Andersons pediatric cardiology 1787
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effectastheothertypesofrejection,withaworse5-yearsurvivalof49%.71
Treatmentofrejectiondependsonmanyfactors,includingthetype,grade,
timeaftertransplantation,clinicalandhemodynamiceffect,andbaseline
immunosuppression.Thereisgeneralagreementthatmildrejectiondoesnot
requirespecificintervention.Moderaterejectionusuallyrequiressomedegreeof
intensificationofimmunosuppression,whichgenerallyincludesanoralor
intravenousbolusofcorticosteroid,andanincreaseinregulartherapies.Any
rejectionwithhemodynamiccompromiserequireshemodynamicsupport
commensuratewiththeclinicalpresentationandaggressiveintensificationof
immunosuppression.
CardiacAllograftVasculopathy
CAVisadiffuse,chronicvascularinjurytothegraft.Ultimatelyischemiaresults
fromcircumferentialthickeningofthevascularintimawithstenosisorocclusion
(Fig.67.22).Classicalclinicalsignsassociatedwithcoronaryarterydiseaseare
rareinrecipientsoftransplantedhearts.Sincethetransplantedheartis
denervated,recipientsmaynotexperiencecharacteristicchestpaineveninthe
faceofsignificantmyocardialischemia.Consequentlythefirstclinical
manifestationsmaybesymptomsofadvanceddisease,includingcongestive
heartfailure,syncope,ventriculararrhythmias,anddeath.CAVisaleading
causeofdeathbeyond3yearsaftertransplantation(seeTable67.3).
FIG.67.22 Cardiacallograftvasculopathy.(A)Coronaryangiography
(singleplane)demonstratingasmooth-caliberregularlumen(arrows).(B
andC)Intravascularultrasoundofthesamevesselatthesitesindicatedby
thearrows.Despitethenormalappearingangiogram,notethedifferencein
intimalthickeningreflectingthepresenceofmoderategraftvasculopathy
asseenbyultrasound.
Riskfactorsforvasculopathyinchildrenareolderdonorage,olderrecipient
age,donorcigaretteuse,recipientblackrace,transplantera,retransplantation
andrepeatedepisodesofcellularrejection.72,73Fig.67.23illustratesfreedom
fromthiscomplicationforthefirst17yearsaftertransplantation,withthesame
datastratifiedbyageshowninFig.67.24.Thisishighlydependentonthe
aggressivenessofscreening.Coronaryangiographyremainsthegoldstandard,
butittendstounderestimateCAVcomparedwithpathologicexaminationor
intravascularultrasound(seeFig.67.22).Inaddition,angiographyprovides
minimalinformationontheimpactofCAVoncardiacfunction.Therevised
ISHLTgradingsystemforCAVendeavorstotakeintoaccountfunctional
parametersfrominvasivemeasurementsorsurrogatemarkersofhemodynamics
fromechocardiography.74Onceallograftvasculopathyisevident
angiographically,short-termmortalityishigh(Fig.67.25).Kindeletal.have
describedanincreasedriskofgraftlossinthesettingofCAVwithleft
ventricularejectionfractionbelow45%,rightatrialpressureabove12mmHg,
and/orpulmonarycapillarywedgepressuregreaterthan15mmHg.11
FIG.67.23 Freedomfromcardiacallograftvasculopathy(CAV)inchildren
afterhearttransplantation.(FromtheregistryoftheInternationalSocietyof
HeartandLungTransplantation.JHeartLungTransplant.
2016;35(10):1185–1195.)
