Andersons pediatric cardiology 1791
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FIG.67.30 Malignancy-freesurvivalinchildrenafterhearttransplantation.
(FromtheregistryoftheInternationalSocietyofHeartandLung
Transplantation.JHeartLungTransplant.2016;35(10):1185–1195.)
PTLDincludesallclinicalsyndromesassociatedwithlymphoproliferation
aftertransplantation.Thespectrumrangesfromamononucleosis-likeillnessto
life-threateningmalignancieswithclonalchromosomalabnormalities.EBV
playsamajorroleintheirdevelopment,withthehighestriskfordevelopment
beingaprimaryinfectionwiththevirus.GiventheirnaivetetoEBV,primary
infection,andthusPTLD,thisproblemismorecommoninchildren.Theriskis
alsorelatedtopatientageattimeoftransplant(infancy,childhood,adolescence),
EBVserostatus,andtheviralload.16,107
Therearenocontrolledclinicaltrialscomparinginterventionsortherapiesfor
thesedisorders.Treatmentsincludereductioninimmunosuppression,antiviral
medication,immunoglobulintherapy,monoclonalantibodiessuchasrituximab,
chemotherapy,tumordebulking,radiationtherapy,and,rarely,bonemarrow
transplantation.108TreatmentforPTLDcarrieswithitahigherriskofrejection
relatedtothereductionofimmunosuppressionrequiredtotreatthedisease.15,16
Growth
Somaticgrowthretardationiscommoninchildrenrequiringcardiac
transplantation.Thisisespeciallytrueofthosewithcongenitalheartdiseaseand
thosewithlongwaitlisttimes.Posttransplantationweightandbodymassindex
Z-scorestrendtowardnormalization,butheightZ-scoresexhibitlesscatchup.109Cardiacgrowthoccursinlinewithsomaticgrowth.110
Development
Assessmentofinfantdevelopmenthasrevealedthatmeandevelopmentalscores
forbothmentalandpsychomotorindexesfallwithinnormallimits,although
theyhavetendedtobeatthelowerendofnormal.111Reportsbeyondinfancy
demonstratearangeofvariationinaspectrumofdevelopmentalparameters
comparedwithanormalpopulation,withoverallcognitivescoresatthelower
limitofnormal.18Focusedstudiesofpatientsbornwithhypoplasticleftheart
syndromehavealldemonstratedlowerscoresonthescalesforinfant
developmentandintelligencecomparedwiththegeneralpopulation,butthe
scoresaresimilartothoseofpatientsundergoingtheNorwoodsequenceof
palliativeprocedures.112
FunctionalStatus,QualityofLife,and
PsychosocialIssues
AfunctionalstatusscoreiscollectedintheUnitedStatesforallpediatricheart
transplantrecipients,andarecentanalysisshowedthatthevastmajorityof
childrenwhosurviveatleast1yearposttransplanthaveanexcellentfunctional
statuswithfewlimitationstodailylife.113
Althoughstudiesarelimited,mostchildrenandfamiliesreportagoodquality
oflifefollowinghearttransplantation.114Alongitudinalstudyofchildrenafter
transplantationofeithertheheartortheheartandlungsshowedacademic
cognitivefunctioningtobeinthenormalrange,withoutchangeasafunctionof
timefromtransplantation.115Performanceatschool,however,wassignificantly
poorerthanthatofhealthychildren,andtheprevalenceofbehavioralproblems
wassignificantlygreater.Similarresultswerereportedinanotherlongitudinal
studythatfollowedchildrenformorethan10years,withpsychologic
functioningoutsidethenormalrangeinone-quarterofpatients.116Pre-and
postoperativepsychologicassessment,andsupportofthepatientandhisorher
familyisclearlyanimportantroleofanycenterinvolvedincardiac
transplantation.ArecentstudyfromStanfordevaluatedqualityoflifeinadults
followingpediatrichearttransplantationandrevealedagoodqualityoflifewith
areasonablelevelofsuccessineducationalandwork-relatedachievements.117
Nonadherence
Nonadherenceremainsasignificantchallengeforchildhoodrecipientsofheart
transplants,especiallyduringadolescence.Adolescenceitselfisindependently
associatedwithworsesurvival.2Thereasonsforthisaremultifactorialand
includebiologicfactors,adherencetomedicalcare,andthenormal
developmentalchallengesofadolescencethataffecttheabilitytotransitionto
self-monitoringandself-care.Thecomplexpsychologicissuesalreadydescribed
combinedwithnormaladolescentdevelopmentaftercardiactransplantation
renderalargepopulationofrecipientsatriskofnonadherence.19,118,119
Nonadherencehasbeenlinkedtolaterejectionandtohighratesofdeathin
adolescents.19Highvariabilityintroughlevelsofmedications,withbothhigh
andlowlevelspresumablyreflectingvariableadherence,isamarkerforchildren
atgreaterriskforrecurrentrejectionandhospitalizationaftertransplantation.120
Thereareincreasingstrategiestorecognizeandmanagenonadherenceincluding
web-basedand/ormedicalappstargetedatthetechnology-savvyadolescent.121
Acoordinatedapproachbetweenpediatricians,thetransplantteam,thepatient,
andthefamilymustbeinplace,119,122particularlyatthetimeoftransitionfrom
pediatrictoadult-basedcare.
TransitiontoAdultCare
Transitionisaplannedprocessthataddressesthemedical,psychologic,and
educationalneedsofadolescentsandyoungadultswithchronicphysicaland
medicalconditionsastheymovefromchild-centeredtoadult-orientedcare.
Medium-termrejectionandgraftlosshasbeenassociatedwithanineffective
transitionprocess.Transitionisnotasingletimepointbutratheraprocessthat
shouldbeinitiatedwithpatientsintheirearlyteensandtheirfamilies;itisan
ongoingeducationalendeavorwiththegoalofprovidinguninterruptedhealth
care.Majoreducationaltopicsincludelearningabouttheirheartdisease,
medicationdosesandsideeffects,andsignsandsymptomsofinfectionand
rejection.Additionalmilestonesandskillsallfocusingonpromotingselfmanagementincludelearningtocontactthepharmacyformedicationrefills,
