prenatal diagnosis of cri0cal cardiac defects a case series · discussion the american heart...

DISCUSSIONTheAmericanHeartAssocia0onreleasedtheirguidelinesontheDiagnosisandManagementofFetalCongenitalCardiacAnomalies,indica0ngthedifferent levelsof care for variousdefects inorder to facilitateprepara0ons fordelivery. Theseguidelineshavehelpedprepare thesepa0entswhoweredelivered inater0aryhospitalwithaspecializedcardiaccareteam,crea0ngan idealsetupforpostnatalevalua0on.Asshown in cases 2 and 5, although the prenatal diagnoses were accurate, the severity of the disease and plausibility and necessity ofinterven0onmays0lldifferpostnatally.

PrenatalDiagnosisofCri0calCardiacDefectsACaseSeriesAvelino,A.V.A.Sec0onofMaternalFetalMedicineDepartmentofObstetricsandGynecology INTRODUCTION

Congenitalheartdiseaseisthemostcommonbirthdefectandoccursinabout6-13livebirths,and25%ofthesearecri0cal.Cri0calCongenitalHeartisusuallydefinedasthoserequiringeithersurgicalorcatheterbasedinterven0onwithin

thefirstyearoflife.Prenataldiagnosisiskeysoeitherfetalinterven0onmaybedoneiffeasible,orsothatdeliveryandimmediatepostnatalevalua0onand

managementcanbeop0mizedinanidealseRng.Thisseriesreviewstheaccuracyofprenataldiagnosisandpostnatalinterven0on.

THECASESFivecasesofpossiblycri0calcardiacdefectswerediagnosedantenatallywithfetalechocardiography:

twocasesofTetralogyofFallot,onecaseofHypoplas0cLeUHeartSyndrome,onecaseofDoubleOutletRightVentriclewithAtroioventricularSeptalDefectandPulmonaryStenosis,andonecaseofCoarcta0onoftheAorta.Prenatalcounselingestablishedthattherewasahighriskofthesecasesthatwouldrequire

immediateneonatalsurgicalorcatheterinterven0on.Fivecasespresentedasfollows.Table1showsmaternaldata,ageofgesta0onondiagnosis,fetalechocardiographyfindingsandimages,postnatalechocardiographyfindingsand

images,cri0calcongenitalheartdiseasescreeningresult,andmanagement.AllbabiespresentedwithcyanosisatbirthexceptforCase5withCoarcta0on.Fetalechocardiographycorrelatedwellwithpostnatalechocardiography.Case1withTetralogyofFallotunderwentsten0ngoftheductusarteriosus,Case2withTetralogyofFallothadIntrauterineGrowthRestric0onandwasadvisedthatbothsurgeryandcatheterinterven0onwerenotfeasible.Case3withHypoplas0cLeUHeartSyndromewasgivenprostaglandinandunderwentductusarteriosussten0ngwithballoonatrialseptostomyand

scheduledforahybridprocedure.ForCase4withDoubleOutletRightVentricle,noimmediateinterven0onwasdonebutwasadviseddefini0vesurgeryaUernutri0onalbuild-up.Case5withCoarcta0onoftheaortaonlyhadasmallaor0cshelf

andsincethebabywasasymptoma0c,wassenthomeandadvisedarepeatechoaUer1month.

CASE PRENATALECHO DELIVERYANDPOSTNATALECHO POSTNATALINTERVENTION

Case139G1P0

28weeks:OverridingoftheAortaAVSDPulmonarystenosisTOF

31weeksPPROMModerateOverridingoftheaortaMalalignmentVSDInfundibularpulmonicvalveatresiaRightVentricularHypertrophyPersistentLeUSVC

Angiographywithsten0ngoftheductusarteriosusviacaro0darteriotomy

Case242

G4P1(1021)GDM,

33weeks:OverridingoftheaortaMalalignedVSDPulmonarystenosisTOF

372/7weeksGrowthRestric0onAtrialseptalaneurysmwithPFOVer0caloriginofatortuousPDAarisingfromtransverseaor0carch,OverridingoftheaortaVSD,Pulmonicatresia

NoneExpiredonthe9thdayoflife

Case331

G1P0

HLHS

24weeks:Mitralandaor0cvalvehypoplasiaDilatedleUventriclewithpoorsystolicfunc0onHLHS

40weeksPROMHypoplas0cLeUVentricleAtrialseptaldefectHypoplas0caor0candmitralvalve

HybridProcedureDuctusarteriorsussten0ngwithballoonatrialseptostomy

Case418

G1P0GDM

28weeks:AVSDDoubleOutletRightVentriclePulmonaryStenosisDORV

38weeksAVSD(RastelliTypeA)DoubleOutletRightVentricleGreatarteriessidebysidePulmonarystenosis,mild

PendingRepair

Case530

G1P0GDMInsulin

34weeks:Coarcta0onoftheAorta

38weeksAor0cShelf(Gradientof10mmHg)

None

*GDM=Gesta*onalDiabetesMellitus;TOF=TetralogyofFallot;HLHS=Hypoplas*cLe?HeartSyndrome;DORV=DoubleOutletRightVentricle;Coarc=Coarcta*onoftheAorta

REFERENCESDonofrioetal.(2014)DiagnosisandTreatmentofFetalCardiacDisease:AScien0ficStatementfromtheAmericanHeartAssocia0on.Circula0on.hcp://circ.ahajournals.org/content/early/2014/04/23/01.cir.0000437597.44550.5d#cited-by