Andersons pediatric cardiology 1904

FIG.73.3 OriginalschematicrepresentationoftheKreutzertechniquefor
thefirstatriopulmonaryanastomosis.Ahomograftwasinsertedbetween
therightatrialappendageandmainpulmonaryarterywithoutvalve
implantationintheinferiorvenacava.(FromKreutzerG,GalíndezE,Bono
H,etal.Anoperationforthecorrectionoftricuspidatresia.JThorac
CardiovascSurg.1973;6[4]:613–621.)

TheatriopulmonaryconnectionbecamethestandardFontanmodification
throughtothelate1980s.However,overthelongterm,thiscirculationwas
associatedwithprogressivedilatationofthesystemicvenousatrium,atrial
thrombus,andintractableatrialarrhythmia.Inaseriesofeleganthydrodynamic
experiments,deLevaldemonstratedtheenergylossassociatedwiththe
atriopulmonaryanastomosisandpotentialforgreatercirculatoryefficiencyif
muchoftherightatriumwasexcludedfromthesystemicatrialpathwaybyusing
aninteratrialpatch.Thistechnique—termedthetotalcavopulmonary
connection4orlateraltunnelFontan—reducedthedegreeofturbulenceand
energylossandimprovedoverallhemodynamics.Shortlyafter,theextracardiac
conduitwasintroducedbyMarcellettietal.byinterposingaprostheticvalveless
conduittoconnecttheinferiorvenacavatothepulmonaryartery.5Thisaimedto
avoidprogressiveatrialdilation,latetachyarrhythmia,andsinusnode
dysfunctionbyreducingthenumberofsuturelinesandthepressureloadwithin
therightatrium(Fig.73.4).


FIG.73.4 Fontansurgicaltechniques:classicalatriopulmonaryFontan
(A),lateraltunnel(B),andextracardiacconduit(C).ASD,Atrialseptal
defect;IVC,inferiorvenacava;RA,rightatrial;RPA,rightpulmonaryartery;
SVC,superiorvenacava.(Fromd'UdekemY,IyengarAJ,CochraneAD,et
al.TheFontanprocedure:contemporarytechniqueshaveimprovedlongtermoutcomes.Circulation.2007;116[11suppl]:I157–164.)

Currently,boththelateraltunnelandextracardiacconduitarewidelyused,

somepreferringtheformertechniqueinyoungerpatientsandthosewith
anomalousdrainageoftheirhepaticveins.Studiesdemonstratecomparable
hemodynamicsinbothcirculations.6–8Neverthelesstheextracardiacconduitis
thepreferredtechniqueinmanycentersbecauseoftheperceptionthatitwillbe
associatedwithareducedlatearrhythmiaburden,althoughtodatethishasnot
beenreliablydemonstrated.9,10


LateOutcomeWithaFontanCirculation
AsthefifthdecadeofFontansurgeryapproaches,theburdenoflatemorbidity
andmortalityhasbecomeapparent,withtheriskofcomplicationsanddeath
increasingthelongerthedurationoftheFontancirculation.11Lateoutcome
studiesreportasurvivalrateof60%to80%20yearspost-Fontansurgery.12–14
Variablecaseselectionanddurationoffollow-uplikelyaccountforthisrangein
outcome.At25yearsafterFontansurgery,almosthalfthecohortispredictedto
faceFontanfailure,definedascirculatorydysfunctionwithlimitedfunctional
capacity(NewYorkHeartAssociation[NYHA]classIIIorIV),Fontan
takedownorconversion,thedevelopmentofdebilitatingcomplications
includingprotein-losingenteropathy(PLE)andplasticbronchitis,theneedfor
cardiactransplantation,ordeath(Fig.73.5).13,14

FIG.73.5 Freedomfromfailure(death,hearttransplantation,reoperation,
orpoorfunctionalstatus)forpatientswithandwithouthypoplasticleftheart
syndrome(HLHS)asreportedbytheAustraliaandNewZealandFontan
Registry.LV,Leftventricle;RV,rightventricle.(FromD'udekemY,Iyengar
AJ,GalatiJC,etal.Redefiningexpectationsoflong-termsurvivalafterthe
fontanprocedure:twenty-fiveyearsoffollow-upfromtheentirepopulation
ofAustraliaandNewZealand.Circulation.2014;130[suppl1]:S32–S38.)