Andersons pediatric cardiology 1292
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2010
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2012
2012
Subramanyan
Holmes
Fatih
Jowett
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2012 Jowett
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2013 Murugan
2015 Gupta
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2015 Gupta
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2015 Bhatla
2016 Gupta
2015 Nicholsona
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1(Fig.
48.8A)
2(Fig.
48.9B)
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1
1
Aorta(lateralaspect)topulmonaryarteryconfluence
Aortatodistalmainpulmonaryartery
Aortatopulmonaryarteryconfluence,verylongcourse
AortatopulmonaryarteryconfluencemimickingdistalAP
window
AortatopulmonaryarteryconfluencemimickingdistalAP
window
Aortatoleftpulmonaryartery
Aortatopulmonaryarteryconfluence
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Ascendingaortatoleftpulmonaryartery
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50
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Aortatopulmonaryarteryconfluence
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Aortatopulmonaryarteryconfluence
70
Double-barreledaorta(left)andleftpulmonaryarteryfrom 55
aorta
aThisshowscasesthatweinterpretasrequiringdualorbilateralfiftharcharteriestoexplainthe
anatomy.
AP,Aortopulmonary.
FifthArchArteryVersusDistalAortopulmonary
Window
Inoneofourpatientswithventricularseptaldefectandpulmonaryatresiainthe
settingofdiscordantventriculoarterialconnections,weencounteredachannel
arisingfromthelateralaspectofascendingaorta(Fig.48.8,left).Thischannel,
alongwithothersdescribedintheliterature,23,56,58–62,64–70couldwellbean
arteryofthefiftharch,ascouldthevesselfoundinthesettingoftetralogyof
Fallotwithpulmonaryatresiashownintheright-handpanelofFig.48.8.This
typeofconnectionhasbeeninterpretedbyotherinvestigatorsasrepresentinga
distalaortopulmonarywindow.23,38,59–61,67Aortopulmonarywindows,however,
areintrapericardial,asopposedtoeitheracollateralchannel,oranarteryofthe
fiftharch,bothofwhichwouldoccupyextrapericardialspace(seeFig.48.8).
Thereforethelocationofthelesionsmeansthattheycannotbeaortopulmonary
windows.Localizationofthechannelwithinoroutsidethepericardium,
nonetheless,althoughreadilyrecognizedduringsurgery,canbeextremely
difficultonpreoperativeimaging.68,70
FIG.48.8 Left,Imagefromapatientwithdiscordantventriculoarterial
connections,ventricularseptaldefect,andpulmonaryatresia.Thechannel
arisesfromtheascendingaortaproximaltotheoriginofthe
brachiocephalicarteryandterminatesattheconfluenceofthepulmonary
arteriesthroughthearterialduct.Thisstronglyfavorsitsoriginasafifth
archartery.Right,ImagefromapatientwithtetralogyofFallotand
pulmonaryatresia.Thechannelfeedingthepulmonaryarteriesarisesfrom
theascendingaortaproximaltotheoriginofthebrachiocephalicartery.
Whentraceddistally,itfeedstheconfluenceofthepulmonaryarteriesin
retrogrademanner—inotherwords,throughtheterminationoftheleftsixth
archartery.Thisagainfavorsitsoriginasafiftharchartery.
ChannelsFromtheAscendingAortatotheLeft
orRightPulmonaryArtery
Irrespectiveofpriorconsiderations,itisnotuncommontofindachannelarising
fromtheascendingaortathat,aftertakingatortuouscourse,terminatesinoneor
otherofthepulmonaryarteries.Inonesuchcaseheldtorepresentafiftharch
artery,thecandidatechannelisshownasarisingfromtheascendingaorta.It
terminatesdirectlyintherightpulmonaryartery(Fig.48.9A).23Asimilarcase
wasreportedbyus,andagaininterpretedinitiallyasanarteryofthefiftharch.
Thechannelarisesfromtheascendingaortaproximaltothebrachiocephalic
arteryandterminatesinthediscontinuousleftpulmonaryartery,havingtakinga
serpentinecourse(seeFig.48.9B).68Furtherinterrogationofthecomputed
tomographicdatasetfromthepatient(Fig.48.10)showswellthe
interrelationshipsoftheoriginsofthebrachiocephalicarteryandtheserpentine
channel.Inthesettingofdiscontinuouspulmonaryarteries,thelocationofthe
originofaserpentinechannelcouldjustaswellbeinterpretedtorepresent
extensiveremodelingofthearterialductasitistorepresentthefiftharchartery.
Andsincethesixtharchisuniversallypresent,probabilitiesfavoritsoriginfrom
thesixthratherthanthefiftharch.
FIG.48.9 (A)Suggestedoriginofavesselfeedingtherightpulmonary
arteryinthesettingofpulmonaryatresiaasanarteryofthefifthpharyngeal
arch.Itisjustaslikelytobeanaberrantarterialductderivedexclusively
fromthesixthaorticarch,sinceitterminatesdirectlyintherightpulmonary
artery.(B)Vascularchannelarisingfromtheascendingaortathat,after
followingatortuouscourse,terminatesinthediscontinuousleftpulmonary
artery.Thepreciserelationshipsoftheoriginsofthechannelandthe
brachiocephalicarteryareshowninFig.48.10.Therightpulmonaryartery
issuppliedbysystemic-to-pulmonarycollateralarteries.Aswasthecase
withthechanneldescribedbyGerlisandcolleagues,23weinitially
interpretedthechannelasseenin(B)asanarteryofthefiftharch.On
reflection,itcouldjustaswellbetheremodeledarterialduct,with
probabilitiesfavoringthelatterinterpretation.
