Prenatal Diagnosis of Cri0cal Cardiac Defects A Case Series · DISCUSSION The American Heart...
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DISCUSSION The American Heart Associa0on released their guidelines on the Diagnosis and Management of Fetal Congenital Cardiac Anomalies, indica0ng the different levels of care for various defects in order to facilitate prepara0ons for delivery. These guidelines have helped prepare these pa0ents who were delivered in a ter0ary hospital with a specialized cardiac care team, crea0ng an ideal setup for postnatal evalua0on. As shown in cases 2 and 5, although the prenatal diagnoses were accurate, the severity of the disease and plausibility and necessity of interven0on may s0ll differ postnatally. Prenatal Diagnosis of Cri0cal Cardiac Defects A Case Series Avelino, A.V.A. Sec0on of Maternal Fetal Medicine Department of Obstetrics and Gynecology INTRODUCTION Congenital heart disease is the most common birth defect and occurs in about 6-13 live births, and 25% of these are cri0cal. Cri0cal Congenital Heart is usually defined as those requiring either surgical or catheter based interven0on within the first year of life. Prenatal diagnosis is key so either fetal interven0on may be done if feasible, or so that delivery and immediate postnatal evalua0on and management can be op0mized in an ideal seRng. This series reviews the accuracy of prenatal diagnosis and postnatal interven0on. THE CASES Five cases of possibly cri0cal cardiac defects were diagnosed antenatally with fetal echocardiography: two cases of Tetralogy of Fallot, one case of Hypoplas0c LeU Heart Syndrome, one case of Double Outlet Right Ventricle with Atroioventricular Septal Defect and Pulmonary Stenosis, and one case of Coarcta0on of the Aorta. Prenatal counseling established that there was a high risk of these cases that would require immediate neonatal surgical or catheter interven0on. Five cases presented as follows. Table 1 shows maternal data, age of gesta0on on diagnosis, fetal echocardiography findings and images, postnatal echocardiography findings and images, cri0cal congenital heart disease screening result, and management. All babies presented with cyanosis at birth except for Case 5 with Coarcta0on. Fetal echocardiography correlated well with postnatal echocardiography. Case 1 with Tetralogy of Fallot underwent sten0ng of the ductus arteriosus, Case 2 with Tetralogy of Fallot had Intrauterine Growth Restric0on and was advised that both surgery and catheter interven0on were not feasible. Case 3 with Hypoplas0c LeU Heart Syndrome was given prostaglandin and underwent ductus arteriosus sten0ng with balloon atrial septostomy and scheduled for a hybrid procedure. For Case 4 with Double Outlet Right Ventricle, no immediate interven0on was done but was advised defini0ve surgery aUer nutri0onal build-up. Case 5 with Coarcta0on of the aorta only had a small aor0c shelf and since the baby was asymptoma0c, was sent home and advised a repeat echo aUer 1 month. CASE PRENATAL ECHO DELIVERY AND POSTNATAL ECHO POSTNATAL INTERVENTION Case 1 39 G1P0 28 weeks: Overriding of the Aorta AVSD Pulmonary stenosis TOF 31 weeks PPROM Moderate Overriding of the aorta Malalignment VSD Infundibular pulmonic valve atresia Right Ventricular HypertrophyPersistent LeU SVC Angiography with sten0ng of the ductus arteriosus via caro0d arteriotomy Case 2 42 G4P1 (1021) GDM, 33 weeks: Overriding of the aorta Malaligned VSD Pulmonary stenosis TOF 37 2/7 weeks Growth Restric0on Atrial septal aneurysm with PFO Ver0cal origin of a tortuous PDA arising from transverse aor0c arch, Overriding of the aorta VSD, Pulmonic atresia None Expired on the 9 th day of life Case 3 31 G1P0 HLHS 24 weeks: Mitral and aor0c valve hypoplasia Dilated leU ventricle with poor systolic func0on HLHS 40 weeks PROM Hypoplas0c LeU Ventricle Atrial septal defect Hypoplas0c aor0c and mitral valve Hybrid Procedure Ductus arteriorsus sten0ng with balloon atrial septostomy Case 4 18 G1P0 GDM 28 weeks: AVSD Double Outlet Right Ventricle Pulmonary Stenosis DORV 38 weeks AVSD (Rastelli Type A) Double Outlet Right Ventricle Great arteries side by side Pulmonary stenosis, mild Pending Repair Case 5 30 G1P0 GDM Insulin 34 weeks: Coarcta0on of the Aorta 38 weeks Aor0c Shelf (Gradient of 10mmHg) None *GDM=Gesta*onal Diabetes Mellitus; TOF=Tetralogy of Fallot; HLHS=Hypoplas*c Le? Heart Syndrome; DORV=Double Outlet Right Ventricle; Coarc=Coarcta*on of the Aorta REFERENCES Donofrio et al. (2014) Diagnosis and Treatment of Fetal Cardiac Disease: A Scien0fic Statement from the American Heart Associa0on. Circula0on. hcp://circ.ahajournals.org/content/early/2014/04/23/01.cir.0000437597.44550.5d#cited-by
Transcript of 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
