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Andersons pediatric cardiology 1271

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recommendedasanadjuncttotherepairofadoubleaorticarch.

RightAorticArchWithAberrantLeftSubclavian
Artery
Contemporarysurgicaltherapyfortherepairofarightaorticarchwithaberrant
leftsubclavianarteryfocusesonthemanagementofthediverticulumof
Kommerell.Thefundamentalaspectoftherepairisligationanddivisionofthe
ligamentumarteriosumwithdivisionofadhesivebandsinvolvingtheesophagus
anddescendingthoracicaorta.Histologicstudieshaveshownthepresenceof
medicalnecrosisinasmanyas505patientswhounderwentdelayedresectionof
adiverticulumofKommerell.102Ithasbeenspeculatedthatthismedialnecrosis
maybeanidusforlateaneurysmformation.Aorticdissection103,104withinthe
diverticulumandruptureofaneurysms105associatedwiththediverticulumhave
beenreportedinadultpatientswhodidnotundergoresectionduringinfancy.For
thesereasons,someauthorsadvocateroutineresectionofthediverticulumof
Kommerellwhenrepairisundertakeninsymptomaticinfantsandchildren.106
Whenthisapproachistaken,theleftsubclavianarteryisreimplantedintothe
leftcommoncarotidartery.Thesiteofresectiononthedescendingaortaiseither
repairedprimarilyorpatched(Fig.47.22).106Inacohortof19adultpatients
undergoingdelayedrepairofadiverticulumofKommerell,theprimary
indicationforsurgicalinterventionwasadiameterthatwas1.5timesthe
diameterofthesubclavianartery.107Ifthiscriterionisnotmetatthetimeof
initialsurgicalintervention,analternativetoresectionofthediverticulumand
reimplantationoftheleftsubclavianarterywouldbetheclassicrepair.The
classicrepairincludesdivisionoftheligamentumarteriosumandaposterior
aortopexyofthedescendingaortatotheperiosteumofaposteriorrib(Video
47.2).Thisisaneffectivestrategyforrelievinganyadditionalextrinsic
compressionbyasmallerdiverticulumwherethesubclavianarteryinsertsonthe
descendingaorta.



FIG.47.22 Strategyforrepairofthedescendingaortaafterresectionofa
diverticulumofKommerellandreimplantationoftheaberrantleft
subclavianartery(LSA)intotheleftcommoncarotidartery.Thedescending
aortaisrepairedprimarilyorpatched.LCA,Leftcoronaryartery;RCA,right
coronaryartery;RSA,rightsubclavianartery.

LeftAorticArchWithAberrantRightSubclavian
Artery
Aleftaorticarchwithaberrantrightsubclavianarteryisquitecommon108andis
oftenasymptomatic.Whensymptomsdooccur,dysphagia(dysphagialusoria)is
mostcommonlyrecognized.91Morerecently,isolatedtracheomalacia(arteria
lusoria)intheabsenceofgastrointestinalsymptomshasbeenreported.109Nearly
everyincisioninthearmamentariumofacardiothoracicsurgeonhasbeenused
torepairthislesion.16,110Teenagersandadultpatientsseemsuitableforcervical
orsurpraclavicularapproaches.111Theretroesophagealrightsubclavianarteryis
ligatedandtransectedfromitsoriginontheproximaldescendingthoracicaorta.
Itisreimplantedintotherightcommoncarotidartery(Fig.47.23A–B).Theright
subclavianarteryshouldbetransectedasclosetothedescendingthoracicaorta
aspossible.Leavingalargevascularstumpmayleadtopersistentsymptoms
afterrepair.112Othercausesforpersistentsymptomsafterrepairincludethe
presenceofaleftligamentumarteriosum.112Althoughmoreinvasive,the
mediansternotomyprovidesaccesstotheentireaorticarchanditsbranches,the
ligamentumarteriosum,andthedescendingthoracicaorta.


FIG.47.23 (A)Intraoperativeimageshowingtheanatomyofapatient
withaleftaorticarchandaberrantrightsubclavianartery(RSA).The
aberrantrightsubclaviancanbeseenarisingposteriortotheesophagus.
(B)ThesamepatienthasundergonedivisionoftheaberrantRSAfromthe
descendingaortaandreimplantationoftheanomalousvesselintotheright

commoncarotidartery.

CircumflexRetroesophagealAorticArch
Thecircumflexaortaisararetypeoftruevascularringanomaly.Knightand
colleaguesoriginallydescribedthislesionin1974.113Itconsistsofa
retroesophagealrightaorticarch,aleft-sideddescendingthoracicaorta,anda
left-sidedligamentumarteriosum.114Thetrachealandesophagealcompression
associatedwithacircumflexaortaisrelatedtotheposteriorpositionoftheaortic



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