Andersons pediatric cardiology 2282

NeurodevelopmentalScreening
ThedecreaseinmortalityseeninsurvivorsofCHDhasallowedcliniciansto
begintoshifttheirfocustowardminimizingmorbidityandoptimizinglong-term
neurodevelopmentalandpsychosocialoutcomes.ChildrenwithCHDarea
heterogenousgroupwithawidespectrumofdiseaseburden,butitisclearthat
evenchildrenwithjustasingleexposuretocardiopulmonarybypassmayrequire
rehabilitativeservicesinchildhood.133ManychildrenwithCHDalsohave
additionalriskfactorsfordevelopmentaldelaysuchasprematurity,underlying
geneticconditions,andexposuretomechanicalcirculatorysupportor
cardiopulmonaryresuscitation.Muchoftheframeworkforthedevelopmental
challengesfacedbysuchchildrenhasbeenextrapolatedfrom2decadesworthof
follow-upprovidedbytheBostonCirculatoryArrestStudy(BCAS)cohort.134
Themostrecentfollow-upofthesechildreninbothelementaryandhighschool
wasnotableforbelow-averageperformanceontestsofmemory,executive
functioning,visualspatialskills,attention,andsocialcognitiondespiteIQ
testing,withapproximately65%ofthecohortrequiringremedialacademic
and/orbehavioralsciencesbyadolescence.135,136Thedevelopmentalprofile
notedinCHDsurvivors133ischaracterizedby:

■Mildcognitivedelaysandreducedschool
performance
■Neuropsychologicalissuesinexecutivefunctioning,
visual-spatialskills,andworkingmemory
■Behavioraldifficultiesincludinginattentionand
increasedimpulsivity
■Difficultywithcommunicationandsocialskills
■Mildfine-andgross-motordelays
Becausethesechildrenmaymanifestmilddelaysinmultipledomains,the
impactoftheirdeficitsisoftenunderappreciated.Milddelaysinmultipleareas
maygounrecognizedwithoutformaltesting.Thiscanresultindelayed

implementationofservicesthatmightimprovedevelopmental,functional,and
academicoutcomes.Asearlydevelopmentalinterventionhasbeenshownto
improveoutcomesinotherhigh-riskpopulations,137,138timely
identification/screeningforneurodevelopmentalandpsychosocialproblemsand
focusedinterventioncoordinatedbythemedicalhomeisparamount.The
AmericanHeartAssociationcurrentlyrecommendsearlyevaluation/screening
andtreatmentforthreecategoriesofpediatricCHDpatientsknowntobeathigh
riskfordevelopmentaldisability(Box89.1)whoshouldreceiveearlyevaluation
andtreatment.133Pediatriciansandcardiologistsmustworktogethertoensure
thathigh-riskchildrenwithCHDarebeingreferredforacomprehensive
neurodevelopmentalevaluation,butalsorecognizefindingswithineach
developmentaldomainthatshouldtriggeramorein-depthevaluationinchildren
whowouldotherwisebeconsideredlowerrisk.


Box89.1

CategoriesofHigh-RiskPatientsWho
RequireDevelopmentalScreening/Referral
1.Survivorsofneonatalorinfantopenheartsurgerya(palliativeor
corrective)
2.Childrenwithcyanoticheartdiseasewhodonotrequireopenheart
surgeryininfancyb
3.ChildrenwithcongenitalheartdiseaseANDotherhigh-risk
comorbidities:
■Prematurity(GA<37weeks)
■Historyofmicrocephalyorabnormalneuroimaging
■Historyofperioperativeseizures
■Developmentaldelayrecognizedininfancy

■Underlyinggeneticabnormalityorsyndrome
■Historyofmechanicalcirculatorysupport(VADorECMO)
■Historyofhearttransplantation
■HistoryofCPR
■Prolongedpostoperativehospitalization(LOS>14days)


CPR,Cardiopulmonaryresuscitation;ECMO,extracorporealmembrane
oxygenation;GA,gestationalage;LOS,lengthofstay;VAD,ventricularassist
device.

aExamplesofcongenitalheartdiseasethatmayrequireinfantopenheartsurgery

include:double-inletleftventricle,double-outletrightventricle,hypoplasticleft
heartsyndrome,interruptedaorticarch,pulmonaryatresiawithintactventricular
septum,tetralogyofFallot,transpositionofthegreatarteries,tricuspidatresia,
totalanomalouspulmonaryvenousconnection.
bExamplesofcyanoticheartdiseaseincludetetralogyofFallotwithpulmonary

atresiaandmajoraortopulmonarycollaterals,Ebsteinanomaly.
Thecurrentalgorithmrecommendsthathigh-riskchildrenshouldreceive
directreferralforaformalmultidisciplinarydevelopmentalevaluationandEarly
Intervention(Fig.89.2).Aformaldevelopmentalassessmentshouldberepeated
at12to24months,3to5years,and11to12yearsofagetoreassessandtreat
ongoingneurodevelopmentalandpsychosocialissuesoridentifylatentissues
thatwerenotpreviouslyappreciated.Childrenidentifiedaslowriskshould
undergoheightenedsurveillanceandscreeningaccordingtothegeneral
AmericanAssociationofPediatricsguidelines,includingthestandard
developmentalscreeningat9,18,30,and48monthsinadditiontoautismspecificscreeningat18to24monthsofage.139