Andersons pediatric cardiology 965
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vasculature.Suchdissectionmayresultinlungparenchymalinjuryand
contusion.Itmayalsodisruptthebloodsupplytotheairways,resultingin
necrosisandinflammationwithanaccumulationofairwaysecretions.64
Excessiveairwaysecretionsmaycauseairflowobstructionandimpairedgas
exchange.Childrenarealsoatriskforreperfusionpulmonaryedema,whichis
commonafterthisprocedure.Theriskofreperfusionedemaisrelatedtothe
degreeofpreoperativestenosisofthereconstructedvessels65butisusuallyselflimitedanddoesnotaffectthedurationofpostoperativemechanical
ventilation.66Diuretictherapyandventilatorstrategiesoptimizingmeanairway
pressuretomaintainfunctionalreservecapacityareoftenhelpfultohasten
improvementofpresumptivereperfusionedema.Patientswhoundergounilateral
unifocalizationrevisionsurgeryorwhorequiremoreextensivereconstructionon
onesideareatriskforsignificantventilation/perfusiondeficits.Elevateddead
spaceventilationisassociatedwithlongerventilatorydysfunction.67Attentionto
lungrecruitment,airwayclearance,andventilation/perfusionmismatchare
importantinthepostoperativemanagementofunifocalization/pulmonaryartery
reconstructionpatients.
Otherpotentialrespiratorycomplicationsareairwayobstructionfromsurgical
rearrangementofMAPCAs,phrenicnerveinjury,pulmonaryhemorrhage,upper
airwayobstruction,andbronchomalacia.Childrenwitha22q11deletionareat
higherriskforpreoperativeairwayabnormalitiesandmalacia,whichmay
worsenaftersurgeryandcannecessitateprolongedmechanicalventilation.68
Earlydetectionandinterventionforrespiratorycomplicationsisimportantand
mayreducethedurationofstayintheintensivecareunitandhospitalization.
Diagnosticmodalitiesthataresometimesindicatedincludechestultrasound,
fluoroscopytoevaluatediaphragmaticmovement,computedtomography,
bronchoscopy,cardiaccatheterization,andlungperfusionscintigraphy,
dependingontheparticularscenario.Postoperatively,childrenmaydemonstrate
diaphragmaticweaknessorparalysisduetophrenicnerveinjury.Insuch
circumstances,ourpracticeistoperformsurgicaldiaphragmaticplicationafter
paradoxicaldiaphragmaticmovementisdocumentedbyfluoroscopyand
dependenceonpositivepressureventilationisdemonstrated.Incasesof
diaphragmaticweakness,musclestrengthgenerallyreturnsasphrenicnerve
injuryrecovers.Inpatientswithtracheobronchialcompressionormalacia,
tracheostomyissometimesnecessaryinchildrenwithprolongedpostoperative
respiratoryfailureand/ordependenceonnoninvasivepositivepressure
ventilationandcanaidindevelopmentalandpulmonaryrehabilitation.
Nutritionalandphysicalrehabilitationareinitiatedearlyaftersurgeryand
continuedthroughoutthepostoperativestay,asthesechildrenrequiresignificant
accessorymusclestrengthinordertopreventatelectasisandlungcollapse.
Enteralfeedsareinitiatedoncepatientsarehemodynamicallystable,andtotal
parenteralnutritionisgiventosupplementnutrition.Afterextubation,patients
mayrequirenoninvasivepositivepressureventilationandareweanedto
supplementaloxygenvianasalcannula.Nasalcannulaoxygensupplementation
maybecontinuedafterdischargefromthehospital.Childrenwithprolonged
exposuretobenzodiazepinesandnarcoticsareweanedslowlyandsometimes
dischargedwhilestillonoralmedications.Allchildrenwithouta
contraindicationreceiveanticoagulationwithaspirinforaminimumof1year
afterunifocalizationsurgery.
NursingConsiderations
ThemainnursingconsiderationsfollowingsurgeryforTOF/PA/MAPCAsare
focusedonclosehemodynamicmonitoring,airwayclearance,andrecognitionof
complications.Uponarrivalfromtheoperatingroom,normothermiais
maintained.Hyperthermiaincreasesmetabolicdemandandpredisposesto
tachyarrhythmias,whereashypothermiacanworsenpostoperativebleeding—a
significantriskinpatientswhohaveundergoneextensivedissectionand
pulmonaryarteryreconstructionwithprolongedcardiopulmonarybypass.Chest
tubeoutputandbloodproductreplacementareimportantindicatorsofthe
potentialneedforsurgicalreexplorationandrequireclosemonitoring.Fluid
shiftsarecommon,asisacutekidneyinjury,requiringclosemonitoringof
hemodynamicparametersandurineoutput.Airwayclearancebeginsinthe
immediatepostoperativeperiodwithfrequentsuctioningoftheendotracheal
tubeandtheinstitutionofmucolytictherapywithnebulizedN-acetylcysteine
andalbuterolonthefirstpostoperativeday.Thebedsidenurseworksclosely
withtherespiratorytherapisttooptimizeairwayclearanceandlungmechanics.
Itisimportanttocontinuepulmonaryclearanceafterextubation,andthebedside
nurseplaysakeyroleinearlymobilization,assistancewithcoughing,and
oropharyngealsuctioning.Theimportanceofthiseffortcannotbe
overemphasized;itcanmakethedifferencebetweenapatientbeingreintubated
andleavingtheintensivecareunit.Appropriatepaincontrolisalsocrucialto
facilitatemobilizationandtheclearanceofsecretions.Bedsidenursesarealso
keyadvocatesforpatientsandfamilies,whohaveoftencomefromlong
distancesandhaveexperiencedfrequenthospitalizationsandsurgeries,whichis
thecaseatourprogram.
Follow-upEvaluationandReintervention
Followingcompleterepair,whethersinglestageorafterpalliation,weperform
anechocardiogramandquantitativelungperfusionscintigraphyathospital
dischargeandrecommendrepeatingthesestudiesseveraltimesoverthefirst12
monthsasameansofscreeningforearlyadverseremodelingofthe
reconstructedpulmonarycirculation.Wealsorecommendaroutinecardiac
catheterization1yearafterrepair.Findingsindicativeofincreasingright
ventricularpressureorprogressivechangesinthedistributionoflungperfusion
onnoninvasiveevaluationmaymeritearliercatheterization.During
catheterization,evenmodestpulmonaryarterystenosisistypicallytreatedwith
balloonangioplastyinanefforttooptimizetheuniformityoflungperfusionand
pulmonaryarterypressure(Figs.36.21and36.22,Videos36.5through36.7),
evenifcentralpulmonaryarterypressureisnormalorlow.Stentimplantationis
avoided.Ifthereismoreextensiveorsevereobstructionalongwithelevated
centralpulmonaryarterypressureandballoonangioplastydoesnotprovide
sufficientrelief,surgicalrevisionisperformed.
