Andersons pediatric cardiology 1866
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OutcomesAlongtheFontanPathway
NeonatalPalliation
PatientsundergoingstagedpalliationforfUVHareathighriskformortality.
Evenseeminglysimpleproceduressuchaspulmonaryarterybandingcarrya
hospitalmortalityof7%.228Similarly,constructionofamodifiedBlalockTaussigsystemic-to-pulmonaryarteryshunthasamortalityof6.7%,anda
centralshunthasariskof7.7%.228TheNorwoodprocedureforfUVHwith
systemicoutflowobstructionisamongthehighest-riskneonatalprocedures
routinelyperformedtoday.Amongprogramswithastronginterestinthissubset
ofpatients,therearereportsofsurvivalgreaterthan95%incertain
subgroups.304–307Theresultsareattributedtotheimprovementsandinnovations
incarecoveredinthischapter.TheSingleVentricleReconstructionTrial
providedachancetoseecontemporaryoutcomesoftheNorwoodprocedure
amongexperiencedcenters.One-yearsurvivalwasonly63.6%forthose
randomizedtoasystemic-to-pulmonaryarteryshuntand73.7%amongthose
whoreceivedtherightventricle-to-pulmonaryarteryconduit.308Recentdata
fromtheSocietyofThoracicSurgeonsCongenitalHeartDatabaseshows
Norwoodsurvivalhasimproved.Amongmorethan100congenitalheart
programsintheUnitedStatesandCanada,thehospitalsurvivalfortheNorwood
procedurehasreached86.3%.228
CurrentInterstageResults
ImprovementsinneonatalpalliationofpatientswithfUVHrevealedthe
increasedriskoftheinterstageperiod—thetimefrominitialpalliationtothe
secondstage,SCPC.Eventhewell-palliatedindividualwithfUVHcontinuesto
havephysiologicrisks,specificallyamultidistributioncirculation,volume
overloadtothesingleventricle,andcyanosis.Increasedphysiologic
vulnerabilitymaybeduetothedevelopmentofrecurrentlesions,suchasshunt
stenosisorarchobstruction,orintercurrentillnessthatwillresultinan
imbalanceintheoxygensupply-demandrelationship.Finally,thechallengesof
heartfailurewithcyanosiscanresultingrowthfailure.Homemonitoringusing,
initially,periodicpulseoximetrytoidentifyexcessivecyanosisandscalesto
identifygrowthfailureandacutedehydrationwasdevelopedtoidentifytheat-
riskpatientbeforecatastrophiccollapse.309Usingthesestrategies,some
programshavenearlyeliminatedinterstagemortality.310TheNationalPediatric
CardiologyQualityImprovementCollaborativetargetedreductionofinterstage
mortalityandhasdemonstratedasignificantreductionfrom9.5%to5.3%.244
CurrentResultsoftheSecondStageorSuperior
CavopulmonaryConnection
HistoricallytheSCPChasbeenalow-riskprocedure;indeed,thefirstreportof
theSocietyofThoracicSurgeonsCongenitalHeartDatabaseidentified73
patientswithhypoplasticleftheartsyndromeundergoingthesuperior
cavopulmonaryanastomosiswithnomortality.311However,withidentification
oftheinterstageperiodasoneofsustainedrisk,theageofSCPChasdecreased
bothasanefforttodecreasetheperiodofinterstagevulnerabilityandasa
strategyforthemanagementofpatientswhofailafterneonatalpalliationof
fUVH.Overallthisstrategyappearstobesuccessfulindecreasinginterstage
mortality.DatafromNationalPediatricCardiologyQualityImprovement
Collaborativedemonstratedlowerinterstagemortalityamongcentersthat
performedSCPCatlessthan5months(5.7vs.9.9months)withnodifferencein
SCPCsurvival,complications,orhospitallengthofstay.238However,earlier
SCPCasastrategyforthepatientdeemedtobeathighriskduringtheinterstage
periodisofquestionablebenefit.MezaandcolleaguesanalyzedtheSingle
VentricleReconstructionTrialdatasetandfoundthatamonglow-oraverage-risk
infants,SCPCbetween3and6monthspost-Norwoodwasassociatedwith
maximal3-yeartransplant-freesurvival.230Inhigh-riskpatients—specifically
thosewithrightventriculardysfunction,thosethatrequiredECMOafterstage1
palliationorhadlowerweightforage—z-scoredidnotbenefitfromearlier
SCPC.TheSingleVentricleReconstructionTrialfoundthehospitalmortalityto
be4.3%andthemedianhospitallengthofstay8days.Riskfactorsformortality
werenon-electiveSCPC,moderateorgreaterAVvalveregurgitation,andthe
needforAVrepair.201ThemostrecentSocietyofThoracicSurgeonsCongenital
Heartdatabasereportshowsanoverallmortalityof1.8%forallpatients
undergoingtheSCPCoverthelast4years.228
CurrentFontanResults
IntheeraofstagedsinglepalliationoffUVH,theperioperativeoutcomesofthe
Fontanareexcellent.Thisislikelyduetocombinationofbetterneonatal
palliationandearlyvolumeunloadingwiththeSCPCaswellasimproved
Fontancandidateselection.Severalrecentseriesnumberinghundredsofpatients
demonstrateamortalityof0.4%to4.0%.296,312–316ThemostrecentSocietyof
ThoracicSurgeonsdatabasereportshowsaFontanmortalitybetween0.5%and
1.2%.317
DespiteimprovementsinacuteoutcomefortheFontan,itisnoteworthythat
longitudinalresultsshowsignificantattritionalongthepathwayfromneonateto
Fontan.DatafromtheSingleVentricleReconstructionTrialshowa6-year
transplant-freesurvivalofonly59%fortheNorwoodwithaBlalock-Taussig
shuntand64%fortherightventricle-to-pulmonaryarteryconduit.318Arecent
analysisoftheAustralianandNewZealandFontanRegistry,whichincludes683
adultsurvivors,providesperspectiveonthelong-termoutcome.Theregistry
includes201atriopulmonaryconnectionsand482totalcavopulmonary
connections(249lateraltunnelsand233extracardiacconduits).Overallsurvival
wasgood;90%at30yearsofageand80%at40yearsofage,butsurvivalatage
30yearswassignificantlyworseforthepatientswithatriopulmonary
connections(P=.03).Therewassignificantlatefunctionalimpairment,with
only53%ofpatientsinNewYorkHeartAssociationfunctionalclassI.Only
41%ofFontanpatientswerefreeofseriousadverseeventsat40yearsofage.
Arrhythmiaswerefoundin136(20%),42(6%)hadreceivedapermanent
pacemaker,45(7%)hadhadathromboembolicevent,and135(21%)requireda
surgicalreintervention.319
