Andersons pediatric cardiology 1351
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FIG.51.9 Noninvasiveevaluationofaortopulmonarywindow.Computed
tomographicthree-dimensionalreconstructionofananteroposterior
window(A)andaxialviewdemonstratingaortopulmonarycommunication
(B,pinkline).(C–D)Two-dimensionalechocardiograminshort-axisview
showingaortopulmonarywindow(arrows).Parasternallong-axisview
includingtwo-dimensionalimaging(E)andcolorflowmapping(F)
demonstratingtypeBinterruptedaorticarch(arrow).Short-axisoblique
ductalviewshowingbranchpulmonaryarteriesandlargearterialductin
two-dimensionalimaging(G)andcolorflowmapping(H).AO,Aorta;AP,
aortopulmonary;LPA,leftpulmonaryartery;PA,pulmonaryartery;PDA,
patentductusarteriosus;RPA,rightpulmonaryartery.
Management
PresenceofanAPwindowisanindicationforsurgery.Theprognosisofan
uncorrectedAPwindowispoor,withreportedmortalityofupto40%dueto
intractableheartfailureinearlyinfancyorthedevelopmentofpulmonary
vasculardiseaseinlaterinfancyorchildhood.24–26Preoperativestabilization
mayincludeeffortsaimedatimprovingsystemicoutputbycontrolling
pulmonarybloodflow(similartosingleventriclepatientsorcommonarterial
trunk)viacontrolledhypercapneatoincreasepulmonaryvascularresistance
afterintubationandmechanicalventilation.Inaddition,treatmentofshockand
metabolicacidosiswithuseoffluidsandinotropes,useofprostaglandinsfora
restrictiveorclosingarterialductinpatientswithassociatedinterruptedaortic
archorcoarctation,andmedicaltreatmentforcongestiveheartfailureare
importantforoptimalpreoperativepatientstability.25,26ForasimpleAP
window,surgicalrepaircanbeperformedwithanormothermiccardiopulmonary
bypassormoderatehypothermia.Anapproachthroughthewindowispreferable
toidentifytheoriginofthecoronaryarteriesandtoincorporatecoronaryostial
originintotheaorta.Theposteriorwallofthewindowisusuallyclosedwiththe
useofpolytetrafluoroethylene,autologousglutaraldehyde-fixedpericardium,or
homograft(Video51.1).13,16,26RepairofacomplexAPwindowcaninvolveuse
ofdeephypothermiccirculatoryarrestorcontinuouscerebralperfusion.The
outcomeofsurgicalrepairinthecurrenteraisexcellentforasimpleAPwindow,
withamortalityapproachingzeroandgoodlong-termoutlook.Long-term
follow-upiswarrantedtorecognizeandtreatbranchPAstenosis,andrecurrent
coarctationinthesettingofinterruptedarchrepair.13,14,16,26Surgicalligation
withoutcardiopulmonarybypasshasbeenperformedforrarerestrictivedistal
defects.26,27
TranscatheterapproachforclosureofanAPwindowislimitedduetothe
largesizeofthedefect,smallsizeofthepatient,andpossiblepresenceofilldefinedcomplexcoronaryarteryanomaliespriortointervention.However,
deviceclosuremaybesuitableforsmalldefects.Earlyexperiencewith
transcatheterclosurewassuccessfulwiththeuseofadouble-umbrellabuttoned
deviceforsmallAPwindows.28,29Fewreportshavedemonstratedsuccessful
closureofisolated,smalldistalAPwindowswithAmplatzervascularplugsand
ductaloccluders.30–33Sivakumarreportedsuccessfulocclusionofmoderate-
sizeddistalAPwindowsmeasuring8.7mmand6mmusingAmplatzerductal
occluderdevices.Theauthorsadviseddetailedevaluationoftherelationshipof
thesemilunarvalves,delineationoftheoriginsofthecoronaryarteries,and
relationshipsofthebranchPAspriortodeviceocclusion,andrecommendedthat
onlydefectswithadequatesuperiorandinferiorrimsshouldbeconsideredfor
transcatheterdeviceclosure.32Aorticrootangiographywilldelineatethesizeof
thedefect.Devicedeploymentisusuallyfromthevenoussideaftercrossingthe
defectfromeithertheaortaorthePA.Afterdeviceplacement,antiplatelet
therapyisadministeredfor6to12months.Transcatheterdeviceclosurehasalso
beenreportedincasesofpostsurgicalresidualAPwindows.34,35
Traumatic/iatrogenicAPfistulaisanunusualcomplicationthatisincreasingly
recognizedinolderchildrenandyoungadultsfollowingPAstentingor
angioplastyafterarterialswitchoperationfortranspositionofgreatarteries.
Treatmentshouldbeinstitutedinatimelyfashionincludingsurgicalcorrection
ortranscatheterclosurewiththeuseofcoveredstentsordevicessuchasthe
Amplatzerductaloccluderorvascularplug(Fig.51.10).17,19
