Andersons pediatric cardiology 1157
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Long-TermOutcome
Survivorsarenowreachingadulthood,buttheirnumbersaresmallduetothe
rarityofthediseaseandhighmortalityinpreviouseras.Fewdataareavailable
aboutlong-termsurvivalandfunctionalstate.Mortalitytendstooccurinthefirst
6monthsoflifeandthesurvivalcurvesflatten(seeFig.43.17).55,114,120The
TorontoHospitalforSickChildrenreported10-yearsurvivalof43%,the
CongenitalHeartSurgeonsstudyreported15-yearsurvivalat58%,theSwedish
Collaborativestudynoted10-yearsurvivalof68%,butmorereassuringly,a
seriesfromtheUniversityofCalifornia,LosAngeles,reporteda10-year
survivalof86%.6,116,118,121Ofthepatients,20%hadlatearrhythmias,andright
atrialdilationwasfoundinallpatients.Atthecurrenttime,predictionoflongertermoutcomeforabiventricularrepaircanonlybemadebydrawingparallels
withpatientswhohaveotherdiseases,suchasthosewhohaveundergone
definitiverepairoftetralogyofFallotandpulmonarystenosis(seeChapter35).
Surprisingly,thereislimitedevidencethatbiventricularrepairisbetterthan
univentricularrepair.Sanghaviandassociates122foundnostatisticaldifference
inexercisecapacitybetweenthosewithbiventricularversusuniventricular
repair.Mostpatientsinbothgroupshadsubnormalpeakoxygenconsumption
andatrendtowardimpairedperformancewithincreasingage.Similarly,EkmanJoelssonandcolleagues,intheSwedishCollaborativeStudy,123foundno
differenceinexercisecapacityinpatientsafterbiventricularversus
univentricularrepair.Decreasedlungfunctionwasnotedinallgroups.Karamlou
etal.124foundatrendtowardhigherVO2inpatientswithbiventricularand1.5ventriclerepairscomparedtouniventricularpatients.However,increased
performancewasstronglyassociatedwiththeinitialtricuspidvalvez-score,
ratherthanconferredbyrepairtype.PeakVO2andmaximumheartratewere
lowerinsurvivorsofpulmonaryatresiathancontrolsregardlessoftheirtypeof
repair.Thestudyalsodemonstratedaninterestingdichotomywherebypatients
withpulmonaryatresiabelievetheyaredoingwelldespiteimportantphysical
limitations.
Numataandcoworkersexploredwhethertherewasanyfunctionalbenefitin
havinga1.5-ventriclerepaircomparedwithauniventricularrepair.At5and10
yearstherewasnodifferenceinexercisecapacity.Importantly,atrialarrhythmias
werecommoninthe1.5-ventriclerepairgroup.125
Forthoseembarkingonatotalcavopulmonarycirculation,mortalitytendsto
occurearlyinchildhood,oftenwithinafewmonthsoftheinitial
procedure.114,126Thereisanongoingmortalityhazardbuttheearlydata
availableindicatethattheinfluenceofcoronaryarterialabnormalitiesmaybe
lessthanpredicted.126,127AstudyfromtheMayoClinicof40patientswho
underwenttheFontanprocedureforpulmonaryatresiafoundthreeoperative
deathsandalsothreelaterdeathsat2.5,8,and8yearspostoperatively.126Cause
ofdeathwaspresumeddysrhythmiaintwopatientsandprotein-losing
enteropathyinthethird.Themedianageofsurvivorswas13years(range,4to
30years);allbutonewereinNewYorkHeartAssociationfunctionalclassIor
II.Thiswasahighlypreselectedgroupwithalowincidenceofcoronaryfistulas
(10%)andRV-dependentcoronarybloodflow(2.5%).
AstudyfromTorontoreportedsurvivalaftertheFontanprocedureof80%at
10yearswithonlyonelatedeath,1yearaftertheprocedure.127Thiswasinspite
ofarelativelyhighoccurrenceofcoronaryfistulas(68%)andRV-dependent
coronarybloodflow(22%).PersistingRVhypertensionandRV-to-coronary
connectionscanleadtoprogressionofcoronaryabnormalitiessuchasstenoses,
ectasia,andinterruptionsthatcanthemselvesleadtosuddendeath.Itis
pertinent,intheTorontostudy,thatpatientswithfistulaeunderwent
thromboexclusion(patchclosure)oftheRV,whichwasbelievedtobeindicated
topreventongoingcoronaryarterydamage.Furtherfollow-upwillberequired
toascertainwhetherthisstrategyleadstoimprovedlateoutcome.
AstudyfromBostonexaminedtheoutcomeof32patientswithRV-dependent
coronarycirculationandfollowingtheuniventricularroute.128Therewasa
surprisinglygoodoutcomewithactuarialsurvivalof81%at5,10,and15years.
Allmortalityoccurredwithin3monthsoftheinitialsystemic-to-pulmonary
shunt.Allpatientswithaortocoronaryatresiadied.Theresearchers’conclusion
wasthat“singleventriclepalliationyieldsexcellentlong-termsurvivaland
shouldbethepreferredmanagementstrategyforthesepatients.”128
ArecentseriesfromColombiaUniversityexaminedtheoutcomeof17
patientsundergoinguniventricularpalliation.TheycomparedthosewithRVdependentcoronarycirculationstothosewithout.Inthiscohort,60%ofpatients
withanRV-dependentcoronarycirculationdied,comparedtononeinthenormal
coronarygroup.Ofnote,2ofthe3survivingpatientswhounderwentFontan
completionwithRV-dependentcoronarycirculationshadevidenceofischemia
duringfollow-up.129
RecommendationsforLong-TermFollow-Up
Forthosewithabiventricularrepairandminimalresidualhemodynamiclesions,
patientsshouldbeseenevery1to3yearsbyacardiologist.Wherethereare
significantresiduallesions,follow-upshouldbeyearlybyanadultcongenital
cardiologist.Similarly,patientswithmixedorfunctionallyuniventricular
circulationswarrantfollow-upinatertiarycenter.Forpatientswithvenous
shuntsortheFontancirculation,strongconsiderationshouldbegiventofull
anticoagulation,particularlyifthereissuspicionofcoronaryarterial
abnormalities.
Exerciselimitationsneedtobereviewedonanindividualbasis,dependingon
typeofsurgicalroutefollowed,theunderlyinghemodynamics,andtheoverall
stateofthepatient.Endocarditisprophylaxisisnotrequiredbuthighlevelsof
dentalhygienearerecommended.
Ateachvisit,itisessentialtoassessresidualmorphologiclesions.Following
abiventricularrepair,thepatientshouldbeacyanotic,withnormalvolume
pulses,althoughthejugularvenouspulsemaybeelevatedandtheright
ventricularimpulseincreased.Therewillusuallybeanormalfirstheartsound
withsinglesecond,whichmaybesplitifahomografthasbeeninserted.
Murmursofresidualpulmonarystenosis,regurgitation,andtricuspid
regurgitationmaybeevident.Hepatomegalymaybepresent.Indeed,iftricuspid
regurgitationissevere,thelivermaybepulsatile.Patientsarepronetoatrial
arrhythmia.
FollowingaFontanprocedure,thepatientshouldbepink,withsaturationsin
the90s.Brachialpulsesmaybeabsentfollowingpreviousarterialshunt
procedures,andthejugularvenouspulsewillbegreatlyelevatedandmayonly
bevisibleonsittingup.Therewillbeasingleheartsound.Theremaybea
murmurfromtricuspidregurgitationorasystolicmurmurcausedbybloodflow
fromahigh-pressureRVintoacoronaryarterialfistula.Hepaticcongestionmay
beevident.
Forpatientswithamixedcirculation,thepatientwillbecyanosedwith
clubbing,erythrocytosis,possiblecontinuousmurmursduetopatentsystemic
shunts,andmayhavefeaturesofeithercirculationdescribedearlier.
Chestradiographywilloftenshowanincreasedcardiothoracicratiowith,in
particular,adilatedrightatrialcontour.Inpatientswithseveretricuspid
regurgitation,theRVmayalsobedilated.Inthosewithamixedcirculation,
theremaybepulmonaryoligemia.
