Andersons pediatric cardiology 1933
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venousatriumbecausefailureoftheFontancirculationischaracterizedbyan
increaseinpulmonarycollateralflowandhighpulmonaryvenousreturn.Others
areexploringthepossibilityofinsertinganassistdeviceinthepositionofthe
missingsubpulmonaryventricle.Thisstrategymaybeparticularlybeneficialfor
thelargenumberofFontancaseswherefailureoccursinthecontextof
preservedsystolicventricularfunction.Althoughthisstrategyisfeasible,its
limitsremaintobedetermined.Anotherlineofresearchisfocusedonthedesign
ofaright-sidedassistdevicefortheFontancirculationthatwouldcompensate
forthemissingsubpulmonaryventricle.422
NovelTreatmentsforLymphaticDrainage
Abnormalities(PlasticBronchitisandProteinLosingEnteropathy)
Itisonlyintherecentyearsthattherelativecontributionofthelymphatic
circulationtothedemiseoftheFontancirculationhasbeenunveiled(see
earlier).Itseemsthattheincreasedvenousloadingoftheliveratthetimeofthe
Fontancirculationisresponsibleforthegenerationofalargeamountoflymph
thatmayoverloadthecapacityofthelymphaticcirculationtobedrainedbythe
thoracicduct.187,197,423Thisoverloadofpressuremayresultinprolongedpleural
effusion,particularlychylothorax,atthetimeoftheFontan,andsubsequently
plasticbronchitisorPLE.Itiscurrentlypossibletointervenedirectlyinand
obstructselectivelytheconnectinglymphaticchannelresponsibleforthese
complications,andsomeearlysuccesseshavebeenreported.Todecreasethe
productionoflymphbytheliver,operationshavealsobeendesignedthatdivert
thehepaticvenouscirculationintothepulmonaryvenousatrium.424,425Another
approachhasbeentoreconnectthethoracicducttoalowervenouspressureby
anastomosingtheinnominateveintothepulmonaryvenousatrium.426Further
studieswillbetterdefinetheroleoftheseproceduresinthetreatmentofthese
debilitatingconditions.
AnnotatedReferences
d'UdekemY,IyengarAJ,GalatiJC,etal.
Redefiningexpectationsoflong-termsurvival
aftertheFontanprocedure:twenty-fiveyearsof
follow-upfromtheentirepopulationofAustralia
andNewZealand.Circulation.2014;130:S32–
S38.
Inthispublicationthelong-termoutcomeisbetter
thananticipated.Thismaybebecausetheseries
ispopulationbasedandthereforeunselectedbut
mayalsobebecausethereisasmaller
proportionofpatientswithhypoplasticleftheart
syndrome—thelatterbeingawell-identifiedrisk
factorforpooroutcome..
ParidonSM,MitchellPD,ColanSD,etal.Acrosssectionalstudyofexerciseperformanceduring
thefirst2decadesoflifeaftertheFontan
operation.JAmCollCardiol.2008;52(2):99–
107.
Inthislargecross-sectionalstudyofchildrenand
adolescentswithaFontancirculation,peak
exercisecapacitywasmarkedlydecreasedwith
onlyapproximatelyone-quarterhavinganormal
peakVO2.Olderageandmalegenderwere
associatedwithalowerexercisecapacity.
Interestingly,submaximalexercisewasless
likelytobeimpaired,suggestingthatthe
absenceofasubpulmonaryventricleimpairs
maximal(anaerobic)exercisetoagreater
extent..
CarinsTA,ShiWY,IyengarAJ,etal.Long-term
outcomesafterfirst-onsetarrhythmiainFontan
physiology.JThoracCardiovascSurg.
2016;152(5)[1355–63e1].
Theonsetofarrythmiaisdetrimentalforthe
Fontancirculation.Inthislargepopulationbasedregistryreport,one-thirdofpatientshada
tachyarrhythmiaorbradyarrhythmia20years
aftertheirFontanoperation.Afterdeveloping
arrythmia,freedomfromdeathwas74%10
yearsandfreedomfromFontanfailure55%.An
atriopulmonaryFontanandatrialisomerism
wereriskfactorsforarrhythmia..
HebertA,MikkelsenUR,ThilenU,etal.Bosentan
improvesexercisecapacityinadolescentsand
adultsafterFontanoperation:theTEMPO
(TreatmentwithEndothelinReceptorAntagonist
inFontanpatients,aRandomized,PlaceboControlled,Double-BlindStudyMeasuringPeak
OxygenConsumption)study.Circulation.
2014;130(23):2021–2030.
Inthisrandomizedtrialtherewasasmallbut
