Andersons pediatric cardiology 1857

identified,plicationofthediaphragmisgenerallysufficienttoweanthepatient
frommechanicalventilation.181–185Attemptsshouldalsobemadetoweanhim
orherfromsedativeagentsandsteroids.Primarypulmonary,neurogenic,and
infectiousetiologiesmustalsobeconsidered.Thedecisiontocommitaneonate
toatracheostomy,withorwithoutchronicmechanicalventilation,isdifficult;
however,onceundertaken,apostoperativetracheostomytypicallyexpedites
weaningfromsedatives,advancingnutritionalstatus,andimprovingneurologic
development.171,186,187
Acutekidneyinjurymaysignificantlydelaypostoperativerecovery.In
neonates,theneteffectofacutekidneyinjuryismoretypicallyimpaired
filtrationratherthansoluteclearance.Aninabilitytomobilizefluidwillrapidly
leadtopoorcardiacandlungcompliance.Filtrationdisordersintheneonatemay
beaddressedwithperitonealdialysis.Theneedfordialysisintheearly
postoperativeperiodfollowingneonatalpalliationforuniventricularheart
diseaseisassociatedwithasignificantlyincreasedmortalityrisk.188–191
Nutritionaldeficienciesmayalsodelayprogress;moststudiesreporttheuse
ofsupplementaltubefeedingof40%ormorefollowingneonatalpalliationfor
fUVH.192–194Theclinicianshouldensurethattheappropriatenutritionalgoals
arebeingmetviaenteralorparenteralmeans.Ifthepatientistoleratingenteral
feeds,acarefulassessmentofthevocalcords,swallowing,andrefluxrisk
shouldbemadepriortotheinitiationoforalfeeds.Oncethepatienthas
achievedastablephysiologicprofile,considerationofasurgicallyplacedgastric
tubewillbepossible.187,195,196

FamilySupportandDischargePlanning
Comprehensivedischargeplanningfollowingsurgicalinterventionsinall
neonatesiscriticalandperhapsmostimportantinthisfragilepatientpopulation.
Aswithallneonatesrequiringinterventionsafterbirth,thebabyhasneverbeen
home,familysupportstructureshavenotyetbeenestablished,andaformalvisit
withtheprimarycareproviderhasnottakenplace.Fortheneonatewitha
multidistributioncirculation,theserisksarecompoundedbyatenuous

physiology,complexhospitalcourse,andahighfrequencyofadditionalmedical
concerns.Aspartofthecomprehensiverisk-reductionstrategydiscussedin
Chapter72,establishmentofamedicalhomeiscritical.Thisrequiresproactive
multidisciplinarycoordinationstartingwellbeforepatientsaremedicallyready
fordischarge.Parentsmustbewelleducatedandasemotionallypreparedas


possiblefortherealitiesofcaringforaneonatewithatenuouscirculation.
Interstagemonitoringprogramsprovideelevatedmonitoringofinfantwellness
aswellasparentalcoping.Parentsshouldbeencouragedtoperformallinfant
care,medicationmeasurement,anddeliverypriortodischargeandmaybenefit
fromtheopportunityto“roomin”for24to48hourspriortodischarge.
Dischargeisoverwhelmingandfear-inducingformanyfamilies,soeducation
shouldbeongoingthroughoutadmissionratherthanclusteredtowardtheendof
thehospitalization.Ideally,overthecourseofadmissiontheparentwillbecome
ascomfortablewiththeneedsoftheinfantasthebedsidestaffandmedicalteam
havebecome.56,197–200


Second-StagePalliation:TheSuperior
CavopulmonaryConnection
StagedsurgicalpalliationforpatientswithafUVHtypicallyincludesan
intermediateorsecond-stageprocedure,thesuperiorcavopulmonary
anastomosis,whicheliminatesvolumeloadontheventricle.Thisreducesboth
wallstressand(potentially)AVvalveinsufficiencyaswellasincreasing
effectivepulmonarybloodflowandachievingamoreefficientin-series
circulation.Inpatientswithapriorsystemic-to-pulmonaryarteryshunt,thisalso
includesincreaseddiastolicpressurewithimprovedcoronaryarteryperfusion
seeBox71.5.201–204

Box71.5

GoalsofSuperiorCavopulmonaryConnection
■Surgicalconnectionofthesuperiorvenacava(e)totheipsilateral
pulmonaryartery
■Reducevolumeload
■Performanceofadditionalprocedures(e.g.,pulmonaryarteryplasty,
septectomy,valvuloplasty)

PreoperativeEvaluation
PreoperativeevaluationoftheinfantwithafUVHpriortotheSCPCrequiresa
numberofcomponentstoassessriskandguidesurgicalplanning(Table71.8).
Traditionallycardiaccatheterization,alongwithechocardiography,hasbeen
usedforthecompleteevaluation,althoughthereisconsiderablevariabilityin
practiceaswellasspecificityandsensitivityinthevariousmodesof
investigation.3,205–210Studiescomparingechocardiographywithangiography
havedemonstratedsuboptimalperformanceofechocardiographyinadequately
evaluatingtherelevantvascularanatomy.Asingle-centerprospective
randomizedtrialcomparingcardiacmagneticresonance(CMR)withcardiac