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appropriatelarge-scalescreeningstrategy.28aFurtherstudyisnecessaryto
determineifthethresholdtomaximizesensitivityandspecificityisdifferentat
moderateorgreateraltitude,asthenormalsaturationofneonatesataltitudeis
around95%.29
InSeptember2011,theUnitedStatesSecretaryofHealthrecommendedthat
pulse-oximetryscreeningbeforedischargeshouldbeaddedtothenewborn
screeningpanelfortheearlydetectionofcriticalCHDforallinfantsborninthe
UnitedStates.By2014,43of50stateshadinstitutedlegislationorregulations
mandatingpulse-oximetryscreeningwithsevenstatesandtheDistrictof
Columbiasupportingpulse-oximetryscreeningasthestandardofcarewithno
legislationinplace.30Therehavenotbeenmanystudiesassessingtheaccuracy
ofscreeningsincetheimplementationofuniversalpulse-oximetryscreening.An
initialstudyintheUKin2011showedafalse-positiverateof0.8%witha
negativepredictivevalueof99.7%.Of20,055newborninfants,24hadcritical
CHD.Ofthese24,6werenotidentifiedbypulse-oximetryscreening.The
criticallesionsmostlikelytobemissedbyusingpulseoximetryasascreening
methodwereobstructivearchlesionssuchascoarctationandinterruptedaortic
arch.31InrecentstudiesintheUnitedStates,pulseoximetryscreeninghasbeen
showntobecosteffective.32


ScreeningandSuddenCardiacDeath
PrimaryPreventionofSuddenCardiacDeath:
ScreeningWithHistoryandPhysical
Examination,Electrocardiography,and
Echocardiography
Afairamountofcontroversyexistswithregardtoprimarystrategiesforthe
preventionofsuddencardiacarrest(SCA)andsuddencardiacdeath(SCD).The
acceptedstandardhadbeenforathoroughcomprehensiveanduniformscreening
history,familyhistory,andphysicalexaminationassuggestedbytheAmerican
HeartAssociationwiththedocumentationof12importantpoints.33The12

importantpointsthatneedtobereviewedareasfollows:
Familyhistory
Prematuresuddendeath
Heartdiseaseinsurvivingrelatives
Personalhistory
Heartmurmur
Systemichypertension
Fatigability
Syncope
Exertionaldyspnea
Exertionalchestpain
Physicalexamination
Presenceofaheartmurmur
Femoralpulses
StigmataofMarfansyndrome
Bloodpressuremeasurement
Thisapproachunderscorestheimportanceofthediscoveryofsignsand
symptomsthatmayultimatelyuncoverunderlyingat-riskabnormalitiesfor
suddencardiacarrest.However,theutilityandsuccessofthisstrategyisless
thanoptimal.Specifically,ithasbeendifficulttothoroughlyanduniformly


achievetheabovestrategy.Studies34,35havesuggestedthat,overall,themajority
ofstatesintheUnitedStateshavenotbeenabletoadoptastrategyforcovering
theimportantpointsrecommendedbytheAmericanHeartAssociation.Asa
result,severalorganizations,includingtheAmericanAcademyofPediatrics,
havesuggestedauniformpreparticipationformwherebythekeyquestionsare
coveredpriortosportsparticipation.36Severalpointsmustbeemphasized.
Firstly,preventionofSCAandSCDcannotbediscussedinthecontextof
athleticparticipationonlyandcannotbedirectedonlytoathletes.An“athlete”

maybesomewhatdifficulttotrulydefinespecifically.Inaddition,inthat
underlyingandundiagnosedcardiacabnormalities,bothstructuralandelectrical,
havethepotentialtocauseSCAinanyat-riskyoungperson,theprotectionas
wellasthestrategiesforpreventionshouldincludeallyouth,notonlythose
arbitrarilydefinedasathletes.Secondly,theefficacyofthehistory,family
historyandphysicalexaminationisfarfromperfect.Thoughtherearesome
retrospectivestudiesthatsuggestthatasmanyas25%to50%ofthosewho
experiencedSCDhadantecedentsymptomssuchassyncope,palpitations,or
chestpain,themajorityofstudieshaveshownarelativelylowyieldforthis
strategy.37–39Andofcoursetheindividualwhotrulyhasnosymptoms,hasa
negativefamilyhistory,andanormalphysicalexaminationwillnotbe
uncoveredbythisstrategyfordiscoveryandprevention.
In2006,Corradopublishedastudyoutliningtheresultsof25yearsofa
mandatoryelectrocardiography(ECG)screeningprogramforcompetitive
athletesintheVenetoregionofItaly.40Corrado'sstudyshoweda90%reduction
intheincidenceofSCDintheathleticpopulationbyprospectivelyidentifying
thoseathleteswitharrhythmogenicrightventriculardysplasia(ARVD)and
hypertrophiccardiomyopathy(HCM)andsubsequentlyexcludingthemfrom
athleticparticipation.However,studiesbyotherinvestigatorswerenotableto
reproducethisexperience.Marondidnotfindsimilaroutcomesinastudy
reportedfromMinnesotain200941andSteinvil(2011)42didnotfindareduction
intheriskofSCDinathletesinIsraelwiththeirmandatoryECGscreening
programin2011.42However,tremendouscontroversyhasbeengeneratedbythis
work.Concernsincludereproducibilityofthedata,ethicalconsiderations
regardingselectingspecificpopulationsandautonomy,generalizabilityto
heterogeneouspopulations,effectsoffalsepositivesandnegatives,andcost
considerations.TheAmericanHeartAssociationhasnotyetembracedand
recommendedastrategyformassECGscreeningforyoungathletesinthe