Andersons pediatric cardiology 1919

complexcongenitalheartdiseaseareatincreasedriskofneurodevelopmental
impairment,particularlythosewithafunctionallyuniventricularheart.220
Althoughstudiesreportalowermeanintelligencequotientforgroupsof
childrenandyoungpeoplewithaFontancirculationcomparedwithhealthy
peers,221,222themajorityofpatientshaveintellectualfunctionwithinthenormal
range.223However,thereisahigherprevalenceofimpairmentsinexecutive
functioning,visualconstructionandperception,fineandgrossmotorskills,
language,attention,andacademicperformanceinchildhoodcomparedwith
populationnorms.220,224–227Riskandseverityofneurodevelopmental
impairmentareassociatedwithindividualfactors(e.g.,presenceofagenetic
syndrome,hypoplasticleftheartsyndrome,structuralbrainabnormalities,
cyanosis,geneticfactors)andenvironmentalfactors(e.g.,prolongeddeep
hypothermiccirculatoryarrestduringcardiacsurgery,postoperativeseizures,
longerlengthofhospitalstay,lowersocioeconomicstatus,greaterpsychological
stress).225,226,228,229Althoughmanypediatriccardiaccenterscurrentlyinclude
neurodevelopmentalclinics,thesameattentionhasnotbeenpaidto
neurocognitivehealthinadultCHDcare.Withouteffectiveinterventionand
support,hardshipsencounteredduringchildhoodcanendureforyearsafter
cardiacdiagnosisandtreatment.Itisalsopossiblefordifficultiestoemergefor
thefirsttimeinadolescenceoradulthood,withheartfailure,atrialfibrillation,
cardiacsurgery,andrecurrentstrokesincreasingvulnerabilitytoneurocognitive
impairmentlaterinlife.

PsychologicalHealth
Illness-relatedstressorscanchallengechildrenandyoungpeople'semerging
copingskillsduringthepeakyearsofonsetformentalhealthdisorders.Children
andadolescentswithaFontancirculation(aged10to19years)havebeenfound
tohavehigherratesoflifetimepsychiatricdiagnosis(65%)comparedwith
healthyreferents(22%),particularlyanxietydisorders(Fontan:35%,referent:
7%)anddisruptivebehaviordisorderssuchasattention-deficit/hyperactivity
disorder(Fontan:34%,referent:6%).230Health-relatedfears,separationanxiety,

bodyimageconcerns,andsleepingdifficultiesarealsocommon.Overall,
patient-specificdemographic,perinatal,medical,andpsychosocialfactorstend
tobebetterpredictorsoflaterpsychologicaloutcomesthanintraoperative
factors.230–232Severalmechanismsforpsychologicalmorbidityincomplex
CHDareimportanttoconsider.Exposuretoearlyphysiologicalrisk,suchasin


uterobrainimmaturity,233perioperativehemodynamicalterations,andsystemic
inflammation,234mayadverselyaffectneurobiologicdevelopmentand
consequentlyalterlong-termresponsestostress,increasingtheriskof
psychologicalmorbidity.Inaddition,studiesofindividualsexposedtohigh
levelsofstressearlyinlifeconsistentlyshowthattheexperienceofearly
adversityisassociatedwithdisruptedchild-parentattachmentandalterationsin
thedevelopmentaltrajectoriesofnetworksinthebrainassociatedwithemotion
andcognition.235
Parents231,236andsiblings237alsoexperiencehigherlevelsofpsychological
distresscomparedwithpopulationnorms,yetthesegroupsmaybevulnerableto
fallingbetweenthecracksintermsofclinicalassessmentandaccessto
evidence-basedemotionalhealthcare.231Parentswithhighdistressreportpoorer
physicalhealth,238greaterparentingburden,239higherhealthserviceuse,240and
moresuicidalideation241comparedwithparentsofchildrenwithcomplexCHD
withlowerdistress.Manywhosufferfromthesedifficultiesneverreceive
psychologicaltreatment.242

ClinicalImplications
Withouteffectiveintervention,psychologicaleffectscanbeenduringandcan
influenceapatient'scapacitytosuccessfullytransitionfrompediatrictoadult
healthservices,withpotentiallylife-threateningconsequences.243,244Evidencebased,theoreticallygroundedinterventionsmaymitigatethedevelopmentof
mentalhealthdifficultiesinresponsetoseriousillnessacrossthefamilysystem.
Regularscreeningandassessmentforpsychologicalmorbidityinpeopleofall

ageswithaFontancirculationandtheirfamiliesareclearlyindicated.230,231In
general,integratingpsychosocialassessmentwithinaclinicalsettingwithwhich
patientsarealreadyengagedisakeyfactordistinguishingsuccessfuland
unsuccessfulearlymentalhealthinterventions,improvingtreatmentuptakeand
mentalhealthoutcomes.245However,atthistimeofwritingthereareno
publisheddataontheefficacyofpsychologicalinterventionsdeveloped
specificallyforchildrenoradultswithaFontancirculation.

FutureDirections
Althoughitisclearweneedtoaddresspsychologicalvulnerabilitiesacrossthe


familysystem,thebestframeworkthroughwhichtodosoremainstobe
determined.Earlytheoriesconceptualizedtheillnessexperienceasatraumafor
thepatient,leadingtoanemphasisontrauma-focusedpsychologicaltherapies.
Despitetheinclusionoflife-threateningillnessesasmeetingcriteriafora
traumaticeventwithintheDiagnosticandStatisticalManualofMental
Disorders,researchershavemorerecentlycritiquedthisdiagnostic
conceptualizationduetothelackofclarityregardingonetriggeringtraumatic
event.Goingforward,weneedtobetterunderstandthemechanismsunderlying
psychologicalmorbidityforchildrenandadultswithaFontancirculationand
theirfamiliesandtocontinueeffortstofosterintegratedmodelsofpsychological
andneurocognitivecare.Theserepresentsomeofthenextfrontiersofresearch
andclinicalpracticeinthefield.