Andersons pediatric cardiology 1331
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inaccessibleepicardiallyandinpatientsrequiringrepairofassociatedcardiac
lesions.Anatriotomyorventriculotomyisnecessarytovisualizethedrainage
point,especiallyinlargedistalfistulas.Severallargeserieshavedescribeduseof
surgicalligationofthefistulaepicardiallywithouttheuseofcardiopulmonary
bypassinmorethan50%ofthepatientsundergoingSC.140,144Themortality
relatedtoSCislessthan1%,142withrareinstancesofperiproceduralST
changes,myocardialinfarctions,arrhythmias,strokes,andresidualshuntsin
approximately10%.144–147
Inthecurrentera,advancesindeliverysystems,microcatheters,andimproved
deviceshaveenabledTCCtoemergeasaneffectiveandsafealternativeto
surgerywithashorterrecoverytime.Thefirsttranscatheterocclusionwas
reportedin1983.148Inthepast2decades,availabilityofvariousdevices,suchas
Gianturcocoils,detachablecoils,detachableballoons,avarietyofAmplatzer
devices(e.g.,ductaloccluder,VSDoccluder,vascularplugs),andrecentlythe
useofmicrovascularplugs,hasallowedsuccessfulTCCofCAFsofvaryingsize
inanyagegroup,includingneonatesandinfants(Fig.50.20).111,114,145,149,150
TheTCCresultsarecomparabletothereportedsurgicalresultswithlow
mortalityrates.145,151PeriproceduralSTchanges,acuteandchronicthrombosis,
myocardialinfarctions,and/orarrhythmiashavebeenreported.111,114,145,151In
thepastthepresenceofmultipledrainagesitesandsmallsizeofthepatientsuch
asneonataltreatmentoflargefistulawereconsideredrelativecontraindications
forTCC.Controlled-releasecoilsandtheuseofmicrocathetershavewidened
thespectrumofindicationsinsmallerfistulas,smallerpatients,andinfistulas
withmultipledrainagepointsusingaretrogradearterialapproach.149,152,153The
aimofTCCistooccludethefeedingarteryproximallyasclosetothecoronary
treeinproximalfistulas(seeFig.50.14A).Indistalfistulas,precisedeliveryof
thedeviceorcoilbeyondthenormalcoronarybranchand/orclosetothe
drainagepointisvital(seeFig.50.14B).Manysmalltomediumsizefistulas
withlongtortuouscourseareaccessiblewiththeuseofmicrocatheters(2.3to3
Fr)coaxiallywithinacoronaryguidecatheter,allowingdeliveryofsmallercalibercoilsandmicrovascularplugs.Inlarge,high-flowfistulas(especially
proximalCAFs),useofanarteriovenousloopmaybeinvaluable(thisapproach
involvespassinganarterialguidewirethatcanthenbesnaredandexteriorized
onthevenoussidetoprovideaguidewirerailthroughthefistula).Thisapproach
facilitatespositioningalargedeliverysystemthroughtheveinoverthe
guidewirerail,permittingdeploymentoflargerdevicespreciselyandsafely
whilelimitingthecaliberofthearterialsheath.Recently,similarlargefistulas
havebeenoccludedeffectivelybyaretrogradearterialapproachbydeploying
microvascularplugsusingmicrocathetersthroughsmaller-calibersheaths.153
FIG.50.20 Transcatheterclosureofcoronaryarteryfistulas(CAFs).
IllustrationsdemonstratingtypesofCAF;Proximal(A)anddistal(D)with
potentialocclusionsites(asterisks).Proximalfistula(B,whitearrow)arising
fromtheleftmaincoronaryarteryproximaltoitsepicardialbranches(black
arrows)drainingtotherightatrium.Vascularplugdeliveryusingthevenous
approach(bycreatinganarteriovenousloop)atthedrainagepoint(C).
DistalrightCAF(E)drainingtotherightventricle.Usinganarterial
approach,retrogradevascularplugdelivery(F)atthedrainagepoint(white
arrow)distaltothelastcoronaryarterybranch.LAD,Leftanterior
descendingartery;LCX,leftcircumflexartery.
Long-TermOutcomesofCoronaryArteryFistula
Closure
Severalcasereports,caseseries,andlong-termstudieshavereportedpostclosure
adversecoronaryeventssuchasdiscretestenosiswithperfusiondefects,154and
acuteandchronicthrombosiswithandwithoutmyocardial
infarction.112–114,127,144,155–159Cheungperformedcoronaryangiogramsafter
CAFclosurein21of41asymptomaticpatientsandfoundthat4patientshad
distalthrombosiswiththreadlikevesselsand2hadcompletethrombosisupto
theproximalstumpwithdistalcollateralvessels.144Similarly,BostonChildren's
Hospitalintheirseriesof76patientsshowed11patientstohavelong-term
adversecoronaryevents,with9ofthemhavingevidenceofthrombosiscausing
infarctionand/orangina.Theclinicalpredictorsofcoronaryeventswereolder
ageatdiagnosis,tobaccouse,diabetes,systemichypertension,andangiographic
featureofCAFdrainagetocoronarysinus.159
Tobetterunderstandtheriskfactorsforcoronaryevents,Gowdaclassified
CAFasproximalanddistal(seeFig.50.14).112,113ProximalCAFsarisefromthe
centralproximalmajorepicardialarteryandarefelttobeatlowriskfor
coronaryeventsafterclosurebecausetherearenonormalnutritivecoronary
branchesarisingfromtheresidualfistulasegment.Theycanundergoeither
proximalordistalclosure;theresidualfistulasegmentwouldthenundergo
thrombosiswithextensionofthethrombusuptotheproximalrunoffmajor
epicardialcoronaryarterywithoutprogressionofthethrombustothenormal
coronarybranchesunlessthereisseverelydilatedadjacentaorticsinus.In
contrast,distalCAFsarisefromthedistalmajorepicardialcoronaryartery.The
proximalconduitcoronaryarteryiseithertortuousordilatedandalsohasnormal
coronaryarterybranchessupplyingthemyocardium.TheseCAFscanundergo
onlydistalclosureatthedrainagesiteorjustdistaltothemostdistalnormal
coronarybranch.Inthesecasestheproximalresidualconduitcoronaryarteryis
atriskforadversecoronaryeventsfromthrombosis,especiallyifitisdilated
(Fig.50.21),andunfavorableremodelingfromdiscreteintimalstenosiscausing
perfusiondefects.154
