Andersons pediatric cardiology 1786
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FIG.67.19 Percentageofchildrenwithtransplantedheartsexperiencing
treatedrejectionbetweendischargeand1-yearfollow-upbyera.(Fromthe
registryoftheInternationalSocietyofHeartandLungTransplantation.J
HeartLungTransplant.2016;35(10):1185–1195.)
FIG.67.20 Kaplan-Meiersurvivalinchildrenwithtransplantedhearts
basedonthepresenceofrejectionwithinthefirstyearaftertransplantation.
(FromtheregistryoftheInternationalSocietyofHeartandLung
Transplantation.JHeartLungTransplant.2016;35(10):1185–1195.)
Thegoldstandardfordiagnosisofrejectionremainstheendomyocardial
biopsy.Thereis,however,tremendousvariabilityintheuseofroutine
surveillancebiopsies,withsomecenterscontinuingthemindefinitelyandothers
discontinuingthemafteraperiodoftimeposttransplant.63Biopsiesaregraded
accordingtotherevisedcriteriaoftheISHLT.64Foracutecellularrejection,
grade0Rindicatesnorejection,grade1Rrepresentsmildrejection,grade2R
indicatesmoderaterejection,andgrade3Rrepresentssevererejection.More
recently,toaidinthediagnosisandtreatmentofAMR,theISHLThasalso
developedapathology-basedAMRgradingsystem.65Therehavebeenmany
effortstoidentifyandvalidatenoninvasivetestsfordiagnosingrejection,
includingechocardiography,intramyocardialelectrography,andtheprofilingof
geneexpression.Asyetnononinvasivetesthasbeendevelopedtoconsistently
andaccuratelydiagnoseand/orpredictrejection.
Theincidenceofacuterejectionpeaksinthefirstmonthaftertransplantation
andthentapersoffby3months66(Fig.67.21).Morethan20%ofpediatricheart
transplantrecipientsexperiencerejectioninthefirstyear,with65%ofrecipients
experiencingatleastoneepisodeofrejectionatanytimeafterheart
transplantation.2,67DatafromPHTShaveshownanoveralldeclineinthe
incidenceandprevalenceofrejectionovertimeinthefirstyearposttransplant.68
Theseepisodesareusuallyasymptomatic,identifiedonlybybiopsy,andare
easilytreated.Thatsaid,boththePHTSandtheISHLTregistriesshowthat
treatedrejectioninthefirstyearposttransplantdecreaseslong-termsurvival.2,69
Nevertheless,acuterejectionhasdecreasedovertime(seeFig.67.19)andisrare
inpediatrichearttransplantrecipientsafterthefirstyearposttransplant.70Data
fromthePHTShaveidentifiedantibody-mediatedrejectionin11%ofpatients,
accountingfor35%ofrejectionepisodes.69
FIG.67.21 Freedomfromfirstacuteepisodeofrejectionshownasa
hazardfunctioncurve.(FromDoddDA,CaboJ,DipchandAI.Acute
rejection:naturalhistory,riskfactors,surveillance,andtreatment.In:
CanterCE,KirklinJK,eds.PediatricHeartTransplantation.ISHLT
MonographSeries,vol.2.Philadelphia:Elsevier;2007:chap.9,fig.3,p.
143.).
Laterejectionmorethan1yearposttransplanthasalsodecreasedovertime,
thoughwithapersistentimpactonCAVandmortality.67Riskfactorsinclude
earlyrejection,anti-HLAantibodies,olderage,AfricanAmericanrace,and
nonadherence.13,71Rejectionwithhemodynamiccompromiserequiringinotropic
supportandtheresultantincreasedmortalityhasnotexperiencedthesameera
