Andersons pediatric cardiology 1349
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FIG.51.6 Featuresofaortopulmonarywindows.(A)Exampleinthe
settingofinterruptionoftheaorticarchattheisthmus,withthearterialduct
supplyingthedistalcirculationfromthepulmonarytrunk.Thearterialtrunks
arephotographedfromthefront.(B)Alargedefect(dashedline)viewed
subsequenttoopeningtheaorta.Notethepresenceoftheseparateaortic
andpulmonaryroots.
FIG.51.7 Asmallaortopulmonarywindowasseenfromthepulmonary
trunk(A)andalargeaortopulmonarywindowextendingtothemarginsof
thepericardialcavityasseenfromtheaorticaspect(B).Notethepresence
oftheanomalousoriginoftheleftcoronaryarteryfromthepulmonarytrunk
(A)andtheoriginoftherightpulmonaryarteryfromtheaorta(B).
Whenlarger,thedefectsextendmoredistallytowardthemarginsofthe
pericardialcavity.Thelargestwindowscanoccupymostoftheadjacentarea
betweentheintrapericardialarterialtrunks(seeFigs.51.6Band51.7B).When
thedeficientareareachestothemarginsofthepericardialcavity,itisfrequentto
findaorticoriginoftherightPA,oftentimesalsoinassociationwithinterruption
oftheaorticarch(seeFig.51.6).Associationwithoriginoftheleftcoronary
arteryfromthepulmonarytrunkisalsofrequent(seeFig.51.7A).
TheprevalenceofAPwindowinpatientswithcongenitalheartdiseaseis
0.1%to0.2%.11,12APwindowcanoccurasanisolatedlesion,oritcanbe
associatedwithothercardiacabnormalitiesin30%to50%ofcases,11,13,14the
mostcommonofwhicharearchabnormalities,specificallyinterruptedaortic
arch(commonlytypeA)andcoarctationoftheaorta,andrarelyVSD,tetralogy
ofFallotandtranspositionofgreatarteries.MoriandcolleaguesclassifiedAP
windowintothreetypes:proximal(typeI),distal(typeII),andtotal(typeIII).
Thisclassificationwasrecentlymodifiedwiththeadditionofanintermediate
category(typeIV)(Fig.51.8).15InterruptedaorticarchinthesettingofAP
windowisnotusuallyassociatedwithDiGeorgesyndrome,suggestingadistinct
malformationunrelatedto“conotruncal”abnormalities.16Abnormaloriginofthe
coronaryarteriesiscommon,withthecoronaryarteriesarisingfrequentlyfrom
theedgeofthedefect.ThesizeofthecommunicationinanAPwindowis
variable,buttheyaregenerallylarge,unrestrictive,andhemodynamically
significant.InrarecasestheAPwindowcanbesmallandpressurerestrictive.
Iatrogenic/traumaticAPwindowhasbeenreportedfollowingballoon
angioplastyofthePAs,afterPAstentplacementforsupravalvarpulmonary
stenosisinpost–arterialswitchpatients.17–19
FIG.51.8 Classificationofaortopulmonarywindowaccordingtothe
SocietyofThoracicSurgeons.(FromBackerCL,MavroudisC.Surgical
managementofaortopulmonarywindow:a40-yearexperience.EurJ
CardiothoracSurg.2002;21:773–779).
