test, andchocardiogram to exclude associated cardiovascular abnor-alities. In some instances, a 24-h ECG recording during

thletic activity may be indicated. Electrophysiologic studiesre indicated in athletes with symptoms of impaired con-ciousness, long-lasting palpitations, or rapid rates in whomn ablation procedure is indicated.

In asymptomatic athletes with no history of palpitationsr tachycardia and no evidence of structural cardiac abnor-alities, further evaluation may not be necessary. However,

he optimal management for these athletes is uncertain, andontinues to be debated (15,16). Furthermore, the youngerhe patient, the less time the patient has had to developymptoms, and so the distinction between symptomatic andsymptomatic WPW syndrome may be less meaningful inhe pediatric population. Sudden death in athletes withre-excitation is rare, and it appears to be confined largely tohose with accessory pathways that have short refractoryeriods. Therefore, it may be advisable in selected asymp-omatic athletes who anticipate moderate or high levelctivity to undergo electrophysiology study to determine thenterograde refractory period of the accessory pathway, theinimum RR interval between pre-excited complexes in

trial fibrillation, and the number of accessory pathways.ndividuals with multiple accessory pathways or ventricularates exceeding 240 beats/min should be offered catheterblation of the accessory pathway (17,18). For those athletesith a history of palpitations, syncope, or near syncope, it isandatory to assess the functional capabilities and electro-

hysiologic properties of the accessory pathway.

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ecommendations:

. Athletes without structural heart disease, without ahistory of palpitations, or without tachycardia (par-ticularly those 20 to 25 years old or more) canparticipate in all competitive sports. However, inyounger age groups, a more in-depth evaluationincluding an electrophysiologic study may be recom-mended before allowing participation in moderate-to high-intensity competitive sports.

. Athletes with episodes of AV reciprocating tachycar-dia should be treated as previously recommended (seesection on Supraventricular Tachycardia). However,it should be appreciated that they can develop atrialfibrillation with rapid ventricular rates. Electricalinduction of atrial fibrillation to determine the short-est QRS interval between two complexes conductedover the accessory pathway during isoproterenol ad-ministration or exercise is recommended. Those ath-letes in whom the shortest cycle length is less than250 ms should undergo ablation of the accessorypathway.

. Athletes with episodes of atrial flutter/fibrillationand syncope or near syncope whose maximal ventric-ular rate at rest (without therapy) as a result ofconduction over the accessory pathway exceeding 240beats/min should be considered for catheter ablationtherapy of the accessory pathway prior to continuingcompetition. Those whose ventricular rate duringisoproterenol administration is less than 240 beats/min and who have no episodes of syncope or nearsyncope appear to be at low risk for sudden cardiacdeath.

. Athletes with no structural heart disease who havehad successful catheter or surgical ablation of theaccessory pathway, are asymptomatic, and have nor-mal AV conduction and no inducible arrhythmia byfollow-up electrophysiologic study can participate inall competitive sports in several days. Those withoutan electrophysiologic study and no spontaneous re-currence of tachycardia for two to four weeks afterablation can participate in all competitive sports.

remature ventricular complexes. Noninvasive tests rec-mmended include a 12-lead ECG and exercise test. Ifhere is evidence to suggest the presence of structural heartisease, an echocardiogram is indicated, and a 24-h ECGecording may be beneficial. Even without evidence oftructural heart disease, if an increase in the number ofremature ventricular complexes or complex ventricularrrhythmias occurs during exercise (with or without treat-ent, or substantial reduction or abolition following a

eriod of deconditioning), further evaluation may be indi-ated. In some of these athletes thought to have a structur-lly normal heart, cardiac catheterization and angiographyay reveal otherwise undetected abnormalities, including

ccult CAD, congenital coronary anomalies, ARVC, car-

iac tumor, or evidence of cardiomyopathy. The moreecently recognized channelopathy known as CPVT shoulde considered (see the following text).Frequent and complex ventricular tachyarrhythmias are

ommon in trained athletes; they are usually unassociatedith underlying cardiovascular abnormalities and do not

ppear to convey increased risk (18). Deconditioning usuallyesults in loss or diminution of these arrhythmias, providingvidence of their benign clinical nature (19).

ecommendations:

. Athletes without structural heart disease who havepremature ventricular complexes at rest and duringexercise, and exercise testing (comparable to the sportin which they compete) can participate in all com-petitive sports. Should the premature ventricularcomplexes increase in frequency during exercise orexercise testing to the extent that they producesymptoms of impaired consciousness, significant fa-tigue, or dyspnea, the athlete can participate in classIA competitive sports only.

. Athletes with structural heart disease who are inhigh-risk groups and have premature ventricularcomplexes (with or without treatment) can partici-pate in class IA competitive sports only. Such ath-letes with premature ventricular complexes that aresuppressed by drug therapy (as assessed by ambula-tory ECG recordings) during participation in thesport can compete in only class IA competitivesports.

entricular tachycardia (VT). Nonsustained or sustainedonomorphic or polymorphic VT is always a potentially

erious occurrence. Noninvasive tests to be performed in-lude a 12-lead ECG, exercise test, and echocardiography.n some patients, 24-h ECG recording during exercise maye indicated. Echocardiography, cardiac catheterization,nd electrophysiologic study should be considered to verifyhat the heart is structurally normal and to establish theechanism or location, or both, of the VT. A possible

xception is the patient with accelerated idioventricularhythm, in which the ventricular rate is similar to the sinusate. In such patients, if they have no significant structuraleart disease, the approach should be similar to that in

ndividuals with premature ventricular complexes.

ecommendations:

. Athletes with a structurally normal heart and mono-morphic nonsustained or sustained VT that can belocalized to a specific site(s) in the heart are candi-dates for a catheter ablation procedure that maypotentially offer a cure. Following such a successfulablation procedure, with subsequent failure to induceVT during electrophysiologic study (EPS) with/with-out isoproterenol when the VT was reproducibly
induced before ablation, the athlete can resume full

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competitive activity within two to four weeks. A moreconservative approach is recommended for the ath-lete who chooses drug suppression because cat-echolamines released during athletic activity cancounter the suppressive effects of the drug, and theVT can re-emerge. In that situation, generally theathlete should not compete in any sports for at leasttwo to three months after the last VT episode. Ifthere have been no clinical recurrences, and the VT isnot inducible by exercise/exercise testing or EPS, andthe athlete has no structural heart disease, all com-petitive sports may be permitted. Because decondi-tioning can result in the loss or lessening of ventriculararrhythmias (19), a short period of deconditioning andretesting can be considered in some athletes.

. For the athlete with structural heart disease and VT,moderate- and high-intensity competition is contra-indicated regardless of whether the VT is suppressedor ablated. Only class IA competitive sports arepermitted.

. An exception to this general recommendation is theasymptomatic athlete with brief (generally less than 8to 10 consecutive ventricular beats) episodes of non-sustained monomorphic VT, rates generally less than150 beats/min, and no structural heart disease estab-lished by noninvasive and invasive tests. These ath-letes do not appear to be at increased risk for suddencardiac death. If exercise testing (preferably by am-bulatory ECG recording during the specific compet-itive activity) demonstrates suppression of the VT orno significant worsening compared with baseline,participation in all competitive sports is permissible.

. The desire of the athlete to continue athletic com-petition should not represent the primary indicationfor use of an implantable cardioverter-defibrillator(ICD). The efficacy with which these devices willterminate a potentially lethal arrhythmia under theextreme conditions of competitive sports, with theassociated metabolic and autonomic changes, andpossible myocardial ischemia, is unknown. In addi-tion, sports with physical contact may result indamage to the ICD and/or lead, preventing normalfunction. For athletes with ICDs, all moderate andhigh intensity sports are contraindicated. Class IAsports are permitted.

entricular flutter and ventricular fibrillation.

ecommendation:

. Athletes with conditions that result in cardiac arrestin the presence or absence of structural heart diseasegenerally are treated with an ICD and cannot partic-ipate in any moderate- or high-intensity competitivesports. However, athletes with ICDs and who havehad no episodes of ventricular flutter or ventricular
fibrillation requiring device therapy for six months A
may engage in class IA competitive sports. Recom-mendations in the section on VT also apply.

irst-degree AV block. In asymptomatic athletes withtructurally normal hearts, if the QRS complex is normal,o further evaluation other than a 12-lead ECG is neces-ary. If the QRS complex is abnormal, or the PR interval isxcessively prolonged (0.3 s or more), an exercise stress test,4-h ECG recording and echocardiogram may be indicated.ossibly an EPS might be necessary to determine the sitend duration of conduction delay.

ecommendation:

. Asymptomatic athletes without evidence of structuralheart disease, in whom the first-degree AV block doesnot worsen with exercise, can participate in all com-petitive sports. If underlying heart disease is present,its nature and severity can independently dictatealternative restrictions.

ype 1 second-degree (Wenckebach) AV block. Wenck-bach AV nodal block can be present in otherwise normal,ell-trained endurance athletes (4). Recommended evalua-

ions include a 12-lead ECG, exercise test, and echocardio-ram. A 24-h ECG recording during athletic activity maye indicated in some athletes. In those athletes with type 1econd-degree AV block and co-existing bundle-branchlock, EPS may be indicated to identify the presence ofis-Purkinje Wenckebach block.

ecommendations:

. Asymptomatic athletes with a structurally normalheart and no worsening or actual improvement of AVblock with exercise or recovery can participate in allcompetitive sports.

. Asymptomatic athletes with a structurally abnormalheart in whom AV block disappears or does notworsen with exercise or recovery can participate in allcompetitive sports, as determined by the limitationsof the cardiac abnormality.

. Asymptomatic athletes in whom type 1 second-degree AV block initially appears or worsens withexercise or during the recovery period should beevaluated further (e.g., for possible intra- or infra-His block) and may require pacemaker therapy. Suchathletes can participate in class IA competitivesports.

. Athletes treated with pacemakers should not engagein competitive sports with a danger of bodily collisionbecause such trauma may damage the pacemakersystem.

ype 2 second-degree (Mobitz) AV block. The evolutionnd treatment of this abnormality is considered to be theame as in acquired complete heart block. It should bereated with permanent pacing before any athletic activity.
thletes with pacemakers should not participate in compet-

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tive sports that pose a danger of bodily collision becauseuch trauma may damage the pacemaker system. Beforellowing athletes to engage in these activities, an exerciseest should be done at the level of activity demanded by thearticular sport to be certain that the paced heart ratencreases appropriately.

ongenital complete heart block. The clinical approacho evaluating the severity of the cardiovascular abnormalityncludes an echocardiogram, 12-lead ECG, 24-h ECGecording during exercise, and exercise stress test (exerciseesting should be performed at the same exercise level ashat during the sports activity).

ecommendations:

. Athletes with a structurally normal heart and normalcardiac function, with no history of syncope or nearsyncope, a narrow QRS complex, ventricular rates atrest greater than 40 to 50 beats/min increasing ap-propriately with exertion, no or only occasional pre-mature ventricular complexes, and no VT duringexertion can participate in all competitive sports.

. Athletes with ventricular arrhythmia, symptoms offatigue, near-syncope, or syncope should have apacemaker implanted before they participate in com-petitive sports. Athletes with pacemakers should notparticipate in competitive sports when the danger ofbodily collision exists because such trauma may dam-age the pacemaker system. Before allowing athletesto engage in these activities, an exercise test shouldbe conducted at the level of activity demanded by theparticular sport so as to be certain that the pacedheart rate increases appropriately.

. Athletes with abnormal hemodynamic status, asthose with an intracardiac shunt, cannot participatein any competitive sports without a pacemaker. Re-strictions are the same as those in recommendation 2.

cquired complete heart block.

ecommendations:

. Patients with acquired complete heart block shouldbe treated with pacing before any athletic activity.

. Athletes with a pacemaker should not participate incompetitive sports posing a danger of bodily collisionbecause such trauma may damage the pacemakersystem.

omplete right bundle-branch block. Evaluation in-ludes a 12-lead ECG, exercise test, and echocardiogram. Inome instances, a 24 h ECG recording may be indicated.

ecommendation:

. Athletes without ventricular arrhythmias who do notdevelop AV block with exercise and who have no
symptoms can participate in all competitive sports p
consistent with their cardiac status. This also appliesto athletes with associated left-axis deviation.

omplete left bundle-branch block. Evaluation includes12-lead ECG, exercise test, and echocardiogram. In some

nstances, a 24-h ECG recording may be indicated. Becausef the rarity of acquired left bundle-branch block in childrennd its association with syncope from presumed paroxysmalV block, an invasive EPS should be considered in youngatients.

ecommendations:

. Adult athletes with acquired left bundle-branchblock should follow the recommendations under thesection entitled Complete Right Bundle-BranchBlock.

. Athletes with a normal HV interval and a normal AVconduction response to pacing can participate inall competitive sports consistent with their cardiacstatus.

. Athletes with abnormal AV conduction characterizedby an HV interval greater than 90 ms or a His-Purkinje block should have pacemaker implantation.They should be restricted from competitive sportsthat hold a danger of bodily collision because suchtrauma may damage the pacemaker system.

nherited arrhythmia syndromes. LONG QT SYNDROME.

he definitive clinical diagnosis of congenital long QTyndrome (LQTS) can be complex (20). Debate continuess to what QTc constitutes the upper limit of normal. Anncreasing proportion of asymptomatic individuals withenetically proven LQTS are found to have a normal restingCG with a heart rate corrected QT interval (QTc) byazett’s formula of less than 460 ms (genotype positive/henotype negative LQTS). In addition, a QTc of 440 ms,sed in the past as an upper limit of normal, is present in faroo many normal individuals (greater than 25%) to serve asmeaningful upper limit cut-off value. In general, a QTc of70 ms or more in males and 480 ms or more in femalesequires further investigation as to the presence of congen-tal (or acquired) causes of QT prolongation. A patient withQTS and a resting QTc of 500 ms or more is generallyonsidered at increased clinical risk for a significant arrhyth-ia (21). One approach to the diagnosis of congenitalQTS is to utilize the “Priori-Schwartz” score that incor-orates QTc, T-wave morphology, symptomatic presenta-ion, and family history into the diagnostic algorithm (21).

“Priori-Schwartz” score of 4 or more suggests highlinical probability for LQTS. In addition, genetic testingor the five cardiac ion-channel genes responsible for 75% ofQTS (LQT1, LQT2, LQT3, LQT5, and LQT6) is nowvailable as a commercial diagnostic test (22).

Mutations involving the structural protein ankyrin-Bnderlie the rare LQT4 form (23). Mutations involving theCNJ2-encoded IK1 potassium channel account for ap-
roximately one-half of Andersen-Tawil syndrome (ATS1)

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haracterized by abnormal U waves, a prolonged QUnterval, periodic paralysis, and facial and skeletal dysmor-hisms. The ATS1 has been annotated in the past asQT7.Physical exertion (particularly swimming) appears to be a

ommon trigger for ventricular arrhythmias in LQT1,hereas individuals with LQT2 seem more at-risk to

uditory/emotional triggers, and patients with LQT3 maye at greater risk during rest and inactivity (24,25). How-ver, exceptions to these genotype-phenotype correlationsinder genotype-specific tailoring of competitive sportsecommendations. The entire personal and family pheno-ype must be incorporated before any eligibility or disqual-fication decision is rendered.

ecommendations:

. Regardless of QTc or underlying genotype, all com-petitive sports, except those in class IA categoryshould be restricted in a patient who has previouslyexperienced either: 1) an out-of-hospital cardiac ar-rest, or 2) a suspected LQTS-precipitated syncopalepisode.

. Asymptomatic patients with baseline QT prolonga-tion (QTc of 470 ms or more in males, 480 ms ormore in females) should be restricted to class IAsports. The restriction limiting participation to classIA activities may be liberalized for the asymptomaticpatient with genetically proven type 3 LQTS(LQT3).

. Patients with genotype-positive/phenotype-negativeLQTS (i.e., identification of a LQTS-associated mu-tation in an asymptomatic individual with a nondiag-nostic QTc) may be allowed to participate in competi-tive sports. Although the risk of sudden cardiac death isnot zero in such individuals, there is no compelling dataavailable to justify precluding these individuals (who arebeing identified with increasing frequency) from com-petitive activities. Because of the strong associationbetween swimming and LQT1, persons with genotype-positive/phenotype-negative LQT1 should refrain fromcompetitive swimming.

. LQTS patients with an ICD/pacemaker should notengage in sports with a danger of bodily collisionbecause such trauma may damage the pacemakersystem. The presence of an ICD should restrictindividuals to class IA activities.

HORT QT SYNDROME. Individuals with the short QTyndrome (SQTS) (QTc less than 300 ms) have a short QTnterval and ventricular refractory period, and at least somef them have “gain-of-function” abnormalities in either IKr

KCNH2) or IKs (KCNQ1) (26).

ecommendation:

. Until the phenotype of SQTS is better understood, a
universal restriction from competitive sports with the
possible exception of class IA activities seems torepresent the most prudent recommendation (27).

ATECHOLAMINERGIC POLYMORPHIC VENTRICULAR

ACHYCARDIA (CPVT). Approximately one-half of patientsith CPVT is pursuant to mutations involving the RyR2-

ncoded ryanodine receptor (sarcoplasmic reticulum cal-ium release channel). Such individuals are vulnerable toxercise-induced VT/ventricular fibrillation.

ecommendations:

. Symptomatic patients have a poor prognosis unlesstreated with an implantable cardioverter-defibrillator(ICD) (28) and all such patients are restricted fromcompetitive sports with the possible exception of min-imal contact, class IA activities. As with LQT1, pa-tients with CPVT should be restricted from competitiveswimming. Asymptomatic patients detected as part offamilial screening with documented exercise- orisoproterenol-induced VT should refrain from all com-petitive sports except possible class IA activities. A lessrestrictive approach may be possible for the genotype-positive/phenotype-negative (asymptomatic, no induc-ible VT) athlete.

RUGADA SYNDROME (BrS). Brugada syndrome (29), char-cterized by an accentuated J-wave primarily in leads V1hrough V3, with ST-segment elevation, often followed by

negative T-wave and an R prime, may be the cause ofudden unexplained death syndrome, typically during sleep.nly 15% to 20% of BrS is established as a channelopathy

ue to mutations involving the SCN5A-encoded alphaubunit of the cardiac sodium channel (30). Individuals withrS and no previous cardiac arrests may have a high risk of

udden death if they have inducibility of ventricular arrhyth-ias and a previous history of syncope (28). Hyperthermia

an potentially unmask the Brugada ECG pattern in pa-ients with BrS, who can then display fever-induced poly-orphic VT. Death often occurs with mild activity or

uring sleep.

ecommendations:

. Although a clear association between exercise andsudden death has not been established, and becauseof the potential impact of hyperthermia, restriction toparticipation in class IA sports seems advisable.

. The presence of an ICD device warrants the samerestrictions to class IA sports as previously outlined.

doi:10.1016/j.jacc.2005.02.014



2. Zehender M, Meinertz T, Keul J, Just H. ECG variants and cardiac
arrhythmias in athletes: clinical relevance and prognostic importance.Am Heart J 1990;119:1378–91.

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3. Bjornstad H, Storstein L, Meen HD, Hals O. Ambulatory electro-cardiographic findings in top athletes, athletic students and controlsubjects. Cardiology 1994;84:42–50.

4. Olgin JE, Zipes DP. Specific arrhythmias: diagnosis and treatment. In:Zipes DP, Libby P, Bonow RO, Braunwald E, editors. Heart Disease:A Textbook of Cardiovascular Medicine. Philadelphia, PA: Saunders,2005:803–63.

5. Allessie M, Ausma J, Schotten U. Electrical, contractile and structuralremodeling during atrial fibrillation. Cardiovasc Res 2002;54:230–46.

6. Antzelevitch C. Molecular genetics of arrhythmias and cardiovascularconditions associated with arrhythmias. J Cardiovasc Electrophysiol2003;14:1259–72.

7. Calkins H, Zipes DP. Hypotension and syncope. In: Zipes DP, LibbyP, Bonow RO, Braunwald E, editors. Heart Disease. A Textbook ofCardiovascular Medicine. Philadelphia, PA: Saunders, 2005:909–19.

8. Ackerman MJ, Khositseth A, Tester DJ, Hejlik JB, Shen WK, PorterCB. Epinephrine-induced QT interval prolongation: a gene-specificparadoxical response in congenital long QT syndrome. Mayo ClinProc 2002;77:413–21.

9. Schott JJ, Alshinawi C, Kyndt F, et al. Cardiac conduction defectsassociated with mutations in SCN5A. Nat Genet 1999;23:20–1.

0. Benson DW, Wang DW, Dyment M, et al. Congenital sick sinussyndrome caused by recessive mutations in the cardiac sodium channelgene (SCN5A). J Clin Invest 2003;112:1019–28.

1. Nattel S, Erlich J. Atrial fibrillation. In: Zipes D, Jalife J, editors.Cardiac Electrophysiology: From Cell to Bedside. Philadelphia, PA:Saunders, 2004:512–22.

2. Furlanello F, Bertoldi A, Dallago M, et al. Atrial fibrillation in eliteathletes. J Cardiovasc Electrophysiol 1998;9:S63–8.

3. Oral H, Strickberger SA. Junctional rhythms and junctional tachycar-dia. In: Zipes D, Jalife J, editors. Cardiac Electrophysiology: FromCell to Bedside. Philadelphia, PA: Saunders, 2004:523–7.

4. Lockwood D, Otomoto K, Wang Z. Electrophysiologic characteristics ofatrioventricular nodal reentrant tachycardia. In: Zipes D, Jalife J, editors.Cardiac Electrophysiology: From Cell to Bedside. Philadelphia, PA:Saunders, 2004:537–57.

5. Pappone C, Santinelli V, Rosanio S, et al. Usefulness of invasiveelectrophysiologic testing to stratify the risk of arrhythmic events inasymptomatic patients with Wolff-Parkinson-White pattern: resultsfrom a large prospective long-term follow-up study. J Am Coll Cardiol2003;41:239–44.

6. Klein GJ, Bashore TM, Sellers TD, Pritchett EL, Smith WM,Gallagher JJ. Ventricular fibrillation in the Wolff-Parkinson-White

7. Pappone C, Santinelli V, Manguso F, et al. A randomized study ofprophylactic catheter ablation in asymptomatic patients with theWolff-Parkinson-White syndrome. N Engl J Med 2003;349:1803–11.

8. Biffi A, Pelliccia A, Verdile L, et al. Long-term clinical significance offrequent and complex ventricular tachyarrhythmias in trained athletes.J Am Coll Cardiol 2002;40:446–52.

9. Biffi A, Maron BJ, Verdile L, et al. Impact of physical deconditioningon ventricular tachyarrhythmias in trained athletes. J Am Coll Cardiol2004;44:1053–8.

0. Ackerman MJ. Cardiac channelopathies: it’s in the genes. Nat Med2004;10:463–4.

1. Priori SG, Schwartz PJ, Napolitano C, et al. Risk stratification in thelong-QT syndrome. N Engl J Med 2003;348:1866–74.

2. Genaissance Pharmaceuticals, Inc. Genaissance Pharmaceuticals, Inc.(GNSC) launches its proprietary FAMILION test for genetic muta-tions associated with sudden cardiac death. Available at: http://www.biospace.com/news_story.cfm?StoryID�16229920&full�1.Accessed October 1, 2004.

3. Mohler PJ, Schott JJ, Gramolini AO, et al. Ankyrin-B mutationcauses type 4 long-QT cardiac arrhythmia and sudden cardiac death.Nature 2003;421:634–9.

4. Schwartz PJ, Priori SG, Spazzolini C, et al. Genotype-phenotypecorrelation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias. Circulation 2001;103:89–95.

5. Choi G, Kopplin LJ, Tester DJ, Will ML, Haaglund CM, AckermanMJ. Spectrum and frequency of cardiac channel defects implicated inswimming-triggered arrhythmia syndromes. Circulation 2004;110:2119–24.

6. Brugada R, Hong K, Dumaine R, et al. Sudden death associated withshort-QT syndrome linked to mutations in HERG. Circulation2004;109:30–5.

7. Gaita F, Giustetto C, Bianchi F, et al. Short QT syndrome: a familialcause of sudden death. Circulation 2003;108:965–70.

8. Sumitomo N, Harada K, Nagashima M, et al. Catecholaminergicpolymorphic ventricular tachycardia: electrocardiographic characteris-tics and optimal therapeutic strategies to prevent sudden death. Heart2003;89:66–70.

9. Brugada J, Brugada R, Brugada P. Determinants of sudden cardiac deathin individuals with the electrocardiographic pattern of Brugada syndromeand no previous cardiac arrest. Circulation 2003;108:3092–6.

0. Brugada P, Brugada R, Mont L, Rivero M, Geelen P, Brugada J.Natural history of Brugada syndrome: the prognostic value of pro-grammed electrical stimulation of the heart. J Cardiovasc Electro-

syndrome. N Engl J Med 1979;301:1080–5. physiol 2003;14:455–7.



Advisory BoardStock

HolderExpert Witness

Testimony

r. Michael J. Ackerman None None None None None

r. N. A. Mark Estes III ● Guidant● Medtronic

● Guidant● Medtronic

● Guidant(Executive Committee)

None None

r. Augustus O. Grant None None None None None

r. Robert J. Myerburg ● Guidant● Procter & Gamble

None ● Procter & Gamble● Reliant Pharmaceutical

None ● 2000, Defense,Lewis vs. Mudge

● 2002, Defense,Weiner vs. Vitello

● 2005, Defense,Ephedra Multi-DistrictLitigation

r. George Van Hare None ● Medtronic None None None● St. Jude Medical

r. Douglas P. Zipes ● Cardiofocus● Janssen● Medtronic

● Medtronic ● Medtronic ● MVMD ● 1996, Defense,Knapp vs. Northwestern

http://www.biospace.com/news_story.cfm?StoryID=16229920&full=1



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1364 Mitchell et al. JACC Vol. 45, No. 8, 2005Task Force 8: Classification of Sports April 19, 2005:1364–7

ask Force 8: Classification of Sportsere H. Mitchell, MD, FACC, Chair

illiam Haskell, PHD, Peter Snell, PHD, Steven P. Van Camp, MD, FACC

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his classification of sports has been developed to allow aundamental question to be addressed: whether it is reason-bly safe to recommend that an athlete with a specificardiovascular abnormality be eligible for a particular com-etitive sport (1,2). We recognize that cardiovascular diseasessessments are imprecise and may change over time and benfluenced by exercise training. Furthermore, there areotentially life-threatening aspects to the nature of the risknvolved. We have attempted to incorporate these realitiesnto the classification system.

Sports can be classified according to the type and inten-ity of exercise performed and also with regard to the dangerf bodily injury from collision, as well as the consequencesf syncope. Exercise can be divided into two broad types:ynamic (isotonic) and static (isometric) (3–6).Dynamic exercise involves changes in muscle length and

oint movement with rhythmic contractions that develop aelatively small intramuscular force; static exercise involvesevelopment of a relatively large intramuscular force with

ittle or no change in muscle length or joint movement.hese two types of exercise should be thought of as the twopposite poles of a continuum, with most physical activitiesnvolving both static and dynamic components. For exam-le, distance running has low static and high dynamicemands, water skiing has principally high static and lowynamic demands, and rowing has both high static andynamic demands.The terms dynamic and static exercise characterize activity

n the basis of the mechanical action of the musclesnvolved and are different from the terms aerobic andnaerobic exercise. The latter characterize activity on theasis of the type of muscle metabolism. Most high-intensitytatic exercise is performed anaerobically, whereas high-ntensity dynamic exercise lasting for more than several

inutes is performed aerobically. However, some dynamicxercises, such as sprinting or jumping, are performedrimarily anaerobically. Thus, many sports are placed in theigh dynamic category, including such diverse activities askiing (cross country), running (distance), soccer, andquash. Because the cardiovascular demands of very highesistance dynamic exercise are similar to sustained staticxercise, those sports that have either a sustained staticomponent or a very high resistance dynamic componentre classified together as high-intensity static exercise (e.g.,eightlifting, gymnastics, and field events [throwing]).The two primary factors determining the cardiovascular

isk of competitive sports are, clearly, the athlete’s abnor-ality and the stress under which it is placed by the sport.
his involves: 1) the specific cardiovascular diagnosis and its c
athophysiological consequences; and 2) the cardiovascularesponse to the demands of the sport during both compe-ition and training, which a competitive athlete in a sportay typically or reasonably be expected to undertake. The

tress of the sport involves both static and dynamic compo-ents that determine the cardiovascular demands of theport. Thus, for athletes with cardiovascular abnormalities,ecommendations regarding eligibility for competition willecognize these factors as well as the attendant psychologicaltresses that invariably accompany competitive athletics.he cardiovascular demands produced by training or com-etition in a particular sport involve the type, intensity, anduration of the activity, with both peak intensity and totalork performed as well as attendant neurohumoral effects

nd environmental factors.

ESPONSE AND ADAPTATION TO EXERCISE

he acute responses of the cardiovascular system to dynamicnd static exercise are summarized in Figure 1 (5,6).ynamic exercise performed with a large muscle mass

auses a marked increase in oxygen consumption (Fig. 1,anel A). There is a substantial increase in cardiac output,eart rate, stroke volume, and systolic blood pressure; aoderate increase in mean arterial pressure; and a decrease

n diastolic blood pressure. Also, there is a marked decreasen total peripheral resistance. Static exercise, in contrast,auses a small increase in oxygen consumption, cardiacutput, and heart rate, and no change in stroke volume (Fig., Panel B). Moreover, there is a marked increase in systolic,iastolic, and mean arterial pressure and no appreciablehange in total peripheral resistance. Thus, dynamic exerciserimarily causes a volume load on the left ventricle, whereastatic exercise causes a pressure load. The cardiovascularesponses during dynamic exercise of a small muscle mass atow resistance or during dynamic exercise of a large muscle

ass at high resistance are similar to the responses duringtatic exercise.

The acute response to both dynamic and static exercisehanges several factors that are important in determiningyocardial oxygen demand: heart rate, wall tension, and

ontractile state of the left ventricle (LV) (7,8). Wall tensions affected by pressure development and ventricular volume.n high-intensity dynamic exercise, there is a large increasen heart rate and an increase in stroke volume that ischieved by both an increase in end-diastolic volumeFrank-Starling mechanism) and a decrease in end-systolicolume (increased contractile state). In high-intensity staticxercise, a smaller increase occurs in heart rate and little
hange occurs in end- and end-systolic volumes of the LV.

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owever, arterial pressure and contractile state of theentricle are increased. Thus, both dynamic and staticxercise increase factors that are important in determiningyocardial oxygen demand.The chronic adaptation of the cardiovascular system to

epeated bouts of dynamic exercise (training) results in anncrease in maximal oxygen uptake (5,6). This is due to anncreased maximal stroke volume and arteriovenous oxygen

igure 1. Cardiovascular response to exercise. (A) Response to dynamicxercise of progressively increasing workload to maximal oxygen consump-ion. (B) Response to a static handgrip contraction at 30% maximaloluntary contraction. ABP (mm Hg) � systolic, mean and diastolicrterial blood pressures; HR (bpm) � heart rate (beats/min); Q (1/min) �ardiac output (liters/min); SV (ml/beat) � stroke volume; TPR (PRU) �otal peripheral resistance in peripheral resistance units; VO2 (ml/min/kg) �xygen consumption (ml/min � body weight in kg). Reprinted with permis-ion from Mitchell JH, Raven PB. Cardiovascular adaptation to physicalctivity. In: Bouchard C, Shephard RJ, Stephens T, editors. Physical Activity,itness and Health: International Proceedings and Consensus Statement (Fig.7.2). Champaign, IL: Human Kinetics Publishers. Copyright 1994 byuman Kinetics Publishers, Inc.

ifference. Athletes who train in sports with a high dynamic o

omponent have a large absolute LV mass and chamber sizeeccentric hypertrophy) (6,9,10). This eccentric hypertrophyevelops gradually and correlates with a high maximaltroke volume and high maximal oxygen uptake. Also, thekeletal muscles involved in the dynamic exercise trainingecome more oxidative and less glycolytic with an increasen the number and size of the mitochondria and anncreased number of capillaries. These changes contribute tohe larger maximal arteriovenous oxygen difference seen inndurance athletes.

The chronic adaptation of the cardiovascular system totatic exercise training results in little or no increase inaximal oxygen uptake. However, athletes who participate

n sports with a high static component also have a large LVass but no increase in chamber size (concentric hypertro-

hy) (6,9,10). In addition, the skeletal muscles involved inhe static exercise training become more glycolytic and lessxidative, and there is an increase in skeletal muscle massrimarily by fiber hypertrophy with a small degree ofyperplasia via stem cell activation.

THLETE’S HEART

s previously mentioned, participation in sports with a highynamic demand (endurance) or with a high static demandpower) causes an increased cardiac mass and structuralemodeling in many athletes. This finding has been shownn detail over three decades in a multitude of echocardio-raphic studies and more recently by cardiac magneticesonance imaging (6,9,10). The changes resulting fromraining include enlargement and increased volume of theight and LV chambers, sometimes accompanied by in-reased thickness of the LV wall, and increased size of theeft atrium, with preservation of systolic and diastolicunction. Extreme changes in cavity dimensions and LVall thickness are most commonly associated with training

n rowing, cross-country skiing, cycling, and swimming, butaradoxically are uncommon as a consequence of training inltra-endurance sports (10). The increased cardiac dimen-ions associated with athletic training are related to bodyurface area or lean body mass and consequently are lessronounced in women (10).Participation in sports with a high static demand (e.g.,

eightlifting or wrestling) is associated with LV wallhickness that is usually normal in absolute terms (less than2 mm) but disproportionately increased in relation toavity size. More substantial LV wall thickness (13 mm orore in men and 12 mm or more in women), occasionally

ncountered in competitive athletes, creates a differentialiagnosis with hypertrophic cardiomyopathy (9,10) (also seeask Force 1).

LASSIFICATION OF SPORTS

classification of sports is provided in Figure 2, whichelates individual competitive sports to the two general types
f exercise: dynamic and static (3–6). Each sport is catego-

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1366 Mitchell et al. JACC Vol. 45, No. 8, 2005Task Force 8: Classification of Sports April 19, 2005:1364–7

ized by the level of intensity (low, medium, high) ofynamic or static exercise generally required to perform thatport during competition. It also recognizes those sportshat pose significant risk due to bodily collision, eitherecause of the probability of hard impact between compet-tors or between a competitor and an object, projectile, orhe ground; as well as the degree of risk to the athlete orthers if a sudden syncopal event occurs. Thus, in terms ofheir dynamic and static demands, sports can be classifiedFig. 2) as IIIC (high static, high dynamic), IIB (moderatetatic, moderate dynamic), IA (low static, low dynamic), ando forth. For example, an athlete with a cardiovascularbnormality that contraindicates a sport that produces aigh pressure load on the LV may be advised to avoid sportslassified as IIIA, IIIB, and IIIC. It should be emphasizedhat in terms of the classification of sports matrix presentedn Figure 2, cardiovascular abnormalities designated asompatible with a high level of intensity in any particularategory also (by definition) permit participation in levels of

igure 2. Classification of sports. This classification is based on peak statowever, that higher values may be reached during training. The increasinxygen uptake (MaxO2) achieved and results in an increasing cardiac outputoluntary contraction (MVC) reached and results in an increasing blood prressure) are shown in green and the highest in red. Blue, yellow, and oemands. *Danger of bodily collision. †Increased risk if syncope occurs.

esser intensity. For example, if class IC sports are appro- n

riate (low static/high dynamic), then so are classes IA andB (low static/low and moderate dynamic).

The sports matrix in Figure 2 should not be regarded asrigid classification, but rather a spectrum in which some

thletes in the same sport could possibly deserve placementn different categories. Furthermore, some sports involveeterogeneity with respect to static and dynamic cardiovas-ular demands in either different athletic disciplines—suchs parallel bars and floor exercises in gymnastics or positionsuch as lineman and running back in football, or goalkeepernd mid-fielder in soccer. We have not formulated suchistinctions in the matrix, but these should be taken intoonsideration when making clinical decisions regarding theligibility and disqualification for competitive sports.

IMITATIONS OF CLASSIFICATION

here are important limitations to the present classificationf sports according to the type and intensity of exerciseerformed, as presented in Figure 2. For example, it does

dynamic components achieved during competition. It should be noted,amic component is defined in terms of the estimated percent of maximalincreasing static component is related to the estimated percent of maximalload. The lowest total cardiovascular demands (cardiac output and blooddepict low moderate, moderate, and high moderate total cardiovascular

ic andg dyn. Theessure

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1367JACC Vol. 45, No. 8, 2005 Mitchell et al.April 19, 2005:1364–7 Task Force 8: Classification of Sports

uring a competitive event, the effects of environmentalactors, electrolyte abnormalities, or the specific trainingegimen used by the athlete. Also, for team sports thelassification is based on the highest cardiovascular demandshat are experienced during competition and does notonsider the different cardiovascular demands of specificositions.During all athletic competitions, the athlete’s emotional

nvolvement can substantially increase sympathetic drive,nd the resulting catecholamine concentrations can increaselood pressure, heart rate, and myocardial contractility,hereby increasing myocardial oxygen demand. Also, thencrease in sympathetic tone can trigger arrhythmias andggravate existing myocardial ischemia. Thus, even in com-etitive sports such as golf or riflery, which have lowyocardial oxygen demands owing to the exercise required,

ubstantial increases may occur because of emotional in-olvement during competition. This problem is difficult (ifot impossible) to quantitate, but it needs to be considered

n determining the eligibility for sports participation ofthletes with existing cardiovascular abnormalities.

Environmental exposure during athletic competition orraining also needs to be considered. Performance at highltitudes or under water may decrease oxygen availability,hereas excessively hot or cold temperatures and highumidity can increase myocardial workload for the same

ntensity of exercise. Another potentially relevant environ-ental factor is air pollution, such as elevated carbononoxide levels in a sport such as auto racing.With the modern application of exercise science to

ompetitive sports, training for competition can, in fact, beore demanding on the cardiovascular system than the

ompetition itself. Many training regimens now use heavyesistance weight training (high static and low dynamicemand) for increasing strength and power in sports that doot include heavy static demands during competition (e.g.,ennis, basketball). This concept that both the dynamic andtatic demands of a sport may be greater during traininghan in competition must be seriously considered when theligibility of an athlete in a given sport is being determined.lso, in some cases where it is found acceptable for the

thlete to participate in the competitive aspect of a specificport but the existing training program is considered too
igorous, it may be possible to modify the training regimen i
o as to reduce the cardiovascular demands to an acceptableevel.

doi:10.1016/j.jacc.2005.02.015


1. Mitchell JH, Blomqvist CG, Haskell WL, et al. Classification ofsports. 16th Bethesda Conference: cardiovascular abnormalities in theathlete: recommendations regarding eligibility for competition. J AmColl Cardiol 1985;6:1198–9.

2. Mitchell JH, Haskell WL, Raven PB. Classification of sports. 26thBethesda Conference: cardiovascular abnormalities in the athlete:recommendations for determining eligibility for competition in ath-letes with cardiovascular abnormalities. J Am Coll Cardiol 1994;24:864–6.

3. Asmussen E. Similarities and dissimilarities between static and dy-namic exercise. Circ Res 1981;48 Suppl 1:I3–10.

4. Mitchell JH, Wildenthal K. Static (isometric) exercise and the heart:physiological and clinical considerations. Annu Rev Med 1974;25:369–81.

5. Mitchell JH, Raven PB. Cardiovascular adaptation to physical activity.In: Bouchard C, Shephard R, Stephen T, editors. Physical Activity,Fitness, and Health: International Proceedings and Consensus State-ment. Champaign, IL: Human Kinetics, 1994:286–98.

6. Gallagher KM, Raven PB, Mitchell JH. Classification of sports andthe athlete’s heart. In: Williams RA, editor. The Athlete and HeartDisease: Diagnosis, Evaluation and Management. Philadelphia, PA:Lippincott Williams & Wilkins, 1999:9–21.

7. Sonnenblick EH, Ross JJ, Braunwald E. Oxygen consumption of theheart: newer concepts of its multifactorial determination. Am J Cardiol1968;22:328–36.

8. Mitchell JH, Hefner LL, Monroe RG. Performance of the leftventricle. Am J Med 1972;53:481–94.

9. Pelliccia A, Maron BJ, Spataro A, Proschan MA, Spirito P. The upperlimit of physiologic cardiac hypertrophy in highly trained elite athletes.N Engl J Med 1991;324:295–301.


ppendix 1

r. Steven P. Van Camp declared that he served as anxpert witness for the following court cases which may beelated to the 36th Bethesda Conference: 2001—Defense,oung athlete with cardiomyopathy dying suddenly; 2002—efense, ephedra ingestion and exercise-related death;

003—Defense, two cases of ephedra ingestion andxercise-related death; 2005—Defense, ephedra ingestionnd exercise-related death; Defense, ephedra ingestion andyocardial infarction. The other authors of this report

eclared that they have no financial relationships with
ndustry or others pertinent to this topic.

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1368 Estes III et al. JACC Vol. 45, No. 8, 2005Task Force 9: Drugs and Performance-Enhancing Substances April 19, 2005:1368–9

ask Force 9: Drugs anderformance-Enhancing Substances. A. Mark Estes III, MD, FACC, Chair
obert Kloner, MD, PHD, FACC, Brian Olshansky, MD, FACC, Renu Virmani, MD, FACC
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thletes commonly use drugs and dietary supplementsecause they hope to improve athletic performance. Theseerformance-enhancing substances include ergogenic andhermogenic supplements, stimulants, anabolic steroids,eptide hormones, and others. Despite aggressive marketingnd user testimonials, scientific studies assessing the benefitsnd risks of any of these substances have not been conducted1–5). Clinical observations indicate some supplements mayave serious side effects including fatal adverse reactions (6–8).thletes should make informed decisions regarding the use ofrugs and dietary supplements with careful consideration ofhat is known and unknown. Health care professionals should

sk about drug and dietary supplements and serve as anducational resource for athletes and athletic organizations (9).

Many drugs and supplements are marketed to improvexercise duration or physical strength, to shorten recovery timerom exertion, to reduce fat, or in other ways to improvethletic performance (1–3,10–13). These substances includenabolic-androgenic steroids and the more than 30 natural andynthetic derivatives including tetrahydrogestrinone (THG).ndrostenedione, commonly referred to as “andro,” and dehy-roepiandrosterone (DHEA) are available in oral form and areold as nutritional supplements. Stimulants include amphet-mines, cocaine, dexadrine, ephedra, ritalin, beta-2 agonists,nd others (1–3,10–13). Peptide hormones and analogues,uch as recombinant erythropoietin (EPO), are used as aharmacologic alternative to “blood doping” or autotransfusion1–3,10–13). Human growth hormone (hCG), chorionic go-adotropin, pituitary and synthetic gonadotropins (LH), andorticotropins (ACTH, tetracosactide) also are used becausehey are believed to improve athletic performance (1–3,10–13).

Dietary supplements are products, other than tobacco,ontaining vitamins, minerals, amino acids, herbs, or otherotanical dietary substances (1–3,10–13). Some substancesuch as vitamins, minerals, bee pollen, caffeine, glycine,arnitine, lecithin, brewer’s yeast, gelatin, creatine, proteinupplements, and others probably have minimal toxicityhen used in recommended doses. Based on serious con-

erns regarding the safety of a popular energy drink withaurine, caffeine, and glucuronolactone, multiple Europeanountries have banned its sale (10). Like most dietaryupplements this drink has not been assessed for efficacy oroxicity in rigorous scientific studies (1–3,10–13).

Other banned drugs that are not considered performance-
nhancing would come under the designation of recreational
rugs (1–5,13). These include alcohol, cannaboids, seda-ives, narcotics, LSD, and others that have the potential tompair cognitive and physical function and are prohibited1–3,12,13). The dietary supplement ephedra (ma huang) isssociated with life-threatening toxicity and death resultingn a ban of its sale by the Food and Drug Administration6–8). Inadequate labeling and insufficient quality controln the production of many nutritional supplements are alsoeasons to recommend that athletes not take dietary supple-ents. Contamination or poor labeling of nutritional sup-

lements are not regarded as adequate defenses by athleticoverning bodies. Recognizing that there may be excep-ional circumstances where an athlete will require an oth-rwise prohibited substance for medical purposes, formalherapeutic exemption mechanisms are available with prioronsideration and approval (11–13).

Athletic governing bodies should provide comprehensiveists of prohibited drugs and dietary supplements (1,11–13).hey should develop a rigorous approach to preventerformance-enhancing and recreational drug and dietaryupplement use. The crucial elements of any programhould include education, counseling, treatment, detection,nd enforcement. Governing athletic bodies should use allvailable resources to enhance, supplement, and coordinatexisting efforts to educate athletes and reinforce the ethicalrinciples inherent in athletic participation. Without suchversight, the integrity of athletics is threatened. Ultimately,thletes must accept responsibility for the decisions theyake regarding the usage of drugs and performance-

nhancing substances.Athletes taking or considering the use of such substances

hould be aware that the safety and efficacy of supplementssed for improving athletic performance have not beenddressed in systematic scientific studies. Trainers, exercisehysiologists, sports nutritionists, athletic governing bodies,r medical organizations should discourage the use ofietary supplements by athletes. Serious side effects mayesult from the use of these substances, including cardiacypertrophy, myocyte necrosis, myocarditis, fibrosis, coro-ary thrombosis, and sudden death (even at recommendedosing) (2,3,6–8,10).

ecommendation:

. Athletes should have their nutritional needs met througha healthy, balanced diet without dietary supplements.

doi:10.1016/j.jacc.2005.02.016

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1369JACC Vol. 45, No. 8, 2005 Myerburg et al.April 19, 2005:1369–71 Task Force 10: Automated External Defibrillators

ASK FORCE 9 REFERENCES1. Estes NAM III, Link MS, Cannom D, et al. Report of the NASPE

policy conference on arrhythmias and the athlete. J CardiovascElectrophysiol 2001;12:1208–19.

2. Kloner RA. Illicit drug use in the athlete as a contributor to cardiacevents. In: Estes NAM III, Salem D, Wang P, editors. Sudden CardiacDeath in the Athlete. Armonk, NY: Futura Pub. Co., 1998:441–52.

3. Cregler LL. Substance abuse in sports: the impact of cocaine, alcohol,steroids, and other drugs on the heart. In: Williams R, editor. TheAthlete and Heart Disease: Diagnosis, Evaluation and Management.Philadelphia, PA: Lippincott Williams & Wilkins, 1998:131–54.

4. DeAngelis CD, Fontanarosa PB. Drugs alias dietary supplements.JAMA 2003;290:1519–20.

5. Stout CW, Weinstock J, Homoud MK, Wang PJ, Estes NAM III,Link MS. Herbal medicine: beneficial effects, side effects, and prom-ising new research in the treatment of arrhythmias. Curr Cardiol Rep2003;5:395–401.

6. Samenuk D, Link MS, Homoud MK, et al. Adverse cardiovascularevents temporally associated with ma huang, an herbal source ofephedrine. Mayo Clin Proc 2002;77:12–6.

7. U.S. Food and Drug Administration. FDA announces plans to prohibit

ouglas P. Zipes, MD, MACC

isproportionately higher-risk subset, compared to the gen-

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sale of dietary supplements containing ephedra. December 30, 2003.Available at: http://www.fda.gov/oc/initiatives/ephedra/february2004/.Accessed October 1, 2004.

8. U.S. Food and Drug Administration. RAND report. Available at:http://www.fda.gov/OHRMS/DOCKETS/98fr/95n-0304-bkg0003-ref-07-01-index.htm. Accessed October 1, 2004.

9. Maron BJ, Thompson PD, Puffer JC, et al. Cardiovascular prepartici-pation screening of competitive athletes: a statement for healthprofessionals from the Sudden Death Committee (clinical cardiology)and Congenital Cardiac Defects Committee (cardiovascular diseasein the young), American Heart Association. Circulation 1996;94:850 – 6.

0. Ireland to review safety of energy drinks. Available at: http://www.foodanddrinkeurope.com/news/ng.asp?id�49957. Accessed October 1,2004.

1. The National Center for Drug Free Sport, Inc. Nutritional supple-ments. Available at: http://www.drugfreesport.com/choices/supplements/index.html. Accessed October 1, 2004.

2. NCAA Web site. Drug testing policy. Available at: http://www1.ncaa.org/membership/ed_outreach/health-safety/drug_testing/index.html. Accessed October 1, 2004.

3. World Anti-Doping Agency. Available at: http://www.wada-ama.org.Accessed November 30, 2004.


Name Consultant Research Grant Scientific Advisory Board Speakers’ Bureau




None

r. Robert Kloner ● Bayer GSK ● CV Therapeutics ● Bayer GSK ● Bayer GSK● Lilly ICOS ● Lilly ICOS ● Lilly ICOS ● Lilly ICOS● Pfizer ● McNeill ● Pfizer ● Pfizer● Schering Plough

r. Brian Olshansky ● Guidant ● Medtronic None ● Astra Zeneca● Medicorp ● Baxter

● Bayer GSK● Reliant Pharmaceutical

r. Renu Virmani None None None None

ask Force 10: Automated External Defibrillatorsobert J. Myerburg, MD, FACC, Chair. A. Mark Estes III, MD, FACC, John M. Fontaine, MD, FACC, Mark S. Link, MD, FACC,

ENERAL CONSIDERATIONS OFARDIAC ARREST RISK AMONG ATHLETES

he incidence of out-of-hospital cardiac arrest among theeneral population of adults is 1 to 2 deaths per 1,000ubjects per year; a figure that represents 50% of allardiovascular deaths (1). For the adolescent and youngdult subgroups, the estimated incidence is 1 per 100,000er year or less. Available data suggest that among theounger population, competitive athletes account for a

ral population in a comparable age group (2–4). Inddition, among the adult population performing condi-ioning activities in health clubs, the probability of cardiacrrest during exertion appears higher than the expected rateor comparable groups generally, especially among personsho exercise despite being habitually sedentary (5).Within the subgroup of the population age 35 years and

lder, coronary heart disease accounts for approximately0% of all sudden cardiac deaths (SCDs), with the cardio-yopathies accounting for another 10% to 15%. In the

ounger age groups, hypertrophic cardiomyopathy, anoma-

http://www.fda.gov/oc/initiatives/ephedra/february2004/

http://www.fda.gov/oc/initiatives/ephedra/february2004/

http://www.fda.gov/OHRMS/DOCKETS/98fr/95n-0304-bkg0003-ref-07-01-index.htm

http://www.fda.gov/OHRMS/DOCKETS/98fr/95n-0304-bkg0003-ref-07-01-index.htm

http://www.foodanddrinkeurope.com/news/ng.asp?id=49957

http://www.foodanddrinkeurope.com/news/ng.asp?id=49957

http://www.drugfreesport.com/choices/supplements/index.html

http://www.drugfreesport.com/choices/supplements/index.html

http://www1.ncaa.org/membership/ed_outreach/health-safety/drug_testing/index.html



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ous coronary arteries, myocarditis, and various other inher-ted disorders that are associated with arrhythmic riskominate the etiologies (1). Although the absolute risk forhe young athlete remains low, the excess compared to theeneral population in their age group and the life expectancyssociated with the underlying diseases in the absence of aardiac arrest, suggests the need for aggressive approaches tohe recognition of individuals at risk, and for systems toespond to unexpected events.

OLE OF AUTOMATED EXTERNALEFIBRILLATORS IN RESPONSE TO CARDIAC ARREST

n attacking the problem of SCD, it is unrealistic to assumehat any single approach—epidemiological surveillance, pri-ary prevention of disease states, clinical interventions of

stablished diseases, or community-based response sys-ems—will have a major impact alone. Each strategy has aole, in part because a large majority of events occurnexpectedly in the out-of-hospital environment and areot predictable with great accuracy by risk profiling in mostlinical circumstances (6).

Because the majority of out-of-hospital cardiac arrestsccur by the initial mechanism of ventricular fibrillation,ommunity-based defibrillation strategies have emerged asne approach to the SCD problem. Time to defibrillation ishe most important factor in survival from out-of-hospitalardiac arrest due to ventricular fibrillation (7). Thus,ommunity deployment of rapid access systems has beenvolving since the late 1960s, when portable defibrillatorsnitially became available. The first systems were fireepartment-based paramedical programs, and were fol-

owed years later by the placement of automated externalefibrillators (AEDs) in the hands of non-conventionalrained responders, such as security guards, police, and flightttendants (8–15). Most recently, defibrillators were madevailable to minimally trained or untrained lay responders inublic locations such as airports, commercial aircraft, super-arkets, stadiums, and hospitals (14–17), and have also

een suggested for private residences or neighborhoods (18).Although survival rates from out-of-hospital cardiac ar-

est remain far lower than desirable, the various out-of-ospital response strategies have improved the survival rates,

argely based on more rapid response times. Those settingsnd strategies that provide response times from witnessednset to initial defibrillation of less than 2 to 3 min canrovide survival outcomes hovering about 50% (16); butates fall sharply with each passing minute thereafter. By 4o 5 min, survival is 25% or less, and less than 10% after 10in (19). As a consequence, despite the apparent value of

onventional emergency medical systems and other vehicle-ased systems such as police responders, additional publicccess systems are anticipated to provide even faster accesso defibrillation. Such systems are being deployed and tested20,21). Each has potential or demonstrates added benefit
o survival rates.
EDs AT SITES OF TRAINING AND COMPETITIONmong young athletes, cardiac arrests generally occur duringr shortly after intense training sessions or competition. Al-hough the incidence of cardiac arrest is extremely low (ap-roximately 1% of that reported in middle-age and older adultopulations), the value of prompt and successful resuscitationnd long-term survival is enhanced by the potential of ex-ended life over many decades (i.e., much longer than is thease for older adults, among whom the extent of underlyingisease results in substantial risk over shorter time periods).hus, it is considered reasonable to have an AED available forse at educational facilities, training centers, and sports arenasnd stadiums, in addition to trained responders among the stafft each (5,21). The AEDs should be deployed in a distributionhat can achieve an anticipated response time of 5 min or less22). When the time from loss of consciousness to availabilityf an AED is 5 min or longer, 30 to 60 s of CPR prior to therst attempt to defibrillate has been shown to provide a survivalenefit (23).

Although there are only anecdotal observations supportinghe feasibility and impact of this strategy, the rationale is clearnd should be promoted. In addition, it should be recognizedhat the availability of AEDs during competitive sportingvents also provides the potential for life-saving support topectators and other bystanders. Nonetheless, the availability ofn AED at a sporting event should not be construed asbsolute protection against a fatal outcome from a cardiacrrest. Neither should it supersede restrictions against partici-ation in competitive sports, based upon underlying cardiacbnormalities, as defined in this document.

ecommendations:

. The AEDs should be available at educational facili-ties that have competitive athletic programs (includ-ing intramural sports and conditioning classes), sta-diums, arenas, and training sites, with trainedresponders identified among the permanent staff.Devices should be deployed so as to provide aresponse time of less than 5 min.

. The initial response to a suspected or identified cardiacarrest should be to contact emergency medical services(e.g., 9-1-1), followed immediately by, or concomitantwith, initiating CPR and deploying the AED.

doi:10.1016/j.jacc.2005.02.017

ASK FORCE 10 REFERENCES1. Myerburg RJ, Castellanos A. Cardiac arrest and sudden cardiac death.

In: Zipes DP, Libby P, Bonow RO, Braunwald E, editors. Braun-wald’s Heart Disease: A Textbook of Cardiovascular Medicine. 7thedition. Philadelphia, PA: WB Saunders Company, 2005:865–908.

2. Thiene G, Basso C, Corrado D. Is prevention of sudden death inyoung athletes feasible? Cardiologia 1999;44:497–505.

3. Corrado D, Basso C, Schiavon M, Thiene G. Screening for hypertrophiccardiomyopathy in young athletes. N Engl J Med 1998;339:364–9.


5. Albert CM, Mittleman MA, Chae CU, Lee IM, Hennekens CH,
Manson JE. Triggering of sudden death from cardiac causes byvigorous exertion. N Engl J Med 2000;343:1355–61.

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1371JACC Vol. 45, No. 8, 2005 Maron et al.April 19, 2005:1371–3 Task Force 11: Commotio Cordis

6. Myerburg RJ. Sudden cardiac death: exploring the limits of ourknowledge. J Cardiovasc Electrophysiol 2001;12:369–81.

7. Cobb LA, Weaver WD, Fahrenbruch CE, Hallstrom AP, CopassMK. Community-based interventions for sudden cardiac death: im-pact, limitations, and changes. Circulation 1992;85:I98–102.

8. Mosesso VN Jr., Davis EA, Auble TE, Paris PM, Yealy DM. Use ofautomated external defibrillators by police officers for treatment ofout-of-hospital cardiac arrest. Ann Emerg Med 1998;32:200–7.

9. White RD, Hankins DG, Bugliosi TF. Seven years’ experience withearly defibrillation by police and paramedics in an emergency medicalservices system. Resuscitation 1998;39:145–51.

0. Kette F, Sbrojavacca R, Rellini G, et al. Epidemiology and survival rateof out-of-hospital cardiac arrest in north-east Italy: the F.A.C.S.study: Friuli Venezia Giulia Cardiac Arrest Cooperative Study. Re-suscitation 1998;36:153–9.

1. Newman MM, Mosesso VN Jr., Ornato JP, et al. Law EnforcementAgency Defibrillation (LEA-D): position statement and best practicesrecommendations from the National Center for Early Defibrillation.Prehosp Emerg Care 2002;6:346–7.

2. Myerburg RJ, Fenster J, Velez M, et al. Impact of community-widepolice car deployment of automated external defibrillators on survivalfrom out-of-hospital cardiac arrest. Circulation 2002;106:1058–64.

3. Capucci A, Aschieri D, Piepoli MF, Bardy GH, Iconomu E, ArvediM. Tripling survival from sudden cardiac arrest via early defibrillationwithout traditional education in cardiopulmonary resuscitation. Cir-culation 2002;106:1065–70.

4. O’Rourke MF, Donaldson E, Geddes JS. An airline cardiac arrest

. A. Mark Estes III, MD, FACC, Mark S. Link, MD,

ubject to another risk for sudden death that occurs in the

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5. Page RL, Joglar JA, Kowal RC, et al. Use of automated externaldefibrillators by a U.S. airline. N Engl J Med 2000;343:1210–6.

6. Valenzuela TD, Roe DJ, Nichol G, Clark LL, Spaite DW, HardmanRG. Outcomes of rapid defibrillation by security officers after cardiacarrest in casinos. N Engl J Med 2000;343:1206–9.

7. Caffrey SL, Willoughby PJ, Pepe PE, Becker LB. Public use ofautomated external defibrillators. N Engl J Med 2002;347:1242–7.

8. Zipes DP. President’s page: the neighborhood health watch program:Save A Victim Everywhere (SAVE). J Am Coll Cardiol 2001;37:2004–5.

9. Holmberg M, Holmberg S, Herlitz J. Incidence, duration and survivalof ventricular fibrillation in out-of-hospital cardiac arrest patients inSweden. Resuscitation 2000;44:7–17.

0. Nichol G, Hallstrom AP, Kerber R, et al. American Heart Associationreport on the Second Public Access Defibrillation Conference, April17–19, 1997. Circulation 1998;97:1309–14.

1. Marenco JP, Wang PJ, Link MS, Homoud MK, Estes NA III.Improving survival from sudden cardiac arrest: the role of the auto-mated external defibrillator. JAMA 2001;285:1193–200.

2. Hazinski MF, Markenson D, Neish S, et al. Response to cardiac arrestand selected life-threatening medical emergencies: the medical emer-gency response plan for schools: a statement for healthcare providers,policymakers, school administrators, and community leaders. Circula-tion 2004;109:278–91.

3. Wik L, Hansen TB, Fylling F, et al. Delaying defibrillation to givebasic cardiopulmonary resuscitation to patients with out-of-hospital

program. Circulation 1997;96:2849–53. ventricular fibrillation: a randomized trial. JAMA 2003;289:1389–95.


Name ConsultantResearch

GrantScientific

Advisory Board Stock Holder Expert Witness Testimony




None None

r. John M. Fontaine None None None None None

r. Mark S. Link None ● Guidant● Medtronic

None None None

r. Robert J. Myerburg ● Guidant None ● Procter & Gamble None ● 2000, Defense, Lewis vs. Mudge● Procter & Gamble ● Reliant Pharmaceutical ● 2002, Defense, Weiner vs. Vitello

● 2005, Defense, Ephedra Multi-District Litigation


● Medtronic ● Medtronic ● MVMD ● 1996, Defense, Knapp vs. Northwestern

ask Force 11: Commotio Cordisarry J. Maron, MD, FACC, Chair

FACC

ENERAL CONSIDERATIONSudden and unexpected deaths of young athletes are mostrequently the consequence of unsuspected cardiovasculariseases (1). However, participants in organized sports are also

bsence of cardiovascular disease—namely, blunt, non-enetrating, and usually innocent-appearing chest blows, trig-ering ventricular fibrillation unassociated with structuralamage to the ribs, sternum, or heart itself (commotio cordis)
2,3). Although the precise incidence during competitive

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1372 Maron et al. JACC Vol. 45, No. 8, 2005Task Force 11: Commotio Cordis April 19, 2005:1371–3

nd recreational sports is unknown, commotio cordis eventsay be a more frequent cause of sudden death than

reviously believed, and also more common than many ofhe cardiovascular diseases that cause these catastrophes (1).

Knowledge of the demographics and clinical profile ofommotio cordis is based largely on information from the.S. Registry (Minneapolis, Minnesota) (2,3). Precordiallows that cause commotio cordis usually are not perceivedo be unusual for the sport or activity involved, nor ofufficient magnitude to trigger arrhythmic sudden death.lthough reported at a wide range of ages (3 months to 50

ears), commotio cordis has a predilection for children anddolescents (mean age 13 years) probably because the youngharacteristically have narrow, pliable chest walls that facil-tate transmission of energy from the chest impact to the

yocardium.Commotio cordis occurs in a wide variety of sports, butost commonly youth baseball (and softball), ice hockey,

ootball, and lacrosse, with death often caused by projectileshat are implements of the competition. Although blows areypically of low energy, projectiles may strike the precor-ium with a range of velocities—paradoxically, and mostommonly, with only modest force such as a pitchedaseball striking a batter at 30 to 40 mph, but also withigh-velocity blows from hockey pucks or lacrosse balls (upo an estimated 90 mph), and frequently with bodily contacto the precordium such as by karate blows or collisionsetween outfielders tracking a baseball in-flight. Collapsean be instantaneous or preceded by brief periods ofonsciousness and physical activity. Despite a structurallyormal heart, survival from commotio cordis is uncommoni.e., only 15%). However, survival from commotio cordisas been reported with increasing frequency associated withrompt cardiopulmonary resuscitation and defibrillation (3).urvivors of commotio cordis should undergo a completeardiac evaluation including electrocardiogram (ECG),olter ambulatory monitoring, echocardiogram, and possi-

ly cardiac catheterization to exclude underlying structuralardiac abnormalities.

Also, many deaths from commotio cordis occur aroundhe home or on the playground in informal activities relatedr unrelated to recreational sports (often involving closeelatives) in which the chest impact is delivered in annnocent fashion; for example, such events have occurred asresult of light blows during playful “shadow boxing” or asremedy for hiccups (3). Unfortunately, some commotio

ordis events have even triggered criminal convictions foranslaughter or murder (4).A swine model that replicates commotio cordis has

rovided important insights into the mechanisms responsi-le for the devastating electrophysiologic consequences ofhese precordial blows (5–7). Determinants of ventricularbrillation following a chest blow include impact deliveredt a wide range of velocities directly over the heart, andiming within a narrow 15-to-30-ms window just prior to
he T-wave peak during the vulnerable phase of repolariza-
ion (representing only 1% of the cardiac cycle) (5–8). Theequirement for such an exquisite confluence of circum-tances may largely explain the uncommon occurrence ofommotio cordis.

In addition, spontaneously aborted commotio cordisvents may occasionally result from blows sustained duringhe QRS complex (depolarization), which trigger transientomplete heart block or nonsustained polymorphic ventric-lar tachycardia in the animal model (5). Basic cellularechanisms responsible for commotio cordis are incom-

letely understood, although selective activation of K�ATP

hannels may play a pivotal role (9).Several strategies for prevention of commotio cordis

vents, including innovations in the design of sports equip-ent, have been considered. Softer-than-normal (“safety”)

aseballs reduce risk for ventricular fibrillation under labo-atory conditions (5,7). Although such projectiles do notrovide absolute protection from sudden death on theaseball field (3), nevertheless there is sufficient reason toncourage the use of such baseballs in organized play atppropriate ages (10). Chest barriers with proven efficacy forouth sports (e.g., baseball, lacrosse, and hockey) have notet been developed, and many of the commercially availableroducts offer no or only incomplete protection againstrovoked arrhythmias (3,11). The continued reports ofommotio cordis events during organized and recreationalports emphasize the importance of more timely resuscita-ive efforts, including immediate access to automated exter-al defibrillators (AEDs) (12,13), and also development ofreventive strategies including design of effective chestarriers (11).

ecommendations:

. Age-appropriate safety baseballs are recommendedfor use in children up to 13 years of age.

. Although chest wall protectors may prevent traumaticinjury in goalies and baseball catchers, insufficient evi-dence is available to recommend universal use of com-mercially available chest barriers for all participants insports, specifically to prevent commotio cordis events.

. AEDs should be available within 5 min after partic-ipant collapse at sporting events.

. Survivors of a commotio cordis with ventricularfibrillation (or a presumed aborted event withoutdocumented ventricular fibrillation) should undergoa thorough cardiac evaluation, including at least12-lead ECG, ambulatory Holter monitoring, andechocardiogram. Standard electrophysiologic testingand an implantable cardioverter-defibrillator are notusually recommended.

. Because data are lacking with regard to the suscepti-bility for recurrent events, eligibility for returning tocompetitive sports in survivors is at present a decisionleft to individual clinical judgment.

doi:10.1016/j.jacc.2005.02.018

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1373JACC Vol. 45, No. 8, 2005 Mitten et al.April 19, 2005:1373–5 Task Force 12: Legal Aspects



2. Maron BJ, Poliac LC, Kaplan JA, Mueller FO. Blunt impact to thechest leading to sudden death from cardiac arrest during sportsactivities. N Engl J Med 1995;333:337–42.

3. Maron BJ, Gohman TE, Kyle SB, Estes NAM III, Link MS. Clinicalprofile and spectrum of commotio cordis. JAMA 2002;287:1142–6.

4. Maron BJ, Mitten MJ, Greene BC. Criminal consequences of com-motio cordis. Am J Cardiol 2002;89:210–3.

5. Link MS, Wang PJ, Pandian NG, et al. An experimental model ofsudden death due to low-energy chest-wall impact (commotio cordis).N Engl J Med 1998;338:1805–11.

6. Link MS, Maron BJ, VanderBrink BA, et al. Impact directly over the car-diac silhouette is necessary to produce ventricular fibrillation in an experi-mental model of commotio cordis. J Am Coll Cardiol 2001;37:649–54.

7. Link MS, Maron BJ, Wang PJ, Pandian NG, VanderBrink BA, Estes

arry J. Maron, MD, FACC, Douglas P. Zipes, MD, M

icipation recommendation for an athlete with a cardiovas-

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8. Link MS, Maron BJ, Wang PJ. Upper and lower limits of vulnerabilityto sudden arrhythmic death with chest wall impact (commotio cordis).J Am Coll Cardiol 2003;41:99–104.

9. Link MS, Wang PJ, VanderBrink BA, et al. Selective activation of theK(�)(ATP) channel is a mechanism by which sudden death isproduced by low-energy chest-wall impact (commotio cordis). Circu-lation 1999;100:413–8.

0. Kyle SB. Youth Baseball Protective Equipment Project Final Report.United States Consumer Product Safety Commission. Washington,DC: 1996.

1. Weinstock J, Maron BJ, Song C, Mane PP, Estes NAM III, LinkMS. Commercially available chest wall protectors fail to preventventricular fibrillation induced by chest wall impact (commotio cordis)(abstr). Heart Rhythm 2004;1:692.

2. Strasburger JF, Maron BJ. Images in clinical medicine: commotiocordis. N Engl J Med 2002;347:1248.

3. Link MS, Maron BJ, Stickney RE, et al. Automated external defibril-lator arrhythmia detection in a model of cardiac arrest due to
NAM III. Reduced risk of sudden death from chest wall blows
(commotio cordis) with safety baseballs. Pediatrics 2002;109:873–7. commotio cordis. J Cardiovasc Electrophysiol 2003;14:83–7.


Name Consultant Research Grant Scientific Advisory Board Expert Witness Testimony

r. N. A. Mark Estes III ● Guidant ● Guidant ● Guidant (Executive Committee) None● Medtronic ● Medtronic

r. Mark S. Link None ● Guidant None None● Medtronic

r. Barry J. Maron None ● Medtronic None ● 1996, Defense, Knapp vs. Northwestern

ask Force 12: Legal Aspects of the6th Bethesda Conference Recommendationsatthew J. Mitten, JD, Chair

ACC


n 1994, when the 26th Bethesda Conference recommenda-ions were formulated, no court had yet considered whether anthlete with a cardiovascular abnormality could be involuntarilyxcluded from a competitive sport if physicians disagreed inheir participation recommendations (1–3). However, newata have subsequently become available, and several highlyisible cases involving the sudden deaths of elite competitivethletes (4,5) have brought medical-legal and liability consid-rations into prominent focus. A 1996 lawsuit brought by atudent-athlete claiming the legal right to play intercolle-iate basketball contrary to a university team physician’sedical recommendation has established a developing legal

ramework for medical decisions regarding the eligibility orisqualification of trained athletes with cardiovascular dis-ase to participate in competitive sports. In this case, Knapps. Northwestern University (6,7), a federal appellate courtecognized the appropriateness of a physician’s reliance onurrent consensus medical guidelines when making a par-

ular abnormality. Consequently, judicial precedent nowrovides some guidance regarding the role of the present6th Bethesda Conference recommendations in resolvingegal issues relating to athletic participation disputes.

In the Knapp case, the court upheld Northwesternniversity’s legal right to accept its team physician’s recom-endation, which was consistent with the then-current

6th Bethesda Conference guidelines, to medically disqual-fy a student-athlete from playing college basketball (6).s a high school senior, Nicholas Knapp suffered sudden

ardiac arrest while playing in an informal basketballame, which required cardiopulmonary resuscitation andefibrillation to restore sinus rhythm. Thereafter, he had aardioverter-defibrillator (ICD) implanted. He resumedlaying recreational basketball without any subsequent car-iovascular events, and three cardiologists medically clearedim to play college basketball.Knapp had received a full athletic scholarship at North-

estern University to play intercollegiate basketball. While
orthwestern honored Knapp’s scholarship, it barred him

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1374 Mitten et al. JACC Vol. 45, No. 8, 2005Task Force 12: Legal Aspects April 19, 2005:1373–5

rom playing on its basketball team based on the teamhysician’s medical recommendation. The team physicianonsidered Knapp’s medical records and history, the 26thethesda Conference recommendations (1), and the opin-

ons of two consulting cardiologists who concluded thatnapp would expose himself to a medically unacceptable

isk for ventricular fibrillation during competitive athletics.All medical experts agreed on the following facts: 1)

napp had suffered a cardiac arrest; 2) even with the ICD,laying college basketball placed Knapp at a higher risk ofudden cardiac death as compared to other male collegeasketball players; 3) the ICD had never been tested underhe conditions of intercollegiate basketball; and 4) no personurrently played or had ever played college or professionalasketball after having an ICD implanted. However, thexperts disagreed whether Knapp should be medically dis-ualified from playing intercollegiate basketball.The trial court ruled that Northwestern should restore

napp’s eligibility to play on its basketball team andandated a courtside defibrillator and cardiologist to be

resent at all games and practices. However, the appealsourt overruled that decision and held that a university haslegal right to establish legitimate physical qualifications for

ts intercollegiate athletes. Northwestern did not violate theehabilitation Act of 1973, a federal law prohibiting dis-

rimination against persons with disabilities, by followingts team physician’s reasonable medical advice. An athlete

ay be medically disqualified and excluded from a sport ifecessary to avoid an enhanced risk of death or serious injuryuring competitive athletics that cannot be eliminated throughhe use of medication, monitoring, or protective equipment.he court explained that Northwestern’s decision to excludenapp from its basketball team was legally justified:

“We do not believe that, in cases where medical expertsdisagree in their assessment of the extent of a real risk ofserious harm or death, Congress intended that the courts—neutral arbiters but generally less skilled in medicine thanthe experts involved—should make the final medical deci-sion. Instead, in the midst of conflicting expert testimonyregarding the degree of serious risk of harm or death, thecourt’s place is to ensure that the exclusion or disqualifica-tion of an individual was individualized, reasonably made,and based upon competent medical evidence. . . . [W]e wishto make clear that we are not saying Northwestern’s decisionis necessarily the right decision. We say only that it is not anillegal one under the Rehabilitation Act” (6).

napp eventually left Northwestern and pursued collegiateasketball at another university where the team physicianleared him to play. Shortly thereafter, his ICD delivered anpparently appropriate shock during a basketball game.

The present 36th Bethesda Conference recommenda-ions update the 26th Bethesda Conference guidelines of994 (1) (which modified the 16th Bethesda Conferenceuidelines of 1984), taking into account the most recent and
elevant developments in the diagnosis and management of fi
ardiovascular disease. These new guidelines represent theost current consensus opinion of a distinguished group of

ardiologists regarding the medical risks of participation inompetitive sports by athletes with cardiovascular abnor-alities. Thus, we anticipate that the 36th Bethesda Con-

erence recommendations will be recognized and acceptedy physicians but also by the legal community and courts, ashe most contemporary consensus opinion of a distin-uished expert panel of cardiologists regarding medicalligibility and disqualification recommendations for com-etitive athletes with cardiovascular disease.The Knapp case recognized the appropriateness of phy-

ician reliance on current consensus guidelines in makingedical clearance recommendations. However, the court

id not rule that the 26th Bethesda Conference guidelinesould always be legally determinative in resolving athleticarticipation disputes involving athletes with cardiovascularbnormalities. Therefore, consistent with legal precedent es-ablished by Knapp vs. Northwestern University, a physicianay justifiably consider and rely upon the updated 36thethesda Conference recommendations in making medicalligibility recommendations for competitive athletes with car-iovascular disease. Nevertheless, the law continues to requirehat these recommendations be applied on an individualizedasis rather than used to exclude categorically all competitivethletes who have a particular cardiovascular abnormality.

The Knapp case establishes an important precedentegarding the medical exclusion of college and high schoolthletes with cardiovascular disease from intercollegiate andnterscholastic athletics, for whom sports is an avocation orxtracurricular activity incidental to one’s education (6,7). Its presently uncertain whether this same legal frameworkill be applied to resolve future participation disputes that

nvolve professional athletes (for whom sports is an income-enerating livelihood) (8). However, it is notable that the.S. Supreme Court recently held that an enhanced risk of

ignificant harm to personal health is a legitimate ground forxclusion from employment (9), which suggests that theegal framework developed in the Knapp case may bepplied to professional sports.

Currently there is no well-defined legal precedent regard-ng a physician’s potential malpractice liability for medicallylearing an athlete with a cardiovascular abnormality toarticipate in a competitive sport contrary to consensusecommendations (10). The law generally requires a physi-ian to have and use the current knowledge, skill, and carerdinarily possessed and employed by members of theedical profession in good standing. The applicable legal

tandard of physician conduct is “good medical practice”ithin the physician’s area of specialty practice, whichepending on the jurisdiction means either “reasonable,”customary,” or “accepted” medical care under the circum-tances (10). This general standard applies to physiciansho provide cardiovascular medical treatment to a compet-

tive athlete, including evaluation of his or her medical
tness to participate in a sport.

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1375JACC Vol. 45, No. 8, 2005 Mitten et al.April 19, 2005:1373–5 Task Force 12: Legal Aspects

Courts generally have recognized “guidelines” establishedy national medical associations as evidence of good medicalractice, but they are not conclusive evidence of the stan-ard of care (11–13). Indeed, consistent with the require-ents of the federal disability discrimination laws as inter-

reted in the Knapp case, it is important to emphasize thathe Bethesda Conference recommendations permit thexercise of a physician’s medical judgment in individualases. The recommendations do not, per se, rigidly restrictlinical practice or medical decision making. A clinician hashe flexibility to deviate from the recommendations if he orhe believes it is in the best interests of a patient-athlete toeach an alternative decision and strategy.

The controlling legal issue is whether adherence to (oreviation from) consensus recommendations is consistentith reasonable, customary, or accepted medical practice in

n individual patient’s case. Although the recommendationsf the 36th Bethesda Conference do not represent formaluidelines endorsed by the American College of Cardiol-gy, they are well-considered views of a group of expertsonvened to address the medical risks imposed by compe-ition on an athlete with a cardiovascular abnormality.herefore, deviations from the 36th Bethesda Conference

ecommendations that are nevertheless consistent with goodedical practice and are protective of an athlete’s health may be

ppropriate in particular cases and do not necessarily createhysician liability for medical malpractice. Conversely, com-liance with the 36th Bethesda Conference recommendations

equirement, and in future legal disputes may form the basis ofsuccessful defense against allegations of malpractice (14).

doi:10.1016/j.jacc.2005.02.019


1. Maron BJ, Mitchell JH. 26th Bethesda Conference: recommendationsfor determining eligibility for competition in athletes with cardiovas-cular abnormalities. J Am Coll Cardiol 1994;24:845–99.

2. Maron BJ, Brown RW, McGrew CA, Mitten MJ, Caplan AL, HutterAM Jr. Ethical, legal, and practical considerations affecting medicaldecision-making in competitive athletes. J Am Coll Cardiol 1994;24:854–60.

3. Mitten MJ, Maron BJ. Legal considerations that affect medicaleligibility for competitive athletes with cardiovascular abnormalitiesand acceptance of Bethesda Conference recommendations. J Am CollCardiol 1994;24:861–3.

4. Maron BJ. Sudden death in young athletes: lessons from the HankGathers affair. N Engl J Med 1993;329:55–7.


6. 101 F. 3d 473 (7th Cir. 1996), cert. denied, 520 U.S. 1274 (1997).7. Maron BJ, Mitten MJ, Quandt EF, Zipes DP. Competitive athletes

with cardiovascular disease—the case of Nicholas Knapp. N EnglJ Med 1998;339:1632–5.

8. Mitten MJ. Enhanced risk of harm to one’s self as a justification forexclusion from athletics. Marq Sports L J 1998;8:189–223.

9. Chevron U.S.A. Inc. vs. Echazabal, 536 U.S. 73 (2002).0. Mitten MJ. Emerging legal issues in sports medicine: a synthesis,

summary, and analysis. St John’s L Rev 2002;76:5–86.1. Stone vs. Proctor, 131 S.E.2d 297, 299 (N.C. 1963).2. Pollard vs. Goldsmith, 572 P.2d 1201, 1203 (Ariz. Ct. App. 1977).3. Swank vs. Halivopoulos, 260 A.2d 240, 242-43 (N.J. Super. Ct. App.

Div. 1969).

s some evidence that a physician has satisfied this legal14. Mitten M. Team physicians and competitive athletes: allocating legal

responsibility for athletic injuries. Univ Pitt L Rev 1993;55:129–60.



Advisory Board Stock HolderExpert Witness

Testimony

r. Barry J. Maron None ● Medtronic None None ● 1996, Defense, Knappvs. Northwestern

r. Matthew J. Mitten None None None None None


● Medtronic ● Medtronic ● MVMD ● 1996, Defense, Knappvs. Northwestern