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SummaryandFutureDirections
AlthoughchildrenwithmildtypesofCHD(thosewhohavenothadanysurgical
orcatheter-basedprocedures)appeartohavenormalCNSand
neurodevelopmentalandpsychosocialoutcomes,220,234childrenwithcCHD
constituteahigh-riskpopulationwithasignificantincidenceofadverse
neurodevelopmental,behavioral,andemotionaloutcomes.Currenttechniques
fordevelopmentalevaluationinneonatesandinfantsareimprecisepredictorsof
lateoutcomes.Evaluationofpreschool-andschool-agedchildrenrevealsa
patternofneurodevelopmentaldysfunctioncharacterizedbymildcognitive
impairment,motordysfunction,impairedvisual-spatialandvisual-motorskills,
andattentionandacademicdifficultiesinmanyofthechildren.Therearemore
significantproblemswithexpressivespeechandlanguageinsome,andahigh
incidenceoflearningdifferences.ThefactorsresultinginCNSinjury(most
commonlyWMI)anddevelopmentaldysfunctioninthesechildrenaremultiple,
interactive,andincompletelyunderstood.Developmentaldysfunctionresults
fromacomplexrelationshipbetweenpatient-specificfactors(genetic
susceptibility,cardiacdiagnosis,fetaldevelopment)andenvironmentalfactors
(preoperativeevents,techniquesofsupportduringsurgicalrepair,postoperative
events,SES,familyfunctioning).Currently,reportedriskfactorsincompletely
explainthepatternsorprevalenceofCNSinjuryfollowingcardiacsurgeryin
infants,suggestingthatas-yet-unidentifiedprenatal,perioperative,andpatientspecificfactorsmaymodulatetheresponsetoCHDandcardiacsurgery,
increasingtheriskofadverseneurodevelopmentalandpsychosocial
consequences.
Currently,importantresearchinvestigationsareunderwayto(1)understand
andimprovethedevelopingbraininthefetuswithcCHD82,235–237;(2)identify
modifiableriskfactorsintheoperatingroomandICUtomaximizelong-term
neurodevelopmentaloutcomes2,238;and(3)developstrategiesafterhospital
dischargetoimprovefamilypsychosocialhealth,childhooddevelopment,longtermpsychosocialoutcomes,andhealth-relatedQOL.Crucialinthiseffortisthe
identificationofanearlypostoperativesurrogatevariable(e.g.,biomarkersand
newerbrainphysiologicandimagingassessments)withgoodpredictivevalidity
forlong-termoutcomes.43,239–240MRIinparticularisshowinggreatpromisein