Andersons pediatric cardiology 1777
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FIG.67.8 Diagnosisdistributionofrecipientsofhearttransplantationby
geographiclocation.CHD,Congenitalheartdisease;DCM,dilated
cardiomyopathy.(FromtheregistryoftheInternationalSocietyofHeartand
LungTransplantation.JHeartLungTransplant.2016;35(10):1185–1195.)
Outcomes
MortalityWhileAwaitingTransplantation
Childrenwaitingforhearttransplantationhavethehighestmortalityinsolid
organtransplantation.5,6Deathwhilewaitingreflectsacombinationof
availabilityofdonororgans;themedicalstateoftherecipient;recipientage,
weightanddiagnosis;andavailabilityofdurablemechanicalcirculatorysupport
options.6,7Althoughmodifyingtheavailabilityoforgansisdifficult,knowledge
oftheotherfactorsthatinfluencemortalityduringtheperiodofwaitingplaysan
importantroleindecisionmakingregardingtheappropriatetimingoflistinga
patientfortransplantation.
AnalysisoftheUSScientificRegistryofTransplantRecipientsdatabasefor
theperiodbetween1999and2006demonstratesmortalityforalllistedpatients
whilewaitingof17%at1year.6Recipientcharacteristicsassociatedwith
increasedwaitlistmortalityincludeextracorporealmembraneoxygenation
(ECMO)support,ventilatorsupport,listingstatus1A,congenitalheartdisease,
renalreplacementtherapy,andnonwhiterace.Akeyfindingisthatwaitlist
mortalityvariesbyasmuchas10-foldbasedonrecipientfactors(5%to39%).
Themortalityforinfantsduringtheperiodofwaitingrangesfrom25%to30%
andhasconsistentlybeenhigherthanthatreportedforolderpatients.Patients
withacardiomyopathydiagnosishavebetterwaitlistoutcomesthanthosewith
congenitalheartdisease.Waitlistmortalityevenwithinthecongenitalheart
diseasegroupvariesbyunderlyingdiagnosisandprevioussurgicalpalliation,
especiallyforthosewithsingle-ventriclephysiology.8
Inthemostrecentera,therehasbeena50%reductioninwaitlistmortality
duetotheadventandmorewidespreaduseofdurableventricularassistdevices
(VADs)formechanicalsupport,withafourfoldhigherlikelihoodofsurvivingto
transplantation.7
SurvivalAfterTransplantation
DatafromtheregistryoftheISHLT2showthatsurvivalaftertransplantationin
themostrecenterais91%and81%at1and5years,respectively,witha10-year
overallsurvivalof66%forthepriorera(Fig.67.9).Lookedatinadifferentway,
thesamedatashowahalf-lifefortransplantation,definedasthetimeto50%
survivalwithoutdeathorretransplantation,of20.7yearsforthoseagedlessthan
1yearattransplantation,18.2yearsforthoseagedfrom1to5years,14years
forthoseagedfrom6to10years,and12.7yearsforthoseolderthan11years—
numbersthatcontinuetoimprovealmostannually(Fig.67.10).These
differencesareevenmoremarkedwhenconditionalsurvivalisexamined,which
excludesmortalityrelatedtotheprocedureitself,withanotableeraeffect(Figs.
67.11and67.12).Infantsandneonatesarerelativelyprotectedfromlater
complications,whereasadolescents,whohavelowermortalityovertheshort
term,areatincreasedriskofdeathortheneedforretransplantationduringlongtermfollow-up.
FIG.67.9 Kaplan-Meiersurvivalcurveoutto25yearsafterheart
transplantationduringchildhoodstratifiedbyera.NA,Notapplicable.(From
theregistryoftheInternationalSocietyofHeartandLungTransplantation.
JHeartLungTransplant.2016;35[10]:1185–1195.)
