www.revportcardiol.org
CASEREPORT
Marfansyndromewithascendingaorticaneurysm:
Valueofcardiaccomputedtomography
PedroJerónimoSousaa,∗,PedroAraújoGonc¸alvesc,d,SérgioBoshoffb,
HugoMarquesc,SaloméCarvalhoa,JoãoMoradasFerreirab,MiguelMotaCarmod,
AnaAleixod,JoséPedroNevesb,MiguelMendesa
aServic¸odeCardiologia,HospitaldeSantaCruz--- CentroHospitalardeLisboaOcidental,Lisboa,Portugal bCirurgiaCardio-Torácica,HospitaldeSantaCruz--- CentroHospitalardeLisboaOcidental,Lisboa,Portugal cCentrodeImagiologia,HospitaldaLuz,Lisboa,Portugal
dCEDOC--- CentrodeEstudosdedoenc¸asCrónicas--- FCM-UNL,Lisboa,Portugal
Received31January2012;accepted2May2012
KEYWORDS
CardiacCT; Marfansyndrome; Aorticaneurysm
Abstract Wereportthecaseofa40-year-oldmanwithknownMarfansyndromewhopre- sentedwithsevereaorticvalveregurgitationsecondarytosignificantaorticrootdilatation.To ruleoutcoronaryarterydiseaseandtoevaluatetherestofthethoracicaortabeforesurgery, cardiaccomputedtomography(CT)wasperformed.Abriefreviewoftheliteratureshows howcardiacCTcan,inselectedcases,ruleoutcoronaryarterydiseasebeforenon-coronary cardiothoracicsurgery.
©2012SociedadePortuguesadeCardiologiaPublishedbyElsevierEspaña,S.L.Allrights reserved.
PALAVRAS-CHAVE
AngioTCcardíaca; SíndromedeMarfan; Aneurismadaaorta
SíndromedeMarfancomaneurismadaaortaascendente---importânciadaangioTC
cardíaca
Resumo Descrevemosocasodeumdoentede40anosdeidadecomSíndromedeMarfan,que apresentaregurgitac¸ãovalvularaórticagrave,secundáriaadilatac¸ãoseveradaraizaórtica. Paraexcluirapresenc¸adedoenc¸acoronáriaeestudarosrestantessegmentosaórticosantesda cirurgia,foirealizadaumaangioTCcardíaca.Umabreverevisãodaliteraturademonstracomo, emdeterminadoscontextos,aangioTCcardíacapodetervantagensnaexclusãodedoenc¸a coronáriaantesdecirurgiacardio-torácicanãocoronária.
©2012SociedadePortuguesadeCardiologia.PublicadoporElsevierEspaña,S.L.Todosos direitosreservados.
∗Correspondingauthor.
E-mailaddress:p965675551@gmail.com(P.J.Sousa).
0870-2551/$–seefrontmatter©2012SociedadePortuguesadeCardiologiaPublishedbyElsevierEspaña,S.L.Allrightsreserved.
http://dx.doi.org/10.1016/j.repc.2012.05.019
60 P.J.Sousaetal.
Casereport
Wereportthecaseofa40-year-oldmanwithnoknown cardiovascularriskfactorswhowasdiagnosedwithMarfan syndromeattheageof32.Hismedicalhistoryincluded gastroesophagealrefluxsyndromeandpreviousophthalmic surgeryforlensdislocationandorthopedicsurgeryonthe tibial-tarsaljoints.Hewasbeingmedicatedwithbisoprolol andpantoprazole.
Fortheprevioussixmonthshehadpresentedworsening exercisedyspneaandhadmarkedlimitationinactivitydue tosymptoms(NYHAclassII---III).
Theechocardiogramshowedsevereaorticvalveregurgi- tationsecondarytosignificantaorticrootdilatation(64mm diameterattheValsalvasinus)andleftventriculardilata- tion,butwithnormalsystolicfunction.
Toexcludecoronaryarterydiseaseandsimultaneously toevaluatetherestofthethoracicaorta,cardiaccom- puted tomography (CT) was performed (including the thoracicaorta).Thisexamruledoutcoronaryarterydisease (Figure1)andconfirmedthepresenceofsignificantaortic rootdilatation(70mm),therestoftheaortabeingnormal (Figure2).
Thepatientwasreferredforsurgery,withnoneedfor invasivecatheterization.HeunderwentaBentallprocedure withimplantationofanaorticconduitandaprostheticaor- ticvalve(29mmSt.Jude).Therewerenocomplications aftersurgery,theechocardiogramshowingtheprosthetic aorticvalvewithnormalmotionandtheconduitfunction- ingcorrectly.Thepatientwasdischargedsixdayslaterand warfarinwasaddedtohismedication.
At6-monthfollow-upthepatientwasasymptomaticand freefromcardiovascularevents.
Discussion
Marfan syndrome is the most common inherited multi- systemic disorder ofconnective tissue,witha reported incidence of2-3 per10000population, withoutgender, racial,orethnicpredilection.1Itscardiovascularfeatures
werefirstoutlinedbyMcKusickin19552;accordingtothe
currentdiagnosticcriteriamajorcardiovascularmanifes- tationsincludedilatationoftheascendingaorta,withor withoutdissection.3
Inthepresenceofascendingaortadilatation,prophy- lacticsurgeryisrecommendedwhenthediameterofthe ascendingaortaattheaorticsinusesreaches4.5cm,orin somecasesevenless(whenthereisafamilyhistoryofaor- ticdissection,inthepresenceofrapidaorticdilatationor severeaorticvalveregurgitation,orwhenavalve-sparing operationispossible).1,4
Inthecasereported,thepresenceofsevereaorticroot dilatation(>60mm)withassociatedaorticvalveregurgita- tionwasaclearindicationforsurgery.
Althoughthepatienthadnoangina,obstructivecoro- naryarterydiseasewasruledoutpriortoaorticsurgery. Thisevaluationshouldbeperformedinasymptomaticmale patientsovertheageof40orpostmenopausalwomen,and isalsoindicatedforpatientswithpreviouscoronaryartery disease,symptomsofleftventriculardysfunction,presumed ischemicmitralregurgitationoroneormorecardiovascular riskfactor.4
RCA
LAD
LCX
OM
Figure1 Cardiaccomputedtomography:multiplanarreconstructionsrulingoutsignificantcoronaryarterydisease.LAD:left anteriordescendingartery;LCX:leftcircumflexartery;OM:obtusemarginalartery;RCA:rightcoronaryartery.
Marfansyndromewithascendingaorticaneurysm 61
Figure2 Aorticrootdilatationseenincardiaccomputedtomographyvolume-renderedimage(A)andinintraoperativephotograph (B).
Traditionally,thestudyofcoronaryarteryanatomyis performedthroughinvasiveangiography.However,thiscan alsobesafelydonewithcardiacCT5,6takingadvantageof
thehighnegativepredictivevalueofthisexam6;thisis
oneindicationforwhichcardiacCTcanreplaceinvasive angiography.7
Thisapproachcanbeappliedtoahighpercentageof patientswhenstudyofcoronaryarteryanatomyisindicated priortovalvularoraorticsurgery,asitismoreconvenient andlessinvasivethaninvasivecoronaryangiography.Fur- thermore,somepatientsubsetsmayderivegreaterbenefit fromthisapproach,whenthereishigheriatrogenicpoten- tial(suchasinthepresenceofaorticdissectionoraortic valvethrombus/vegetations)orwhencoronarycatheteriza- tionwouldbedifficult(suchasinthepresenceofaortic rootdilatationoranomalouscoronaryarteryorigin).Inthese cases,cardiacCT,avoidingtheneedforpotentialprolonged invasivecardiaccatheterization,reducesnotonlyradia- tionandcontrastdoses,butalsotheriskofcomplications. Amongthelatter,aorticdissectionhasbeendescribedasa rare(0.04%)butseriouscomplicationofcardiaccatheter- izationandwasfoundtobe associatedwith theuseof non-conventionalcatheters.8
Therisk of contrast-induced nephropathy should be weighedwhencoronaryangiographyisconsidered.Since therearenodifferencesinthecontrastdoseusedincoro- naryangiographyperformedinvasivelyorbycardiacCT,9this
riskshouldbesimilarwithbothtechniques.
Inthecasereported,thereweretworiskfactorsfor complicationswithinvasivecoronaryangiography:thepres- ence of aorticroot dilatation and severe aortic valve regurgitation.This favoredthechoice ofcardiacCTto studythecardiacanatomy,whichwassafe,ruledoutcoro- naryarterydiseaseandsimultaneouslyprovideddetailed anatomyofthethoracicaorta.
Ethicaldisclosures
Protectionofhumanandanimalsubjects.Theauthors
declarethatnoexperimentswereperformedonhumansor animalsforthisstudy.
Confidentialityofdata.Theauthorsdeclarethattheyhave followedtheprotocolsoftheirworkcenteronthepublica- tionofpatientdataandthatallthepatientsincludedinthe studyreceivedsufficientinformationandgavetheirwritten informedconsenttoparticipateinthestudy.
Righttoprivacyandinformedconsent.Theauthorshave obtainedthewritteninformedconsentofthepatientsor subjectsmentionedinthearticle.Thecorrespondingauthor isinpossessionofthisdocument.
Conflictsofinterest
Theauthorshavenoconflictsofinteresttodeclare.
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ARTIGO 10/ MANUSCRIPT 10:
Pleasecitethisarticleinpressas:BorgesSantosM,etal.Diagnosticyieldofcurrentreferralstrategiesforelective coronaryangiographyinsuspectedcoronaryarterydisease----AnanalysisoftheACROSSregistry.RevPortCardiol.2013.
http://dx.doi.org/10.1016/j.repc.2012.11.008