Andersons pediatric cardiology 1328
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FIG.50.13 Aproposedmanagementflowchart.aAdviseofrisksofbrain
abscessandhemorrhagicstrokesthatmaymodifystrokemanagement
(earlyMRIscan,cautionwiththrombolysis).bCautionrequiredifpreexisting
severepulmonaryhypertensionispresentwhenrisk-benefitconsiderations
change(increasedriskofproceduralrelatedmortality;reducedbenefitform
preventionofparadoxicalembolicstrokes.CT,Computerizedtomography;
HHT,hereditaryhemorrhagictelangiectasia;PAVM,pulmonary
arteriovenousmalformation.(FromShovlinCL.Pulmonaryarteriovenous
malformations.AmJRespirCritCareMed.2014;190[11]:1217–1228.)
CoronaryArteryFistulas
Morphology
Coronaryarteryfistulas(CAFs)arerarecongenitalanomalies,representing
anomaliesofmyocardial-coronaryarteryinteraction.105–107CAFsrepresent
communicationsbetweencoronaryarteriesandeitheracardiacchamberor
vessel(e.g.,coronarysinus,thesuperiorvenacava,pulmonaryarteryorveins),
bypassingthecapillarybed.Itstrueincidenceisunknownorhighlyspeculative
becausemanylesionsaresmallandneverdetected.However,theyarethemost
commonhemodynamicallysignificantcongenitalcoronaryartery
anomalies.108,109CAFsmaybeseenin0.3%to0.4%ofpatientswithcongenital
heartdisease,109,110in0.06%ofchildrenundergoingcardiaccatheterization,110
andin0.1%to0.2%ofalladultpatientswhoundergoselectivecoronary
angiography.111ThemajorityofCAFsarecongenitalinorigin,butothersmay
beacquiredaftercardiactraumaoriatrogenic(aftersurgeryormyocardial
biopsy).ACAFmaybeasolitarylesionorassociatedwithcongenitalheart
disease(mostcommonlytetralogyofFallotincludingpulmonaryatresia,atrial
septaldefect,andventricularseptaldefect).111TheCAFmayoriginatefroma
majorcoronaryarteryoritsbranchesandterminateinanyofthecardiac
chambers,greatveins,orpulmonaryarteries.ACAFpredominantlyoriginates
fromasinglefeedingartery,butrarelymultiplearteriescanfeedasingleCAF
(Fig.50.14).112–114WhenCAFsaretiny,theyareusuallyasymptomaticandare
detectedincidentallyduringdiagnosticechocardiographyorcardiac
catheterization.InlargeCAFsthefistulasegmentand/orfeedingconduit
coronaryarterycanbedilatedandtortuouswithaneurysmalsegments(Fig.
50.15).ThemajorityofCAFsarisefromtherightcoronaryartery(20%to60%),
followedbyleftanteriordescendingarteryandleftcircumflexartery.Asnoted
inmostlargeseries,theydrainpredominantlytotheright-sidedheartstructures;
themostcommonsitesofdrainagearetherightventricleandpulmonaryartery
followedbyrightatrium,coronarysinus,leftventricle,andleftatrium(Fig.
50.16).111,114
FIG.50.14 Proximalcoronaryarteryfistula(CAF)arisingfromtheleft
maincoronaryartery(A);notetheentirefistulasegmentdoesnothaveany
coronarynutritivebranches.DistalCAF(B)arisingfromadistalmajor
epicardialcoronaryartery(arrow).Theproximalconduitcoronaryartery
(leftanteriordescendingartery;LAD)istortuousanddilatedbutprovides
normalcoronarybranchessupplyingthemyocardium.Asterisksindicate
sitesofpotentialtranscatheterorsurgicalclosure.LCX,leftcircumflex
artery.(FromGowdaST,LatsonLA,KuttyS,etal.Intermediatetolongtermoutcomefollowingcongenitalcoronaryarteryfistulaeclosurewith
focusonthrombusformation.AmJCardiol.2011;107:302–308.)
FIG.50.15 (A)Smallcoronaryarteryfistulacommunicatingwiththe
pulmonaryartery(arrow).(B)Severelydilatedrightcoronaryartery(arrow)
duetoalargedistalrightcoronaryarteryfistula.
