Andersons pediatric cardiology 1333
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CAF.AllproximalCAFthataremoderateorlargeinsizewithorwithout
symptomscanundergoclosure,preferablyasclosetotheoriginofthefistula
fromtheadjacentnormalcoronaryarterybranchaspossible(seeFig.50.14A).
Pharmacotherapycanincludeantiplatelettherapyfor1year.However,the
decisiontocloseornottoclosedistalCAFsthataremediumtolargeremain
unclear,complicated,andcontroversial.Closureofmedium-sizedistalCAFata
youngeragemayberecommendedduetofavorableremodelingandminimal
riskforpostclosurecoronaryevent.However,observationwithantiplatelets
indefinitelywithoutinterventionremainsanoption.
FIG.50.23 Coronaryarteryfistula(CAF)treatmentalgorithm.*Highrisk
forcoronaryeventsfollowingclosure,decisiontotreatbasedonimmediate
andlifelongriskofoneoptionovertheother.AC,Anticoagulation;IV,
intravenous;SC,surgicalclosure;TCC,transcatheterclosure.(From
GowdaST,ForbesTJ,SinghH,etal.Remodelingandthrombosis
followingclosureofcoronaryarteryfistulawithreviewofmanagement:
largedistalcoronaryarteryfistula—tocloseornottoclose?Cathet
CardiovascIntervent.2013;82[1]:132–142.)
PatientswithlargedistalCAFs(whethersymptomaticorasymptomatic)areat
highestriskforcoronaryeventspostclosure.Thereforethedecisiontotreatis
bestmadeonassessmentofimmediateandlifelongriskofinterventionversus
observation.ThelargedistalCAFpatientswhoaresymptomatic
(hemodynamicallysignificantrunoff,heartfailure,endocarditis,etc.)require
treatment(TCCorSC).Becausetheyareathighriskforthrombosisand
coronaryevents,aggressivepostclosureanticoagulationwithintravenousheparin
followedbywarfarinorlow-molecular-weightheparinfor6to12monthsis
recommended,andantiplatelettherapyfor1yearorindefinitelyforresidual
coronarydilationiswarranted.Follow-upanatomiccoronaryarteryevaluationat
6to12monthsshouldhighlightremodelingsequelaeincludingthrombosisand
residualcoronarydilationthatwillfurtherdictatetypeanddurationof
anticoagulationmanagement.
ForpatientswithlargedistalCAFsthatareasymptomatic,thebesttreatment
approachisunclear.ClosureofalargedistalCAFcancausestasisandthrombus
formationintheresiduallargedilatedconduitcoronaryarterysegment,leading
tosymptomaticorasymptomaticmyocardialinfarction.112,113,127,151
Interestingly,GowdaalsonotedseverallargeasymptomaticdistalCAFpatients
whodidnotundergoTCCorSCandremainedasymptomaticwithoutcoronary
eventsduringlong-termobservationandfollow-up.112Thesepatientshadnatural
restrictionatthedrainagepoint;consequentlytheywereasymptomatic
(spontaneouspartialclosure).Thusnoninterventionwithanticoagulation
(antiplatelets)maybeanoptionforasymptomaticsignificantlylargedistalCAF
irrespectiveoftheage.112AftercompleteorpartialCAFclosuremeticulous
follow-upisessentialandshouldincludedetailedanatomicandfunctional
coronaryarteryevaluation.
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