Andersons pediatric cardiology 1358
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ClinicalManifestations
SinusofValsalvaaneurysmsthathavenotrupturedusuallydonotpresentwith
anysymptomsdirectlyassociatedwithdilatationofthesinuses.Inthese
instances,symptomsaccompanyotherassociateddiagnoses(e.g.,from
connectivetissuedisordersorinfections).Thrombimayforminthedilated
sinus,inwhichcasesymptomsfromsystemicembolizationofthrombican
manifest.
PatientswhopresentwitharupturedsinusofValsalvaaneurysmhaveahighly
variablepresentation.Somepatientsmaybeasymptomaticandthelesiononly
detecteduponwork-upforaheartmurmur.Inothercases,symptomsofacute
rupturecanoccur.Theseconsistofacuteonsetofsharpchestpain,dyspnea,
fatigue,andhemodynamiccompromise/collapse.Therupturemayoccurwithor
withoutactivity.Rarely,evensuddendeathcanoccur,particularlyifthe
aneurysmrupturesintothepericardium.
PhysicalfindingsinpatientswithrupturedsinusofValsalvaaneurysms
includeanillappearance,tachypnea,andboundingpulses.Thecardiac
examinationrevealsahyperdynamicprecordiumandto-and-froorcontinuous
murmur.Findingsofassociatedlesions(e.g.,aVSDoraorticregurgitation)are
difficulttoseparatefromthefindingsoftherupturedaneurysm.
Investigations
ForpatientswithsinusofValsalvaaneurysmsthathavenotruptured,inaddition
toechocardiography,diagnosticimagingincludingcardiacMRIandCTscans
shouldbeperformedaccordingtoguidelines/institutionalpracticesforroutine
surveillanceaccordingtotheunderlyingdisease.
RupturedsinusofValsalvaaneurysmsoftenpresentasadiagnosticdilemma
andaremisdiagnosedornotrecognizedatall.Theelectrocardiogram
demonstratesST-Twaveabnormalities,andthechestradiographshowschamber
enlargementintowhichtheaneurysmhasruptured.Forthemostpart,witha
highindexofsuspicion,rupturedsinusofValsalvaaneurysmscanbediagnosed
bytransthoracicechocardiographyalone(Fig.51.19)andcanbebetter
visualizedwithtransesophagealechocardiographybeforeoratthetimeof
correction(Fig.51.20).However,itisnotuncommonfortherupturedaneurysms
tobeconfusedwithVSDsornotrecognizedinthepresenceofaVSD.Recently,
withtheadventofthree-dimensionalechocardiography,thecorrectdiagnosiscan
bemade.72Inaddition,thismodalityprovidesanatomicdetailsthatare
invaluablefortheeventualtreatment.72,73CardiacMRIandCTscansalso
providevaluableanatomicinformation(andfunctionalinformationinthecaseof
MRI),whichcanbeusedtohelpplantheeventualcorrectiveprocedure.74
FIG.51.19 A9-year-old,asymptomaticgirlpresentedtoacardiologistfor
evaluationofaheartmurmur.Aninitialwork-upincludingatransthoracic
echocardiogramsuggestedthediagnosisofaventricularseptaldefect.
Furtherdetailedechocardiographyconfirmedthatitwasarupturedsinusof
Valsalvaaneurysm(left,arrow)fromtherightcoronarycusptotheright
atrium.Holodiastolicflowreversalwasseenintheaorta.Dopplercolorflow
mappingdemonstratesflowfromtherightaorticsinustotherightatrium
(right).
FIG.51.20 Transesophagealechocardiographypriortosuccessful
operativerepairinthepatientdescribedinFig.51.19demonstratesthe
sinusofValsalvaaneurysm(left,arrow)withcorrespondingDopplercolor
flowmapping(right).
