Andersons pediatric cardiology 1274
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PulmonaryArterySlingandInnominate
ArteryCompressionSyndrome
PulmonaryArterySling
Earlyrecognitionisimportant.Generalsupportiverespiratorycareshouldbe
givenbeforeproceedingtosurgery.Respiratoryinfectionmaybedifficultto
clearcompletelybeforesurgerybecauseofpersistentretentionofsecretions.The
firstsurgicalrepairofpulmonaryarteryslingwasbyWillisJ.PottsatChildren's
MemorialHospitalin1954.131Earlyexperiencewithsurgicalrepairof
pulmonaryarteryslingwasassociatedwithsignificantoperativemortality,50but
smallsingleinstitutionserieshavebeenreported.132Earlyattritionwasrelatedto
patientsrequiringconcomitantsurgicalinterventionfortrachealstenosis.The
incidenceofcongenitaltrachealstenosiswithleftpulmonaryarteryslinghas
beenreportedtobeashighas50%to65%.75,133Fiberopticbronchoscopyto
assessforthepresenceofcompletetrachealringsismandatorywhenthe
diagnosisofleftpulmonaryarteryslingismade.Concomitanttrachealrepairhas
beenrecommendedinpatientswhodemonstratecompletetrachealrings.134
However,someauthorsadvocatereimplantationoftheleftpulmonaryartery
alone,eveninthepresenceofcompletetrachealrings,ifthediameterofthe
tracheaisgreaterthan3mm135oriftheratioofstenosislengthtototaltracheal
lengthishigh.136Echocardiographyshouldalsobedonebecauseofthe
associationofthislesionwithothercardiacdefects.137Thiswillallowcomplete
assessmentofallassociateddefectsandcarefulplanningofthesurgical
procedure.Therepairisapproachedviamediansternotomyandmostoftenuses
cardiopulmonarybypass.Thebypassstrategyisguidedbytheneedfor
interventiononthetracheaandthepresenceorabsenceofassociatedcardiac
defects.Theductusarteriosusisligatedanddivided.Anyintracardiacdefectsare
repairedfirstwithcardioplegicarrest.Theanomalousleftpulmonaryarteryand
tracheacanberepairedwiththeheartbeating.Thecourseoftheanomalousleft
pulmonaryarteryisbetweenthetracheaandesophagustothelefthilum.Once
transectedfromtheposteriorwalloftherightpulmonaryartery,itisdelivered
frombehindthetracheaandimplantedintotheleftsideofthemainpulmonary
artery(Fig.47.27).Thestrategyforrepairofcongenitaltrachealstenosishas
evolvedoverseveraldecades.81Severaltechniqueshavebeenreportedand
includeresectionwithprimarytrachealanastomosis(Fig.47.28),138tracheal
autograft,139andpericardialpatchtracheoplasty.140Theadventofslide
tracheoplasty82,141forlongsegmenttrachealstenosishasrevolutionizedthecare
ofchildrenwithcongenitalstenosis.Itsversatilityandreproducibilityhavemade
slidetracheoplastytheprocedureofchoiceforchildrenwithcongenitaltracheal
stenosisregardlessofthelengthofthestenosis.142,143
FIG.47.27 Repairofpulmonaryarteryslingbyreimplantationoftheleft
pulmonaryartery(LPA)intothemainpulmonaryartery.MPA,Main
pulmonaryartery;RPA,rightpulmonaryartery.
FIG.47.28 Corkscrewtrachea.(A)Beforerepair.(B)Afterrepair.(C)
Repairbyresectionofthestenoticsegmentandend-to-endrepair.(D)
Post-repaircomputedtomographicimageoftheairway.
Outcomes
Theoutcomesofrepairofpulmonaryarteryslingareeradependent.Earlysingle
institutionreports,withsmallnumbersofpatients,revealedanoperative
mortalityashighas14%.144Currentexpectationsshouldbeanoperative
mortalitythatapproacheszero.Insomecenters,theeffectofconcomitant
trachealsurgeryhasbeenneutralizedbytheapplicationofslidetracheoplasty.145
Thefateoftheleftpulmonaryarteryhasbeenvariable.146Earlierreportsshowed
noevidenceofflowtotheleftlungonlong-termfollow-up147eveninpatients
