Box80.1
PredictorsofMaternalCardiacComplications
inWomenWithHeartDisease
Cardiaceventsbeforepregnancy
Cardiacmedicationsbeforepregnancy
Smokinghistory
NewYorkHeartAssociationfunctionalclassIIIorIV
Cyanosisoroxygensaturation<90%
Systemicventricularsystolicdysfunction
Subpulmonaryventricularsystolicdysfunction
Leftheartobstruction
Pulmonaryatrioventricularvalveregurgitation
Systemicatrioventricularvalveregurgitation
Mechanicalprosthesis
Elevatedbrainnatriureticpeptidelevels
ABritishworkinggroupcreatedaconsensus-basedclassificationofriskfor
womenwithcardiacconditionsundergoingpregnancythatcategorizestherisk
usingtheWorldHealthOrganization(WHO)classificationandprimarilylesionspecificelements.1Theoriginalclassificationhasbeenmodifiedand
subsequentlybeenshowntobeapredictorofcardiaccomplicationsinwomen
withheartdisease.9–11High-risklesionsaccordingtotheWHOclassificationare
showninTable80.2.9Pregnancyiscontraindicatedinwomenwithcardiac
lesionsassociatedwithextremelyhighriskofmaternalmorbidityandmortality,
includingseveresystemicventriculardysfunction(systolicfunction<30%),
peripartumcardiomyopathywithanyresidualleftventriculardysfunction,severe
mitralstenosis,severesymptomaticaorticstenosis,Marfansyndromewithan
aorticrootdiametergreaterthan45mm,andpulmonaryarterialhypertension
fromanycauseincludingEisenmengersyndrome.Otherfactorsthatincrease
maternalcardiacriskduringpregnancyincludecomorbidmedicalconditions
suchasdiabetesandhypertensionaswellastheuseoffertilitytherapy.12
Althoughtherisksofadversematernaleventsduringpregnancyandtheearly
peripartalperiodarereasonablywelldescribed,fewdataareavailable
concerninglatematernaloutcomesinwomenwithcongenitallymalformed
hearts.Ingeneral,womenwhodevelopcomplicationsduringpregnancyareat
higherriskofcomplicationslateafterpregnancy(Fig.80.3).13
Table80.2
High-RiskCardiacLesionsAccordingtotheWorldHealth
OrganizationClassification
WHO
Risk RiskofPregnancy
Class9
III
Significantincreaseintheriskofmaternalmortalityorseveremorbidity.
Expertcounselingrequired.Ifpregnancyisdecidedupon,intensive
specialistcardiacandobstetricmonitoringneededthroughoutpregnancy,
childbirth,andthepuerperium.
IV
Extremelyhighriskofmaternalmortalityorseveremorbidity;pregnancy
contraindicated.Ifpregnancyoccurs,terminationshouldbediscussed.If
pregnancycontinues,careasforclassIII.
Examples
Mechanicalvalve
Systemicrightventricle
Fontancirculation
Cyanoticheartdisease
(unrepaired)
Othercomplexcongenital
heartdisease
Marfansyndrome
withoutaorticdilation
Aortadilation40–45
mminMarfan
syndrome
Aorticdilation45–50
mminaorticdisease
associatedwith
bicuspidaorticvalve
Pulmonaryarterial
hypertensionofanycause
Severesystemicventricular
dysfunction(LVEF<30%,
NYHAIIIorIV)
Previousperipartum
cardiomyopathywithany
residualimpairmentofleft
ventricularfunction
Severemitralstenosis,
severesymptomaticaortic
stenosis
Marfansyndromewith
aortadilated>45mm
Aorticdilatation>50
mminaorticdisease
associatedwith
bicuspidaorticvalve
Nativeseverecoarctation
LVEF,Leftventricularejectionfraction;NYHA,NewYorkHeartAssociation;WHO,WorldHealth
Organization.
FromRegitz-ZagrosekV,BlomstromLundqvistC,BorghiC,etal.ESCGuidelinesonthe
managementofcardiovasculardiseasesduringpregnancy:theTaskForceontheManagementof
CardiovascularDiseasesduringPregnancyoftheEuropeanSocietyofCardiology(ESC).Eur
HeartJ.2011;32:3147–3197.
FIG.80.3 Adversecardiaceventslateafterpregnancyinwomenwithand
withoutcomplicationsduringpregnancy.Theredlinerepresentsthelate
outcomesinwomenwhohadmaternalcardiaccomplicationsduring
pregnancy;thebluelinerepresentswomenwhodidnothavematernal
cardiaccomplicationsduringpregnancy.Time0,dateofpregnancy.(From
BalintOH,SiuSC,MasonJ,etal.Cardiacoutcomesafterpregnancyin
womenwithcongenitalheartdisease.Heart.2010;96:1656–1661.)
Womenwithcardiacdiseasealsohaveanincreasedriskofadversefetaland
neonataleventsincludingprematurebirth,weightatbirthsmallforgestational
age,respiratorydistresssyndrome,intraventricularhemorrhage,andfetalor
neonataldeath.14Thisriskisamplifiedbyspecificmaternalcardiacriskfactors
andfurtheramplifiedbyconcomitantmaternalnoncardiacriskfactorsfor
adversefetalandneonataloutcomes.Fetalrisksareinverselyrelatedtomaternal
cardiacoutput.15,16Thereisalsoanincreasedriskoftransmissionofheart
diseasetooffspring.Baselineprobabilityofacongenitallymalformedheartin
theabsenceofanaffectedrelativeisfrom0.4%to0.6%butincreasesabout10foldwhenaparentisaffected,withsomestudiessuggestingahigherrateof
transmissionwhenthemotheristheaffectedparent.Withsomelesionsthe
likelihoodoftransmissionishigher.Inautosomaldominantsyndromes—suchas