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ThisspecializeddiagnosticexaminationisanextensionofthestandardultrasoundfetalassessmentdescribedinthePracticeParameterforthePerformanceofStandardDiagnosticObstetricUltrasoundExaminationsandtheAmericanCollegeofObstetriciansandGynecologistsPracticeBulletinNo.175:UltrasoundinPregnancy.cationsandResponsibilitiesofPhysiciansinterpretingorperformingthistypeofultra-soundexaminationshouldmeetthespeciedAIUMTrainingGuidelinesinaccordancewithAIUMaccreditationpolicies.SonographersperformingtheultrasoundexaminationshouldbeappropriatelycredentialedinthespecialtyareainaccordancewithAIUMaccreditationpolicies.Physiciansnotpersonallyperformingtheexaminationmustprovidesupervision,asdenedbytheCentersforMedicareandMedicaidServicesCodeofFederalRegulations42CFR410.32.RequestfortheExaminationThewrittenorelectronicrequestforanultrasoundexaminationmustoriginatefromaphysicianorotherappropriatelylicensedhealthcareproviderorundertheprovidersdirection.Theclinicalinformationpro-videdshouldallowfortheperformanceandinterpre-tationoftheappropriateultrasoundexaminationandshouldbeconsistentwithrelevantlegalandlocalhealthcarefacilityrequirements.IndicationsClinicalindicationsforfetalechocardiographyareoftenbasedonavarietyofparentalandfetalriskfac-torsforCHD.However,mostCHDcasesarenotassociatedwithknownfetaland/ormaternalriskfac-torsbut,rather,areoftensuspectedatthetimeofananatomicultrasoundsurvey.Forfetusessuspectedofhavinganabnormalfetalheartatthetimeofabasicordetailedanatomicultrasoundexamination,referralforfetalechocardiographyisindicated,astheriskofcantdiseaseishigh.ForpregnanciesatlowriskforCHD,cardiacscreeningultrasoundisprimarilyusedtoexaminethefetalheartasapartofastandardsecond-trimesterobstetricultrasoundexamination.Whenriskiselevatedabovethatofthegeneralpopu-lation,referralforfetalechocardiographymaybeindicateddependingonthelocalresources,clinicalsettings,examineravailability,andresultsofafetalcardiacscreeningevaluation.Althoughpreciseestimatesofriskareoutsidethescopeofthisdocument,thefollowingisalistofcom-monfetalandmaternalconditionsassociatedwithanincreasedriskofCHDFetalFactorsFetalechocardiographyisindicatedifthereis:SuspectedcardiacstructuralanomalySuspectedabnormalityincardiacfunctionHydropsfetalisPersistentfetaltachycardia(heartrate�180beatsperminute)Persistentfetalbradycardia(heartrate120beatsperminute)orasuspectedheartblockFrequentepisodesorapersistentlyirregularcardiacMajorfetalextracardiacanomalyNuchaltranslucencyof3.5mmorgreateroratorabovethe99thpercentileforgestationalageChromosomalabnormalitybyinvasivegenetictest-ingorwithcell-freefetalDNAscreeningMonochorionictwinningFetalechocardiographymaybeconsideredifthereis:Systemicvenousanomaly(eg,apersistentrightumbilicalvein,leftsuperiorvenacava,orabsentductusvenosus)Greater-than-normalnuchaltranslucencymeasure-mentbetween3.0and3.4mmMaternalorFamilialDiseaseorMaternalEnvironmentalExposureFetalechocardiographyisindicatedifthereis:Pregestationaldiabetesregardlessofthehemoglo-binAGestationaldiabetesdiagnosedintherstorearlysecondtrimesterAIUMPracticeParameterforthePerformanceofFetalEchocardiographyJUltrasoundMed2020;39:E5E16 Invitrofertilization,includingintracytoplasmicsperminjectionPhenylketonuria(unknownstatusoraper-iconceptionalphenylalaninelevel�10mg/dL)Autoimmunediseasewithanti-SjogrensyndromerelatedantigenAantibodiesandwithaprioraffectedfetusFirst-degreerelativeofafetuswithCHD(parents,siblings,orpriorpregnancy)First-orsecond-degreerelativewithdiseaseofMendelianinheritanceandahistoryofchildhoodcardiacmanifestationsRetinoidexposureFirst-trimesterrubellainfectionFetalechocardiographymaybeconsideredifthereis:Selectedteratogenexposure(eg,paroxetine,carba-mazepine,orlithium)Antihypertensivemedicationlimitedtoangiotensin-convertingenzymeinhibitorsAutoimmunediseasewithSjogrensyndromerelatedantigenApositivityandwithoutaprioraffectedfetusSecond-degreerelativeofafetuswithCHDOtherConsiderationsLimiteddataexisttosupporttheutilityoffetalecho-cardiographyforthefollowingisolatedconditions,givenminimalrisktothefetusandpotentialdifinimplementingfetalechocardiographyasroutineinsomeclinicalsettings.Adetailedfetalanatomicultra-soundexamination(CurrentProceduralTerminologycode76811),whichincludesanevaluationofthefetalheart,maybeappropriateinstead,withfetalechocar-diographyperformedonlyifanabnormalityisObesity(bodymassindex30kg/mSelectiveserotoninreuptakeinhibitorantidepres-santexposureotherthanparoxetinesoftmarkerforaneuploidyintheabsenceofkaryotypeinformationAbnormalmaternalserumanalytes(eg,-fetopro-teinlevel)IsolatedsingleumbilicalarteryEarlierstudiesmayhavepreviouslysuggestedanincreasedriskoffetalheartdiseaseforcertainconditionsorexposuresthathavenotbeenborneoutinlargerfollow-upstudies.Fetalechocardiographyinthesecasesisonlyindicatediftheresultsofadetailedfetalultrasoundexamination(CurrentProceduralTer-code76811)areabnormal.Thesecondi-tionsinclude:GestationaldiabetesdiagnosedafterthesecondWarfarinexposureAlcoholexposureEchogenicintracardiacfocusMaternalfeverorviralinfectionwithseroconver-siononlyIsolatedCHDinarelativefurtherremovedfromseconddegreetothefetuscationsoftheExaminationThefollowingsectiondescribesrequiredandoptionalelementsforfetalechocardiography.TechnicalConsiderationsFetalechocardiographyiscommonlyperformedbetween18and22weeksgestationalage,althoughsomecardiacstructuresmaybebettervisualizedbeforeorafterthisperiod.VariousformsofCHDmayalsoberecognizedatearlystagesofpregnancy,includingduringthenuchaltranslucencyexamination.Optimalviewsaretypicallyobtainedwhenthecardiacapexisup)towardthetransducer.Howeverevaluationsoftheatrialandventricularseptaandwallthicknessareimprovedwhentheultrasoundbeamistangentialorper-pendiculartothesestructures.Technicallimitations(eg,maternalobesity,fetalposition,andadvancedgesta-tion)mayimpedeadetailedevaluationofcardiacanat-omyduetopoorpenetrationandposterioracousticshadowing,especiallyduringthethirdtrimester.Optimizingtransducerplacementonthemater-nalabdomen,applyingadequatetransducerpressure,andchangingthematernalpositionaretechniquesthatmayimprovefetalpositioningandimagequality.Systemsettingsshouldbeadjustedwithanemphasisonmaintaininghighframerates(eg,usinganarroweldofview,smallimagingdepth,singleacousticfocus,andnarrowcolorDopplerultrasoundregionofinterestbox)withapplicationofacceptableacousticAIUMPracticeParameterforthePerformanceofFetalEchocardiographyJUltrasoundMed2020;39:E5E16 outputlevelsundertheALARA(aslowasreasonablyachievable)principle.Thedegreeofimagemagnitionshouldbeadjustedsothattheheartllsaboutone-thirdoftheimagingsectordisplay.Insomecases,itmaybenecessarytoreexaminethepatientatadif-ferenttimeduringgestationiftheheartispoorlyvisu-alizedduetotechnicalfactors.CardiacImagingGuidelines:BasicApproachThefetalechocardiogramisadetailedevaluationofcardiacstructureandfunction.Thisassessmentinvolvesasequentialsegmentalanalysisof4basicareasthatincludethesitus,atria,ventricles,andgreatarteriesandtheirconnections.Thisanalysisincludesaninitialassessmentofthefetalright/leftorientation,followedbyanassessmentofthefollowingsegmentsandtheirrelationships:Visceral/abdominalsitus:Positionofthestomach,portalvein,descendingaorta,andinferiorvenacavaintheaxialviewoftheabdomenCardiacapexpositionandcardiacaxisintheaxialviewofthechestSystemicandpulmonaryvenousconnectionsSystemicvenousanatomy,includingnormal/abnormalvariations(eg,ductusvenosus)Pulmonaryvenousanatomy,notingnormalcon-nectionofatleastonerightandoneleftpulmo-naryveinAtrialanatomy(includingtheseptum,foramenovale,andseptumprimum)Atrioventricularconnections(includingoffset-tingofthemitralandtricuspidvalves)Rightandleftventricularanatomy(includingtheRelativeandabsolutesizesSystolicfunctionGreatarteries(aorta,mainandbranchpulmonaryarteries,andductusarteriosus):VentricularconnectionsVesselsize,patency,andow(bothvelocityandRelativeandabsolutesizesoftheaorticisthmusandductusarteriosusPulmonaryarterybifurcationPositionofthetransverseaorticarchandductusarteriosusrelativetothetracheaThefollowingconnectionsshouldbealsoevalu-atedaspartofasegmentalanalysis:Atrioventricularjunction:anatomy,size,andfunc-tion(stenosisorregurgitation)ofatrioventricular(eg,mitralandtricuspidorcommonatrioventricu-lar)valvesVentriculoarterialjunction:anatomy,size,andfunction(stenosisorregurgitation)ofsemilunar(eg,aorticandpulmonaryortruncal)valves,includingassessmentsofboththesubpulmonaryandsubaorticregionsGrayscaleImagingKeyscanningplanescanprovideusefuldiagnosticinformationaboutthefetalheart(Figures1Theevaluationshouldincludethefollowinganatomicregions,includingtheupperabdomenforsitus,car-diacchambers,valves,vessels,andpericardium:Four-chamberview,includingpulmonaryveinsLeftventricularoutowtractRightventricularoutowtractBranchpulmonaryarterybifurcationThree-vesselview(includingaviewwithpulmo-naryarterybifurcationandamoresuperiorviewwiththeductalarch)Short-axisviews(forventriclesandowtracts)Long-axisview(ifclinicallyrelevant)AorticarchDuctalarchSuperiorandinferiorvenaecavaeColorDopplerUltrasoundColorDopplerultrasoundshouldbeusedtoevalu-atethefollowingstructuresforpotentialSystemicveins(includingsuperiorandinferiorvenaecavaeandductusvenosus)Pulmonaryveins(atleasttwo:onerightveinandoneleftvein)AtrialseptumandforamenovaleAtrioventricularvalvesAIUMPracticeParameterforthePerformanceofFetalEchocardiographyJUltrasoundMed2020;39:E5E16 Figure1.Representativescanplanesforfetalechocardiographyincludeanevaluationofthe4-chamberview(1),leftandrightarterialout-owtracts(2and3,respectively),twovariantsofthe3-vesselview,onedemonstratingthemainpulmonaryarterybifurcation(4)withanothermoresuperiorplanethatdemonstratestheductalarch(5),andthe3-vesselandtracheaview(6).Notallviewsmaybeseenfromasinglecephalictransducersweepwithoutsomeminoradjustmentsinthepositionandorientationofthetransducerduetoanatomicvaria-tionsandthefetallie.AscAoindicatesascendingaorta;DAo,descendingaorta;LA,leftatrium;LV,leftventricle;PA,pulmonaryartery;RA,rightatrium;RV,rightventricle;andTr,trachea. AIUMPracticeParameterforthePerformanceofFetalEchocardiographyJUltrasoundMed2020;39:E5E16 Figure2.Sagittalviewsofthesuperiorandinferiorvenaecavae(1),aorticarch(2),andductalarch(3).Thescananglebetweentheductalarchandthoracicaortarangesbetween10and19duringpregnancy,asillustratedbythe4-chamberviewdiagram(lowerright).Aoindi-catesdescendingaorta;AoRoot,aorticroot;DA,ductusarteriosus;IVC,inferiorvenacava;LA,leftatrium;LV,leftventricle;PV,pulmonaryvalve;RA,rightatrium;RPA,rightpulmonaryartery;RV,rightventricle;andSVC,superiorvenacava. AIUMPracticeParameterforthePerformanceofFetalEchocardiographyJUltrasoundMed2020;39:E5E16 Figure3.Highshort-axisview(1),lowshort-axisview(2),andlong-axisview(3)ofthefetalheart.Aoindicatesaorticvalve;LV,leftventricle;PA,pulmonaryartery;RA,rightatrium;andRV,rightventricle. AIUMPracticeParameterforthePerformanceofFetalEchocardiographyJUltrasoundMed2020;39:E5E16E11 VentricularseptumSemilunarvalvesDuctalarchAorticarchPulsedDopplerUltrasoundPulsed-waveDopplerultrasoundshouldbeusedtoevaluatethefollowing:RightandleftatrioventricularvalvesRightandleftsemilunarvalvesPulmonaryveins(atleasttwo:onerightveinandoneleftvein)DuctusvenosusSuspectedstructuralorowabnormalityoncolorDopplerimagingPulsed-waveDopplerultrasoundmayalsobeclinicallyrelevantforevaluatingtheductusarteriosus,systemicveins(eg,superiorvenacava,inferiorvenacava,andhepaticveins),aorticarchattheisthmus,branchpulmonaryarteries,middlecerebralartery,andumbilicalarteryorvein.HeartRateandRhythmAssessmentDocumentationoftheheartrateandrhythmshouldbemadebycardiaccyclelengthmeasurementsobtainedbytheDopplertechniqueorM-modeinter-rogation.Anormalfetalheartrateatmidgestationis120to180beatsperminute.Ifbradycardiaortachy-cardiaisdocumented,oriftherhythmisnotedtobeirregular,adetailedassessmentofatrialandventricularcontractionsshouldbeperformed.CardiacBiometryNormalrangesforfetalcardiacmeasurementshavebeenpublishedaspercentilesandscoresthatarebasedongestationalageorfetalbiometry.Individ-ualmeasurementsshouldbedeterminedfrom2-dimensional(2D)imagesandincludethefollowingparameters:Aorticandpulmonaryvalveannulusinsystole(absolutesizewithcomparisonofleft-toright-sidedvalves)Tricuspidandmitralvalveannulusindiastole(abso-lutesizewithcomparisonofleft-toright-sidedvalves)Additionalfetalcardiacbiometrycanalsobeper-formedforsuspectedstructuralandfunctionalcardiacanomalies,includingbutnotlimitedto:RightandleftventricularlengthsAorticarchandisthmusdiametermeasurementsfromthesagittalarchviewor3-vesselandtracheaviewwithcomparisonoftheaorticisthmustoductusarteriosusMainpulmonaryarteryandductusarteriosusEnd-diastolicventriculardiameterjustinferiortotheatrioventricularvalveleaetsintheshort-orlong-axisviewThicknessoftheventricularfreewallsandinter-ventricularseptumindiastolejustinferiortotheatrioventricularvalvesCardiothoracicratioAdditionalmeasurementsifclinicallyrelevant,Systolicventriculardimensions(shortorlongaxisviews)TransverseatrialdimensionsBranchpulmonaryarterydiametersCardiacFunctionAssessment(IfClinicallyRelevant)Rightandleftheartfunctionshouldbequalitativelyassessed.Signsofcardiomegaly,atrioventricularvalveregurgitation,andhydropsfetalisarekeycirculatoryndingsthatcanindicatefetalcardiacdysfunctionandshouldbenotedifpresent.Ifcompromisedfunctionissuspected,aquantitativeassessmentofheartfunctionmaybeperformedusingseveralmeasures,includingbutnotlimitedtofractionalshortening,42,43ventricularstrain,44,45andthemyocardialperformanceindex.ComplementaryImagingStrategies(IfClinicallyOtheradjunctiveimagingmodalities,suchas3-and4-dimensionalultrasound,havebeenusedtoevaluateanatomicdefectsandtoquantifyfetalhemodynamicparameters,suchascardiacoutput.AdjunctiveDopp-lermodalitiesincludetissueandcontinuouswaveDopplerultrasound.Additionalfetalcardiacfunc-tionalassessmentmodalitiessuchastricuspidannularplanesystolicexcursionandthesphericityindexhavealsobeenreported,althoughtheirroleinclinicalcareshouldbeconsideredinvestigationalatthistime.AIUMPracticeParameterforthePerformanceofFetalEchocardiographyJUltrasoundMed2020;39:E5E16 cDocumentationofHeartViewsInadditiontostill-frameacquisitionandstoragedocumentingthegrayscale,color,andpulsedDopplerviews,thefollowingmotionvideoclipsshouldbeobtainedforroutinedocumentation.Iftherearesuspectedstructuralorfunctionalcardiacanomalies,additionalmotionvideoclipsshouldbeconsidered.Requiredclipsinclude:Axialsweepfromthestomachtotheuppermedias-tinum,toincludethe4-chamberview,arterialout-owtracts,aswellasthe3-vesselandtracheaviewFour-chamberview:2DandcolorDopplerLeftventricularoutowtractview:2DandcolorDopplerultrasoundRightventricularoutowtractview:2DandcolorDopplerultrasoundThree-vesselandtracheaview:2DandcolorDopp-lerultrasoundSagittalviewoftheaorticandductalarches:2DandcolorDopplerultrasoundAccurateandcompletedocumentationisessentialforhigh-qualitypatientcare.Writtenreportsandultrasoundimages/videoclipsthatcontaindiagnosticinformationshouldbeobtainedandarchived,withrecommenda-tionsforfollow-upstudiesifclinicallyapplicable,inaccordancewiththeAIUMPracticeParameterforDocumentationofanUltrasoundExaminationAcompleteevaluationcanonlybeaccomplishedifacquisitionofanalogrecordingsordigitalmotionvideoclips,inconjunctionwithstillimages,isusedasastandardpartofeveryfetalechocardiogram.EquipmentSpecicationsAnultrasoundexaminationofthefetalheartshouldbeconductedusinganultrasoundsystemequippedwiththeabilitytoobtainM-mode,pulsedDoppler,andpower/colorDopplerimages.Sector,curvilinear,andendovaginaltransducersareusedforthispurpose.Useof3-and4-dimensionaltechnologyandcontinuouswaveDopplerultrasoundisoptionalifclinicallyrelevant.Thetransducershouldbeadjustedtooperateatthehighestclinicallyappropriatefrequency,usingacousticpowersettingsthatfollowtheALARAprinciple.Atrade-offexistsbetweenimageresolutionandbeampenetration.Withmodernequipment,fetalimagingstudiesperformedfromtheanteriorabdomi-nalwallcanusuallyusefrequenciesthatvarybetween1and9MHz,dependingonthebodyhabitusofthepatient.Furthermore,acousticshadowingandthematernalbodyhabitusmaylimittheabilityofhigher-frequencytransducersfromprovidinggreateranatomicdetailforthefetalheart.Endovaginalscansshouldbeperformedusingfrequenciesof5MHzorhigher.QualityandSafetyPoliciesandproceduresrelatedtoqualityassuranceandimprovement,safety,infectioncontrol,andequipmentperformancemonitoringshouldbedevel-opedandimplementedinaccordancewiththeAIUMStandardsandGuidelinesfortheAccreditationofUltrasoundPractices.ALARAPrincipleThepotentialbenetsandrisksofeachexaminationshouldbeconsidered.TheALARAprincipleshouldbeobservedforfactorsthataffecttheacousticoutputandbyconsideringthetransducerdwelltimeandtotalscanningtime.FurtherdetailsonALARAmaybefoundinthecurrentAIUMpublicationUltrasoundSafetyFetalSafetyDiagnosticultrasoundstudiesofthefetusaregener-allyconsideredsafeduringpregnancy(ConclusionsRegardingEpidemiologyforObstetricUltrasound).Diagnosticultrasoundshouldbeperformedonlywhenthereisavalidmedicalindication(PrudentUseinPregnancy).Thelowestpossibleultrasonicexpo-suresettingshouldbeusedtogainthenecessarydiag-nosticinformationundertheALARAprinciple.Theoutputdisplaystandard,anon-screenreal-timedisplayofacousticoutput,shouldbevisibleandmonitoredforthethermalindex(TI)andmechanicalindex(MI).Thedwelltimeshouldbekepttoamini-mum.ATIforsofttissue(TIs)shouldbeusedbeforeAIUMPracticeParameterforthePerformanceofFetalEchocardiographyJUltrasoundMed2020;39:E5E16 10weeksgestation,andaTIforbone(TIb)shouldbeusedatorafter10weeksgestationwhenboneossi-cationisevident(RecommendedMaximumScanningTimesforDisplayedThermalIndex(TI)ValuesDopplerultrasoundmaybeusedtoanswerspe-cclinicalquestions.SpectralpulsedDopplerultra-soundisassociatedwithhigherenergyoutputandshouldbeusedjudiciouslyaspartofanevaluationforanomalies.Thepromotion,selling,orleasingofultra-soundequipmentformakingkeepsakefetalvideosisconsideredbytheUSFoodandDrugAdministra-tiontobeanunapproveduseofamedicaldevice.Useofadiagnosticultrasoundsystemforkeepsakefetalimaging,withoutaphysiciansorder,maybeinviolationofstatelawsorregulations.InfectionControlTransducerpreparation,cleaning,anddisinfectionshouldfollowmanufacturerrecommendationsandbeconsistentwiththeAIUMGuidelinesforCleaningandPreparingExternal-andInternal-UseUltrasoundTransducersBetweenPatients,SafeHandling,andUseofUltrasoundCouplingGel.EquipmentPerformanceMonitoringMonitoringprotocolsforequipmentperformanceshouldbedevelopedandimplementedinaccordancewiththeAIUMStandardsandGuidelinesfortheAccreditationofUltrasoundPracticesThisparameterwasdevelopedbytheAIUMincollabo-rationwiththeAmericanCollegeofObstetriciansandGynecologists(ACOG),AmericanCollegeofRadiol-ogy(ACR),AmericanSocietyofEchocardiography(ASE),FetalHeartSociety(FHS),InternationalSocietyofUltrasoundinObstetricsandGynecology(ISUOG),SocietyforMaternal-FetalMedicine(SMFM),andSocietyofRadiologistsinUltrasound(SRU).Weareindebtedtothemanyvolunteerswhocontributedtheirtime,knowledge,andenergytodevel-opingthisdocument.AppreciationisparticularlyextendedtoKathiKeatonMinton,MA,RDMS,RDCS,forheradministrativeassistanceduringthedevelop-mentofthisdocumentandtoVictoriaWebster,MA,RT(MR),CNMT,RDMS,forheroriginalillustrations.CollaborativeCommitteeWesleyLee,MD,chairTracyAnton,BS,RDMS,RDCSJoshuaA.Copel,MDGreggoryR.DeVore,MDAnjaliKaimal,MD,MASIsabelleA.Wilkins,MDCarolB.Benson,MDMaryC.Frates,MDMaryT.Donofrio,MDAnitaJ.Moon-Grady,MDMaryT.Donofrio,MDAnitaJ.Moon-Grady,MDLaurentJ.Salomon,MD,PhDAlfredAbuhamad,MDTheodoreJ.Dubinski,MDPaulaJ.Woodward,MDAIUMClinicalStandardsCommitteeBryannBromley,MD,chairJamesM.Shwayder,MD,JD,vicechairNirviDahiya,MDRobGoodman,MD,MBBChir,MBARachelLiu,MDJeanLeaSpitz,MPH,CAE,RDMSJohnPellerito,MD,immediatepastchairAIUMExpertAdvisoryGroupHarrisL.Cohen,MDLisaHornberger,MDElenaSinkovskaya,MDJuliaSolomon,MDLamiYeo,MDOriginalcopyright2010;Revised2019,2013;Renamed20151.DonofrioMT,Moon-GradyAJ,HornbergerLK,etal.Diagnosisandtreatmentoffetalcardiacdisease:ascienticstatementfromAIUMPracticeParameterforthePerformanceofFetalEchocardiographyE14JUltrasoundMed2020;39:E5E16 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PRACTICEPARAMETERSAIUMPracticeParameterforthePerformanceofFetalEchocardiographyheAmericanInstituteofUltrasoundinMedicine(AIUM)isamultidisciplinaryassociationdedicatedtoadvancingthesafeandeffectiveuseofultrasoundinmedicinethroughprofessionalandpubliceducation,research,developmentofclinicalpracticeparameters,andaccreditationofpracticesperformingultrasoundexaminations. ©2019bytheAmericanInstituteofUltrasoundinMedicine JUltrasoundMed2020;39:E5E160278-4297www.aium.org