Andersons pediatric cardiology 1269
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reportedinacasewithtetralogyofFallotandpulmonaryatresiawhentheaortic
archisrightsided.85Avascularcoursesimilartotheslinghasbeendescribed
involvingthearterialduct.86Inthisinstance,theductarosefromtheright
pulmonaryarteryandcoursedleftwardthroughthespacebetweenthedistal
tracheaandesophagustoconnecttothedescendingaortaontheleftside.Itis
alsosuggestedthatanaberrantleftsubclavianarterycanarisefromthe
descendingaortaandcoursethroughthespacebetweenthetracheaand
esophagus,1butinthisinstanceitismorelikelythatthearteryisacollateral
vesselratherthantheleftsubclavianartery.
ClinicalManifestations
Pulmonaryarterialslingusuallypresentsinthefirstyearoflife,although
asymptomaticolderchildrenandadultshavebeenreported.77Thereisaslight
malepreponderance.77Thedominantclinicalmanifestationsaredueto
obstructionoftheairwaysandincludestridor,wheeze,andcough.Stridoris
oftenexpiratorybutcanbeinspiratoryorbiphasic.Lesscommonly,patients
presentwithsymptomsfromesophagealcompression,suchasdysphagiaand
vomiting.Rarely,theslingisfoundasanincidentallesionduringcardiacor
pulmonaryinvestigationsforotherreasons.
DiagnosticInvestigation
Thechestradiographsometimesprovidescluestothediagnosis(seeFig.
47.20C).Whenpresent,abnormalbranchingoftheairwaycanbeappreciatedin
awell-takenfrontalimage.TheinvertedTappearanceofthetrachealbifurcation
atalevellowerthannormalisanimportantsign.Narrowingoflongorshort
trachealandbronchialsegmentscanalsoberecognized.Thelungsmayshow
variouscombinationsofhyperinflationand/oratelectasis,accordingtothe
associatedtracheobronchialnarrowingandretentionofsecretions.The
pulmonaryvascularitymaybeasymmetric,withtheleftlungor,lesscommonly,
rightlungshowingless-prominentvascularitythanitspartner.Barium
esophagographyisdiagnostic.77Onthelateralview,theleftpulmonaryarteryis
seenasaroundstructurebetweentheair-filleddistaltracheaandbarium-filled
esophagus,causingananteriorindentationintheesophagus.Barium
esophagography,nonetheless,shouldbeavoidedincriticallyillneonates,
especiallyiftheyareventilatordependent.78
Echocardiographyrevealscontinuationofthepulmonarytrunktotheright,
withoutoriginofthenormalleftpulmonaryartery,afeaturethatmaysuggest
unilateralabsenceoftheleftpulmonaryarteryoraberrantoriginoftheleft
pulmonaryarteryfromtheascendingaorta.Theoriginoftheleftpulmonary
arteryfromtherightpulmonaryarteryisappreciatedwhenthepulmonarytrunk
isfollowedtotheright(Fig.47.21).Echocardiographyisalwaysessentialwhen
assessingpatientswithaslingbecauseassociatedcongenitalcardiacdiseaseis
common.
FIG.47.21 Echocardiographicimagesshowingaleftpulmonaryarterial
