Andersons pediatric cardiology 1330
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Inaddition,acquiredatheroscleroticcoronaryarterydiseaseinadultsmaybe
discoveredduringinvestigationsfortheCAFthatmayrequiresubsequent
management.MultisliceCTangiographycanbeusedtodemonstratethelength,
tortuosity,anddimensionsoftheaneurysm(Fig.50.19)inCAFpatients.136
Cardiacmagneticresonanceangiographymayprovideinformationregarding
cardiacfunction,quantificationofthebloodflowwithinthefistula,and
assessmentofmyocardialviability.137,138
FIG.50.17 Transthoracicechocardiographicimagesofcoronaryartery
fistula(CAF).Short-axistwo-dimensionalviewdemonstratingaseverely
dilatedproximalleftcoronaryartery(A,arrow)withDopplercolorflow
mapping(B).TheCAFdrainsintotherightatrium,asseeninatwodimensionalview(C)andwithDopplercolorflowmapping(D).
FIG.50.18 Coronaryarteryfistula(CAF)evaluationbyangiography.
Aortograms(AandC)helptoidentifythesiteoforigin(rightcoronary
artery)andsize(large)ofCAF.Selectiveballoonwedge(B,whitearrow)
coronaryangiographyallowsdetailedevaluationofcoronaryarteryfistula
drainage(BandD)andhighlightsadjacentcoronaryarterybranchesand
theirrelationshiptothedrainagesite(D,blackarrows).
FIG.50.19 Multislicecomputerizedtomography(CT)angiographyof
coronaryarteryfistulas(CAFs).Selectiveleftcoronaryangiography(A)and
correspondingCTangiography(B)demonstratingleftcircumflexCAF
withinseverelydilatedaneurysmaldrainagesite.Selectiverightcoronary
angiography(C)andcorrespondingCTangiography(D)showinga
medium-sizedtortuousrightcoronaryarteryfistulaanditsdrainagesite
(arrowinC).LCx,Leftcircumflexartery.
Management
ThenaturalhistoryofCAFsremainspoorlydefined.Thetremendous
heterogeneityinrelationtosize,symptoms,andageatpresentationposesa
significantchallengetodefinitionoftheoptimalmanagementapproach.Small
CAFsaregenerallyasymptomaticwithpropensityforspontaneousclosureon
long-termfollow-up.115,116,118ThemediumtolargeCAFsareusually
asymptomaticinchildrenbutbecomeincreasinglysymptomaticinadultsafter
20yearsofage.108TheAmericanHeartAssociationguidelinesforCAF
managementsuggestthatlargeCAFregardlessofsymptomatologyandsmallto
moderateCAFmanifestingmyocardialischemia,arrhythmia,unexplained
ventricularsystolicordiastolicdysfunctionorenlargement,orendarteritis
shouldundergoTCCorSCafterdelineationofanatomiccourseandthepotential
tofullyobliteratethefistula.PatientswithsmallasymptomaticCHFshouldnot
undergoclosure.138Thetreatmentgoalistooccludethefistulatoprevent
hemodynamicconsequenceswithfavorableremodelingandtoprovideeventfreelong-termsurvival.Inthepast,SCofthefistulawasadvocated139–142and
wasfeasibleinallagesandforallsizes.143Itremainsthepreferredapproachin
patientswhoundergooperativerepairforothercardiovascularindicationsandin
complexlargetortuousCAFthatarenotaccessibletotranscatheterapproach.In
patientswithlargeaneurysmaldilationsofthefistula,thesurgicalapproach
allowsexcisionorreductioninsizeoftheaneurysm.141SCpredominantly
involvestheuseofcardiopulmonarybypass,especiallyforthelesionsthatare
