To repair many types of single ventricle heart defects, including hypoplastic left heart syndrome (HLHS), tricuspid atresia, double outlet left ventricle (DOLV), ...Skiptocontent StagedReconstructionHeartSurgeryOutpatientAppointments215-590-4040SecondOpinions,ReferralsandInformationAboutOurServices267-426-9600ContactUsOnlineTorepairmanytypesofsingleventricleheartdefects, includinghypoplasticleftheartsyndrome(HLHS),tricuspidatresia,doubleoutletleftventricle(DOLV),someheterotaxydefects,andothercongenitalheartdefects, surgeonsoftenperformaseriesofopenheartproceduresoverseveralyears.Thisiscalled“stagedreconstruction.”Surgeonsreconfiguretheheartandcirculatorysystemduringtheseprocedures.Aftertheoperationsforsingleventricledefects,theheartfunctionslikeaone-sidedpumpwithtwochambers.Theheartnolongerreceivesdeoxygenatedbloodfromtheveins.Instead,thisbloodflowsdirectlytothelungs.Theheartreceivesoxygenatedbloodfromthelungsandpumpsittothebody.ThisiscalledFontancirculation.Stage1:NorwoodprocedureThisoperationwilloccurwithinseveraldaysofbirth.Dependingonthetypeofheartdefect,differentsurgicalproceduresmaybeused,includingtheNorwoodprocedure.Thepurposeofthisoperationistoensurethatblood-flowiscontrolledenoughtopreventdamagetotheheartandlungs,andthatenoughbloodisreachingthelungstokeepthechildaliveuntilthesecondoperation.Stage2:Hemi-FontanorGlennoperationCalledthehemi-FontanorGlennoperation,thesecondprocedureusuallyoccurswithinsixmonthsofbirth.Duringthissurgerythesuperiorvenacava —alargeveinthatcarriesdeoxygenatedbloodfromtheupperbodyintotheheart — isdisconnectedfromtheheartandattachedtothepulmonaryartery.Afterthisoperation,deoxygenatedbloodfromtheupperbodygoestothelungswithoutpassingthroughtheheart.Stage3:FontanprocedureThisoperation,calledtheFontanprocedure,occursatapproximately 11/2 to3yearsofage.Duringthissurgerytheinferiorvenacava,alargeveinthatcarriesdeoxygenatedbloodfromthelowerbodyintotheheart,isdisconnectedfromtheheartandattachedtothepulmonaryartery.Afterthisoperation,allofthede