Andersons pediatric cardiology 1804
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AnatomicConsiderationsinthe
FunctionallyUniventricularHeart
RobertH.Anderson,DianeE.Spicer
Abstract
Formanyyearsthe“univentricularheart”or“singleventricle”hearthas
beenasubjectofcontention.Thisisbecauseinheartsthatclearlypossess
twoventricularchambers,oneofthetwoisdeniedventricularstatus.The
classicexampleofthelesionthusconsideredtobeuniventricularwasthe
arrangementfoundinthesettingofdouble-inletleftventricle.Theartificial
natureofsuchadescriptionwasrevealedbycomparingheartshaving
double-inletleftventricleswithconcordantventriculoarterialconnections
andtheclassicvariantoftricuspidatresia.Atthetimewhendouble-inlet
leftventriclewasconsidereduniventricular,itwasgenerallyacceptedthat
tricuspidatresiawasa“biventricular”heart.Comparisonofthevariants
describedabove,however,showedthatthe“infundibularoutletchamber”in
double-inletleftventriclewasindistinguishablefromtherightventriclein
tricuspidatresia.Bothchamberswereexamplesofincompleteright
ventricleslackingtheirinletcomponent.Comparisonwithotherexamples
ofheartshavingeitherdoubleinlets,orabsenceofoneatrioventricular
connection,showedthatitwastheatrioventricularconnectionratherthan
theventricularmassthatwastrulyuniventricular.Withtheadventofthe
Fontancirculation,itsoonbecameevidentthatotherhearts,whichwere
obviouslybiventricular,wereunabletosupportthearrangementinwhich
eachoftheventriclessuppliedoneorotherofthecirculations.Inother
words,itwasrealizedthat,althoughheartswiththeuniventricular
atrioventricularconnectioncouldneverbefunctionallyuniventricular,some
heartswithbiventricularatrioventricularconnectionsmightbefunctionally
univentricular.Theanatomyoftheheartsfoundinpatientsundergoing
functionallyuniventricularpalliationisthesubjectoftheinitialsectionof
thischapter,alongwiththecomponentsthatfollowouranatomic
introduction.Webeginouranatomicassessmentbydescribingingreater
detailtheevolutionofthecurrentconceptofthefunctionallyuniventricular
arrangement.Wealsodiscusstheanatomicarrangementtobefoundinthe
settingofthehypoplasticleftheartsyndrome,pulmonaryatresiawithan
intactventricularseptum,double-inletventricle,absenceofone
atrioventricularconnection,straddlingatrioventricularvalves,unbalanced
atrioventricularseptaldefect,andcomplexformsofdouble-outletventricle.
Weconcludethesectionwithabriefanalysisofthepresumed
