Andersons pediatric cardiology 1788
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FIG.67.24 Freedomfromcardiacallograftvasculopathy(CAV)inchildren
afterhearttransplantationstratifiedbyagegroup.(Fromtheregistryofthe
InternationalSocietyofHeartandLungTransplantation.JHeartLung
Transplant.2016;35(10):1185–1195.)
FIG.67.25 Kaplan-Meiersurvivalfollowingdiagnosisofcardiacallograft
vasculopathy(CAV)inchildrenafterhearttransplantationstratifiedbyage
group.(FromtheregistryoftheInternationalSocietyofHeartandLung
Transplantation.JHeartLungTransplant.2016;35(10):1185–1195.)
Becauseofthelimitationsofangiography,intravascularultrasoundhasbeen
proposedasabettermeansofidentificationandstudyoftheprogressionof
epicardialCAV.Theearlyenthusiasmhasbeentemperedbytechnicalissues,
cost,andlackofmeaningfulendpointsformanagementand/orprognosis.Itsuse
iscurrentlylimitedtoolderchildrenowingtothesizeoftheavailablecatheters;
mostoftenitplaysanancillaryroleindecisionmaking.75TheutilityofMRIis
beingexploredbuthasnotyetreachedthelevelofreplacingangiography,either
fromanimagingperspectiveor,morerecently,assessmentofthe
microcirculation.76Opticalcoherencetomographyisusedinafewcentersasa
supplementarytool,butithasnotreachedwidespreadapplicationinthepediatric
population.77
Lipid-loweringtherapy,usingstatins,hasbeenshowntoplayaroleinthe
preventionofCAV.Inadults,pravastatinhasbeenshowntoreducetheincidence
ofacuterejectionassociatedwithhemodynamiccompromise,toimprove
survivalat1year,andtoreducethedevelopmentofCAV.78Thiswas
independentofthelevelofcholesterol,suggestingthattheeffectofstatins
extendsbeyondthemanipulationoflipids.Similarfindingshavebeenreported
withsimvastatin.79Therearelimiteddatainchildren,withtwostudiesshowing
alowerincidenceofCAVduringtreatmentwithsimvastatinoratorvastatin.80,81
Alargerretrospectiveregistry-basedanalysisshowednoimpactonoutcomes.82
Nevertheless,statinusehasbeenincorporatedinmostofmaintenancetherapies
forpediatrichearttransplantrecipients.
TreatmentofestablishedCAVischallenging,especiallyinchildren.Thereare
onlythreereportsofinterventionsinasmallnumberofchildren,making
definitiverecommendationsimpossible.83–85Themajorityofpatientsarenot
amenabletothesetechniquesowingtothediffusenatureofthedisease.
UltimatelypatientswithmoderatetosevereCAVandevidenceofgraft
dysfunctionmaybecandidatesforretransplantation.
Retransplantation
Retransplantationmakesupaverysmallproportionofhearttransplantsin
childrenannuallyandismorecommoninNorthAmericathantherestofthe
world(seeFigs.67.4to67.8).Overall,numbershavedecreasedoverthelast
decade,withlessthan25reportedtotheISHLTregistryin2014(Fig.67.26).
Thereisongoingcontroversyregardingtheroleofretransplantation,giventhe
decreasedlong-termsurvivalandanincreaseintransplant-relatedmorbidities,86
especiallyinthecontextoforgandonorshortages(Fig.67.27).Overallsurvival
followingretransplantationapproachesthatofprimarytransplantationwhen
retransplantationoccurslaterthan1yearafterprimarytransplantation(Fig.
67.28).Retransplantationforearlygraftfailureoracuterejection,theusual
causesofdeathinthefirstyear,carriesahighmortality(Fig.67.29).Forpatients
transplantedwithin1yearoftheirprimarytransplant,the1-yearactuarial
survivalis55%,comparedwithalmost90%forthoseretransplantedbeyond5
yearsaftertheprimarytransplant.
FIG.67.26 Retransplantationinchildrenbyyear.(Fromtheregistryofthe
InternationalSocietyofHeartandLungTransplantation.JHeartLung
Transplant.2016;35(10):1185–1195.)
