Andersons pediatric cardiology 1837
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71
FontanPathwayFromBirthThrough
EarlyChildhood
JamesS.Tweddell,RonaldA.Bronicki,JoshuaW.Salvin,MaryamY.Naim,
ChristineM.Riley,GilWernovsky
Abstract
Functionallyuniventricularhearts,sometimesreferredtoassingle
ventricles,areabroadgroupofcongenitalheartdefectsinwhichdivision
intoseparatepulmonaryandsystemicpumpingchambersisnotpossible.
Forindividualswithafunctionallyuniventricularheart,theultimategoalis
theFontancirculation,inwhichthecavalveinsaredirectlyconnectedto
thepulmonaryarteries,restoringin-seriescirculationandrelieving
cyanosis.Treatmenteffortsfrombirtharedirectedatcreatingtheideal
Fontancandidateandincludepreservingsystolicanddiastolicventricular
function,minimizingatrioventricularvalveregurgitation,eliminatingany
mechanicalobstructiontopulmonarybloodflow,minimizingpulmonary
vascularresistance,andensuringatrioventricularsequentialrhythm.Inthe
currentera,palliationisaccomplishedinthreestagesofsurgical
intervention:theneonatalperiod,thesuperiorcavopulmonaryconnection
(accomplishedduringinfancy),andthecompletionFontanprocedure,
generallycompletedby4yearsofage.Thischapterdetailsthemanagement
ofthepatientalongtheFontanpathway,includingneonatalstabilization
andselectionoftheinitialsurgicalpalliation,thechallengesofthe
interstageperiod,thesuperiorcavopulmonaryconnectionandcompletion
Fontan,includingpatientselectionandpostoperativemanagement.
Ultimately,long-termsurvivaloftheindividualwithfunctionally
univentricularheartispossible,butlifeexpectancyfallsshortofnormaland
thereisanongoinglifelongriskofheartfailureduetotheabsenceofa
pulmonaryventricle.Ongoingchallengesincludeincreasingthedurability
oftheFontancirculation,optimizingoutcomeofhearttransplantation,and
developingdurablemechanicalcirculatorysupportforthepatientwitha
functionaluniventricularheart.
Keywords
Functionallyuniventricularheart;Singleventricle;Fontan;Bidirectional
Glennshunt;Hemi-Fontan;Norwoodprocedure;Damus-Kaye-Stansel
Goodjudgementcomesfromexperience.Experiencecomesfrombad
