Andersons pediatric cardiology 2127
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women,avasovagalreactionwilloccurwhenthecervixisinstrumentedfor
placementofadevice.Thiscanbeparticularlyhazardousinwomenwith
pulmonaryhypertensionandthosewiththeFontancirculation.Womeninwhom
pregnancycarriesaveryhighriskshouldconsidersterilization.Vasectomyis
sometimesagoodalternative,butthepossibilitythatthehealthymalepartner
mayoutlivehisspouseandwishtofatherchildrenwithanewpartnershouldbe
considered.Emergencycontraception(“themorning-afterpill”)isavailablein
formscontainingestrogenandprogestin,orprogestinonly,andissafefor
womenwithcongenitalheartdisease.
GeneralApproachtoRiskAssessment
Maternalcardiacdiseaseisariskfactorformaternal,fetal,andneonatal
complicationsduringpregnancy,andcarefulriskstratificationisanimportant
aspectofcareofthepregnantpatientwithheartdisease.Whenpregnantwomen
withcardiacdiseasearebeingevaluated,anassessmentismadeoftheriskof
adversematernalcardiacevents,withfetalandneonatalrisksconsidered
separately.Cardiacmorbidityandmortalityinpregnantwomenwithheart
diseaseinthreeofthelargecohortstudiesareshowninFig.80.1.3Theriskof
pregnancyvariesaccordingtomanyfactors,whicharediscussedindetailinthe
followingtext.
FIG.80.1 Morbidityandmortalityinwomenwithheartdisease:Results
fromtheCARPREGstudy,theZAHARAstudy,andtheROPACstudy.
CARPREG,CanadianPregnancyandHeartDiseaseStudy4;ZAHARA,
ZwangerschapbijvrouwenmeteenAangeborenHARtAfwijking
(PregnancyinWomenwithCongenitalHeartDisease)7;ROPAC,Registry
ofPregnancyandCardiacdisease.10(FromGreutmannM,SilversidesCK.
TheROPACregistry:amulticentrecollaborationonpregnancyoutcomesin
womenwithheartdisease.EurHeartJ.2013;34:634–635.)
Toestimatethematernalcardiacriskinpregnancy,generalpredictorsofrisk
(e.g.,maternalfunctionclassorthepresenceofcyanosis)shouldbeintegrated
withlesion-specificrisks(e.g.,specificrisksinwomenwiththeMustard
operation)andindividualclinicalvariables.In2001,aCanadianconsortium
(CanadianPregnancyandHeartDiseaseStudy:CARPREGStudy)prospectively
developedandvalidatedthefirstgeneralriskindexforpredictingriskin
pregnantwomenwithheartdisease.4TheCARPREGriskscorehas
subsequentlybeenrevisedandtheCARPREGIIriskindexincludes10risk
predictors:fivegeneralpredictors(priorcardiaceventsorarrhythmias,poor
functionalclassorcyanosis,high-riskvalvedisease/leftventricularoutflowtract
obstruction,systemicventriculardysfunction,nopriorcardiacinterventions);
fourlesionspecificpredictors(mechanicalvalves,high-riskaortopathies,
pulmonaryhypertension,coronaryarterydisease);andonedelivery-of-care
predictor(latepregnancyassessment)5(Fig.80.2).InadditiontotheCARPREG
studies,othergroupshavedevelopedriskpredictionmodels6–8;Box80.1shows
thereportedpredictorsofadversecardiacoutcomesduringpregnancy.
FIG.80.2 CardiacDiseaseinPregnancy(CARPREGII)riskscore:
incidenceofadversecardiaceventsduringpregnancyinwomenwithheart
disease.The10predictorsintheCARPREGIIriskindexareshowninthe
box.Eachpredictorisassignedapointscore,andthesumofpoints
correspondstoariskofcardiaccomplicationsduringpregnancy.Risk
scoresareshownonthex-axis.Thebarsshowthepredicted(redbars)
andobserved(yellowbars)frequencyofadversecardiaceventsinthe
derivationgroupandtheeventrateinthevalidationgroup(bluebars).
(FromSilversidesCK,GrewalJ,MasonJ,etal.Pregnancyoutcomesin
womenwithheartdisease:theCARPREGIIstudy.JAmCollCardiol.
2018;71[21]:2419–2430.)
