Andersons pediatric cardiology 957
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1.Characterizethepulmonarycirculation.
▪IdentifytheoriginandcourseofallMAPCAs.
▪Imagetheaortafromtheroottotheproximalabdominal
descendingsegment.
▪Confirm/excludeMAPCAsfromheadandneckarteries
andcoronaryarteries.
▪AssessrelationshipofMAPCAstothemajorairways.
▪Documentthepresence/absenceofcentral(mediastinal)pulmonary
arteriesandwhichlungsegmentstheysupply.
▪Definethesourceofbloodflowtoalllungsegments.
▪MAPCAs
▪Centralpulmonaryarteries
▪Pulmonaryveinwedgeangiogramsinlobes/segments
withoutevidenceofcentralsupply
▪Documentthepresence,site,androbustnessofconnections
betweenMAPCAsandthecentralpulmonaryarterysystem.
▪IdentifystenosesinMAPCAsandpulmonaryarteries.
▪Measurepulmonaryarterypressuresdistally(beyondanystenoses)
andpressuregradientsacrossanystenosis.
2.Documentaorticarchsidednessandbranching.
3.Defineoriginsandproximalepicardialcourseofthecoronaryarteries.
4.Assessforinterventricularpressuregradient(restrictivenessofventricular
septaldefect).
5.Confirm/clarifyanyotheranomaliesdetectedorsuspectedby
echocardiography.
MAPCA,Majoraortopulmonarycollateralartery;PA,pulmonaryatresia;
TOF,tetralogyofFallot.
FIG.36.12 Angiogramsofthreemajoraortopulmonarycollateralarteries
(MAPCAs)inapatientwithtetralogyofFallot/pulmonaryatresia/MAPCAs
andAlagillesyndrome.(A–B)Anteroposteriorandlateralimagesofthe
samerightlungMAPCA;(C–D),separateMAPCAstotherightupperlobe
(C,shortredarrow),withaconnectiontothecentralpulmonaryarteries
(longredarrow),andtheleftlowerlobe(D).Allthevesselsaremarkedly
hypoplastic.
FIG.36.13 Seriesofselectiveangiogramsfromthesamepatientwhose
aortogramisdepictedinVideo36.2.(A)Left-sidedmajoraortopulmonary
collateralartery(MAPCA)dividessoonafteritsoriginfromthedescending
aorta.Bothbranches,depictedindividuallyin(B)and(C),arestenotic
proximally(asterisks).(B–C)Furtherselectiveimagesofthebranchesof
thecollateralin(A)demonstratestenosisofthesegmentalbranchesas
well.ThisisanexampleofaMAPCAthatarisesfromtheaortaasasingle
vesselbutrequiresseparateunifocalizationoftheinferiorandsuperior
branchesin(B)and(C).(D)Thecatheterhasbeenadvancedtothecentral
pulmonaryarterythroughitsconnectiontoaMAPCAintheleftlung(note
cathetercourse)andshowsagood-sizedcentralpulmonaryarterythat
arborizestoaportionoftherightlung.(E–F)Theremainderoftheright
lungissuppliedbytwoothercollateralsfromthedescendingaorta(E)and
therightsubclavianartery(F).AlloftheseMAPCAsexcept(F)areseenon
thedescendingaortograminVideo36.2.
