functionallyuniventricularcirculations.Differentstrategiesareadopted
dependingonwhetheritisdeemedlikelythatoneortheotherrepairispossible
(Table43.3).
Table43.3
OptionsforManagementforEachInitialStrategy
Initial
Strategy
Biventricular
repair

Procedures
Catheter

Wireperforation+balloondilation
Laserperforation+balloondilation
Radiofrequencyperforation+balloondilation
Surgery
Pulmonaryvalvotomy
Pulmonaryvalvectomy
Transjunctionalpatch
Monocusphomograft
Anyofabovewithsystemic-to-pulmonaryshunt
Univentricular Balloonatrial
Surgery Systemic-toSuperiorcavopulmonary Totalcavopulmonary
repair
septostomy
pulmonaryshunt
anastomosis
connection

FromQuailMA,DaubeneyPEF.Pulmonaryatresiawithintactventricularseptum.In:Gatzoulis

MA,WebbGD,DaubeneyPEF,eds.DiagnosisandManagementofAdultCongenitalHeart
Disease.3rded.Philadelphia:Elsevier;2017:503–512.

ForpatientswithadiminutiveRV,whichwouldneversupportthepulmonary
circulation,theultimateaimwouldbeamodifiedFontan-typecirculationin
whichsystemicvenousreturnwasdirectlytothepulmonaryarteries.This
strategywouldcommenceinitiallywithconsiderationofaballoonatrial
septostomy.Unlesstheovalforamenissubstantial,aseptostomyis
recommendedbecauseafterconstructionofasystemic-to-pulmonaryarterial
shunt,theforamencanbecomerestrictive,eitherimmediatelyorovertime.55
Followingseptostomy,theshuntisconstructed,33usuallyaleft-orright-sided
modifiedBlalock-Taussigshunt.Thenextprocedure,performedattheageof3
to24months,wouldbeabidirectionalcavopulmonaryanastomosis.TheFontan
circulationwouldbecompletedattheageof24to60months.
SurgicalmanagementofanRVofgoodsizeisaimedatachievinga
biventricularcirculation,inwhichtheRVcanpumpbloodtothelungs(see
Table43.3).Thisrequiresconsiderationofthesizeandfunctionofthetricuspid
valveandRV.Wherethereisagood-sizedRVandinfundibulum,apulmonary
valvotomyorvalvectomycanbeperformed,33withorwithoutcardiopulmonary
bypass,andwithorwithoutcreationofasystemic-to-pulmonaryarterialshuntas
anadjuvanttopulmonaryflow.Wherethereisalesssubstantialinfundibulum,a


transjunctionalpatchcanbeperformedwithalimitedventriculotomy,or
alternativelyapulmonaryhomograftinserted.Wherethereisdoubtabout
whethertheRValonecanprovideenoughforwardflowtothepulmonary
arteries,atransjunctionalpatchcouldbecombinedwithasystemic-topulmonaryarterialshunt.84However,ifthereissignificanttricuspid
regurgitation,a“circularshunt”mayoccur:bloodflowthroughtheshuntwill
providepulmonarybloodflow,however,someflowsretrogradethroughthe
pulmonaryoutflowtractandbacktotherightatrium.Thisisaparticularly

challengingphysiologytomanage.Ultimately,astherightventricular
hypertrophyregressesandcomplianceimproves,theshuntcanbeclosedinthe
catheterizationlaboratoryorsurgically.Inthoseinwhomthisinitialapproach
failedtoleadtogrowthoftheRV,therightventricularhypertrophycanbe
debulkedandcombinedwithrepairofthetricuspidandpulmonaryvalvesas
required.85
Whereforwardflowthrougharepairedrightventricularoutflowis
insufficienttopreventcyanosis,abidirectionalcavopulmonaryanastomosiscan
alsobecreated.Thishasbeentermedthe“one-and-one-half(1.5)ventricle
repair”andmaybeasuitablelong-termpalliationforsomebipartiteRVs.86
Variationsofthesestrategieshavebeendescribed,wherebycontinuityis
maintainedbetweentherightatriumandthesuperiorcavalvein.87
Forpatientswitharightventriculardependentcoronaryarterialcirculation,
otherprocedureshavebeenproposed,suchasclosureofthetricuspidvalveat
thesametimeasconcurrentprocedures,88–90orconstructionofaconduitfrom
theaortatotheRV.91–93Morerecently,ithasbeenshownthatasuccessful
outcomecanbeachievedbyfollowingastagedpalliationdirectedtoward
completionofafenestratedFontancirculation.94InsomecasesofsevereRVdependentcoronarycirculation,cardiactransplantationmaybethebestlongtermsolution.
Variouscontroversiesstillremain.Whetheraprocedureontheright
ventricularoutflowtractshouldbeperformedinallcases,evenwhentheRVis
diminutive,exceptincasesofrightventriculardependence,isunresolved.
Whetherallsuchproceduresshouldalsobeaccompaniedbyconstructionofa
systemic-to-pulmonaryarterialshuntisalsonotclear.Theneedfora
transjunctionalpatchneedsclarification.Finally,theneedforanearlyprocedure
ontherightventricularoutflowtracttooptimizerightventriculargrowthmust
beresolved.Becausemostpatientshavemembranousratherthanmuscular


atresiawithapatentinfundibulum,andoftenawell-developedrightventricular
cavity(seeFig.43.15),theusualsurgicalstrategiesrecommendedforthisgroup

includeneonatalvalvotomy,valvectomy,andtransjunctionalpatchingwithor
withoutconcomitantplacementofasystemic-to-pulmonaryshunt.

CatheterManagement
Sincetheearly1990s,therehasbeenanincreasingtrendtowardperforationof
theatreticpulmonaryvalveastheprimaryprocedure(seeFig.43.16andVideos
43.6to43.13).55,75–79,95–110Insomecases,thearterialductisalsostented.107,111
Astechnicalsuccessinperforatingtheatreticvalvehasincreased,theplaceof
thistechniqueinthemanagementofthosewithgood-sizedRVshasbeenhotly
debated.Thisissueremainsunanswered,butfromapragmaticstance,thechoice
ofsurgicalversuscathetervalvotomyinanindividualinstitutionshouldbe
determinedbasedonthetechniquethatcausestheleastmortalityandmorbidity.
Ductalstentingmaybeperformedasanalternativetoasurgicalsystemic-topulmonaryarterialshunt.Thisproceduremayalsobeperformedasanadjunctat
thetimeoforafterradiofrequency/laser-assistedpulmonaryvalvotomy.112
Finally,thereisincreasingexperiencewithbilateralpulmonaryarterybanding
andmaintenanceofthepatencyofthearterialductwithprostaglandin,whichis
acommonstrategywhileawaitingcardiactransplantation.

OutcomesofIntervention
Afterhypoplasticleftheartsyndrome,thislesionhasprovedtobeoneofthe
mostdisappointingtotreat.Improvementinoutcomeoverthepast20yearshas
beenextremelyslow(Table43.4),withoccasionalexceptions.113InFig.43.17,
survivalcurvesareshownasdrawnfromthepopulation-basedstudyconducted
intheUnitedKingdomandIreland,stratifiedforinitialprocedureandthepartite
stateoftheRV.9,55
Table43.4
ComparativeMortalityFromtheFiveLargestStudiesofPulmonary
AtresiaWithIntactVentricularSeptum
Study/Center


Years

Study
Number

Survivalat1
Year

Survivalat5
Years

Reference