Andersons pediatric cardiology 978
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FIG.37.3 Heartsfrompatientswithtransposition.(A)Patientwithmirrorimagedatrialappendages.Notetheleft-sidedmorphologicallyrightatrial
appendage.Thestarshowstheright-sidedmorphologicallyleftappendage.
Thereisleft-handventriculartopology,withthemorphologicallyright
ventriclebeingleftsided,indicatingthepresenceofconcordant
atrioventricularconnectionsbutwithdiscordantventriculoarterial
connections.(B)Inthispatienttheatrioventricularconnectionsare
concordantinthesettingofusualatrialarrangement.Thestarshowsthat
themorphologicallyrightappendageisrightsided.Thediscordantly
connectedaortaistotheleftofthepulmonarytrunk.Bothofthesehearts
areexamplesofl-transpositionthatisnotcongenitallycorrected.
Whentheventricularseptumisintact,theaortaalmostalwayshasacomplete
muscularinfundibulum,whiletheleafletsofthepulmonaryvalveareinfibrous
continuitywiththoseofthemitralvalve(Fig.37.2).Variationsinthis
infundibularmorphology(e.g.,thepresenceofbilaterallycompletemuscular
infundibulums)arenotseennearlyasfrequentlywhentheventricularseptumis
intactaswhenthereisaventricularseptaldefect(discussedlater).
CoronaryArteries
Wheneverthepositionoftheaorticrootisabnormal,theoriginsofthecoronary
arteriesdeviatefromthosefoundinthenormalheart.Withoutexception,the
arteriescontinuetoarisefromoneorother,orboth,ofthoseaorticsinusesthat
face,orareadjacentto,thepulmonarytrunk(Fig.37.4).
FIG.37.4 Heartwithatrialchambersandarterialtrunksremoved,
photographedfromtheatrialaspect.Notetheanteriorandright-sided
locationofthediscordantlyconnectedaorta.Asisalwaysthecaseinthe
settingoftransposition,thecoronaryarteriesarisefromtheaorticsinuses
adjacenttothepulmonarytrunk,inthisinstancewithonecoronaryartery
arisingfromeachsinus.
Theepicardialcourseofthearteriesarisingfromtheseadjacentsinusesis
muchlesspredictable.Itisbesttouseadescriptiveapproachtoaccountforthe
multiplevariations,takingnoteseparatelyoftheoriginsofthethreemajor
coronaryarteriesfromtheaorticsinusesandtheircourserelativetothevascular
pedicle.Particularattentionshouldbepaidtoanyintramuralcourse,alongwith
thelocationofthearterysupplyingthesinusnode.Althoughthecoronary
arteriesalwaysarisefromoneorbothoftheaorticsinusesthatareadjacentto
thepulmonarytrunk,thepositionofthesesinusesinspacecanvaryaccordingto
therelationshipofthearterialtrunks.Therefore,whendescribingthesinusal
origin,itisadvantageoustouseasystemthatisindependentofspatial
relationships.Thiscanbeachievedbyconsideringtheaorticsinusesasthey
wouldbevisualized,figurativelyspeaking,byanobserverstandinginthe
