Andersons pediatric cardiology 1051

encouraging.68–71
Itcanbeverychallengingtoachieveabiventricularrepairinpatientswith
noncommittedinterventricularcommunications.Reportedoutcomeistherefore
biasedbythemultitudeofsurgicaltechniquesusedandthevariabilityofthe
anatomy.Althoughanoncommittedinterventricularcommunicationhasbeen
reportedasposingahigherrisk,63,65othergroupsdidnotfindworse
outcomes.62,72,73Successfulbiventricularrepaircanbeachievedinasubstantial
numberofwell-selectedpatientswithfavorableresults.61,73
Intheverycomplexcasesthatmakebiventricularrepairchallenging,
functionallysingle-ventriclephysiologyandstagedconversiontotheFontan
circulationmaybeavaluablealternative.63Patientswithnoncommitted
interventricularcommunication,inparticular,tendtobeallocatedtofunctionally
singleventricularpalliation.20Ifassociateddefects―suchasstraddlingofthe
atrioventricularvalvesorhypoplasiaofaventricle―arepresent,thefunctionally
single-ventriclepathwayshouldbepreferredoverbiventricularrepair.61Inour
experience,riskfactorsforachievingafunctionallysingle-ventriclerepairwere
laterbirthcohort,hypoplasticleftventricle,double-inletleftventricle,an
interventricularcommunicationinanonsubaorticposition,absenceofhepatic
venousmalformations,andfetaldiagnosis.20
Reinterventioniscommonandreachesratesof50%.70Thisratedoesnot
differbetweensurgicalstrategiesbuttherespectivereasonforreintervention
changeswiththeanatomicsubtype.67Subaorticvalveobstruction,ratherthan
rightventricularoutflowtractpathology,wasthemainreasonforreoperationin
bothpatientswithintraventricularrepairorintraventricularrepairand
concomitantrightventricularoutflowtractprocedureasopposedtoaorticrelatedoraorticvalve–relatedproblemsinpatientsafterthearterialswitch
procedures.67Inpatientsafterbiventricularrepair,rightandleftventricular
outflowtractprocedures,aswellasreinterventiononthepulmonaryarteries
weremostcommon.20PatientsconvertedtotheFontancirculation
predominantlyunderwentfenestrationclosureandcoilocclusionofcollateral
vessels.20
Fewdataareavailableonthepostoperativeoutcomeofdouble-outletleft

ventricle.Inanolderseriesreportedbytheauthors,survivalwas75%at1year
and67%at18years,withnosignificantdifferencebetweenfunctionally
univentricularandbiventricularstrategies.19Inamorerecentanalysis,survival
of87%afterbiventricularrepairhasbeenreportedbyothers.74Eventhough


patientoutcomesmaybeconsideredsimilartothoseofpatientswithdoubleoutletrightventricle,66thehighproportionoffunctionalabnormalitiesandthe
morphologicheterogeneityofthisdefectmandatecarefulinterpretationofthe
resultsandanindividualapproachtothemanagementofthesepatients.


AnnotatedReferences
vanPraaghR,DavidI,WrightGB,vanPraaghS.
LargeRVplussmallLVisnotsingleRV.
Circulation.1980;61(5):1057–1058.
Thiscrucialconcept,statedinalettertotheeditor,
pointedoutthatitwasphilosophicallyunsound
tobasedefinitionsofagivenstructureononeof
itspartsthatwasvariable.Instead,they
establishedthecrucialprincipleofthe
morphologicalmethod,statingthatthe
structuresbeidentifiedonthebasisoftheirmost
constantcomponents..
EbadiA,SpicerDE,BackerCL,FrickerFJ,
AndersonRH.Double-outletrightventricle
revisited.JThoracCardiovascSurg.2017;1–8.
Adetaileddiscussiononthevariabilityof
infundibularmorphologyindouble-outletright
ventricle..
VanMieropLHS,WiglesworthFW.Pathogenesis

oftranspositioncomplexes:II.Anomaliesdueto
faultytransferoftheposteriorgreatartery.AmJ
Cardiol.1963;12(2):226–232.
Anearlydiscussionofthemorphogenesisofdoubloutletrightventricle,whichclaimsthatdoubleoutletleftventricleisanembryological