Thomas W.Rowland,M.D..
Department of Pediatrics
Baystate Medical Center
Springfield,Massachusetts

KEY POINTS

■The risk ofsudden cardiac death in an apparently healthy young athlete is extraordinarilysmall.Approximately 10-13 such cases are reported annually in the United States.Evenassuming this represents under-reporting,the chance of such a tragedy in a given athleteis probably less than 1∶250,000.
■Four cardiovascularabnormalities account for the great majority of reported cases of sudden cardiac death inyoung athletes:hypertrophic cardiomyopathy(a pathologically enlarged heart),congenital(inherited) abnormalities of the coronary arteries,dissecting aortic aneurysms (bulging ofthe aorta with blood passing between layers of the arterial wall) in athletes with Marfansyndrome(a congenital disorder of connective tissue),and congenital stenosis(narrowing)ofthe heart valve leading to the aorta.
■Routine physicalexamination during the pre-season medical evaluation can detect those with Marfan syndromeand aortic stenosis,and athletes with hypertrophic cardiomyopathy and coronary arteryabnormalities may give a history of chest pain or fainting with exercise. Athletes withthe latter two conditions,however,often go undetected.
■Additionaldiagnostic procedures such as echocardiography and electrocardiography are not recommendedfor routine screening of athletes because the techniques are not cost-effective and have ahigh rate of false positive findings.
■Futuredevelopments,including genetic screening and the availability of defibrillators at thesport site,may decrease the incidence of sudden death.The current limitation in ourability to identify athletes at risk necessitates acceptance that participation in sportscarries some risk.

INTRODUCTION

Thesudden cardiac-related death of an athlete during a sports event is a particularlypoignant tragedy.Participation in sports--a wholesome pursuit that provides importantphysical and psychosocial benefits--is not expected to place young,apparently healthycompetitors at risk for cardiovascular collapse.When sudden deaths occur in elite-levelathletes who have national media exposure,the concerns become magnified.How safe is sportsplay?What causes these tragic events?How can they be prevented?
In addressingthese questions,particular attention has often been focused on health-careproviders,who,it has been assumed,should be able to identify the young athlete at risk andthus prevent sudden cardiac death in sports.It is probably true that unexpectedcardiac-related deaths occur only in athletes who have some underlying heart disease ordysfunction (i.e.,it is unlikely that a normal heart can be "worked todeath").With their extensive diagnostic tools,it seems that health-care providersshould be able to detect athletes who are afflicted by such conditions,restrict theirsports competition,and thereby diminish or eliminate the risk of sudden death duringsports participation.
In accord withthis strategy,national groups have been organized to formulate and publish guidelines fordetection and prohibition of athletes at risk from further participation in sports(Maronet al.,1994;1996b).It is clear,however,that not all underlying disease can be detected bypreparticipation screening.Therefore,using current screening methods,it is not possible toprevent all sudden unexpected death in young athletes.Moreover,restriction of theadolescent with certain high-risk cardiac conditions from participating in sports may dolittle to protect against the danger of sudden death.Still,identifying those conditionsthat can be detected by history and physical examination,with subsequent restriction fromsports participation,is an important means of reducing risks for these youngsters

INCIDENCE OF SUDDEN CARDIACDEATH

Fortunately,theimmense tragedy of sudden death in athletes is very rare.Each year,10-13 such occurrencesare reported in the United States(Maron et al.,1996a;Van Camp et al.,1995),probablyunderestimating the true incidence.If approximately 4 million youngsters are involved incompetitive sports,the statistical chance of an apparently healthy adolescent dying fromunexpected heart disease during a year of sports participation is no more than1:250,000.Putting this into perspective with other risks,approximately 14,700 adolescentsdie each year in motor vehicle accidents;400 from fires,and 250 as a result of inhaledpoisonous fumes(National Safety Council,1985).
Certain groups ofathletes may be at greater risk.Sudden cardiac death appears to be disproportionally morecommon in males,African Americans,and in competitors in basketball and football(Maron etal.,1996a).The explanation for these trends is unclear,but they may reflect intensity ofplay or gender/racial influences on the frequencies of certain cardiovascularabnormalities.Most sudden deaths have occurred during or immediately after training orcompetition.This time course supports the prohibition of identified at-risk athletes fromsports participation.

CAUSES OF SUDDEN DEATH

Manycardiovascular abnormalities can create risk for sudden death,presumably by causinginsufficient blood flow or oxygen supply to the heart muscle and/or by causing anincreased propensity for fatal arrhythmias of the ventricles of theheart.Fortunately,these diseases are uncommon in the pediatric age group.Autopsy studiesof young athletes indicate that four cardiovascular conditions account for approximately80 percent of unexpected sudden deaths (Table 1).These conditions are 1)diseases of theheart muscle,especially those accociated with an enlarged heart(hypertrophic cardiomyopathy);2)congenital anomalies of the coronary arteries that prevent an adequatesupply of blood to the heart nuscle;3)rupturing of the aorta due to a hereditary weaknessin the vessel wall(aortic aneurysm usually associated with Marfan syndrome);and 4)valvular aortic stenosis(a thickening of the aortic valve that prevents adequate flow ofblood out of the left ventricle into the coronary arteries and other major arteries of thebody).Unfortunately,it is not possible to specifically define the magnitude of risk ofdeath during sports participation for these abnormalities because such statistics arebased on the number of athletes who were allowed to participate and who subsequentlydied.In other words,the number of athletes with unsuspected cardiovascular defects whoplay without complications is unknown.

HypertrophicCardiomyopathy

Hypertrophic cardiomyopathy is the predominant cause ofsudden unexpected cardiac death in athletes,accounting for one-half of cases in mostreports.In its full-blown expression,this disease is characterized by unexplained,dramaticventricularthickening,most prominently in the wall between the two ventricles,with virtualobliteration of the left ventricular cavity.Milder forms have also been observed inautopsy specimens following sudden death.
The mechanism for sudden death in patients with hypertrophic cardiomyopathy isuncertain.However,it is not difficult to predict that inadequate blood flow inthe coronaryarteries,insufficient filling of the ventricles,and predisposition to fatal disturbancesin the normal rhythm of the electrochemical impulses that stimulate ventricularcontractions might all play important roles.Significant obstruction to the flow of bloodfrom the left ventricle into the aorta is seen in only a minority of cases and isgenerally not related to symptoms or risk of death.
Initially,it was thought that the prognosis for patients with hypertrophiccardiomyopathy was grim,with mortality rates as high as 2-4% per year(i.e.,20-40% ofpatients would not survive 10 years after diagnosis). However,subsequent studies suggestedthat the natural history of these patients is variable,and individuals with hypertrophiccardiomyopathy have been described who toleratedyears of intense participation inathletics without complications (Maron & Klues,1994).
Participation in physical activity increases the risk of sudden death withhypertrophic cardiomyopathy,but the physical activity is typically not participation insports.Among 78 abults,37% died at rest or during sleep,24%during mild exertion,and 29%with vigorous activity(running,hiking)(Maronet al.,1982).As illustrated in this report,thedisproportionately large percentage who died during physical activity has prompted therecommendation that those with hypertrophic cardiomyopathy be restricted fromparticipationin competitive athletics.
The effectiveness of this strategy is limited by the difficulty in detectingathletes with hypertrophic cardiomyopathy during the preparticipationmedicalexamination.Careful recording of the athlete's medical history mayprovide some clues.Hypertrophic cardiomyopathy is an inherited conditionwith variable symptoms and signs,anda positive family history can be obtainedin about 20% of cases.Approximately one-half ofpatients may describe symptoms of chest pain,dizziness,fainting,and abnormalbreathlessness,particularly with exercise.
Unfortunately,the results of the physical examination may be deceptively“normal.”Aheart murmur is typically absent or unimpressive unless the flowof blood from the leftventricle to the aorta is obstructed.The peripheralpulses can be bifid (two parts),and anymurmur may intensify with standing or during a Valsalva maneuver(forcefully contractingthe abdominal muscles while holding one's breath),but such clues are not always evident.
The electrocardiograms in most patients with hypertrophic cardiomyopathydemonstrateare abnormal,but the most definitive test for hypertrophic
cardiomyopathy is the echocardiogram,in which sound waves are passed throughthe chest toenable the examiner to visualize heart structures while the heart is beating.Theechocardiogram for a person with hypertrophic cardiomyopathy typically demonstrates thatthe thickness of the septum orwall between the two ventricles exceeds 18 mm and that thereis a generalthickening of the heart muscle.There may also be abnormal movements ofthemitral valve,through which blood passes from the left atrium to the leftventricle,along with abnormal filling of the left ventricle during the relaxation (diastolic)phase of the cardiac cycle,and,in some patients,varying
degrees of obstruction of blood flow leaving the left ventricle.
While a diagnosis of hypertrophic cardiomyopathy can be established confidently whenall these features are present,certain features(e.g.,leftventricular enlargement andatypical electrocardiogram)are also observed in the normal response to long-term intensiveathletic training(“the athlete's heart”).Therefore,in some athletes,the health-careprovider is faced with the dilemma of trying to decide if these findings reflect a highlytrained athlete who can be encouraged to continue participation
or represent a mild form of hypertrophic cardiomyopathy,a sign that the athlete shouldstop participating in sports.
Several criteria have been suggested to help make this differential diagnosis.Thediagnosis of true hypertrophic cardiomyopathy is suggestedby a) a ventricular septalthickness greater than 18 mm,b) normal or smallleft ventricular end-diastolic cavity dimension,c) failure of myocardial hypertrophy to regress following a period of detraining,d) evidence that ventricular thickening has been inherited, and e) abnormalpatterns of left ventricular filling patterns as shown with Doppler echocardiography(Maronet al.,1995).

Coronary Artery Abnormalities

Atherosclerotic coronary artery disease is the mostcommon cause of sudden cardiac death in adults,but this is not a consideration in youngathletes except in extremely rare situations (i.e.,inheritedhypercholesterolemia).However,congenital structural abnormalities of the coronary vesselsmay create a risk for sudden death in children and adolescents,and such deathscan beprecipitated by participation in vigorous physical activity.
Several forms of coronary artery abnormalities have been observed at autopsyfollowing sudden unexpected death in young athletes.These include abnormal origin of thecoronary arteries from the aorta,incomplete development of the arteries,coronary arteriesburied deep within the heart nuscle,and anomalous origin of the left coronary artery fromthe pulmonary artery instead of the aorta.At autopsy,investigators have also notedthickening of the walls of coronary arteries causing narrowing of critical coronary arterybranches (e.g.,those supplying the atrioventricular node,which transmits criticalelectrical impulses from the atria to the ventricles).The specific mechanism underlyingdeath in these conditions is uncertain,but is presumably causes an insufficient flow ofblood to the heart muscle when intense physical activity places greater demands onmyocardial metabolism.
Congenital abnormalities of the coronary arteries that predispose an individual tosudden death are extraordinarily rare in the general population.That they rank as thesecond most common cause of sudden cardiac death in young athletes is a reflection of thedifficulty in recognizing such abnormalities clinically.While cases of fainting duringexercise occasionally lead directly to the diagnosis of coronary artery abnormalities,themost common initial presentation of this condition is sudden collapse and death of apreviously apparently healthy athlete.
The risk and occult nature of congenital abnormalities of the coronary arteries washighlighted by the sudden death of the basketball star Pete Maravich.Following a long andhighly successful career as one of the best college and professional players ever,Maravichdied suddenly following participation in a recreational game when he was 40-yearsold.Autopsy revealed a complete absence of the left main coronary artery.The rightcoronary artery served the left coronary distribution,with marked underdevelopment of theleft anterior descending branch and widespread patches of dead heart muscle (Van Camp& Choi,1988).

Marfan Syndrome

Marfan syndrome is an inherited diseasecharacterized by laxity of connective tissue.Research has identified defective formationof a specific protein in patients with this syndrome, an expression of one of severalchromosomal abnormalities.In the full-blown disorder,individuals have heart disease(dilated aorta and regurgitation of blood through heart valves),abnormalities of theeyes(dislocated lens and myopia),musculoskeletal disorders (spinal curvature and adepressed sternum),tall stature,abnormally long and slender fingers and toes,andhyperextensible joints.Commonly,however,patients with Marfan syndrome present with varyingcombinations of these findings.
The cardiovascular abnormalities present the greatest risk for mortality andmorbidity in Marfan patients.Progressive widening of the root of the aorta where it leavesthe left ventricle is common,with a risk of eventual formation and sudden rupture of anaortic aneurysm.This defect is typically accompanied by abnormalities of the aortic andmitral valves and insufficient blood flow into the aorta.Efforts to prevent rupture of anaortic aneurysm include the use of beta-blocker medication and surgery to narrow theaortic root when it reaches a critical diameter of 55-60 mm. The risk of rupturedaortic aneurysms from sports play in athletes with Marfan syndrome was demonstrated by thesudden death of the volleyball player Flo Hyman ,the 6-foot 5-inch star of the 1984 UnitedStates Olympic team,who collapsed and died
during a game in Japan in 1985.Autopsy revealed findings typical of Marfan syndrome,with aruptured aortic aneurysm and accumulation of blood in the pericardial sac surrounding theheart.This tragedy resulted in an awareness that tall athletes with undiagnosed Marfansyndrome might be at risk and led to recommendations for restricting such individuals fromparticipating in certain types of physical activity (i.e.,avoidance of resistancetraining,sports that might involve a blow to the chest,and endurancetraining)(Braverman,1998;Pyeritz & McKusick,1979).
The diagnosis of Marfan syndrome rests with clinical recognition of its typical
physical features as described earlier.Cardiac examination of most patients with Marfansyndrome reveals abnormal sounds associated with a dysfunctional mitral valve.
A careful family history may reveal other members who have Marfan syndrome,buthaving several tall,healthy relatives is more likely to be evidence of familial tallstature.Chest x-rays and electrocardiograms are typically normal in patients with
Marfan syndrome.Suspicion of Marfan syndrome on preparticipation screening should
rompt referral to a cardiologist for assessment of cardiac manifestations of thisdisease.The diagnosis is supported by echocardiographic findings of mitral valve prolapsein conjunction with widening of the aortic root.(Prolapse of the mitral valve is excessivebackward movement of the valve into the left atrium during contraction of the leftventricle.This allows regurgitation of blood into the
atrium,which reduces flow into the aorta.)

Valvar Aortic Stenosis

Among the cardiac diseases that cause sudden deathin athletes,congenital narrowing of the aortic valve (valvar aortic stenosis)is the mostprevalent in the population at large.Yet this anomaly accounts for only a small percentageof sudden-death reports.The explanation presumably lies in the fact that,as opposed to theother causes that are difficult to detect,patients with aortic stenosis exhibit typicalphysical findings that are easily observed.
Patients with valvar aortic stenosis have an obstructed flow of blood from the leftventricle into the aorta,and risk of sudden death is presumably created by eitherinadequate blood flow to the heart muscle during exercise or marked elevation of leftventricular pressure accompanying physical activity that triggers a strong vagal nervereflex to disrupt the heartbeat rhythm(Rowland,1995).Risk of sudden death,whick appears tobe more common during vigorous physical activity,is high only in those with significantobstruction of the aorta as indicated by abnormalities in cardiac pressures,an abnormal electrocardiogram,and/or chest pain.Would-be athletes with valvar aortic stenosis who areat risk of sudden death while participating in sports should be easily recognizable duringa cardiac examination by the presence of specific heart murmurs.Such finding should promptreferral to a cardiologist.

SCREENING FOR RISK

Among the millions of young athletes who undergopreparticipation medical evaluation each year,very few are at risk for sudden cardiacdeath.The magnitude of these tragedies,however,demands an effective plan to minimize theiroccurrence.It is important,then,to determine the most accurate yet efficient methods forrecognizing these “needles in the haystack.”
A substantial reduction in the incidence of sudden death in athletes requires thatscreening tools be effective in recognizing the four high-risk conditions outlinedabove.Unfortunately,the medical history and physical examination,mainstays of thepreparticipation evaluation,are likely to be effective in detecting only two of theseconditions-aortic aneurysms associated with Marfan syndrome and congenital valvar aortic stenosis.
Recognition of hypertrophic cardiomyopathy becomes more problematic.Description inthe medical history of chest pain,dizziness,fainting,or abnormal breathlessness duringphysical activities,or a family history of sudden early death would provide valuableclues.However,these features are not evident in a majority of cases,A harsh murmur duringsystole detected with the stethoscope placed along the left border of thesternum,particularly if it increases when the patient performs a Valsalva maneuver,shouldalso raise suspicion.Athough the effectiveness of history and physical examination indetecting hypertrophic cardiomyopathy during the preparticipation evaluation is notdefinitively known,the available information is pessimistic.For instance,in a recentcompilation of 134 cardiac deaths in athletes,only one of 48 cases of hypertrophiccardiomyopathy had previously been recognized(Maron et al.,1996a).Congenital coronaryartery abnormalities will probably escape detection by history and examination,butpatients'reports of exercise-induced chest pain,dizziness,or fainting should promptfurther evaluation.
In summary,a careful history and physical examination are important in identifyingcertain individuals who would be at risk for sudden cardiac death during sportsparticipation.However,it is unlikely that such a preparticipation evaluation will reducethe current rate of these tragedies.This conclusion has stimulated efforts to find moreaccurate means of identifying those at risk.
Echocardiography will effectively identify individuals with hypertrophiccardiomyopathy and has therefore been suggested as a routine screening tool in thepreparticipation assessment.A quick,inexpensive cardiac ultrasound study that is bothsensitive and specific to hypertrophic cardiomyopathy should reduce the incidence ofsudden cardiac death in athletes by one-half.Unfortunately,echocardiography does notsatisfy these criteria.Echocardionraphy is expensive (＄500-1000 for a complete study)andrequires prohibitively-costly equipment(＄100,000-＄200,000).At＄500 per athlete,itwould cost ＄2 billion annually to identify 3-5 athletes who would otherwise die ofhypertrophic cardiomyopathy,Performing an abbreviated echocardiogram with donatedequipment is also not a cost-effective approach that would allow widespread use ofechocardiograms for screening millions of athletes.
Another serious drawback of routine screening by echocardiography is the effect offalse positive results(Braden & Strong,1988).While full-blown hypertrophiccardiomyopathy can be fairly easily detected,a large overlap may occur in heart-wallthickness values that represent milder cardiomyopathy,the“athlete's heart,”or normalanatomic variant.Considering that mild ventricular thickening would be detected in tens ofthousands of normal youngsters,such findings“would generate heavyemotional,financial,and medical burdens for the athlete,family,team,and institution byvirtue of the uncertainty created and the requirement for additional testing"(Maronet al.,1996b).
Similar objections can be raised about the use of the electrocardiogram as ascreening tool.Consequently,it has been recommended that a medical history and physicalexamination constitute routine preparticipation screening and that other cardiac studiessuch as echocardiography,electrocardiography,and exercise stress tests be reserved forsecondary evaluation of those with suspected heart disease (Braden & Strong,1988;Maronet al.,1996b).

OTHER APPROACHES TO MINIMIZING SUDDEN DEATH INATHLETES

Hypertrophic cardiomyopathy and Marfan syndrome areinherited conditions,causing hope that a chromosomal marker might be developed to permitan easy means of identifying athletes with these conditions.Unfortunately,such a test isnot yet available.When the gene coding for the protein that is flawed in Marfan syndromewas identified,it was expected that a clinical test for Marfan syndrome was athand.Unfortunately,each patient with Marfan syndrome appears to possess a unique mutationof this gene,making genetic testing for the disease
impossible (Braverman,1998).
A similar situation exists with hypertrophic cardiomyopathy.While certain geneticmarkers of this disease are known,there is insufficient precision to allow their use inits detection.Still,given the limitations of the current screening approach,the potentialablility to detect those at risk by a simple blood test is attractive.Future developmentsin genetic analysis may create an accurate,low-cost method.
Recognizing the inability of preparticipation screening to identify all those atrisk,the availability of automatic external defibrillators at sports events has beenproposed (Cantwell,1998).This proposal is based on 1)the assumption that the mode of deathis sports is often ventricular fibrillation,and 2)the development ofrelatively-inexpensive defibrillators that can be effectively used by non-medicalpersonnel.The availability of such defibrillators,in fact,has been increasingly common atcollgeg and professional sports events as well as non-sports settings to prevent suddendeath before emergency personnel can respond.Concerns regardingsafety,cost-effectiveness,efficacy,and legal issues still need to be addressed,but thesedevices provide hope that the incidence of sudden death in athletes might be decreased.

SUMMARY

Each year in the United States,only about one dozenapparently healthy young athletes die suddenly from undiagnosed diseases of the heart andblood vessels.Still,everything possible should be done to minimize thesesuddendeaths,which
often occur during training or competition in sports.most of these deaths can beattributed to abnormalities in the structure and function of the heart muscle itself,thecoronary arteries that supply blood to the heart,the walls of the aorta,or the heart valvethat leads to the aorta.
Some of the cardiovascular abnormalities that may lead to sudden death can be
detected by routine physical examination during the preparticipation evaluation,but othersmay not be found without more sophisticated testing.Unfortunately,additional
diagnostic procedures such as echocaridography and electrocardiography are not recommendedfor routine screening of athletes because the techniques are not cost-effective and have ahigh rate of false positive findings.Future developments,
including genetic screening and the availability of defibrillators at the sport site,maydecrease the incidence of sudden death.The current limitation in our ability to identifyathletes at risk necessitates acceptance that participation in sports carries some risk.

REFERENCES

Braden,D.S.,and W.B.Strong (1998).Preparticipationscreening for sudden cardiac
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Cantwell,J.D.(1998).Automatic external defibrillators in the sports arena.
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Maron,B.J.,and H.G.Klues(1994).Surviving competitive athletics with hypertrophiccardiomyopathy.Am.J.Cardiol.73:1098-1104.
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Maron,B.J.,J.Shirani,L.C.Poliac,R.Mathenge,W.O.Poberts,and F.O.Mueller(1996).Suddendeath in young competitive athletes.JAMA 276:199-204.
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Supplement

IDENTIFYING ATHLETES AT RISK FORSUDDEN DEATH

A careful medical history and cardiacphysical examination are the best tools available for detecting heart disease that mightpose a risk for participating in sports.Unfortunately,some cardiac abnormalities that cancause death during sports training and competition are not likely to be detected duringroutine examinations.Nonetheless,the health-care provider performing such evaluations canreduce the risk of sudden death by paying attention to several key aspects of the historyand physical examination.Medical History
■A history of chest pain,dizziness,fanting,or abnormal breathlessness and fatigue duringexercise is a “red flag” for possible risk from sports participation.In fact,suchsymptoms may be the only obvious clues to the presence of high-risk cardiovasculardisease.
■Unexpected sudden death of a family member at a young age should raise suspicion of
possible inherited cardiac disease.A family history of unusually tall individuals withheart and/or eye problems,particularly if tall stature is not evident in other familymembers,suggests Marfan syndrome,a serious risk factor for sudden death.
■A history of heart palpitations or heart murmur may signal a need for cardiacconsultation.
Because children and abolescents often have insufficient knowledge to respondappropriately to medical-history questions,parents should complete preparticipationmedical history forms.Physical Examination
■Physical signs of Marfan syndrome should be assessed:tall stature,long arms andfingers,sunken sternum,abnormal spinal curvature,flat feet,and high arched palate.Thediagnosis of Marfan syndrome is supported by a history of nearsightedness (or dislocatedlens of the eye) and certain abnormal heart sounds.
■Athletes with significant and harsh heart murmurs should be referred to acardiologist.This is particularly true if the murmur is heard with the stethoscope placedon the back,if it becomes louder while the athlete is sitting up or while he or shecontracts the abdominal muscles while holding the breath,or if it is accompanied by otherabnormalheart sounds.
■Blood pressure should be obtained,and femoral pulses should be evaluated to rule outnarrowing of the aorta.A biphasic arterial pulse suggests the possibility of a
pathologically enlarged heart.
A focused cardiac history and careful physical examination with attention to
the points above can be performed rapidly and should be a routine part of
preparticipation screening.Any questions raised in this assessment should be
brought to the attention of a cardiologist before the athlete is allowed to
participate in strenuous activity.■