Andersons pediatric cardiology 1801
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SECTION6
FunctionallyUniventricularHeart
OUTLINE
68IntroductionThe“FontanPathway”
69AnatomicConsiderationsintheFunctionallyUniventricular
Heart
70PhysiologicPrinciplestoMaximizeOutcomeinPatientsWitha
FunctionallyUniventricularHeart
71FontanPathwayFromBirthThroughEarlyChildhood
72InterstageManagement
73Longer-TermOutcomesandManagementforPatientsWitha
FunctionallyUniventricularHeart
68
Introduction
TheFontanPathway
GilWernovsky,JamesS.Tweddell
Keywords
Fontan;SingleVentricle;HypoplasticLeftHeartSyndrome;Interstage;
CongenitalHeartDisease
Inthelate1960s,twosurgicalteams(oneledbyFranỗoisFontaninBordeaux,
France,theotherbyGuillermoKreutzerinSóoPaolo,Brazil)1,2concurrently
introducedwhatisperhapsthemostdisruptivesurgicalinnovationin
congenitalcardiacsurgeryaprocedurethatsimultaneouslyeliminatedvolume
loadandcyanosisamongpatientswithafunctionallyuniventricularheart.These
operationswereinitiallyappliedtopatientswithrightheartobstructivelesions
(tricuspidatresiainparticular)andweretermedrightheartbypassprocedures.
Thesuccessofthesepioneeringoperationshasbeenfollowedbymultiple
surgicalmodificationsoftheoriginalconcept.Withalong-termpalliativeoption
nowavailableforthepatientwithafunctionallyuniventricularheart,additional
ingeniousandequallydisruptiveoperationswerecreated,includingthe
Norwoodandhybridprocedures,thesuperiorcavopulmonaryconnection(the
bidirectionalGlenn),andothers.Thesenewerprocedureshavecontinuedto
undergomodificationstobetterallowsurvivalininfancyandminimizerisk
factorsfortheeventualmodifiedFontan(orperhapsmorecorrectlyFontanKreutzer)procedure.Incombination,thesecomplexsurgicalprocedurescanbe
consideredstagedreconstruction.Alongwithmedicalmanagementand
monitoring,theyhavecometobeknowncolloquiallyastheFontanpathway.
Inthistextandelsewhere,congenitalcardiacanomaliesaretypically
describedmorphologically,followedbyadescriptionofthephysiology,surgical
