Andersons pediatric cardiology 1357
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Management
Mostaorto–leftventriculartunnelsrequiretreatmentsoonerratherthanlater,
althoughspontaneousclosureofanaorto–leftventriculartunnelhasbeen
reported.47Medicaltreatmentconsistsofmanagementofheartfailuresymptoms
withdiuretics,angiotensin-convertingenzymeinhibitors,andinextremecases,
mechanicalventilationwithinotropicsupport.Ultimately,surgicalor
transcathetercorrectionofthelesionisrequired.Surgicalcorrectionismost
commonlyperformed,whichmayinvolveclosureoftheaorticend,ventricular
end,bothendsandplicationoftheascendingaorta,andplicationofthe
tunnel,47,57amongothertechniques.Itisimperativetopayattentiontothe
coronaryarteriesduringtheoperationbecausedistortionoftherightcoronary
arterymayoccur56insomeinstances.Deviceocclusionofthetunnelhasbeen
reportedwithdifferentdevicesinaorto–leftventriculartunnelswithfavorable
anatomyforcatheter-basedclosure.58–60Aswithsurgicaltechniques,care
shouldbetakentoevaluatethecoronaryarteriesatthesametimeduetothe
possibilityofcoronaryarteryimpingement/interferenceincoronaryarteryflow
fromdevices.Earlyrepairisgenerallyrecommendedtopreventtheprogression
ofnativeaorticvalveregurgitation,whichisrelativelycommon.57,61,62Some
patientswillalsorequirenativeforaorticvalvesurgery/replacement(upto50%)
infollow-up.61,62However,ifoperatedonsoon,theneedforsurgicalrepairof
theaorticvalve/aorticvalvereplacementmaybedecreased.47
AneurysmsoftheSinusesofValsalva
ThesinusesofValsalvaarethecomponentsofthearterialrootsthatsupportthe
leafletsofthearterialvalve.Althoughitispossibletofindaneurysmaldilation
ofthepulmonaryvalvarsinuses,whendescriptionsareprovidedofsuch
dilation,theyrefertothesinusesoftheaorticroot.63Theaneurysmaldilation
canbecongenitaloracquired,althoughtheso-calledcongenitalvariantsalmost
certainlyreflectacquiredchangeinanaorticrootpronetopermitsinusal
dilation.Thisaneurysmaldilationisfoundmostfrequentlyinthesettingof
connectivetissuedisorders,suchasMarfansyndrome,orLoeys-Dietz
syndrome.Inthesesettings,thedilationtypicallyinvolvesallthreevalvar
sinuses,leadingtoaorticregurgitationunlessappropriatelytreated.Aneurysmal
dilationisalsofrequentinthesettingoftheaorticvalvewithtwoleaflets.These
variantsarediscussedingreaterdetailinChapters45and59.Thischapteris
concernedwithdilationofindividualvalvarsinuses.Theaneurysmaldilation
canafflictanyofthethreesinuses,buttherightcoronarysinusismost
frequentlyinvolved(Fig.51.18),followedbythenoncoronary,ornonadjacent,
sinus.
FIG.51.18 Aneurysmoftherightcoronaryaorticvalvarsinus.(A)
Herniationofthewallofthesinus.(B)Theaneurysmalsinusextendsinto
therightventricle,openingbetweenthehingesoftheseptaland
anterosuperiorleafletsofthetricuspidvalve.
Theleftcoronarysinusisleastfrequentlyaffected.Itisanabnormalityinthe
relationshipbetweenthearterialwallsofthesinusesandtheirsupporting
ventricularstructuresthatisthelikelycauseofthedilation.Aneurysmaldilation
ofthesinusesiscloselyrelatedtothechangesseeninthesettingofthedoubly
committedandjuxtaarterialVSD.Inthelattersetting,itisagaintheright
coronarysinusthatismostfrequentlyinvolved,withthelackofthe
subpulmonaryinfundibulumagainpointingtodeficienciesinthesupportofthe
sinusalwallsasthelikelymorphogeneticfactor.
AneurysmsofthesinusesofValsalvamaybeclassifiedinanumberofways.
Theymaybeconsideredacquiredorcongenitaland,inaddition,rupturedor
nonruptured.Theaorticsinusesarenormallydilatedcomparedwiththerestof
theaorticrootandareknownastheright,left,andnoncoronary(posterioror
nonfacing)sinuses.64Thisisbasedonoriginsofcoronaryarteries,butthe
originsofthecoronaryarteriesarealsohighlyvariable.ThesinusesofValsalva
aremadeupmainlyfromelastictissue,andhistologicexaminationhasshown
thatthewallsofthesinusesarethinnercomparedwiththesurrounding
sinutubularridge.65CausesofaneurysmsofthesinusesofValsalvaarevariable.
Therearemanyinfectiouscauses,includingsyphilis,tuberculosis,andvarious
formsofendocarditis.Aortopathies,bicuspidaorticvalves,oranyconnective
tissuedisordercanresultinaneurysmsofthesinusesofValsalva.Althoughitis
difficulttodiscernfromtheliteraturethetrueincidenceofsinusofValsalva
aneurysms,ithasbeensuggestedthatruptureofthesinusofValsalvaismore
commonintheAsianpopulation.66,67RupturedsinusofValsalvaaneurysmsare
associatedwithothercongenitalheartlesions,themostcommonbeingaVSD
andaorticregurgitation.Theymostcommonlyoriginatefromtherightcoronary
sinus,followedbythenoncoronarysinusandrarelyfromtheleftcoronary
sinus.66–68Giventhatthemostcommonoriginisfromtherightcoronarysinus,
themostcommonsitesofruptureareintotherightventricleandrightatrium,
followedlesscommonlybytheleftventricle,leftatrium,andPA.Rupture
outsidethecardiacchambersintothepericardiumisanexternallyrare
occurrence.69–71
