Andersons pediatric cardiology 2129
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theNoonan,Holt-Oram,Williams,andMarfansyndromesandthe22q11
deletionsyndrome—theriskis50%,thoughpenetrationandphenotypic
expressionmayvary.
SpecificCardiacLesions
Left-to-RightShunts
Simpleleft-to-rightshunts—includingthoseproducedbyaninteratrial
communication,ventricularseptaldefect,andpatencyofthearterialduct—are
generallywelltoleratedduringpregnancy.Theincreaseinvolumeloadis
counteractedtosomeextentbythefallinperipheralvascularresistance.
Complicationsthathavebeendescribedincludearrhythmias,cardiacfailure,and
paradoxicalembolism.17–20However,thesearerare;inaliteraturereview,
arrhythmiaswerereportedinonly1of123pregnanciesinwomenwithatrial
septaldefectsandinnopregnanciesinwomenwithventricularseptaldefects.
Noheartfailurewasreportedineithergroup.21
Atrioventricularseptaldefectsaremorecomplexandcanbeassociatedwith
regurgitationacrossboththerightandleftsidesofthecommonatrioventricular
junction.Comparedwithwomenwithsimpleshuntlesions,thosewith
atrioventricularseptaldefectaremorelikelytoexperiencecardiac
complications.18,22Whenintracardiacshuntsareassociatedwithpulmonary
hypertension,theriskishigherandmainlyattributabletothepulmonary
hypertension,discussedseparatelyfurtheron.
AorticStenosisandOtherLeftVentricular
OutflowTractLesions
Abicuspidaorticvalveisthemostcommoncauseofanobstructedleft
ventricularoutflowtractinwomenofchildbearingage.Asmallernumberof
casesaresecondarytosubvalvarorsupravalvarstenosisorotherabnormalities
atthevalvarlevel.Severeobstructionmaynotbewelltoleratedduring
pregnancybecausetheincreasedstrokevolumemayprovokeleftventricular
failure.Furthermore,thepressure-loaded,marginallycompensated,
hypertrophiedventriclemaypoorlytoleratelossofpreloadordepressionof
function,sohemorrhageortheeffectsofgeneralorregionalanestheticagents
canleadtohemodynamicembarrassment.Duringpregnancy,womenwithsevere
obstructionareatriskforangina,functionaldeterioration,cardiacfailure,and
arrhythmiasaswellassuddendeath,althoughadversematernalcardiacevents
arenotascommonasdescribedinearlyreports.Maternalcardiaccomplications
havebeenreportedinapproximately5%ormoreofpregnancies.23–26Asmany
astwo-fifthsofpatientswithseverestenosishaverequiredinterventionwithina
fewyearsafterpregnancy,sothispossibilityshouldbeaddressedduring
prepregnancycounseling.26Balloonaorticvalvoplastyandaorticvalvarsurgery
havebeenperformedsuccessfullyduringpregnancy.Becauseoftherisktothe
fetus,suchinterventionsshouldbeperformedonlyiftherearenoother
alternatives.Despiterelativelyreassuringmaternaloutcomes,fetal,neonatal,and
obstetriccomplicationsarecommoninwomenwithaorticstenosis.Womenwith
moderateorsevereaorticstenosiswhohavebeenpregnanthavehigherratesof
latecomplicationsascomparedwithwomenwithsimilaraorticstenosiswho
havenotbeenpregnant.27Aorticinsufficiency,ontheotherhand,isgenerally
welltoleratedunlessitissevereandassociatedwithdepressedleftventricular
function.
AorticCoarctation
Mostwomenwithcoarctationoftheaortahavehadsometypeofrepair.Repair
maybeassociatedwithlatesequelae,suchasrecoarctation,aneurysmsatthe
repairsite—especiallywhenDacronhasbeenusedforapatch—and
pseudoaneurysms.Thusimagingofthesiteofrepair,usuallybymagnetic
resonanceimaging,isoptimalpriortoconception.Patientswithunrepaired
coarctationorthosewithrepairedcoarctationandresidualorrecurrent
obstructionaresubjecttoupperbodyhypertension.Antihypertensivetreatment
directedattheupperbodymayexacerbatehypotensiondistaltothecoarctation,
andthistheoreticallycouldcompromiseplacentalperfusion.Incontemporary
studies,maternalmortalityinwomenwithrepairedcoarctationisrare,but
womenareatincreasedriskforpregnancy-inducedhypertension,preeclampsia,
andcomplicationsrelatedtotheassociatedbicuspidaorticvalve.28–30Dissection
oftheaortahasbeenreported.
PulmonaryValvarStenosis
Womenwithpulmonaryvalvarstenosistoleratepregnancywellinspiteofthe
pregnancy-associatedincreaseinpreload.
