Andersons pediatric cardiology 967
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Surgicaltechniqueforreconstructionofperipheralpulmonaryartery
stenosisandothercomplexperipheralreconstructions.AnnThoracSurg.
2016;102:e181–e183.)
Inourexperience,formanypatientsthefirstinterventionafterunifocalization
andcompleterepairisfordysfunctionoftherightventricle-to-pulmonaryartery
conduit,whethersurgicalreplacement,transcatheterpulmonaryvalve
replacement,orangioplasty/stentinginpatientswithasmallconduitandearly
obstruction.45Therearenomajortechnicaldifferencesbetweensurgicalor
transcatheterconduitreplacementinthiscohortcomparedwithothers.70The
samefactorsthatpredictearlierconduitreinterventioninothersurgicallesions
wereassociatedwithshorterfreedomfromconduitproceduresinourexperience,
includingyoungerageandsmallerconduitsize.Asnotedearlier,atthetimeof
conduitreplacement,anynecessarypulmonaryarteryrevisionisperformedas
well.
Outcomes
Werecentlyreportedoutcomesinaconsecutiveseriesof458patientswith
TOF/PA/MAPCAs(34withvalvarseverepulmonarystenosisratherthanatresia)
whounderwentsurgeryatourcenterfromNovember2001throughApril
2016.45Approximatelyone-thirdofthesepatientshadundergoneoneormore
priorsurgicalprocedureselsewhere.Therestunderwenttheirinitialsurgical
managementatourcenter,whichconsistedofasingle-stagecomplete
unifocalizationandrepairin74%,anaortopulmonarywindowin16%(most
withacompletelydualsupplycirculation),andunifocalizationtoashuntor
anotherpalliationin20%.Themedianintensivecareunitstaywas9daysand
themediandurationofhospitalizationwasapproximately2weeks.Early
mortalityintheentirecohortwas3.5%;overall,84%werealivewithacomplete
repairatthemostrecentfollow-upand4%werealivewithapalliated
circulation.Therightventricularpressureearlyaftercompleterepairaveraged
35mmHg,andtherightventricle–to–aorticpressureratiowas0.35.Atfollowup,pulmonaryarterypressuresweresimilarlylow.45,71Asnotedearlier,our
follow-uprecommendationsincluderoutinecatheterization1yearafterrepair,
anditisnotuncommonforballoonangioplastytobeperformedatthattime,
evenforminorstenoses,inanefforttomaintainthelowesttotalpulmonary
resistancepossible(seeFigs.36.21and36.22,andVideos36.8and36.9).
Surgicalreinterventiononthepulmonaryarterieswasuncommon;whenitwas
performed,itwasoftenatthetimeofconduitreplacementandinvolvedonly
unilateralintervention.72Contrarytowidespreadopinion,inthisandprior
studies44nonconfluentorabsentnativecentralpulmonaryarteriesdidnot
constituteariskfactorforworseoutcome,eitherintermsofsurvival,
reintervention,orpulmonaryarterypressures.Althoughearlypostrepairright
ventricularpressureswerelow,ahigherrightventricle–to–aorticpressureratio
wasstillassociatedwithmortalityovertime,aswasalargernumberof
unifocalizedMAPCAs.Amongtheentiretreatedcohort,patientswhosefirst
surgeryatourcenterwascompleterepairhadbettersurvivalovertime,and
thosewithchromosomalabnormalities(achromosome22q11deletion,Alagille
syndrome,oranotherchromosomalanomaly)wereathigherrisk.On
multivariableanalysis,unifocalizationtoashuntasthefirstoperationand
chromosomalanomalywereassociatedwithworsesurvival.
Itisdifficulttocompareoutcomesbetweenmanagementstrategiesfor
TOF/PA/MAPCAsduetovariabilityinthenumberandsequencingof
procedures,incompletedataonpulmonaryarterypressures,lackofacleartime
thresholdfordefiningsuccess,andlackoflong-termfollow-up.However,given
theultimateobjectiveofachievingacompletelyseparatedcirculationwiththe
lowestpossiblerightventricularpressure,acrudecompositemetricforassessing
outcomesisthelikelihoodofsurvivingwithacompleterepair(including
completeclosureofventricularseptaldefect)andacceptablylowright
ventricularorpulmonaryarterypressure.Inanefforttoprovidesome
comparativecontextonoutcomes,wecalculatedthepercentageofpatientswho
achievedthisdesiredclinicaloutcomeinourexperienceandpriorreportsthat
includedsufficientdatatoallowanestimate.Inthe458-patientcohortfromour
centerreportedrecently,45furtheranalysisrevealedthat84%ofpatientssurvived
completerepairwithlowsystolicrightventricular/pulmonaryarterialpressure
(≤50%ofsystolicaorticpressure)and79%werealivewithcompleterepairand
lowrightventricularpressureatthemostrecentfollow-upevaluationamedian
of4.7yearsafterthefirstsurgeryatourcenter.Thefiguresweresimilarforthe
subsetofpatientswhoseinitialmanagementwasatourcenter(i.e.,excluding
thosewhohadundergonesurgerypriortoreferral).Althoughthiscross-sectional
estimatedoesnottakeintoaccountpotentialtemporalvariabilityinachieving
thedesiredoutcome,reportsfromothercenterssuggestasubstantiallylower
likelihoodofsurvivalwithacompleterepairandlowrightventricularpressure,
typicallybelow40%(Table36.1).Althoughthiscomparisonreliesonanumber
ofassumptionsanddoesnotincludeseveralimportantseriesfromcenterswitha
largeexperienceduetoinsufficientdatatoestimatethecompositemetric,20,22,36
itprovidesageneralsenseoftherelativeoutcomesachievedwithourstrategy.
Table36.1
EstimatedFrequencyofDesiredClinicalOutcomeinPreviously
ReportedSeries
Study
Carottiet
al.17
Soquetet
al.31
Dragulescu
etal.24
Iyeret
al.29
Guptaet
al.27
Chenet
al.19
Songet
al.30
No.of
MedianorMeanDuration
Patients ofFollow-Up(Years)
90
3.7
Approximate%ofPatientsAliveWithCompleteRepair
andRV/PAPressure≤50%ofSystemica
<40
37
4.6
<35
20
8.4
<35
58
3.6
<35
104
10.2
<30
69
2.8
<25
40
4.5
<20
aEstimatesarereportedaslessthanavalue(e.g.,<40%)becauseformoststudiesanaverageor
medianrightventricletoaorticpressureratiowasreported.Thusacorrespondingpercentage
rangeofpatientswitharatio≤0.5wasapproximatedconservatively,withtheestimatereportedin
thiscolumnatthehigherendofthecalculatedrange.
Thistableincludespriorreportswithadequatedatatoestimatethecompositeoutcomemetric.
Severalimportantserieswerenotincludedduetoinsufficientdatatoestimatethepercentageof
patientswiththedesiredclinicaloutcome.
PA,Pulmonaryatresia;RV,rightventricle.
Usingaprogrammaticapproachthatemphasizesearlycomplete
unifocalizationandrepairwithincorporationofalllungsegmentsandextensive
lobarandsegmentalPAreconstruction,wehaveachievedexcellentresultsin
patientswithnativediseaseaswellasthosewhohaveundergonevarious
interventionspriortoreferraltoourcenter.Patientswithchromosomal
anomaliesexperienceworseoutcomesforreasonslikelyrelatedinpartto
extracardiacfactors.Someofthepreoperativeevaluationsrecommendedinthis
chapterareaimedinpartatreducinglikelyriskfactorsrelatedtothesegenetic
anomalies.Althoughitisdifficulttodrawcomparisonsbetweendifferentstudies
andapproachesbyindirectcomparisonoftheliterature,thenumberofpatients
