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Diagnosisandinitialmanagementofcyanoticheartdiseaseinthenewborn
OfficialreprintfromUpToDate
www.uptodate.com2016UpToDate
Diagnosisandinitialmanagementofcyanoticheartdiseaseinthenewborn
Author
RobertLGeggel,MD
SectionEditors
DavidRFulton,MD
LeonardEWeisman,MD
DeputyEditor
CarrieArmsby,MD,MPH
Alltopicsareupdatedasnewevidencebecomesavailableandourpeerreviewprocessiscomplete.
Literaturereviewcurrentthrough:Jul2016.|Thistopiclastupdated:Jul15,2016.
INTRODUCTIONCyanoticlesionscompriseapproximatelyonethirdofpotentiallyfatalformsofcongenitalheart
disease(CHD)[1,2].Earlyrecognition,emergentstabilization,andtransporttoanappropriatecardiaccarecenterare
criticallyimportantintheoutcomeofnewbornswiththeselesions.
TheevaluationandinitialmanagementofcyanoticCHDinthenewbornarepresentedhere.Thecausesofneonatal
cyanoticcardiaclesionsarediscussedseparately.(See"Cardiaccausesofcyanosisinthenewborn".)
PRENATALDIAGNOSISPrenatalsonogramsoftenidentifystructuralmalformationsincludingcongenitalheartdisease
(CHD),diaphragmatichernia,andcongenitalcysticadenomatoidmalformationhowever,thesensitivityofCHDdetection
ishighlyvariabledependingonoperatorexpertise,gestationalage,fetalposition,andtypeofdefect.Asaresult,prenatal
sonographywillmisssomepatientswithcyanoticCHD.(See"Fetalcardiacabnormalities:Screening,evaluation,and
pregnancymanagement".)
POSTNATALDIAGNOSISInaffectedneonateswhoarenotidentifiedbyprenatalsonography,aclinicaldiagnosisof
cyanoticcongenitalheartdisease(CHD)isbasedonhistory,physicalfindings,chestradiography,andhyperoxiatest.The
diagnosisisconfirmedbyechocardiography.
Centralcyanosiscausedbyreducedarterialoxygensaturationisgenerallyperceptiblewhenthereducedhemoglobinlevel
exceeds3g/dL(figure1)[3].Itcanresultfromseveraldifferentpathologicmechanismsthatarecausedbycardiac
disorders,pulmonaryabnormalities,orhemoglobinopathies(table1)[3].(See"Cardiaccausesofcyanosisinthe
newborn",sectionon'Noncardiaccausesofcyanosis'and"Overviewofcyanosisinthenewborn",sectionon
'Hemoglobinconcentration'and"Overviewofcyanosisinthenewborn",sectionon'Centralcyanosis'.)
ThemorecommonformsofcyanoticCHDmaybeclinicallydistinguishedfromeachotherandothercausesofcentral
cyanosisbaseduponthephysicalexamination,chestradiography,andelectrocardiography(table2).Thehyperoxiatestis
alsousedtodifferentiatecyanoticCHDfromcyanosisduetorespiratorydisease.Echocardiographyconfirmsthe
diagnosisanddeterminestheunderlyingcardiacanatomyandfunction.
HistoryAthoroughhistorymayidentifymaternalmedicalorprenatalconditions,orfamilyhistoryofCHDthat
increasestheriskofCHD,andisdiscussedseparately.(See"Identifyingnewbornswithcriticalcongenitalheartdisease",
sectionon'History'.)
PhysicalexaminationThephysicalexaminationmaybeusefulindifferentiatingCHDfromothercyanoticdisorders,
suchasrespiratorydiseaseorsepsis,whichhaveoverlappingclinicalfindings.Onceacardiacetiologyisdetermined,the
examinationalsoprovidescluestotheunderlyingspecificcardiacdefect(table2).Thedifferentialdiagnosisandthe
clinicalfindingsthatdistinguishCHDfromotherdisordersarediscussedinthefollowingtopicreviews.(See"Identifying
newbornswithcriticalcongenitalheartdisease",sectionon'Physicalexamination'and"Cardiaccausesofcyanosisinthe
newborn",sectionon'Noncardiaccausesofcyanosis'.)
ThefollowingdiscussionsummarizeskeyfindingsthataresuggestiveofCHDand/orspecificcardiaclesions.
VitalsignsInacyanoticneonate,thepulse,respiratoryrate,oxygensaturation,andbloodpressure(measuredin
therightarmandeitherleg)maybesimilarbetweenCHDandothercauses.Insomecases,findingsmaypointtoa
specificcardiaclesion.
Abloodpressuregradientbetweenthearmsandlegs,orweakenedorabsentfemoralpulses,suggestsleft
ventriculardysfunctionassociatedwithseverecoarctationoftheaortaorinterruptedaorticarch.Iftheductus
arteriosusiswidelypatent,nogradientmaybedetectedbetweenthearmsandlegsinthesedisorders.
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Severerespiratorydistressmanifestedbytachypnea,retractions,andgruntingusuallyindicatesarespiratory
problem.However,somestructuralheartdiseasescanpresentwithsimilarsymptoms.Examplesofthelatter
includeobstructedtotalanomalouspulmonaryvenousconnectionandleftsidedobstructivedisease(eg,hypoplastic
leftheartsyndrome[HLHS],criticalvalvaraorticstenosis,andseverecoarctationoftheaorta).Infantswithcyanotic
CHDusuallypresentwithcyanosiswithmildorabsenttachypnea.(See"Identifyingnewbornswithcritical
congenitalheartdisease",sectionon'Respiratorysymptoms'.)
Vitalsignscanbenormalinsomecyanoticinfantswithstructuralheartdisease.Theymayalsobenormalin
polycythemia,pulmonaryarteriovenousmalformation,ormethemoglobinemia.
Peripheralcyanosisassociatedwithtachycardia,tachypnea,andhypotensionoftensuggestssepsis.However,itis
alsoimportanttoconsiderleftheartobstructivelesionswithheartfailuresuchasHLHS,criticalaorticstenosis,and
severecoarctationoftheaortainthedifferentialdiagnosisofperipheralcyanosis.(See"Cardiaccausesofcyanosis
inthenewborn",sectionon'Heartfailure'.)
PulseoximetryIncyanoticneonates,transcutaneousoxygensaturation(ie,pulseoximetry)shouldbemeasured
frompreductal(righthand)andpostductalsites(rightorleftfoot).Oxygensaturationvaluesarereducedwithcentral
cyanosisandusuallynormalwithperipheralcyanosis.Adifferenceinvaluesatthetwositesidentifiespatientswith
differentialcyanosis.Inpatientswitharightaorticarch,thepreductalsaturationshouldbemeasuredinthelefthand.The
routineuseofpulseoximetrytoscreenforneonatalcriticalcardiacdiseasehasbeenshowntobeaneffectivescreening
testandisdiscussedseparately.
SecondheartsoundThesecondheartsound(S2)isnormallysplitininspiration(aorticcomponentbefore
pulmonarycomponent).Splittingisusuallyaudiblein66percentofinfantsat16hoursofageandin80percentby48
hours[1].(See"Identifyingnewbornswithcriticalcongenitalheartdisease",sectionon'Physicalexamination'.)
InthefollowingformsofcyanoticCHD,althoughpotentiallydifficulttoappreciateinatachycardiacillneonate,theS2
appearstobesinglewithoutsplittingduringauscultation(table2):
Intranspositionofthegreatarteries,thepulmonaryarteryislocatedposterioranddirectlybehindtheaorta.Thus,the
aorticcomponentofS2isloudbecauseofitsanteriorlocationandthesofterpulmonarycomponentisoften
inaudible.
Pulmonaryatresia,truncusarteriosus,orHLHSwithaorticatresiahaveonlyasinglesemilunarvalve,soS2has
onlyonecomponent.
IntetralogyofFallot(TOF),thediminishedpulmonaryvalveexcursionassociatedwithpulmonarystenosismakes
thepulmonarycomponentofS2softanddifficulttodetect,especiallyifthereislatepeakingoftherightventricular
outflowtractsystolicmurmur.(See"Pathophysiology,clinicalfeatures,anddiagnosisoftetralogyofFallot".)
S2isnormallysplitinpatientswithEbstein'sanomalyofthetricuspidvalveandwidelysplitinpatientswithtotal
anomalouspulmonaryvenousconnection,whichisalsodifficulttoappreciateinatachycardiacillneonate.
MurmurApathologicmurmurisaudibleinmostcommonformsofcyanoticCHD(table2).
PatientswithTOFtypicallyhaveamurmurcausedbypulmonarystenosis(movie1).TOFassociatedwith
pulmonaryatresiaoftenhasamurmurassociatedwithapatentductusarteriosus(movie2)oraortopulmonary
collateralsthatcanbedetectedaspulmonaryvascularresistancefalls.
Asoft,outflowsystolicmurmurisheardinpulmonaryatresiawithintactventricularseptum,HLHS,ortruncus
arteriosus.Inalloftheselesions,thecardiacoutputcrossesasinglesemilunarvalve,andthevolumeofbloodflow
causestheassociatedmurmur.Somepatientswithtruncusarteriosusalsohaveadiastolicmurmuroftruncalvalve
regurgitation.
Pulmonaryatresiaisoftenassociatedwithtricuspidregurgitation.Thisproducesasystolicmurmurattheleftlower
sternalborder.
Tricuspidatresiaisusuallyassociatedwithaventricularseptaldefect(movie3)andpulmonarystenosis(movie1),
whichcreatesystolicmurmurs.
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ThetricuspidvalveinEbstein'sanomalyisnearlyalwaysregurgitantandproducesasystolicmurmurattheleft
lowersternalborder.
Dtranspositionofthegreatarteries(DTGA)withanintactventricularseptumandnopulmonarystenosistypically
hasnomurmur.
Descriptionsandexamplesofinnocentandpathologicmurmursininfantsandchildrenareprovidedseparately.(See
"Approachtotheinfantorchildwithacardiacmurmur".)
HepatomegalyHepatomegalyoftenoccursinpatientswithheartfailureduetoleftsidedobstructivelesions(eg,
HLHS,coarctation,criticalaorticstenosis,andcardiomyopathy)orinfradiaphragmatictotalanomalouspulmonaryvenous
connection.Apalpableliverinthemidlinesuggestscomplexcongenitalheartdisease(heterotaxysyndromes)associated
withaspleniaorpolysplenia.
Someinfantswithpulmonarydiseasecanappeartohavehepatomegaly,butthisiscausedbydisplacementbyaflattened
diaphragmduetohyperinflation.Theliverspanisnotenlargedinthesepatients.
ChestradiographAchestradiographishelpfulindifferentiatingbetweencardiacandpulmonarydisorders.
Examinationofthelungfieldsidentifiesmajorpulmonarycausesofcyanosisincludingpneumothorax,pulmonary
hypoplasia,diaphragmatichernia,pulmonaryedema,pleuraleffusion,orairwaydisease.
Threefeaturesofthechestradiographthatcanbesuggestiveofspecificcardiaclesionsareheartsizeorshape,
pulmonaryvascularmarkings,andsitusoftheaorticarch.
Heartsizeorshape
Heartsize
Patientswithleftsidedobstructivelesionsmayhavecardiomegalyduetoheartfailure.
Extremecardiomegalysuggestslesionsassociatedwithadilatedrightatriumsincethischamberisvery
compliant.TheseincludepulmonaryatresiawithintactventricularseptumorEbstein'sanomaly.
HeartshapeCharacteristicabnormalitiesofheartshapeareassociatedwithspecificlesions.
TOFBootshaped(coeurensabot)contour(image1).
DTGAEggonastringpatterncausedbyanarrowmediastinalshadowproducedbytheanteriorposterior
ratherthanrightleftrelationshipofthegreatarteries.
PulmonaryvascularmarkingsThepatternofpulmonarybloodflowdependsuponthespecificcardiaclesion.
AlthoughdecreasedpulmonaryvascularmarkingsoccurinmostcyanoticCHDlesions,theyareincreasedinpatientswith
truncusarteriosusormixinglesions,suchascommonatrioventricularcanal,aspulmonaryvascularresistancefallsafter
delivery.
InDTGA,vascularmarkingsmaybeasymmetric.Inthiscondition,therightpulmonaryarterybranchesfromthemain
pulmonaryarteryalongthelongaxisoftheleftventriclewhiletheleftpulmonaryarterybranchesacutely.Thisanatomy
oftenpromotespreferentialincreasedflowtotherightlungandasymmetricbloodflowwithreducedmarkingsintheleft
lung.
Pulmonaryvenouscongestionduetoheartfailureischaracterizedbyindistinctvascularmarkingsspreadinginabutterfly
distributionfromthecentralregionofthechest.Thisisoftenseeninobstructedtotalanomalouspulmonaryvenous
connectionorfailureduetoleftsidedobstructivelesions(HLHSorseverecoarctationoftheaorta)orcardiomyopathy.
SitusofaorticarchThesitusoftheaorticarchisdefinedbywhichofthemainstembronchithearchcrosses.This
isbestdeterminedbytheindentationofthetracheaontheanteroposteriorimageindicatingthesidetowardswhichthe
aorticarchiscoursing.Thenormalanatomyisaleftsidedaorticarchwithindentationoftheleftsideofthetracheaasthe
archcrossesovertheleftmainstembronchus.
Arightaorticarchresultsinanindentationontherightsideofthetrachea.Approximately20percentofpatientswithTOF
(image1)[4],and30percentwithtruncusarteriosus[5]havearightaorticarch.BecauseTOFismuchmorecommon
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thantruncusarteriosus,arightaorticarchinacyanoticinfantusuallysuggestsTOF.Arightaorticarchmayalsobe
associatedwithotherlesions,suchastranspositionofthegreatarteries.
ElectrocardiogramInthefetus,therightventriclehasalargervolumeloadthantheleftventriclesincethereislimited
pulmonaryflowandthusreducedbloodvolumeintheleftheart.Asaresult,thenormalneonatalelectrocardiogram(ECG)
hasrightaxisdeviation(QRSaxis+90to+180degrees)andaprecordialpatternofrightventricularhypertrophy.
AlthoughtheECGmaybenormalinmanycyanoticheartlesionsduringtheneonatalperiod,somelesionsareassociated
withspecificpatterns(table2).Theseincludethefollowing:
Lesionsassociatedwithasmallrightventriclehavethefollowing:
Leftaxisdeviationforage(forpulmonaryatresiaintactventricularseptumtypically+30to+90degreesfor
tricuspidatresiawithnormallyrelatedgreatarteriestypically30to90degrees)
RightatrialenlargementTallpeakedPwavesmosteasilyidentifiedinleadII
Leftventricularhypertrophy
HLHSoftenhasmarkedrightventricularhypertrophy(increasedQRSvoltageintherightandanteriorlead)and
decreasedleftventricularforcesinthelateralprecordialleads.
Ebstein'sanomalyhasrightatrialenlargementandoccasionallyadeltawaveofWolffParkinsonWhitesyndrome.
HyperoxiatestThehyperoxiatestisusefulindistinguishingcardiacfrompulmonarycausesofcyanosis.
InCHDassociatedwithintracardiacrighttoleftshuntingresultingincyanosis,bloodinthepulmonaryveinsisfully
saturatedwithoxygeninambientair.Administeringhigherconcentrationsofinspiredoxygenincreasestheamountof
dissolvedoxygen,buthasminimaleffectonoxygentensionlevelsbecausethereisnoeffectonthedeoxygenated
bloodthatisshuntedtothesystemiccirculation.
Incontrast,patientswithpulmonarydiseasehavepulmonaryvenousdesaturation.Supplementaloxygen
administrationinpulmonarydiseasetypicallyincreasespulmonaryvenousoxygenlevelsandimprovessystemic
oxygenation.
Inthehyperoxiatest,arterialoxygentensionismeasuredintherightradialartery(preductal)whilethepatientbreathes
100percentoxygenconcentrationfor10minutes.Oxygencanbeadministeredviaahoodorendotrachealtubeifthe
patientisalreadyintubated.Asignificantincreaseinsystemicarterialoxygensaturationandpartialpressureofarterial
oxygen(PaO2)above150mmHgduringthehyperoxiatestmakesitmorelikelythatthepatienthaspulmonarydisease.
However,failuretoincreaseoxygensaturationandPaO2doesnotdefinitively"rulein"cyanoticCHD,assevereformsof
lungdiseaseorpersistentpulmonaryhypertensionofthenewbornmaynotincreasetheoxygensaturationandPaO2with
thehyperoxicchallenge.
Atranscutaneousoxygenmonitorcanbeusedtoassesswhetherarterialoxygentensionrisesinresponsetothe
increasedinspiredoxygenconcentration,therebyavoidingarterialpunctureforbloodsampling.However,anabnormalor
equivocalresponsemustbeverifiedbymeasurementofanarterialbloodgas.(See'Arterialbloodgas'belowand"Oxygen
monitoringandtherapyinthenewborn".)
InterpretationThepreductaloxygentensionwhilebreathing100percentoxygenconcentrationrarelyexceeds150
mmHgincyanoticheartdiseaseandusuallyexceedsthisvalueinpulmonarydisease(table3)[6].Inthesecases,an
echocardiogramisneededtoestablishtheunderlyingdiagnosis.
ThelevelofPaO2in100percentoxygenalsohelpstodistinguishamongthetypesofcyanoticheartdisease(table4).
Patientswithlesionssuchastranspositionofthegreatarteriesorseverepulmonaryoutflowobstructiongenerally
havePaO2<50to60mmHgduringtheadministrationof100percentoxygen.
Incontrast,inpatientswithmixinglesionsinvolvingbothrighttoleftandlefttorightshunting,suchastruncus
arteriosusorsingleventriclewithpatentductusarteriosus,thesystemicoxygentensionmayincreasewith
administrationof100percentoxygen.Inthesecases,supplementaloxygenmaydecreasepulmonaryvascular
resistance,therebyincreasingpulmonaryflow.Theincreasedpulmonaryflowmixedwithafixedamountofsystemic
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venousreturnresultsinincreasedaorticoxygenation,typicallytoPaO2valuesof75to150mmHg,butrarelyhigher
[6].Inthesepatients,thechestradiographtypicallydemonstratescardiomegalyandprominentpulmonary
vascularity.
Increasedoxygenationwithsupplementaloxygenisalsoseenwhenpulmonarydisease,suchaspulmonaryedema
orpneumonia,isassociatedwithcyanoticCHD.However,thePaO2rarelyexceeds150mmHg.
Apulseoximetershouldnotbeusedforthehyperoxiatestbecauseitmaynotdetectaninadequateincreaseinoxygen
tension.Becauseofthecharacteristicsoftheoxygendissociationcurve,normalhemoglobinisfullysaturatedwithoxygen
whenthearterialPO2exceeds70mmHg(figure2).Therefore,apatientreceiving100percentinspiredoxygen
concentrationcouldhaveanoxygensaturationofnearly100percentassociatedwithanarterialPO2of75mmHg,avalue
thatisabnormal.Thus,discriminationbetweencardiacmixinglesionsandpulmonarydiseasemaybelimited.However,
pulseoximetrymaybehelpfulifsaturationremainslessthan93to95percent.(See"Overviewofcyanosisinthe
newborn",sectionon'Fetalhemoglobin'.)
EchocardiographyEchocardiography,includingimaging,andpulsedandcolorDopplerinterrogationofflowpatterns
providesadefinitivediagnosisofCHDwithinformationoncardiacanatomyandfunction.Echocardiographyshouldbe
performedinanynewbornwithcentralcyanosiswhohasfailedahyperoxiatestorhasanequivocalresult.Other
indicationsforechocardiographyincludebloodpressureorpulsedifferentialbetweenupperandlowerextremities,
cardiomegaly,murmur,orcyanosis.
OthertestsOthertestsincludedintheevaluationofaneonatewithcyanosisincludearterialbloodgas,completeblood
count,andbloodcultures.
ArterialbloodgasAbloodgasmeasurementonanarterialsampleshouldbeobtainedinanynewbornwith
cyanosis.AnarterialbloodgasmeasurementprovidesinformationonthePaO2,PaCO2(partialpressureofarterialcarbon
dioxide)(indicativeofadequateventilation),andarterialpH.
AnarterialPO2valueprovidesmorespecificdatathanoxygensaturation.Becauseoftheincreasedaffinityoffetal
hemoglobinforoxygen,arterialPO2valuesatagivenlevelofoxygensaturationareoftenlowerinnewbornsthanin
adults.(See"Overviewofcyanosisinthenewborn",sectionon'Fetalhemoglobin'.)
AnelevatedarterialPCO2valueoftenindicatesthepresenceofpulmonarydisease.ArterialPCO2mayalsobe
increasedinheartfailureduetopulmonarycongestion.
AreducedpHlevelraisesconcernaboutpoorcardiacoutputandpendingshock,whichcanbeseenincasesof
severehypoxemiaand/orheartfailure.(See"Etiology,clinicalmanifestations,andevaluationofneonatalshock",
sectionon'Laboratoryfindings'.)
Patientswithmethemoglobinemiatypicallyhavelowoxygensaturationandnormaloxygentension.Inthis
uncommoncondition,thebloodhasachocolatebrowncoloranddoesnotbecomeredwhenexposedtoair(figure3).
CompletebloodcountNewbornswithcyanosisshouldhaveacompletebloodcountanddifferentialanalysis,
whichmayhelpdifferentiateCHDfromnoncardiacdisorders.Asexamples,anelevatedhematocritorhemoglobin
concentrationidentifiespatientswithpolycythemia,whereasanelevatedordecreasedwhitebloodcellcountor
thrombocytopeniasuggestspossiblesepsis.(See"Neonatalpolycythemia"and"Clinicalfeatures,evaluation,and
diagnosisofsepsisintermandlatepreterminfants",sectionon'Otherinflammatorymarkers'.)
SepsisevaluationBecausesepsisisacommondisorderinthedifferentialdiagnosisofcyanoticCHD,ablood
cultureshouldbeobtained.Urinalysisandurineculturearealsousuallyobtained.Dependinguponthelevelofclinical
suspicion,alumbarpunctureshouldbeperformed,withanalysisandcultureofthecerebrospinalfluid.Empiricalantibiotics
aregenerallygivenuntilcultureresultsareavailable.(See"Clinicalfeatures,evaluation,anddiagnosisofsepsisinterm
andlatepreterminfants",sectionon'Differentialdiagnosis'and"Managementandoutcomeofsepsisintermandlate
preterminfants",sectionon'Initialempirictherapy'.)
INITIALMANAGEMENTNewbornswithcyanosisrequireimmediateassessment,generalsupportivecarethat
maintainsadequatetissueperfusionandoxygenation,andspecifictherapywhenanunderlyingcauseisknown.Specific
interventionsforneonatalcyanoticcongenitalheartdisease(CHD)includeadministrationofprostaglandinE1(alsoreferred
toasalprostadil)andcardiaccatheterpalliativeorcorrectiveprocedures.
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GeneralsupportivecareInitialmanagementbeginswithgeneralcarethatincludescardiorespiratorysupportand
monitoringtoensuresufficientorgan/tissueperfusionandoxygenation.Ifthereisrespiratorycompromise,anadequate
airwayshouldbeestablishedimmediatelyandsupportivetherapy(eg,supplementaloxygenand/ormechanicalventilation)
institutedasneeded.Patientswithhypotensionorpoorperfusionrequirecardiopulmonaryresuscitation.
Vitalsignsshouldbemonitoredandvascularaccessestablishedforsamplingofbloodandadministrationofmedications.
Placementofsecureintravenousandintraarterialcathetersismosteasilyaccomplishedviatheumbilicalvessels.This
willenableefficientcorrectionandmonitoringofacidbasebalance,metabolicderangements(eg,hypoglycemia,
hypocalcemia),andbloodpressure.Inotropicagentssuchasdopamineordobutaminemaybenecessarytocorrect
hypotension.
Ininfantswithseverepolycythemia(>70percent),anisovolumetricpartialexchangetransfusionshouldbeperformedwith
salinetoreducethehematocrit.(See"Neonatalpolycythemia".)
Ifcyanosisisduetoacquiredmethemoglobinemia,theoffendingagentisremoved.Inseverecases,methyleneblue,1
percentsolution,inadoseof1to2mg/kg(0.1to0.2mL/kgofa1percentsolution)isinfusedintravenouslyover5to10
minutes.Thedosecanberepeatedinonehourifneeded.Congenitalmethemoglobinemiadoesnotrespondtomethylene
blue.(See"Clinicalfeatures,diagnosis,andtreatmentofmethemoglobinemia",sectionon'Treatmentofhereditary
methemoglobinemia'.)
AntibioticsSepsiscanleadtocyanosisandleftventriculardysfunctionorpulmonarydisease.Asaresult,unless
anotherspecificetiologyispromptlyidentified,broadspectrumantibioticsshouldbeinitiated(ampicillinandgentamicin)
afterobtainingbloodandurinecultures.(See'Sepsisevaluation'aboveand"Managementandoutcomeofsepsisinterm
andlatepreterminfants",sectionon'Initialempirictherapy'.)
SpecificCHDmeasuresAninfantwhofailsthehyperoxiatestanddoesnothavepersistentpulmonaryhypertension
ofthenewbornorachestradiographconsistentwithlungdiseaseislikelytohaveacyanoticCHD.Inmostcases,
cyanoticCHDisdependentuponapatentductusarteriosus(PDA)forpulmonaryorsystemicbloodflow.Closureofthe
ductusarteriosuscanprecipitaterapidclinicaldeteriorationwithsignificantlifethreateningchanges(ie,severemetabolic
acidosis,seizures,cardiogenicshock,cardiacarrest,orendorganinjury).Asaresult,infantswithductaldependent
lesionsareatincreasedriskfordeathandsignificantmorbidityunlessinterventionsareinitiatedtomaintainpatencyofthe
ductusarteriosusforductaldependentlesions,ensureadequatemixingofdeoxygenatedandoxygenatedblood,orrelieve
obstructedbloodflow.(See"Identifyingnewbornswithcriticalcongenitalheartdisease",sectionon'Cyanosis'and
"Identifyingnewbornswithcriticalcongenitalheartdisease",sectionon'Postnataldiagnosis'.)
ProstaglandinE1Ininfantswithorwhohaveaclinicalsuspicionforaductaldependentcongenitalheartdefect,
prostaglandinE1(alprostadil)shouldbeadministereduntiladefinitivediagnosisortreatmentisestablished[7].
Theinitialdoseisdependentontheclinicalsetting,astheriskofapnea,oneofthemajorcomplicationsofprostaglandin
E1infusion,isdosedependent.
Iftheductusisknowntobelargeinapatientwithductdependentphysiology,theinitialdoseis0.01mcg/kgper
minute.ThisscenariotypicallyisseeninpatientswithechocardiographicconfirmationofalargePDAwhoarecared
forinatertiarycenterthatprovidestreatmentforneonateswithcyanoticheartdisease.
Iftheductusisrestrictiveorthestatusoftheductusisunknown,theinitialdoseis0.05mcg/kgperminute.Thisis
thestandarddoseusedinpatientswhorequiretransporttoacenterwithexpertiseinthecareofneonateswith
cyanoticheartdisease.
Thedoseofprostaglandincanbeincreasedasneededtoamaximumdoseof0.1mcg/kgperminute.
ComplicationsofprostaglandinE1infusionincludehypotension,tachycardia,andapnea[8].Asaresult,aseparate
reliableintravenouscathetermustbeinplacetoprovidefluidsforresuscitation.Intubationequipmentshouldbe
immediatelyavailablebecauseapneacanoccuratanytimeduringinfusion.
DeteriorationoftheclinicalstatusafterstartingprostaglandinE1usuallyindicatesthepresenceofrarecongenitalcardiac
defectsassociatedwithpulmonaryvenousorleftatrialobstruction.Theseincludeobstructive(usuallyinfradiaphragmatic)
totalanomalouspulmonaryvenousconnectionorvariousconditionsassociatedwitharestrictiveatrialseptum(eg,
hypoplasticleftheartsyndrome,cortriatriatum,severemitralstenosisoratresia,orDtranspositionofthegreatarteries
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associatedwithrestrictiveatrialshunting).Thesepatientsrequireurgentechocardiographyfollowedbyinterventional
cardiaccatheterizationorsurgery[9].
CardiaccatheterizationCardiaccatheterinterventionscaneitherbepalliativebyimprovingcyanosisorbe
correctivebyrelievingobstructiontoflow.
BalloonatrialseptostomycanrelievemarkedcyanosisinpatientswithDtranspositionofthegreatarteries
associatedwithrestrictiveatrialshunting,andinpatientswitharestrictiveatrialseptumassociatedwithleftsided
obstructivedisease.InpatientswithDtranspositionofthegreatarteries,thisprocedurecanbeperformedatthe
bedsideunderechocardiographicguidance.(See"ManagementandoutcomeofDtranspositionofthegreatarteries",
sectionon'Balloonatrialseptostomy'and"Hypoplasticleftheartsyndrome",sectionon'Initialmedical
management'.)
Balloonvalvuloplastycanbeeffectiveinpatientswithcriticalpulmonarystenosisoraorticstenosis.Selected
patientswithpulmonaryatresiaarealsocandidatesforballoonvalvuloplastyiftheobstructionismembranous,the
tricuspidannulusandrightventricularsizeareadequatetosupportatwoventriclerepair,andthecoronarycirculation
doesnotdependupontherightventricle[10].(See"Valvaraorticstenosisinchildren",sectionon'Firstline
treatment'.)
Transcatheterocclusionofpulmonaryarteriovenousmalformationscanalsobeperformed[11].
TransportWhenitisnecessarytotransferaneonatewithcyanoticCHDfromthebirthhospitaltoanothermedical
facilitywithpediatriccardiologyexpertise,thepatientshouldbeintubatedandmechanicallyventilatedpriortoandduring
transportifprostaglandinE1(alprostadil)isbeingadministered[9].
AlthoughanAustralianreportsuggestedthattransportedinfantswithsuspectedCHDwhoreceivealowdoseof
prostaglandinE1(lessthan0.015mcg/kg/min)maynotrequiremechanicalventilation,significantlimitationsofthestudy
(ie,retrospectivenatureofthestudyandthenonsystematicuseofmechanicalventilationandprostaglandinE1)dictate
cautioningeneralapplicationoftheseresults[12].Untilcontrolledtrialsareperformed,wecontinuetointubateand
ventilateallinfantspriortotransportiftheyarereceivingprostaglandinE1becausetheyareatriskforapnea.However,
intubationisnotroutinelyperformedbyalltertiarycaretransportteams.
INFORMATIONFORPATIENTSUpToDateofferstwotypesofpatienteducationmaterials,"TheBasics"and"Beyond
theBasics."TheBasicspatienteducationpiecesarewritteninplainlanguage,atthe5thto6thgradereadinglevel,and
theyanswerthefourorfivekeyquestionsapatientmighthaveaboutagivencondition.Thesearticlesarebestfor
patientswhowantageneraloverviewandwhoprefershort,easytoreadmaterials.BeyondtheBasicspatienteducation
piecesarelonger,moresophisticated,andmoredetailed.Thesearticlesarewrittenatthe10thto12thgradereadinglevel
andarebestforpatientswhowantindepthinformationandarecomfortablewithsomemedicaljargon.
Herearethepatienteducationarticlesthatarerelevanttothistopic.Weencourageyoutoprintoremailthesetopicsto
yourpatients.(Youcanalsolocatepatienteducationarticlesonavarietyofsubjectsbysearchingonpatientinfoandthe
keyword(s)ofinterest.)
Basicstopics(see"Patientinformation:Totalanomalouspulmonaryvenousconnectioninchildren(TheBasics)"and
"Patientinformation:TetralogyofFallot(TheBasics)")
SUMMARYANDRECOMMENDATIONSCyanoticcardiaclesionsaccountforapproximatelyonethirdofpotentially
fatalcasesofcongenitalheartdisease(CHD).
AlthoughprenatalsonogramsoftenidentifyCHD,thesensitivityofthetestishighlyvariableanddependson
operatorexpertise,gestationalage,fetalposition,andtypeofdefect.Asaresult,prenatalsonographywillmiss
somecasesofcyanoticCHD.(See"Fetalcardiacabnormalities:Screening,evaluation,andpregnancy
management".)
InneonateswithcyanoticCHDwhoarenotidentifiedbyprenatalsonography,aclinicaldiagnosisisbasedon
history,physicalfindings,chestradiography,andhyperoxiatest,andconfirmedbyechocardiography(table2).(See
'Postnataldiagnosis'above.)
ThehistorymayidentifymaternalorperinatalconditionsandafamilyhistoryofCHDthatareriskfactorsfor
CHD.(See"Identifyingnewbornswithcriticalcongenitalheartdisease",sectionon'History'.)
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ThephysicalexaminationmayprovideinformationthatdifferentiatesCHDfromothercyanoticdisorderssuch
asrespiratorydiseaseorsepsis,andmayalsosuggestspecificcardiacdefects(table2).Physicalfindings
associatedwithCHDincludeanabnormalheartrate,heartsounds,pathologicmurmurs,bloodpressure
gradientbetweentheupperandlowerextremities,andhepatomegaly.(See'Physicalexamination'above.)
Achestradiographmayhelpdifferentiatebetweencardiacandpulmonaryetiologiesforneonatalcyanosis.
Abnormalfindingsinthelungfieldsareindicativeofapulmonarycause,whereasincreasedheartsize,
abnormalheartshape,andarightsidedaorticarcharesuggestiveofCHD.(See'Chestradiograph'above.)
Inacyanoticinfant,thehyperoxiatestmaybehelpfulindistinguishingcardiacfrompulmonarycausesof
cyanosis(table3andtable4).(See'Hyperoxiatest'above.)
EchocardiographyprovidesadefinitivediagnosisofCHDandinformationoncardiacanatomyandfunction.
Echocardiographyshouldbeperformedinanynewbornwithcentralcyanosiswhohasfailedahyperoxiatestor
hasanequivocalresult.(See'Echocardiography'above.)
Theinitialmanagementofnewbornswithcyanosisincludesgeneralsupportivecaretoensureadequatetissue
perfusionandoxygenation.
Thedifferentialdiagnosisforneonatalcyanosisincludessepsisasaresult,werecommendadministeringempiric
antibiotictherapyafterobtainingurineandbloodculturesinneonateswithcyanosis,inwhomnounderlyingcause
hasbeenidentified(Grade1A).(See"Clinicalfeatures,evaluation,anddiagnosisofsepsisintermandlatepreterm
infants",sectionon'Differentialdiagnosis'and"Managementandoutcomeofsepsisintermandlatepreterm
infants",sectionon'Initialempirictherapy'.)
IninfantswithaclinicalsuspicionforductaldependentCHD,werecommendprostaglandinE1(alprostadil)
administrationuntiladefinitivediagnosisofanonductaldependentlesionisestablished(Grade1A).Inpatientswith
ductaldependentCHD,werecommendprostaglandinE1administrationuntilcorrectivetreatmentisperformed.(See
'ProstaglandinE1'aboveand"Identifyingnewbornswithcriticalcongenitalheartdisease",sectionon'Cyanosis'and
"Identifyingnewbornswithcriticalcongenitalheartdisease",sectionon'Postnataldiagnosis'.)
IfitisnecessarytotransferaneonatewithcyanoticCHDwhoisreceivingprostaglandininfusion,thepatientshould
beintubatedandmechanicallyventilatedpriortoandduringtransportbecauseapneaisaknowncomplicationof
prostaglandintherapy.
Cardiaccatheterinterventionsincludepalliativeproceduresthatreducecyanosisbyimprovedmixingofoxygenated
anddeoxygenatedblood,orcorrectiveproceduresthatrelieveobstructivebloodflow.(See'Cardiaccatheterization'
above.)
ACKNOWLEDGMENTTheeditorialstaffatUpToDatewouldliketoacknowledgeLaurieBArmsby,MD,who
contributedtoanearlierversionofthistopicreview.
UseofUpToDateissubjecttotheSubscriptionandLicenseAgreement.
REFERENCES
1.DuffDF,McNamaraDG.Historyandphysicalexaminationofthecardiovascularsystem.In:Thescienceand
practiceofpediatriccardiology,GarsonAJr,BrickerTM,FisherDJ,NeishSR(Eds),WilliamsandWilkins,
Baltimore1998.p.693.
2.ReportoftheNewEnglandRegionalInfantCardiacProgram.Pediatrics198065:375.
3.LeesMH.Cyanosisofthenewborninfant.Recognitionandclinicalevaluation.JPediatr197077:484.
4.ZuberbuhlerJR.TetralogyofFallot.In:Heartdiseaseininfants,children,andadolescents,5thed,Emmanouilides
GC,RiemenschneiderTA,AllenHD,GetgesellHP(Eds),WilliamsandWilkins,Baltimore1995.p.998.
5.MairDD,EdwardsWD,JulsrudPR,etal.Truncusarteriosus.In:Heartdiseaseininfants,children,and
adolescents,5thed,EmmanouilidesGC,RiemenschneiderTA,AllenHD,GetgesellHP(Eds),Williamsand
Wilkins,Baltimore1995.p.1026.
6.JonesRW,BaumerJH,JosephMC,ShinebourneEA.Arterialoxygentensionandresponsetooxygenbreathingin
differentialdiagnosisofcongenitalheartdiseaseininfancy.ArchDisChild197651:667.
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Diagnosisandinitialmanagementofcyanoticheartdiseaseinthenewborn
7.DonofrioMT,MoonGradyAJ,HornbergerLK,etal.Diagnosisandtreatmentoffetalcardiacdisease:ascientific
statementfromtheAmericanHeartAssociation.Circulation2014129:2183.
8.LewisAB,FreedMD,HeymannMA,etal.SideeffectsoftherapywithprostaglandinE1ininfantswithcritical
congenitalheartdisease.Circulation198164:893.
9.MarinoBS,BirdGL,WernovskyG.Diagnosisandmanagementofthenewbornwithsuspectedcongenitalheart
disease.ClinPerinatol200128:91.
10.CheathamJP.Thetranscathetermanagementoftheneonateandinfantwithpulmonaryatresiaandintactventricular
septum.JIntervenCardiol199811:363.
11.FletcherSE,CheathamJP,BolamDL.Primarytranscathetertreatmentofcongenitalpulmonaryarteriovenous
malformationcausingcyanosisofthenewborn.CatheterCardiovascInterv200050:48.
12.BrowningCarmoKA,BarrP,WestM,etal.Transportingnewborninfantswithsuspectedductdependentcongenital
heartdiseaseonlowdoseprostaglandinE1withoutroutinemechanicalventilation.ArchDisChildFetalNeonatalEd
200792:F117.
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GRAPHICS
Cyanosisandhemoglobinconcentration
Thearterialoxygensaturationlevelatwhichcyanosisisdetectableat
differenttotalhemoglobinconcentrationsisillustratedabove.The
solidredportionofeachbarrepresents3g/dLreducedhemoglobin.
Reproducedwithpermissionfrom:LeesMH.Cyanosisofthenewborninfant.J
Pediatr197077:484.Copyright1970Mosby.
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Causesofcentralcyanosisintheneonate
Diseasecategory
Primaryunderlyingmechanism
Airwayobstruction
Choanalatresia
Laryngotracheomalacia
Macroglossia
Hypoventilation
Micrognathiaorretrognathia(eg,PierreRobin
syndrome)
Cardiac
Congenitalcyanoticheartdisease
Righttoleftshunting
Heartfailure/pulmonaryedema
ImpairedalveolararterialdiffusionandV/Q
mismatch
Hematologic
Hemoglobinopathies(eg,methemoglobinemia)
Impairedoxygensaturation
Polycythemia
Elevatedhemoglobinresultinginlowoxygen
saturation
Metabolic
Severehypoglycemia
Inbornerrorsofmetabolism
Hypoventilationduetodecreasedorabsent
respiratoryeffortsecondarytolethargy,seizures,
and/orapnea
Neurologic
Centralnervoussystemdepression
Apneaofprematurity
Infection(eg,meningitis,encephalitis)
Intraventricularhemorrhage
Hypoventilation
Maternalsedation
Seizure
Neuromusculardisorder
Neonatalmyastheniagravis
Phrenicnerveinjury
Hypoventilation
Spinalmuscularatrophytype1(Wernig
Hoffmandisease)
Pulmonary
Parenchymaldisease
Atelectasis
Alveolarcapillarydysplasia
Lobaremphysema
Pneumonia
Pulmonaryhypoplasia
V/Qmismatch
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Pulmonaryhemorrhage
Respiratorydistresssyndrome(Hyaline
membranedisease)
Transienttachypneaofthenewborn
Pulmonaryfibrosis
Impairedalveolararterialdiffusion
Pulmonaryedema
ImpairedalveolararterialdiffusionandV/Q
mismatch
Nonparenchymaldisease
Pleuraleffusion
Pneumothorax
V/Qmismatch
Other
Persistentpulmonaryhypertensionofthe
newborn
Righttoleftshunting
V/Q:ventilation/perfusion.
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Typicalphysicalexamination,chestradiography,andelectrocardiography
findingsinsomeformsofcyanoticheartdisease
Diagnosis
Physical
exam
S2
Chest
radiography
Murmur
Heart
size
PBF
Percent
RAA
Electrocardiogram
QRSaxis
Hypertrophy
Incidence
(per
100,000
live
births)*
TGA
single
none
90to150
nml
21
TOF
single
sys
boot
20
90to150
nml
20to26
HLHS
single
sys
vc
90to150
LVforces
16
PAIVS
single
sys
30to90
LVH,RAE
PS
single
sys
30to90
RVH,RAE
TAPVC
split
sys
,nl
,
vc
90to150
RAE
Tricuspid
atresia
single
sys
30to90
LVH,RAE
Truncus
arteriosus
single
sys/
dias
30
90to150
nml
Ebstein's
split
sys
90to150
RAE
NOTE:Thistablerepresentsthecommonpresentationofeachlesion.Variationsdooccur.For
example,tricuspidatresiausuallyisassociatedwithasmallventricularseptaldefectandpulmonary
stenosissomepatientswiththisdiagnosiscanhavealargeventricularseptaldefect,nopulmonary
stenosis,andincreasedpulmonarybloodflow.
dias:diastolicHLHS:hypoplasticleftheartsyndromeLV:leftventricularLVH:leftventricularhypertrophynl:
normalnml:normalforneonate(rightventricularpredominance)PAIVS:pulmonaryatresiaintactventricular
septumPBF:pulmonarybloodflowPS:pulmonarystenosisRAA:rightaorticarchRAE:rightatrial
enlargementsys:systolicTAPVC:totalanomalouspulmonaryvenousconnectionTGAIVS:dtranspositionof
thegreatarteries,intactventricularseptumTOF:tetralogyofFallotvc:venouscongestion.
*IncidencefromreportofNewEnglandRegionalInfantCardiacProgram.Pediatrics198065:375.
PatientswithTAPVCassociatedwithpulmonaryvenousobservationhavenormalheartsizeandvenous
congestiononthechestradiographywhilethosewithoutobstructionusuallyhavecardiomegalyandincreased
pulmonarybloodflow.
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TetralogyofFallot
Thischestradiographdemonstratessomeoftheclassicfindingsof
tetralogyofFallot.Thelungsarehyperinflated,andthereisoverall
decreasedpulmonaryvascularity.Thereisarightaorticarch.The
cardiacapexisupturnedduetorightventricularenlargement.
CourtesyofJeanneChow,MD,andChildren'sHospitalBoston.
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Hyperoxiatestresultsinneonateswithcyanosis
PaO 2
(percent
saturation)
whenFi0 2 =
1
PaO 2 (percentsaturation)
whenFiO 2 =0.21
PaCO 2
Normal
>70(>95)
>300(100)
35
Pulmonarydisease
50(85)
>150(100)
50
Neurologicdisease
50(85)
>150(100)
50
Methemoglobinemia
>70(<85)
>200(<85)
35
Parallelcirculation*
<40(<75)
<50(<85)
35
Mixingwithreduced
PBF
<40(<75)
<50(<85)
35
Mixingwithout
restrictedPBF
40to60(75to93)
<150(<100)
35
Cardiacdisease
Preductal
Postductal
Differentialcyanosis
70(95)
<40(<75)
Variable
35to50
Reversedifferential
cyanosis
<40(<75)
>50(>90)
Hyperoxiatest:Thetypicalresultsofpartialpressureofoxygen(PaO 2 )andpercentofoxygen
saturationfollowingadministrationofroomair(FiO 2 =0.21)or100percentoxygen(FiO 2 =1)to
neonateswithdifferentcausesofcyanosis.
FiO 2 :fractionalinspiredoxygenconcentrationPBF:pulmonarybloodflow.
*Dtranspositionofthegreatarterieswithorwithoutventricularseptaldefect.
Tricuspidatresiawithpulmonarystenosisorpulmonaryatresia,pulmonaryatresiaorcriticalpulmonary
stenosiswithintactventricularseptum,tetralogyofFallot.
Truncusarteriosus,totalanomalouspulmonaryvenousconnectionwithoutobstruction,hypoplasticleftheart
syndrome,singleventriclewithoutpulmonarystenosisorpulmonaryatresia.
Persistentpulmonaryhypertensionofthenewborn,interruptedaorticarch,severecoarctation.
Dtranspositionofthegreatarteriesassociatedwitheithercoarctationorsuprasystemicpulmonaryvascular
resistance.
Reproducedwithpermissionfrom:MarinoBS,BirdGL,WernovskyG.Diagnosisandmanagementofthenewborn
withsuspectedcongenitalheartdisease.ClinPerinatol200128:91.Copyright2001.
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MeanPaO 2 valuesinvarioustypesofcyanoticheartdisease
MeanPaO 2 (mmHg)
Conditions
Roomair
100percentoxygen
FiO 2 =0.21
FiO 2 =1
Commonmixinglesion*
42
64
HLHS
47
79
POTO
39
45
TGA
30
39
Meanarterialpartialpressureofoxygen(PaO 2 )forspecificcyanoticheartdefectsduringhyperoxia
testwithadministrationofroomairor100percentoxygen.
FiO 2 :fractionalinspiredoxygenconcentrationHLHS:hypoplasticleftheartsyndromePOTO:pulmonary
outflowtractobstructionTGA:transpositionofthegreatarteries.
*Lesionsincludetotalanomalouspulmonaryvenousconnection,tricuspidatresiawithlargeventricularseptal
defect,truncusarteriosus,doubleoutletrightventricle,andsingleventriclewithoutpulmonarystenosis.
LesionsincludetetralogyofFallot,tetralogyofFallotassociatedwithpulmonaryatresia,tricuspidatresia
associatedwithsmallventricularseptaldefect,doubleoutletrightventricleassociatedwithpulmonarystenosis,
andsingleventricleassociatedwithpulmonarystenosis.
Datafrom:JonesRW,BaumerJH,JosephMC,ShinebourneEA.Arterialoxygentensionandresponsetooxygen
breathingindifferentialdiagnosisofcongenitalheartdiseaseininfancy.ArchDisChild197651:667.
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Factorsthataffecttheneonataloxygen
dissociationcurve
Theoxygendissociationcurveofhumanbloodandtheeffectsof
changesintheH +ionconcentration,Pco 2 ,temperature,andlevelof
2,3diposphoglycerate(2,3DPG)aredepictedabove.Forfetal
hemoglobin,thenormalcurve(a)isshiftedtotheleft(b).
PCO 2 :partialpressureofcarbondioxidePO 2 :partialpressureofoxygenO 2 :
oxygen.
Reproducedwithpermissionfrom:LevinAR.Managementofthecyanotic
newborn.PedAnn198110:127.Copyright1981SLACK,Inc.
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Methemoglobinemia
Samplesofbloodwithvaryingmethemoglobinlevelsdisplayedonwhiteabsorbentmaterial.
Reproducedfrom:ShihanaF,DissanayakeDM,BuckleyNA,DawsonAH.Asimplequantitativebedside
testtodeterminemethemoglobin.AnnEmergMed201055:184.Illustrationusedwiththe
permissionofElsevierInc.Allrightsreserved.
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ContributorDisclosures
RobertLGeggel,MDNothingtodisclose.DavidRFulton,MDNothingtodisclose.LeonardEWeisman,MD
Grant/Research/ClinicalTrialSupport:VaxImmune[Ureaplasmadiagnosis,vaccines,andantibodies].
Consultant/AdvisoryBoards:GlaxoSmithKline[Malariavaccine]NIAID[Staphylococcusaureus(Mupirocin)].Patent
Holder:BaylorCollegeofMedicine[Ureaplasmadiagnosis,vaccines,antibodies,processforpreparingbiological
samples].EquityOwnership/StockOptions:VaxImmune[Ureaplasmadiagnosis,vaccines,andantibodies].Carrie
Armsby,MD,MPHNothingtodisclose.
Contributordisclosuresarereviewedforconflictsofinterestbytheeditorialgroup.Whenfound,theseareaddressedby
vettingthroughamultilevelreviewprocess,andthroughrequirementsforreferencestobeprovidedtosupportthecontent.
AppropriatelyreferencedcontentisrequiredofallauthorsandmustconformtoUpToDatestandardsofevidence.
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