Andersons pediatric cardiology 1359
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Management
LargenaturalhistorystudiesonrupturedsinusofValsalvaaneurysmsare
unavailableintheliteratureduetotherelativelylowincidence,andearlyreports
consistedofisolatedcasesorcaseseries.However,mostpatientswerefoundto
havediedwithinayearofrupturefromcongestiveheartfailureorendocarditis
orpriortotheeraofsurgicalrepair.75
ThemanagementofsinusofValsalvaaneurysmsassociatedwithotherlesions
isbasedontheunderlyingdiseaseprocess(e.g.,connectivetissueorinfectious
process).Inaddition,thismayconsistofβ-blockers,angiotensin-converting
enzymeinhibitors,angiotensinIIreceptorantagonists,andlifestyle
modificationswithrespecttosportsrestrictions.Surgicalmanagementstrategies
aredictatedbyprogressioninthesizeoftheaneurysm,inadditiontothe
absolutesizeoftheaorticsinuses.
Ingeneral,althoughmedicalmanagementinpatientswithrupturedsinusof
Valsalvaaneurysmsmayhelptocontrolsymptomstosomeextent(with
diuretics,angiotensin-convertingenzymeinhibitors,andcardiorespiratory
supportinextremecases),definitivecorrectionofthedefectiswarrantedina
timelyfashion.Surgicaltreatmentconsistsofprimaryclosureoftheruptured
aneurysmorpatchclosureofthedefectandcanbeapproachedfromtheaortic
end,theendofthechamberintowhichtherupturehasoccurred,orboth
ends.67,68,76–79Concomitantaorticvalvesurgery/replacementmayalsoneedto
beperformed.Infollow-up,progressionofaorticinsufficiencycanoccur,
necessitatingaorticvalvereplacement.68,77–79Residualfistulous
communicationsmaybepresentinfollow-upandinsomeinstancesalsorequire
repair.67,68,76–79
SincethefirstreportfromCullenandcolleagues,80therehavebeennumerous
reportsandseriesofclosureofrupturedsinusofValsalvacommunicationsinthe
cardiaccatheterizationlaboratory.Thedefectcanbeclosedfromaretrograde
approachorantegradeapproachfacilitatedbyanarteriovenousguidewireloop.
Themajorityoftheserupturedaneurysmscanbeoccludedinthecardiac
catheterizationlaboratorywiththemanyoptionsofdevicesavailableatthe
interventionalcardiologist'sdisposalinthecurrentera(e.g.,VSD,patentductus
arteriosusandatrialseptaldefectoccluderdevices).Infourrelativelylarge
transcatheterseriesreportingoutcomeswithpatientnumbersrangingfrom,17–25
successfulocclusionwasachievedin84%to94%ofpatients.81–85Residual
shunt(inmostinstancesresolving)anddevice-relatedaorticregurgitation
occurredinaminorityofpatients.Inalargereviewof877patientsundergoing
correctionofrupturedsinusofValsalvaaneurysms(741surgical,136
transcatheterclosure),transcatheterclosurewasfavorablewithregardtoefficacy
andsafetyprofile.83Givenourcurrentknowledge,itwouldseemthat
transcatheterclosureofrupturedsinusofValsalvaaneurysmsisanacceptable
alternativetosurgeryandshouldbeconsideredunlessconcomitantlesionsneed
tobesurgicallyaddressed.Regardlessofwhethertranscatheterorsurgical
closureisperformed,patientsshouldbefollowedforlife,withparticular
emphasisonaorticregurgitation,residualfistulouscommunications,and
coronaryarterycompromise.
TripleOutletsFromtheCardiacBase
Theselesionsareveryrare.Theyarealsomostunexpectedmorphologic
findings,86althoughthefindingofthreearterialtrunksistheusualarrangement
inreptilesandcrocodilians.8Ascongenitalmalformations,weareawareof
descriptionsofmalformationsoftheoutflowtractoftherightventriclesuchthat
eachPAhasadirectoriginfromtheventricularroof.Thiscanseemingly
produceatriple-outletventriclewhenallarterialrootsarisefromtheright
ventricle.87Aswasexplainedwhendiscussingthedevelopmentalissues,we
havenowassociatedacomparablearrangementoftheaorticroot,whereby
duplicatedrootsfedtheaortictrunk,againinthesettingofapatientwithdoubleoutletrightventricle(Fig.51.21).9
FIG.51.21 Duplicationoftheaorticroot.(A)Atfirstlook,thereisadouble
outletfromtherightventricle(arrow).(B)However,theaorticrootis
duplicated.Thereforethepatientexhibitedtripleoutletfromtheright
ventricle.(CourtesyDr.AshishKatewa,Chhatishgarh,India.)
Thefirstdescriptionoftriarterialheart,alsotermedtritruncalheart,wasby
Diaz-Gongorain1982.86Thisheartwasdescribedtohaveapulmonarytrunk
arisingfromtherightventricleandgivingrisetotheleftPAandarterialduct,
whiletheaortaarosefromtheleftventricleinnormallocationposteriortothe
