Andersons pediatric cardiology 1792
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understandingwhenandhowtocontactthetransplantteam,andpracticingthe
schedulingoftheirownappointmentsandarrangingtransportationforclinic
visits.Theimportanceofandapproachestotransitionforchildhoodrecipientsof
hearttransplantationcontinuetoevolve.123
TransplantationoftheHeartandLungs
Transplantationofthehearttogetherwiththelungshaslimitedapplicationin
children,with10orlessbeingreportedtotheregistryoftheISHLTannually
since2007.124Themainindicationremainspulmonaryarterialhypertensionin
61%andcysticfibrosisin28%.Mostoccurinchildrenandadolescentswithno
reportsofinfantheart-lungtransplantssince2008andonlytwosince2000
(Video67.2).Postoperativetransplant-relatedmorbiditiesaresimilartothosefor
isolatedtransplantationoftheheartandthelungs,withtheissuesrelatingto
transplantationofthelungspredominatingandgenerallyguidingtheclinical
courseandplansfortreatment.Long-termoutcomesarewellbelowthosefor
transplantationoftheheartandhaveshownnoimprovementoverthelast
decade,withamediansurvivalof4.8yearsforpulmonaryarterialhypertension,
3.8yearsforcysticfibrosis,and2.4yearsforcongenitalheartdisease.124
FutureDirections
Overall,thesurvivalforchildrenundergoingtransplantationofthehearthas
improvedremarkablyduringthepast20years,andthefunctionalstatusofmost
patientsisexcellent.Despitethis,thereisacontinuedriskoflatemorbidity
relatedtoimmunosuppression.Mortalitywhilewaitingfortransplantation,
thoughimproved,remainsunacceptablyhigh—especiallyforinfantsandthose
withcongenitalheartdisease.Improvedmanagementofheartfailureand
mechanicalsupportoptionsnowmoreeffectivelybridgeolderchildrenand
adolescentstotransplantation.Keyopportunitiesincludethedevelopmentof
mechanicalsupportoptionsforinfantsandsmallerchildrenandthosewith
congenitalheartdisease,newerimmunosuppressantagentstomitigateantibodymediatedrejection,andnoninvasivelong-termsurveillancestrategies.
