Weinsteinet
al.54
Valeskeet
al.56
Halaweishet
al.72
Jabbaret
al.73
Hogansonet
al.74
Arnaoutakis
etal.60

2014 5

—

EXCOR

2014 1

19,male

EXCORBiVAD

3/5transplantedat1,3,and229days;other2diedat
>360daysofsupport
Transplantedat23days

2015 1

14,male

EXCOR

Transplantedat179days

2015 1

23,male

HeartMateII

Transplanted>3yearsofsupport

2015 1

4,male

EXCOR

Transplantedat26days

HVAD,TAH,
EXCOR,Thoratec
pVAD

3/4transplanted

Pohetal.75


2017 5

ROTAFLOW,
EXCOR

Woodset
al.76

2017 3

HVAD

2/5transplanted;1dischargedhomeinvegetative
state,2diedfrommultiorganfailureandembolic
event
3/3transplantedat148,272,and271d

SynCardia
databasea

—

5

SynCardiaTAH

3/5transplanted

CCHMCb


—

3

HeartMateII,
CentriMag,
HeartWare

2/3transplanted

Total

2017 4

35

18,
male
14,
male
5,
male
23,
male
—

12,
male
13,
male

17,
male
31,
male
12,
male
5,
male
18,
male
24,
female
15,
male
25,
male
37,
male

23/35(66%)positiveoutcomec

aCommunicationwithleadresearcheratSynCardia.
bCommunicationwithCincinnatiChildren'sHospitalMedicalCenter.
cNumberdoesnotaccountforallpatientsduringthistimeframe,asnotallwerereported.

BiVAD,Biventricularassistdevice;pVAD,peripheralVAD;RVAD,rightventricularassistdevice;
TAH,totalartificialheart;VAD,ventricularassistdevice.

Patientswithlateend-stageFontanfailureinwhichthereissignificantend-



organdysfunction(e.g.,protein-losingenteropathy,plasticbronchitis,cachexia)
areknowntobepoortransplantcandidateswithsignificantperitransplantissues,
aswellasinferiorlong-termoutcomes.Oneoftheselatecomplicationsin
isolationdoesnotprecludetransplantation,butasapatientaccumulatesmultiple
comorbidities,onemustquestionwhatweareaffordingthemwithatransplant.
Thegoalisnottolimpthesepatientstotransplantknowingtheirchanceofbeing
alivein5yearsisnotgreat;rather,weshouldattempttomakethembetter
candidatesfortransplant.Perhapsinthesepatients,aTAHcanhelptoresuscitate
themnotonlybecauseofthesupraphysiologiccardiacindex(4L/minperm2)
but,moreimportantly,alowcentralvenouspressureof3to5mmHg.Itisthe
latterthataVADnorevenafreshtransplantcanaffordthepatient.Webelieve
thisuniquehemodynamiceffectallowsfortheresuscitationofkidneysandlivers
thoughttohavefailedsecondarytocongestion,aswellasallowingproteinlosingenteropathytoresolve.Inaddition,theabilitytonutritionallyand
physicallyrehabilitatepriortotransplantshouldnotbeunderestimated.The
TAHhasbeenusedfivetimes(personalcommunicationwithSyncardia),witha
60%survivalinfailingFontanprocedures,includingtheonereportedby
Rossanoetal.77AlongwithsmallerfutureTAHsizes(50cc),theuseofvirtual
surgicalimplantationcanhelptoestablishfitinsmallerpatientswithunique
palliatedcongenitalanatomy,39expandingeligibilityforfutureTAHcandidates.
Inall,theexperiencewithVADsfollowingstageIpalliationhasbeendismal
andrequiresextremecautionwhencontemplatingtheiruseoverECMO.In
patientswithSVPandafterGlennorFontanpalliations,MCShasproventobea
meaningfulwayofbridgingpatientstotransplantationwithgoodposttransplant
survival.Evenstill,thoughtfulpatientanddeviceselection,aswellastimingof
support,isessentialforgoodoutcomes.

ElevatedPulmonaryVascularResistance
Manypatientswithlong-standingheartfailure,especiallyfromCHDor
restrictivephysiology,presentwithmarkedlyelevatedpulmonaryvascular

resistanceandmaybeconsidered“untransplantable.”AperiodofLVADsupport
andmaximalmedicalmanipulationofthepulmonaryvascularresistanceoften
lowersthisresistancesignificantly,asseenbyrepeatcatheterizations.These
patientsthenareeligiblefortransplant,whichistheexpectedcourseforthevast
majorityofsuchpatientsashasbeendocumentedbymultipleseries.78,79In
addition,nowthatpediatricpatientsgreaterthan25kgarealmostexclusively


supportedbyanintracorporealCFVAD,thereislessconcernoverwhetherthe
pulmonaryvascularresistancechanges.Regardless,LVADsupportinthese
patientscanallowthemtobedischargedwiththeirdevicesandresumealmosta
normallifestyle.

AdultCongenitalHeartDisease
Thenumberofadultswithcongenitalheartdisease(ACHD)haslongsince
surpassedthenumberofchildrenwithCHDandrepresentsanincreasing
proportionofhearttransplantrecipients.Thesepatientsfaceahigherearly
mortalityrateandhigherriskofretransplantation.Thesepatientsarealsolikely
tohavelongermeanwaitingtimesandsignificantlyhigherpulmonaryvascular
resistances.80–82Itisthereforelikelythatincreasingnumbersofthesepatients
willrequireVADimplantationpriortohearttransplantation;however,Gelowet
al.confirmedthatVADuseislesscommoninACHDpatientslistedfor
transplantcomparedwithnon-ACHDpatients(3%vs.17%).83Thisrepresents
significanthesitationtosupportthesepatientsmechanically,whichwecan
surmiseissecondarytotheircomplexityandpresentationatadultprogramswho
maynotbeasfamiliarwithsomeofthemorecomplexanatomyandphysiology.
TheauthorsconcludedthisisclinicallyrelevantbecausereducedVADuse
appearstocontributetolowerlistingstatusandimpactsorganallocationto
ACHDpatients,aswellaswaitlistandtransplantsurvival.Theyfoundthatthe
differencehasgrownincreasinglydisparateovertimeandsuggestedthat

separateorganallocationforCHDpatientsmaybejustified.Inresponsetothis
issue,theOrganProcurementandTransplantationNetworkBoardofDirectors
reviewedorganallocationpolicyandissuedastatementtoRegionalReview
BoardstogivehigherconsiderationtoincreasingthelistingstatusofACHD
patients,althoughitdidnotultimatelychangethecurrentpolicy.84
ManyACHDpatientshaveSVHsortranspositionofthegreatarterieswith
eitherasystemicmorphologicleftventricleorasystemicmorphologicright
ventricle.BothadultandpediatricpatientswithSVHhavebeensupportedwith
VADs,asnotedpreviously.Considerationmaybegiventoeithercommonatrial
orventricularcannulation,dependingonresidualventricularfunctionandthe
riskofthromboembolism.PatientswithbothL-transpositionofthegreatarteries
andD-transpositioncorrectedbyanatrialswitchprocedure(Senningor
Mustard)aresusceptibletofailureofthesystemicmorphologicrightventricle,
necessitatinghearttransplantation.VADshavegenerallybeendesignedfor