Andersons pediatric cardiology 2130
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TetralogyofFallot
MostpatientswithtetralogyofFallotwillhaveundergoneanintracardiacrepair,
andmanywillhavepulmonaryregurgitationand/orrightheartdilationand
dysfunctionlateaftertheirrepair.Otherresiduaandsequelaeincludesurgical
scarsandpatchesthatcanactasasubstrateforarrhythmia,residualshunts,and
leftventriculardysfunction.IngeneralwomenwithtetralogyofFallotoftendo
wellinpregnancy,althougharrhythmiasandright-sidedheartfailurecan
occur.31–33Womenwithseverepulmonaryregurgitationandrightventricular
(RV)dysfunction,RVhypertrophy,orbranchpulmonarystenosisareat
increasedriskofdevelopingrightheartfailureduringpregnancy.33Insmall
series,RVdilationwasincreasedinwomenwhohadbeenpregnantcompared
withwomenwhohadneverbeenpregnant.34
EbsteinAnomaly
Ebsteinanomalycomprisesabroadspectrumofseverity.Thosewithsevere
formsofthediseasemaypresentearlyinlifewithcyanosisorright-sided
cardiacfailure,whereasthosewithmildformsmayfirstbedetectedincidentally
inadulthood.Theabilityofthehearttotoleratetheincreaseddemandsof
pregnancyisdependentonthesizeandfunctionofthefunctionalrightventricle,
thedegreeoftricuspidregurgitation,andthepropensitytoarrhythmias.Women
withinteratrialcommunications(atrialseptaldefectorpatentforamenovale)are
atriskforright-to-leftshuntingiftheyareunabletoadapttotheincreased
preload,andwomenwhoenterpregnancywithanestablishedright-to-leftshunt
attheatriallevelarelikelytodemonstrateworseninghypoxemiaandcyanosisas
pregnancyproceeds.Despitethesepotentialproblems,reportedpregnancy
outcomeshavebeenfavorable.35–37
CompleteTranspositionoftheGreatArteries
Atrialswitchprocedures(MustardorSenningoperation)weretheearlier
techniquesusedtorepairpatientswithtransposition,albeitnowtheseare
supersededinmostinstancesbythearterialswitchoperation.Mostwomenwith
transpositionnowofchildbearingagewillhavehadanatrialrepairininfancy;as
aconsequencetheywillhavethemorphologicallyrightventricleandtricuspid
valvesupportingthesystemiccirculation,whichisassociatedwithvariable
degreesofsystemicventriculardysfunctionandsystemicatrioventricularvalvar
regurgitation.Additionalsequelaethatmayaffectpregnancyincludea
propensitytoatrialarrhythmias,sinusnodaldysfunction,andobstructionorleak
acrosstheatrialbaffle.Cardiacfailure,functionaldeterioration,andarrhythmias
arethemaincomplicationsreportedduringpregnancy.38,39Late
echocardiographicevidenceofsystemicventriculardilationhasbeenreportedin
almostone-thirdofsuchwomen,anddeteriorationinthefunctionofthe
systemicventricleinone-quarter.40Inaddition,obstetriccomplications,suchas
prematureruptureofmembranes,prematurelabor,andprematuredeliveryare
frequent.38,39
Althoughonlylimitedoutcomedataareavailableforwomenwitharterial
switchoperationswhohavemorerecentlyreachedchildbearingage,41itis
anticipatedthatwomenwithoutmajorresiduaandsequelaeafterrepairwilldo
well.
CongenitallyCorrectedTransposition
Congenitallycorrectedtranspositionisalsoassociatedwithamorphologically
rightventriclesupportingthesystemiccirculation.Frequentlyassociated
anomaliesincluderegurgitationoftheabnormalsystemicatrioventricularvalve,
ventricularseptaldefect,pulmonarystenosis,and/ordisturbancesof
atrioventricularconduction.Althoughmaternaldeathshavenotbeenreported,
cardiacfailure,arrhythmias,endocarditis,stroke,andmyocardialinfarction(in
thesettingofasinglecoronaryartery)havebeendescribedascomplicationsof
pregnancy.42,43
FunctionallyUniventricularHeartsandthe
FontanCirculation
TheFontanprocedure,initiallydevelopedasapalliationtoimprove
hemodynamicsandrelievehypoxemiainpatientswithtricuspidatresia,hasbeen
extendedtoindividualswithothercomplexcongenitalcardiaclesionsnot
amenabletobiventricularrepair.Despiteoverallbenefit,patientsremainwith
functionallyuniventricularphysiologyandhavelimitedabilitytoincrease
cardiacoutput.LatecomplicationsinpatientswiththeFontancirculationinclude
elevatedrightatrialandsystemicvenouspressures,arrhythmias,ventricular
dysfunction,protein-losingenteropathy,andthromboemboliccomplications.All
theseproblemscanbeprovokedoraggravatedbytheadditionalhemodynamic
loadofpregnancy.WomenwiththeFontancirculationmustbeeducatedabout
thepotentialmaternalrisksofpregnancy;iftheychoosetobecomepregnant,
theymustbemonitoredclosely.Maternalarrhythmias,heartfailure,and
hemorrhagic/thromboticandnoncardiaccomplicationsarereported.44–47
Miscarriagesarecommonandhavebeenreportedinhalfofthosebecoming
pregnant.45
CyanoticCardiacDisease
Cyanosisisthevisiblemanifestationofmaternalhypoxemia.Inawomanwith
manifestorpotentialhypoxemiaowingtoright-to-leftshunting,itshouldbe
establishedwhethertheshuntisduetopulmonaryhypertension(often
Eisenmengersyndrome)oranothercause,sincepulmonaryhypertensionitself
impartsanextremelyhighrisktopregnancy,asdiscussedlater.Eveninthe
absenceofpulmonaryhypertension,womenwithcyanoticdiseaseareatriskfor
adversematernalcardiaceventsduringpregnancy,inparticularcardiacfailure,
arrhythmias,thrombosis,embolism,andendocarditis,althoughdeathisrare.48–51
Thepregnancy-inducedfallinsystemicvascularresistancewillfacilitaterightto-leftshunting,especiallywhentheshuntisattheleveloftheventriclesor
greatarteries.Fetaloutcomesarepoor.Infantsarebornsmallforgestationalage.
Livebirthsarecompromised,andprematurityiscommon.Ifmaternalsaturation
ofoxygenislessthan85%,onlyone-eighthoffetusesprogresstobeborn
alive.48
PulmonaryHypertension
Inspiteofadvancesintreatment,pulmonaryhypertensioncontinuestoimparta
veryhighriskforpregnancy.Theincreasedvolumeloaddirectedthroughthe
high-resistancepulmonarycircuitwillprovokeelevationsinsubpulmonary
ventricularpressure,potentiallycausingsubpulmonaryventricularfailure,and
willaugmentaright-to-leftshuntifpresent,therebyworseninghypoxemia.
Hypoxemiaactsasapulmonaryvasoconstrictor,thusestablishingavicious
cycle.Inaddition,pulmonarythrombosisandpulmonaryembolusaremore
likelyduringpregnancyandmayfurtherincreasepulmonaryvascularresistance.
Challengesatdelivery—includinglossofblood,epiduralanesthesia,and
adversehemodynamicresponsesassociatedwithexpulsiveeffortsofthemother
