8:DiseasesoftheAorta

Overview
Aorticdiseaseisdiscussedinthischapter's2modulesonaneurysmdiseaseandtheacutesyndromesofdissection, intramuralhematoma,andpenetratingulcer,respectively.Patientassessment,management,andsurveillanceareemphasized, ashighlightedinrecentguidelines.

Authors
PatrickT.O'Gara,MD,FACC EditorinChief ThomasM.Bashore,MD,FACC AssociateEditor JamesC.Fang,MD,FACC AssociateEditor GlennA.Hirsch,MD,MHS,FACC AssociateEditor JuliaH.Indik,MD,PhD,FACC AssociateEditor DonnaM.Polk,MD,MPH,FACC AssociateEditor SunilV.Rao,MD,FACC AssociateEditor

8.1:AneurysmDisease
Author(s): EricM.Isselbacher,MD

LearnerObjectives
Uponcompletionofthismodule,thereaderwillbeableto: 1. Screenfirstdegreerelativesoftheaffectedindividualinordertodetectunrecognizedaneurysms,becauseaorticrootand ascendingthoracicaorticaneurysms(TAAs)maybefamilial. 2. Recognizetheassociationbetweenbicuspidaorticvalve(BAV)andascendingTAAs,andmakecertainthattheaortahas beenimagedinpatientswithaBAV. 3. Monitoraneurysmgrowthwithsurveillanceimaginginordertomaketimelyreferralsforaorticrepair. 4. Comparetheriskofaorticdissectionorruptureagainsttheriskofaorticrepairfortheindividualpatientwhendecidingon thetimingoftheaorticrepair.

AbdominalAorticAneurysms
Aorticaneurysmsmayinvolveeithertheabdominalaorta,thethoracicaorta,or, rarely,bothsegments(thoracoabdominalaorticaneurysms).Abdominalaortic aneurysms(AAAs)aremuchmorecommonthanTAAs.Importantly,amongthose withananeurysminonesegmentoftheaorta,thereisa25%chanceofhavinga concomitantaneurysminvolvinganotheraorticsegment.Itisthereforeimportantto imagetheentireaortaofaffectedpatientstolookforthepresenceofaneurysms elsewhere. ThetrueprevalenceofAAAsisnotwelldefined,duetovariationinbothdiagnostic criteriaandthebaselineriskofthepopulationsscreened.However,screeninga population65yearsofagehasrevealedaprevalenceof57%amongmenand approximately1%amongwomen.Therateofaneurysmdiagnosishasbeen increasingoverthepastseveraldecades.ThemajorityofAAAsarisebelowtherenal arteries(infrarenalabdominalaneurysms),whereasaminorityariseabovethelevel oftherenalarteries(suprarenalaneurysms).Stagnationofbloodmayresultin thrombusformationalongtheaneurysmwallthethrombusmayembolizedistally, anditspresencealsoappearstoincreasetheriskforaneurysmgrowthandrupture. ThereareanumberofimportantriskfactorsforAAA,assummarizedinTable1. Atherosclerosis,whichhadlongbeenconsideredthecauseofAAAs,clearly contributestotheprocessofaneurysmformation,butthereisalsoevidencethat genetic,environmental,hemodynamic,andimmunologicalfactorscontributeas well.Thereishistologicalevidenceofinflammatoryinfiltrateswithinthewallsof aneurysms.Moreover,certainmatrixmetalloproteinases(MMPs),enzymes producedbysmoothmuscleandinflammatorycells,candegradeelastinand collagen,theprimarycomponentsoftheaorticextracellularmatrixthatgivetheaortic wallitstensilestrength.ThelevelsofsomeMMPsaresignificantlyhigherinthe wallsofaneurysmscomparedwithcontrols.Conversely,inanimalmodelsofAAA, treatmentwithdoxycycline,whichinhibitsMMPsthroughamechanismunrelatedto itsantibioticactivity,leadstolowerlevelsofMMP9intheaorticwall,reduced degradationofmedialelastin,andreducedexpansionofaneurysms. Earlydatafromhumantrialshaveshownpromisingresults,butrandomizedclinical trialshavenotyetbeenperformed,sotheuseofsuchtherapiesisnotcurrently recommended.StatintherapyhasalsobeenshowntoreduceMMP9expression andtoprotectagainstaneurysmgrowthinamousemodel,regardlessofthe cholesterollevel.Additionally,inhumansundergoingelectiveAAArepair, preoperativestatintherapyisassociatedwithadecreaseinMMP9levelsinthe aorticwall.Onemetaanalysisoffiveobservationalstudiesfoundsignificantly sloweraneurysmgrowthamongpatientstreatedwithstatins,butanotherstudy showednobenefit1 unfortunately,therearenocompletedrandomizedcontrolled trials.Betablockers,angiotensinconvertingenzymeinhibitors,angiotensinreceptor blockers,andaspirinmayalsohavepotentialbenefitsintreatingAAAs,butthese therapieshaveyettobestudiedinprospectivecontrolledclinicaltrials. MostpatientswithAAAsareasymptomatic,andtheiraneurysmsarediscovered incidentallyonphysicalexaminationorviaimagingorderedforanotherindication. Patientswhohavesymptomsusuallypresentwithgnawingpainlocatedinthe hypogastriumorlowerback.Aneurysmruptureisassociatedwithabruptonsetof pain,alongwithabdominaltendernessandapulsatileabdominalmass. SeveralimagingtechniquescanidentifyandsizeAAAs.Abdominalultrasonography isacceptedasthemostpracticalwaytoscreenforAAAs.Thistechniqueis inexpensiveandwithoutrisk,butitislimitedintheaccuracyofitsmeasurements anditsabilitytoimagethesuprarenalaortaandthemesentericandrenalarteries well.Computedtomographyangiography(CTA),ontheotherhand,isextremely accurateinimagingandsizingallsegmentsoftheaorta,aswellasindefining branchvesselanatomy.However,CTAismoreexpensive,andrequirestheuseof iodinatedcontrastandionizingradiation. TherehasbeenmuchdebatehistoricallyaboutthevalueofscreeningforAAAs. However,datafrommultiplelargeprospectivetrialshaveconvincinglydemonstrated

Table1

Table2

Figure1

Figure2

thatscreeningatriskpopulationsiseffectiveand,moreimportantly,costeffective. TheUSPreventiveServicesTaskForcehasconcludedthattheevidencesupports onetimeultrasonographyscreeningforAAAsamongmen6575yearsoldwhoare currentorformersmokers.2 Manyexpertsalsorecommendscreeningthosewho haveafirstdegreerelativewithanAAA.Atpresent,however,thereisnoevidenceto supporttheroutinescreeningofwomen. ThemajorriskassociatedwithAAAsisthatofrupture.Amongtheparticipantsin theUnitedKingdomSmallAneurysmTrialwhosufferedarupturedAAA,25%died beforereachingthehospital,another50%diedatthehospitalpriortoaorticrepair, andtheoverall30daysurvivalwasjust11%.3 ItisthereforecriticaltorepairAAAs whentheyarefirstrecognizedbysizecriteriatobeatsignificantriskofrupture.The riskofruptureincreaseswithaneurysmdiameter,asshowninTable2. AlthoughAAAsarelessprevalentamongwomen,theyaremorepronetorupture andtendtoruptureatsmalleraorticdiameterscomparedwithmen.Inorderto preventaneurysmrupture,electiverepairofasymptomaticAAAsisrecommended whenthediameterreaches5.5cm.Ingoodsurgicalcandidatesandwomen,many expertslowerthethresholdto5.0cm,especiallyamongpatientsofasmallerbody sizeorwithafamilyhistoryofrupture.4 Opensurgicalrepairrequiresopeningtheaneurysmandinsertinganartificialtube graftiftheaneurysmextendsdistally,thentherepairmaybecarriedintothe commoniliacarterieswithabranchedgraft.Alternatively,AAAscanoftenberepaired viaaminimallyinvasiveapproachcalled"endovascularaorticrepair"(EVAR),with theuseofapercutaneouslyimplanted,endovascular,coveredstentgraftthatserves tobridgetheaneurysmsacandtoexcludeitfromthecirculation(Figure1).The stentgraftmaybeastraighttubeorbifurcated,withbranchesextendingintothe commoniliacarteries. Whenattempted,stentgraftsaresuccessfullydeployed98%ofthetime.However, onelimitationofEVARisthatonlyaboutonehalfofpatientswithAAAshaveanatomy thatissuitablefortheprocedure.Asecondlimitationoftheprocedureisthefrequent occurrenceofendoleaks,inwhichthereispersistenceofbloodflowintothe excludedaneurysmsac.Suchendoleaksmayoccurbecauseoffailureto adequatelysealtheproximalordistalendofthestentgraft(typeIendoleak)or, moreoften,duetoretrogradeflowfromsmallbrancharteries(suchastheinferior mesentericorlumbararteries)backintoaneurysmsac(typeIIendoleak).Often, additionalpercutaneousproceduresareneededtotreattheendoleakleftuntreated, theymayleavethepatientatriskforcontinuedaneurysmexpansionandrupture. Patientswithendoleaks,therefore,requiremonitoringwithsurveillanceimaging. Multipleprospectiverandomizedtrialshaveshownsignificantlowerearlymortality amongthosetreatedwithEVARcomparedwithopenrepair.However,both theEVAR1(EndovascularAneurysmRepair1)trial5 andtheDREAM(Dutch RandomizedEndovascularAneurysmRepair)trial6 demonstratedthat,inthelong term,therewerenosignificantdifferencesintotalmortalityoraneurysmrelated mortality(Figure2).Moreover,inthelongterm,EVARwasassociatedwithincreased complicationratesandtheneedformorereinterventions(evenasfaras8yearsout fromtheindexprocedure).Thus,EVARoffersnoclearlateadvantageoveropen repairamongthosewhoaregoodcandidatesforeitherprocedure.Consequently, thechoiceofwhichapproachtotakeforaorticrepairshouldbeindividualizedfor eachpatientandbasedonage,comorbidities,patientpreference,andaortic anatomy.

RiskFactorsforAbdominalAorticAneurysms Table1

AnnualRiskofAbdominalAorticAneurysmRupturevs.AneurysmDiameterintheUnitedKingdomSmallAneurysmTrial Table2 AdaptedwithpermissionfromBrownLC,PowellJT.Riskfactorsforaneurysmruptureinpatientskeptunderultrasoundsurveillance.AnnSurg 1999230:28996.

EndovascularAbdominalAorticAneurysmRepair(EVAR) Figure1 Theimageintheupperleftdemonstratesthetypicalappearanceofaninfrarenalabdominalaorticaneurysm.PanelA:Anendografthasbeen introducedretrogradefromtheaccesssiteanddeployedacrosstheaneurysmsac,withonelimbextendingintotheipsilateralcommoniliac artery.Theproximalendofthegraftneedsalandingzonethatisofrelativelynormaldiameter.PanelB:Barbsorhooksattheproximalend preventdistalmigration.Aguidewireisthenadvancedintothegraftretrogradefromthecontralateralside.PanelC:Thecontralateraliliacartery limbofthegraftisintroducedovertheguidewireandexpanded.PanelD:Theipsilaterallimbisfullydeployedandanendovascularballoonis inflatedalongthestentgrafttosecurefixationoftheanastomoticsites.Thebluearrowsindicatemovementoftheguidewire. ReproducedwithpermissionfromGreenhalghRM,PowellJT.Endovascularrepairofabdominalaorticaneurysm.NEnglJMed2008358:494 501.

KaplanMeierEstimatesforTotalSurvivalandAneurysmRelatedSurvivalDuring8YearsofFollowUp Figure2 Amongpatientsrandomlyassignedtoeitherendovascularrepairoropenrepairofanabdominalaorticaneurysm,anearlybenefitwithrespectto aneurysmrelatedmortalityintheendovascularrepairgroupwaslostbytheendofthestudy,atleastpartiallybecauseoffatalendograft ruptures(adjustedhazardratiowithendovascularrepair,0.9295%confidenceinterval[CI],0.571.49p=0.73).Bytheendof8yearsof followup,therewasnosignificantdifferencebetweenthetwogroupsintheriskofdeathfromanycause(adjustedhazardratio,1.0395%CI, 0.861.23p=0.72). ReproducedwithpermissionfromTheUnitedKingdomEVARTrialInvestigators.Endovascularversusopenrepairofabdominalaorticaneurysm. NEnglJMed2010362:186371.

ThoracicAorticAneurysms
TAAsarelesscommonthanAAAs.TAAsmayinvolvetheaorticroot,ascending aorta,aorticarch,ordescendingaortawhentheyextendbelowthediaphragm,they arereferredtoas"thoracoabdominalaorticaneurysms."Aneurysmsoftheaortic rootandascendingaortaaremostcommon,andusuallyoccurasaconsequence ofunderlyingmedialdegeneration(previouslytermed,cysticmedialnecrosis). Medialdegenerationisnotableforthelossofsmoothmusclecellsandelasticfiber degeneration,resultinginweakeningoftheaorticwall.Aneurysmsofthe descendingaortaaremoreoftenassociatedwithatherosclerosis.Theknown etiologiesofTAAsarelistedinTable3. MedialdegenerationiswellrecognizedinassociationwithMarfansyndrome,which isaconnectivetissuedisorderassociatedwithadecreasedamountofelastininthe aorticmediaandabnormalelasticpropertiesoftheaorticwall.Marfansyndromeis anautosomaldominantheritabledisorderduetomutationsinthegeneFBN1, whichencodesforfibrillin1,acomponentofthemicrofibrilsofelastin. Dietzandcolleagueshavedemonstratedthatfibrillin1notonlyisofstructural importance,butalsoplaysaroleinregulatingtransforminggrowthfactor(TGF)in Marfansyndrome,theabnormalelastinleadstoexcessiveTGFsignaling,whichin turnleadstoelasticfiberfragmentationandprogressiveaorticrootenlargement.In amousemodelofMarfansyndrome(withfibrillin1mutationsandaorticroot enlargement),treatmentwithaTGFneutralizingantibodyresultedinreducedTGF signalingintheaorticmediaandarrestedaorticgrowth.LosartanisalsoaTGF inhibitor,andsimilarlypreventsaorticaneurysmsinthemousemodelofMarfan syndrome.7 BAVdiseaseisanimportantriskfactorforaorticrootandascendingTAA.BAV diseaseisthemostcommoncongenitalvalvedisorder,occurringin1.4%ofthe generalpopulation.Menareaffectedfourtimesascommonlyaswomen.Itwas oncethoughtthattheaorticenlargementseeninpatientswithBAVdiseasewasa consequenceofassociatedbicuspidaorticstenosis,anditwasthereforereferredto as"poststenoticdilatation."However,currentevidencearguesotherwise. Approximately50%ofpatientswithBAVshaveenlargementoftheproximalaorta, andtheprevalenceisindependentofthefunctionofthevalve,meaningthataortic dilatationisseenasoftenamongpatientswithregurgitantornormallyfunctioning BAVsaspatientswithstenoticvalves.Moreover,amongpatientsundergoingaortic valvereplacementsurgeryforaorticstenosis,75%ofthosewithanativeBAVare foundtohavemedialdegenerationcomparedwithonly14%ofthosewithanative tricuspidaorticvalve. BAVdiseasemaybeheritable,as20%willhaveanaffectedfirstdegreerelative, butnosingleresponsiblegenehasyetbeenidentified.Becauseaorticenlargement issocommon,allpatientswithaBAVshouldbeformallyevaluatedforevidenceof aorticrootorascendingaorticdilatation.TheaortopathyassociatedwithBAV diseasecanextendtotheleveloftheligamentumarteriosumandincludes coarctation. Ascendingaorticaneurysmsmaybeidiopathic,andinmanysuchcases,aortic histologyrevealsmedialdegenerationsimilartowhatisseeninMarfansyndrome andBAVdisease.Suchaneurysmsmaybesporadic,butin20%ofcases,other familymembersmaybeaffected,suggestingaheritableconditionthatisreferredto as"familialTAAsyndrome."Mostpedigreessuggestanautosomaldominantmode ofinheritance,butthereismarkedvariabilityinexpressionandpenetrance. Mutationshavebeenidentifiedinapproximately20%ofaffectedfamilies,including thegenesACTA2,TGFBR2,andMYH11.Theroleofgenetictestinginpatientswith TAAsremainstobeclarified.Nevertheless,giventhefactthatMarfansyndrome, BAVs,andidiopathicTAAscanbeheritable,firstdegreerelativesofaffected individualsshouldbescreenedforthoracicaorticdiseasewithdiagnosticimaging. MostTAAsareasymptomaticandarenotdetectableonaroutinephysical examinationthelargemajorityarediscoveredincidentallyonanimagingstudy orderedforanotherreason.WhenTAAspresentclinically,itisusuallydueto

Table3

Table4

Figure3

Figure4

Figure5

Figure6

Figure7

Figure8

enlargementoftherootorascendingaortathatleadstotetheringoftheaorticvalve leaflets,resultinginincompletevalveclosureandaorticinsufficiencytheaortic insufficiencymaypresentearly,asadiastolicheartmurmur,orlate,asheartfailure. Lessoften,TAAspresentwithsymptomscausedbyamasseffect,suchascough, dysphagia,orhoarsenessduetotracheal,esophageal,orrecurrentlaryngealnerve compression,respectively.LargeorrapidlyexpandingTAAsmayalsopresentwith symptomsofchestorbackpain. TAAscanbedetectedbyCTA,magneticresonanceangiography(MRA), echocardiography,andaortography.CTAisusedmostoftenforevaluationofthe thoracicaorta,asitprovidesoutstandinganatomicdetailandaccuratesizingofthe aorta.MRAalsoimagestheaortawell,althoughitislessconvenientandcannotbe usedinpatientswithpacemakersorimplantablecardioverterdefibrillators. Transthoracicechocardiographyisverygoodatimagingtheaorticroot,butisless reliableinimagingtheascendingaorta.Transesophagealechocardiography imagesthethoracicaortawell,butisminimallyinvasive.Invasiveaortographycan identifydilatedaorticsegments,butitcannotbeusedtosizetheaortaaccurately. MostTAAsgrowovertime.Theratesofgrowtharegreaterforlargeversussmall aneurysms,forpatientswithMarfansyndromeorwithachronicaorticdissection, andforaneurysmsinvolvingthedescending,ratherthantheascending,aorta. SimiliartoAAAs,theriskofTAAdissectionorruptureincreaseswithaorticdiameter and,althoughdissectioncanoccuratanydiameter,theriskrisesabruptlyforaortic diametersof6cm.Themortalityassociatedwithaorticdissectionorruptureis high,whereasthemortalityofelectiverepairisreasonable,sothegoalistorepair theaortapriortoanyaorticcatastrophe.Theindicationsforrepairofasymptomatic TAAs,basedonthe2010AmericanCollegeofCardiologyFoundation/American HeartAssociation(ACCF/AHA)guidelines,8 arelistedinTable4.However,the timingofelectiveaorticrepairmustbeindividualizedforeachpatient,weighingthe riskofsurgeryagainsttheriskofaorticdissectionorrupture. OpenrepairisrequiredforproximalTAAs.Iftheascendingaortaaloneisenlarged, asimpleinterpositiontubegraftshouldsuffice(Figure3).Iftheaorticrootis enlarged,traditionalsurgeryrequiredsacrificingtheaorticvalve,withresectionofthe rootandreplacementoftherootandvalvecollectivelywitha"compositeaorticgraft," alsocalledtheBentallprocedure(Figure4).However,inthemodernera,iftheaortic valveleafletsarehealthy,thenativevalvecanbepreservedandresuspendedwithin theprosthetictubegraft,inwhatisknownasvalvesparingaorticrootrepair,also calledtheDavidprocedure(Figure5). Whentheaorticarchisdilated,atotalarchreplacementisusuallyrequired.Most often,thisisperformedusingamultilimbedprostheticarchgraft,towhicheacharch vesselisanastomosedindividually(Figure6).Themajorriskofarchrepairisbrain injury,eitherfromanoxiaorembolizationofdebris.Methodsofcerebralprotection includedeephypothermiccirculatoryarrest,retrogradecerebralperfusion,or antegradecerebralperfusion.Mostsurgeonsnowuseantegradecerebralperfusion viacannulationoftherightaxillaryartery,9 whichhasbeenassociatedwith significantlyimprovedoutcomes.Morerecently,forveryhighriskpatientswitharch aneurysms,ahybridprocedurehasbeenintroducedinwhichthearchis debranchedbybypassingthebrachiocephalicarteriesusingatrifurcatedtrunkfrom theproximalascendingaorta,andthenanendovascularstentgraftisdeployed acrossthearchaneurysm.10 Aneurysmsofthedescendingthoracicaortahavetraditionallybeenrepairedwith opensurgeryviaaleftthoracotomy(Figure7).However,theprocedureisassociated withsignificantmorbidityandamortalityrateofapproximately10%.Themostfeared complicationispostoperativeparaplegiasecondarytoimpairmentofthearterial bloodsupplytothespinalcord,withanincidenceofapproximately5%inhigh volumecenters. Anumberoftechniqueshavebeenintroducedtoreducetheriskofischemicspinal cordinjury,includingcerebrospinalfluiddrainage,epiduralcooling,the reimplantationofpatentcriticalintercostalarteries,theuseofintraoperative somatosensoryormotorevokedpotentialmonitoring,atriofemoral(i.e.,leftatrialto femoral)bypasstomaintaindistalaorticperfusionduringsurgery,andthe maintenanceofsufficientlyhighsystemicarterialpressureduringthefirstseveral

daysaftersurgery. Inrecentyears,thoracicendovascularaorticrepair(TEVAR)withstentgraftinghas emergedasapromisingalternativetoopensurgicalrepairforaneurysmsofthe descendingthoracicaorta(Figure8).TheTEVARprocedureisfarlessinvasiveand isassociatedwithalowerpostoperativemorbiditythanopenrepair.Atpresent,there arenorandomizedprospectivetrialscomparingTEVARtoopenrepair. InamulticenterprospectivenonrandomizedphaseIIstudyoftheGoreTAGgraft, TEVARimplantationwassuccessfulin98%ofpatients,andthe30dayeventrates were3%forparaplegiaand2%fordeath,whichweresignificantlybetterthanthe respectiveeventratesinanopensurgicalcontrolpopulation.11At5years, aneurysmrelatedmortalitywaslowerfortheTEVARpatientsthanforopencontrols, at3%versus12%,respectively.12Yet,despitethisadvantage,therewasno differenceinallcausemortalityat5years,likelyreflectingthefactthattheTEVAR patientpopulationstendtobeolderandhavenumerouscomorbidities. CandidacyforTEVARrequiressuitableaorticanatomy,includingfavorableproximal anddistallandingzones.Asisthecasewithstentgraftingoftheabdominalaorta, evenaftersuccessfuldeploymentofendovascularstentgraftsinthethoracicaorta, patientsmaybeleftwithendoleaks.Theaverageprevalenceofendoleaksat30 daysis10%,withtypeIendoleaksoccurringmostcommonlywithTEVAR,reflecting thechallengesofachievinganoptimalsealattheproximalattachmentsite.13 TEVARpatientsrequireannualsurveillanceimagingwithCTAtomonitorfor endoleaks,toconfirmthattheaneurysmsacisnotexpanding,andtoassessboth stentgraftintegrityandresidualthoracicaorticanatomy. BetablockershavelongbeenthemainstayofmedicaltherapyforTAAs.Beta blockershavebeenshowntosignificantlyreducetherateofaorticgrowthinpatients withMarfansyndrome,althoughtheirbenefitintreatinganeurysmsofother etiologieshasnotbeenproven.Asnotedpreviously,losartanhasbeenshownto reducetherateofaneurysmgrowthinamurinemodelofMarfansyndrome.Thereis anongoingmulticenterrandomizedprospectiveclinicaltrialcomparingtheuseof betablockersversuslosartaninyoungpatientswithMarfansyndrome.Therealso existlimitedtrialdatatosuggestthatangiotensinconvertingenzymeinhibitorsmay alsoslowtherateofaorticgrowthinpatientswithMarfansyndrome.Whichever agentisprescribed,thegoalofmedicaltherapyisasystolicbloodpressureinthe lownormalrange(e.g.,110120mmHg). Anotherelementofmedicaltherapyissurveillanceimagingtomonitorforaortic growth.Inmostcases,theimagingshouldberepeatedannually,andwhenthe aorticdiameternearsthethresholdforintervention,patientsshouldbereferredtoan appropriateaorticspecialistforevaluation.

EtiologyofThoracicAorticAneurysms Table3

RecommendedSizeThresholdsforElectiveThoracicAorticRepairinAsymptomaticPatients Table4 AdaptedwithpermissionfromHiratzkaLF,BakrisGL,BeckmanJA,etal.2010ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVMGuidelines forthediagnosisandmanagementofpatientswiththoracicaorticdisease.AReportoftheAmericanCollegeofCardiologyFoundation/American HeartAssociationTaskForceonPracticeGuidelines,AmericanAssociationforThoracicSurgery,AmericanCollegeofRadiology,American StrokeAssociation,SocietyofCardiovascularAnesthesiologists,SocietyforCardiovascularAngiographyandInterventions,Societyof InterventionalRadiology,SocietyofThoracicSurgeons,andSocietyforVascularMedicine.JAmCollCardiol201055:e27e129.

InterpositionGrafttoRepairanAneurysmoftheAscendingThoracicAorta Figure3 MassachusettsGeneralHospitalThoracicAorticCenter,usedwithpermission.

CompositeAorticGrafttoRepairanAneurysmoftheAorticRoot Figure4 MassachusettsGeneralHospitalThoracicAorticCenter,usedwithpermission.

ValveSparingAorticRootRepairforaRootAneurysm Figure5 MassachusettsGeneralHospitalThoracicAorticCenter,usedwithpermission.

TotalArchReplacementforAorticArchAneurysm Figure6 MassachusettsGeneralHospitalThoracicAorticCenter,usedwithpermission.

OpenSurgicalRepairofaDescendingThoracicAorticAneurysm Figure7 MassachusettsGeneralHospitalThoracicAorticCenter,usedwithpermission.

EndovascularStentGraftingofaDescendingThoracicAorticAneurysm(TEVAR) Figure8 MassachusettsGeneralHospitalThoracicAorticCenter,usedwithpermission.

KeyPoints
MultiplelargeprospectivetrialshavedemonstratedthatscreeningatriskpopulationsforAAAsiscosteffective, andtheUSPreventiveServicesTaskForcesupportsscreeningbyultrasonographyforAAAsamongmen6575 yearsoldwhoarecurrentorformersmokers. ElectiverepairofasymptomaticAAAsisrecommendedatadiameterof5.5cm,althoughingoodsurgical candidatesandwomen,manyexpertslowerthatthresholdto5.0cm. Multipleprospectiverandomizedtrialshaveshownsignificantlowerearlymortalityamongthosetreatedwith EVARcomparedwithopensurgeryrepair,yetsuchtrialshavefoundnosignificantdifferenceinlongertermtotal mortality. InpatientswithMarfansyndrome,theabnormalelastinleadstoexcessiveTGFsignalingthat,inturn,leadsto elasticfiberfragmentationandprogressiveaorticrootenlargement. BAVisanimportantriskfactorforaorticrootandascendingaorticaneurysms. Marfansyndrome,BAV,andidiopathicthoracicaorticaneurysmscanbeheritablethus,firstdegreerelativesof affectedindividualsshouldbescreenedforthoracicaorticdiseaseusingdiagnosticimagingstudies. Traditionalsurgeryforreplacingadilatedaorticrootrequiredsacrificingtheaorticvalve,withresectionoftheroot andreplacingtherootandvalvecollectivelywitha"compositeaorticgraft,"whichisalsoknownastheBentall procedure. Inthemoderneraofaorticrootsurgery,iftheaorticvalveleafletsarehealthy,thevalveispreservedand resuspendedwithinaprosthetictubegraftinwhatisknownasa"valvesparingaorticrootrepair,"ortheDavid procedure.

SuggestedReading
1. GreenhalghRM,BrownLC,PowellJT,andtheUnitedKingdomEVARTrialInvestigators.Endovascularversus openrepairofabdominalaorticaneurysm.NEnglJMed2010362:186371. 2. ChaikofEL,BrewsterDC,DalmanRL,etal.Thecareofpatientswithanabdominalaorticaneurysm:theSociety forVascularSurgerypracticeguidelines.JVascSurg200950(4Suppl):S2S49. 3. HiratzkaLF,BakrisGL,BeckmanJA,etal.2010ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVMGuidelinesfor thediagnosisandmanagementofpatientswiththoracicaorticdisease.AReportoftheAmericanCollegeof CardiologyFoundation/AmericanHeartAssociationTaskForceonPracticeGuidelines,AmericanAssociationfor ThoracicSurgery,AmericanCollegeofRadiology,AmericanStrokeAssociation,SocietyofCardiovascular Anesthesiologists,SocietyforCardiovascularAngiographyandInterventions,SocietyofInterventionalRadiology, SocietyofThoracicSurgeons,andSocietyforVascularMedicine.JAmCollCardiol201055:e27e129. 4. CoadyMA,IkonomidisJS,CheungAT,etal.Surgicalmanagementofdescendingthoracicaorticdisease:open andendovascularapproaches:ascientificstatementfromtheAmericanHeartAssociation.Circulation 2010121:2780804. 5. HabashiJP,JudgeDP,HolmTM,etal.Losartan,anAT1antagonist,preventsaorticaneurysminamousemodel ofMarfansyndrome.Science2006312:11721.

References
1. TwineCP,WilliamsIM.Systematicreviewandmetaanalysisoftheeffectsofstatintherapyonabdominalaortic aneurysms.BrJSurg201198:34653. 2. USPreventiveServicesTaskForce.Screeningforabdominalaorticaneurysm:recommendationstatement.Ann InternMed2005142:198202. 3. BrownLC,PowellJT.Riskfactorsforaneurysmruptureinpatientskeptunderultrasoundsurveillance.AnnSurg 1999230:28996. 4. BrewsterDC,CronenwettJL,HallettJWJr,etal.Guidelinesforthetreatmentofabdominalaorticaneurysms. ReportofasubcommitteeoftheJointCounciloftheAmericanAssociationforVascularSurgeryandSocietyfor VascularSurgery.JVascSurg200337:110617. 5. GreenhalghRM,BrownLC,KwongGP,etal.Comparisonofendovascularaneurysmrepairwithopenrepairin patientswithabdominalaorticaneurysm(EVARtrial1),30dayoperativemortalityresults:randomisedcontrolled trial.Lancet2004364:8438. 6. PrinssenM,VerhoevenEL,ButhJ,etal.,onbehalfoftheDutchRandomizedEndovascularAneurysm Management(DREAM)TrialGroup.Arandomizedtrialcomparingconventionalandendovascularrepairof abdominalaorticaneurysms.NEnglJMed2004351:160718. 7. HabashiJP,JudgeDP,HolmTM,etal.Losartan,anAT1antagonist,preventsaorticaneurysminamousemodel ofMarfansyndrome.Science2006312:11721. 8. HiratzkaLF,BakrisGL,BeckmanJA,etal.2010ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVMGuidelinesfor thediagnosisandmanagementofpatientswiththoracicaorticdisease.AReportoftheAmericanCollegeof CardiologyFoundation/AmericanHeartAssociationTaskForceonPracticeGuidelines,AmericanAssociationfor ThoracicSurgery,AmericanCollegeofRadiology,AmericanStrokeAssociation,SocietyofCardiovascular Anesthesiologists,SocietyforCardiovascularAngiographyandInterventions,SocietyofInterventionalRadiology, SocietyofThoracicSurgeons,andSocietyforVascularMedicine.JAmCollCardiol201055:e27e129. 9. KulikA,CastnerCF,KouchoukosNT.Outcomesaftertotalaorticarchreplacementwithrightaxillaryartery cannulationandapresewnmultibranchedgraft.AnnThoracSurg201192:88997. 10. MilewskiRK,SzetoWY,PochettinoA,MoserGW,MoellerP,BavariaJE.Havehybridproceduresreplacedopen aorticarchreconstructioninhighriskpatients?Acomparativestudyofelectiveopenarchdebranchingwith endovascularstentgraftplacementandconventionalelectiveopentotalanddistalaorticarchreconstruction.J ThoracCardiovascSurg2010140:5907. 11. MakarounMS,DillavouED,KeeST,etal.Endovasculartreatmentofthoracicaorticaneurysms:resultsofthe phaseIImulticentertrialoftheGORETAGthoracicendoprosthesis.JVascSurg200541:19. 12. MakarounMS,DillavouED,WheatleyGH,CambriaRP,andtheGoreTAGInvestigators.Fiveyearresultsof endovasculartreatmentwiththeGoreTAGdevicecomparedwithopenrepairofthoracicaorticaneurysms.JVasc Surg200847:9128. 13. RicottaJJII.Endoleakmanagementandpostoperativesurveillancefollowingendovascularrepairofthoracic aorticaneurysms.JVascSurg201052:91S9S.

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8.2:AcuteAorticSyndromes
Author(s): BradleyA.Maron,MD PatrickT.OGara,MD,FACC

LearnerObjectives
Uponcompletionofthismodule,thereaderwillbeableto: 1. Defineeachoftheacuteaorticsyndromes. 2. Identifyriskfactorsassociatedwiththedevelopmentofacuteaorticdissectionandaorticrupture. 3. Recognizestrengthsofandlimitationstocontemporaryimagingmodalitiesavailablefordiagnosingacuteaortic syndromes. 4. Recommendappropriatemedical,endovascular,orsurgicaltreatmentforpatientswithacuteaorticsyndromes. 5. Recommendappropriateposthospitaldischargecareforacuteaorticsyndromepatients.

Introduction
Theacuteaorticsyndromesencompassaconstellationofanatomicandpathophysiologicalchangestothenormal structureandfunctionoftheaorticwall,whichthreatencentralaorticpressure,vitalorganperfusion,andsurvival.These includeaorticdissection,intramuralhematoma(IMH),penetratingaorticulcer(PAU),rapidaneurysmexpansion,and traumainducedaorticrupture.1 Recentlydescribedgenetic,epidemiological,andclinicaladvancesprovideclinicianswithagreaterrangeoftoolswith whichtoidentifyatriskpatientsanddiagnoseacuteaorticsyndromesearlierinthenaturalhistoryofthesediseases. Promptdeliveryofappropriatemedical,endovascular,and/orsurgicaltherapyisacriticaldeterminateofoutcomeinthis patientpopulation.

Classification
Theacuteaorticsyndromesaredefinedaccordingtotheiranatomicallocation (Table1).Acuteaorticdissectionismostcommonlydescribedaccordingtothe DeBakey2 orStanford3 classificationschemes(Figure1).IntheDeBakeysystem,a typeIdissectionoriginateswithintheascendingaortaandextendsbeyondtheorigin oftheinnominateartery.ThisisincontrasttoatypeIIdissection,whichisconfined totheascendingaorta,oratypeIIIdissectionthatinitiatesdistaltotheoriginofthe leftsubclavianartery. TheStanfordclassificationmodel,whichwillbereferencedintheremainderofthis module,dividesdissectionsintotwotypes:involvementoftheascendingaorta constitutesatypeAdissection,whereasadissectionthatdoesnotinvolvethe ascendingaortaisatypeBdissection.Inthisschema,archdissectionsarenot strictlydefined,butaremostoftenaggregatedwiththemostaffectedportionof contiguousaorta.

Table1

Figure1

TheAcuteAorticSyndromes Table1

AorticDissectionTypeAccordingtotheDeBakeyandStanfordClassificationSystems Figure1 ReproducedwithpermissionfromNienaberCA,EagleKA.Aorticdissection:newfrontiersindiagnosisandmanagement:PartI:frometiologyto diagnosticstrategies.Circulation2003108:62835.

Epidemiology
Decliningautopsyrates,misdiagnosis,andanunknowncontributionofacuteaorticsyndromestoprehospitalsudden deatharefactorsthatobscurethetrueincidenceoftheseevents.1,4Dataderivedfromlargeinternationalregistries estimatethattheincidenceofaorticaneurysmanddissectioninthegeneralpopulationis16.3and9.1per100,000men andwomen,respectively,withameanageof63years.5,6 Importantly,diseaseratesvary(greatly)inaccordancetothesamplepopulation.Forexample,patientswithMarfan syndrome(MFS),whichafflicts1in5,000individuals,aresignificantlymorelikelytodevelopanaorticdissection comparedtothegeneralpopulation(seethenextsectiononPredisposingRiskFactors).IMHisbelievedtoaccountfor upto13%ofacuteaorticsyndromes.5,6AorticrupturemayoccurasacomplicationoftypeAdissection,butisrare outsidethesettingoftrauma.Bysomeestimates,aorticruptureaccountsfor20%ofmotorvehiclecollisionrelated fatalities.

PredisposingRiskFactors
Thelikelihoodofdevelopinganacuteaorticsyndromeisinfluencedbygenetic and/oracquiredriskfactorsthatweakenthemediallayeroftheaorticwall(Table2). "Cysticmedialdegeneration"describesthenoninflammatorylossofelasticfibersin theaorticmediaduetotheaccumulationofmucopolysaccharideandvascular smoothmusclecelldegeneration.Cysticchangesareinfactnotcommonlypresent. Thesemaladaptivechangestothecellulararchitectureoftheaortaareobservedin acuteaorticdissectionduetoawidevarietyofetiologiesthus,thepresenceof medialdegenerationisnotpathognomonicforanysinglecauseofaorticdissection. GeneticRiskFactors MarfanSyndrome Numerousgeneticetiologiesareimplicatedinthepathophysiologyofacuteaortic syndromes.MarfanSyndromeisthemostcommongeneticcauseofacuteaortic dissectionandoccursowingtomutationsinthegeneencodingforfibrillin1(FBN 1),whichisamajorcomponentofmicrofibrilsthatformastructuresupporting sheatharoundelastin.Dysfunctionalmicrofibrilsresultinthelossofintercellular adhesionsand,ultimately,inacardiovascularsyndromethatincludesaneurysmof theaorta(mostoftenaffectingtherootandsinusesofValsalva),dilationofthe proximalmainpulmonaryartery,myxomatousthickeningoftheatrioventricular valves,mitralvalveprolapse,andmitralannularcalcification.7 ThediagnosisofMFSisestablishedprimarilyonclinicalgrounds.Inthesettingofa familyhistoryofMFS,thepresenceofectopialentisoraorticdilatation(z score2for thoseolderthan20yearsofage)issufficienttosecurethediagnosis.8 Sinceupto onethirdofcasespresentinpatientswithoutanaffectedparent,7 strategiesto diagnoseMFSexistthatrelyonclinicalexaminationfindings,imaging,and moleculartesting(Table3). Furthermore,MFSvariantsthataffectprimarilythecardiovascularsystem(i.e.,forme frustes)havebeendescribed,andareimportanttorecognizeclinicallyduetotheir associationwithaorticaneurysmand/ordissection.TheseincludetheMASS phenotype(myopia,mitralvalveprolapse,nonprogressiveaorticrootdilation, skeletalabnormalities,andstriae)andfamilialthoracicaorticaneurysmdisease (FTAAD).FTAADhasalsobeenmappedtomutationsinMYH11andACTA2.911 EhlersDanlosSyndrome VascularEhlersDanlosSyndrome(EDS)(i.e.,EDStypeIV)isarareautosomal dominantdisorderthatoccursduetoamutationintheCOL3A1geneencodingfor typeIIIprocollagensynthesis.Notably,uptoonehalfofcasesarenotinheritedand arebelievedtobesporadic.12InvascularEDS,aorticaneurysm,dissection,and rupturehavebeenreported,andmortalityratesareelevatedsignificantlycompared tothenormalpopulation,withcumulativesurvivalofapproximately50%atage40.13 BicuspidAorticValveDiseaseandOtherGeneticRiskFactors Bicuspidaorticvalve(BAV)diseaseisthemostcommoncongenitalheartdefect affecting12%ofthegeneralpopulation,andispresentinamaletofemaleratioof 3:1.14Comparedtothegeneralpopulation,affectedindividualsdemonstratea relativeriskforaorticdissectionandaorticaneurysmof8.5and86.2,respectively.15 AlthoughthemechanismbywhichtoaccountfortheaortopathyofBAVis unresolved,disruptedlaminarflowwithinthepostvalvularsegmentoftheproximal aortaisassociatedwithmedialdegeneration,smoothmusclecelldisarray,and fragmentationofelastin.14Othergeneticsyndromesassociatedwithanincreased rateofaorticaneurysm/dissectionincludeaorticcoarctation,Noonansyndrome, Turnersyndrome,andpolycystickidneydisease. AcquiredRiskFactors

Table2

Table3

Systemichypertensionandtobaccousearethemostcommonacquired(i.e., modifiable)riskfactorsforaorticdissection.Recreationaluseofcocaineand methamphetaminearerecognizedincreasinglyasstimulatorsofaorticvascular injury.Otheracquiredriskfactorsforaorticdissectionincludeinflammatory diseasesofthelargearteriessuchasTakayasudisease,giantcellaortitis,Behhet disease,relapsingpolychondritis,systemiclupuserythematosus,and,rarely, inflammatoryboweldiseaseassociatedvasculitis.16However,vasculopathies characterizedbysignificantreplacementfibrosisandsubsequentscarringofthe bloodvesselwall,asoccursinresponsetoTreponemapalliduminfection(i.e., syphilis),arenotassociatedwithincreasedriskforaorticdissection. Changesincirculatingbloodvolume,increasedlevelsofthevasculareffectors relaxinandestrogen,Valsalvamaneuvermediatedincreasesinintrathoracic pressureduringlabor,andundiagnosedMFSorotherconnectivetissuediseases areproposingfactorsforaorticdissectionduringpregnancy(whichalsoincludes theearlypostpartumphase).Althoughtheseeventsarerareandmostcommonly occurinthecontextofapreviouslyunrecognizedconnectivetissueabnormality,upto 50%ofalldissectionsinwomenyoungerthan40yearsofageoccurinthe peripartumperiod.17 Iatrogeniccausesofaorticdissectionaccountforupto5%ofallevents,andoccur mostcommonlyduringcardiacsurgeryorcatheterization.Peripheralvascular disease,tobaccouse,ahistoryofsystemichypertension,andthepreprocedural presenceofanatheroscleroticplaqueorPAUareassociatedwithincreasedriskof iatrogenicdissection.18

GeneticandAcquiredRiskFactorsforAcuteAorticSyndromes Table2

SelectedCriteriafortheDiagnosisofMarfanSyndrome Table3 AdaptedwithpermissionfromLoeysBL,DietzHC,BravermanAC,etal.TherevisedGhentnosologyfortheMarfansyndrome.JMedGenet 201047:47685.

Pathophysiology
AorticDissection Thereisapositive,sigmoidrelationshipbetweenthoracicaorticdiameterand probabilityofaorticdissection/rupturewithasteepupwardinflectionpointat6cm (Figure2).19Nevertheless,dissectionwilloftenoccuratmuchsmalleraortic diameters.20 In8090%ofcasesofacutesyndromes,anintimaltearisidentifiedatautopsyor throughadvancedimagingoftheaorta,andismostlikelytooccuratpointsofhigh shearormechanicalstress,suchaswithinafewcentimetersoftheaorticvalvefor typeAdissectionsandjustdistaltotheinsertionoftheligamentumarteriosumfor typeBdissections.16Propagationofthedissectinghematomamayoccur anterogradeorretrograde,resultingininvolvementofboththeascendingand descendingaorta.Reentrysitesmaybemultiple. Thecoagulationstatusofbloodcontainedwithinthefalselumenofadissection influencesthenaturalhistoryofthedisease.Generally,completethrombosisofthe falselumenisafavorablefinding,asitisindicativeofa"sealedoff"dissection.In contrast,partialthrombosisofthefalselumenpredictsfuturedissectionassociated complications,possiblyduetoincreasedcompressionofthetruelumenbythefalse lumen,orowingtosustainedinflammationmediatedinjurytotheaorticwall. Tsaiandcolleaguesshowedthatpartialfalselumenthrombosiswasassociated withreducedlongtermsurvivalamongpatientswithtypeBaorticdissectionand proposedthathemodynamicforceswithinthefalselumenleadultimatelyto progressiveaneurysmformationand/orrupture.21Bloodwithinthefalselumenthat isfreeflowingindicatescommunicationbetweenthefalseandtruelumen.Patency ofthefalselumeniscommonamongpatientswithMFSandaorticdissection,and couldbeariskfactorforlatecomplications. Clinicalmanifestationsofaorticdissectionareintimatelyrelatedtothesiteofentry, courseofthepropagatinghematoma,andbranchvesselcompromise.The dissectionplaneofatypeAdissectionmaypropagateretrogradeacrosstheorigin ofthecoronaryarteries,particularlytherightcoronaryartery,causingmyocardial ischemia,orinvolvetheaorticannuluswithresultantvalvularregurgitation.Rupture intothepericardialspacecanresultintamponadeandearlydeath.Endorgan malperfusionsyndromesduetoaorticbranchvesselcompromiseequatewith increasedmorbidityandmortality.16 AorticIntramuralHematoma "Aorticintramuralhematoma"isdefinedasacollectionofbloodwithinthemediaof theaortaintheabsenceofdissectionflaporadetectableentrytear(Figure3).16Itis thoughttooccurasaresultofspontaneousbleedingwithinthewalloftheaortafrom damagedvasavasorum.DiagnosisofIMHismostoftenaccomplishedusing transesophagealechocardiography(TEE),computedtomographicangiography (CTA),ormagneticresonanceangiography(MRA),withdemonstrationofpartialor circumferentialwallthickeningorthepresenceofafreshhematomainthevessel wall.CTAoutperformsTEEasadiagnosticimagingmodality,andacquisitionofan initialnoncontrastimageismostinformative. ThenaturalhistoryofIMHisvariable:spontaneousresorptionmayoccurinupto onethirdofcases,althoughifpresentintheascendingaorta,adiameterof>11mm ispredictiveofdissectioncomplications,death,andtheneedforsurgical repair.16,22,23Theincidenceratesofaorticregurgitationandpulsedeficitsarelower withIMHcomparedwithdissection.Overtime,approximately10%ofpatientswith IMHwilldevelopaclassicdissectionand5060%willdeveloptrueorfalseaortic aneurysms.24 PenetratingAtheroscleroticAorticUlcer Theperturbationofstableaorticatheroscleroticplaquesfrominflammationorshear
Figure2

Figure3

Figure4

stressmayresultinsubsequenterosionacrosstheinternalelasticmembraneof theaorta,allowingfortheformationofabloodfilledfalsespacewithinthewallofthe aorta(Figure4).PAUdepth>1.0cm,diameter>2.0cm,andlocationintheproximal aspectofthedescendingaortaareassociatedwithworseoutcomes.For uncomplicatedlesions,serialimagingandaconservativemedicalstrategyare recommended(seesectiononClinicalManagementofAorticDissection). AcuteAneurysmExpansion Acuteaneurysmexpansionisanunderrecognizedcauseofseverechestpainand portendsimpendingaorticrupture.Acuteaneurysmexpansionhasbeendescribed asacomplicationofmostaortopathies,includingaortitis,aorticdilationduetoMFS, andatheroscleroticaorticdisease.Forthesepatients,promptsurgicalevaluationis indicated.

InfluenceofAorticSizeonCumulative,LifetimeIncidenceofNaturalComplicationsofAorticAneurysm Figure2 Aninflectionpointintheriskofaneurysmcomplications(i.e.,dissection,rupture)isobservedinnonMarfansyndromepatientsatanaortic diameter>6cm(bluearrow). ReproducedwithpermissionfromElefteriadesJA.Naturalhistoryofthoracicaorticaneurysms:indicationsforsurgery,andsurgicalversus nonsurgicalrisks.AnnThoracSurg200274:S187780.

AorticIntramuralHematomaCapturedonComputedTomographicAngiogram Figure3 Contrastnonenhancingcircumferentialregion(arrows)oftheaortarepresentsanaorticintramuralhematoma. ReproducedwithpermissionfromTakahashiK,StanfordW.MultidetectorCTofthethoracicaorta.IntJCardiovascImaging200521:14153.

PenetratingAtheroscleroticUlcerCapturedonTransesophagealEchocardiography Figure4 *penetratingatheroscleroticulcer ReproducedwithpermissionfromFirschkeC,OrbanM,AndrssyP,LangeR,SchmigA.Imagesincardiovascularmedicine.Penetrating atheroscleroticulceroftheaorticarch.Circulation2003108:e145.

ClinicalPresentation
History Alowclinicalindexofsuspicionforaorticdissectionisnecessarytoavoid misdiagnosis.Pointofcareclinicalpredictionscaleshavebeenrecentlyvalidatedto provideasimpleanduniformstrategyfordeterminingthelikelihoodofdissectionat thepatient'sbedside(Table4).25 Chestpainisthemostcommonpresentingsymptomamongpatientswithacute aorticdissection(Table5).Thequalityofchestpaintendstobedescribedby patientsassevere,ripping,ortearing.Radiationofdiscomfortanteriorlyis suggestiveoftypeAaorticdissection,whereasradiationofdiscomforttothelower backorabdominalregionissuggestiveoftypeBdissection. Syncopeisaparticularlyconcerningsymptomofaorticdissection,andshouldraise immediateconcernforcerebralmalperfusionorcardiactamponade.Othermajor symptomsthatareassociatedwithworseoutcomeinaorticdissectioninclude abdominalpainandparaplegia,whichmayoccurinthepresenceofimpairedblood flowtothespinalcord. PhysicalExamination Patientswithacuteaorticdissectionoftenpresentacutelyill.Hypertensionmaybe observedineithertypeAortypeBaorticdissection.Ifpresent,anaorticregurgitation murmurisoftenfaint,shortinduration,andlowinpitch.Identifyingpulsedeficitsis critical,asthispredictsmortalityinacuteaorticdissection.26Patientsshouldalso beevaluatedforclinicalsignsofcardiactamponadesuchaspulsusparadoxusand elevationofthejugularvenouspressure.Transudativepleuraleffusions(left>right) arereportedinacutetypeBaorticdissection,andshouldbeconsideredinpatients forwhomdullnesstopercussionordecreasedbreathsoundsareobservedon clinicalexamination.
Table4

Table5

ThePercentageofPatientsintheInternationalRegistryofAcuteAorticDissection(IRAD)(19962009)WithEachof12HighRiskClinicalMarkers ObservedattheTimeofPresentationWithAcuteAorticDissection Table4 SBP=systolicbloodpressure. Theaorticdissectiondetection(ADD)scoreaimstoenhanceearlydiagnosisofacuteaorticdissection.TheADDscoreiscalculatedby determiningthenumberofcategoriesinwhichanyof12highriskclinicalfeaturesarepresentinpatientswithsymptomssuggestiveofacute aorticdissection.Forexample,inapatientwithafamilyhistoryofaorticdisease(category1)andknownthoracicaneurysm(alsocategory1), theADDscorewouldbe1.Likewise,theADDscoreis2inapatientwithMarfansyndrome(category1)andabloodpressuredifferential (category3).AretrospectiveanalysisoftheInternationalRegistryofAcuteAorticDissectiondeterminedthatamong2,538patientswithacute aorticdissection,95.7%hadanADDscore1.TheADDscalemaythereforeprovidetheclinicianwithasimpleandeffectivebedsidemethodto informfurtherdiagnostictestingand/ortreatmentinpatientswithsuspectedaorticdissection.Importantly,thenegativepredictivevalueforacute aorticdissectioninpatientswithanADDscoreof0hasnotyetbeenestablished. TableadaptedwithpermissionfromRogersAM,HermannLK,BooherAM,etal.Sensitivityoftheaorticdissectiondetectionriskscore,anovel guidelinebasedtoolforidentificationofacuteaorticdissectionatinitialpresentation:resultsfromtheInternationalRegistryofAcuteAortic Dissection.Circulation2011123:22138. LegendreproducedwithpermissionfromMaronBA,O'GaraPT.Pathophysiology,clinicalevaluation,andmedicalmanagementofaortic dissection.In:Creager,Beckman,Loscalzo,eds.VascularMedicine:ACompaniontoBraunwald'sHeartDisease.2nded.Inpress.

TheAverageSensitivityofVariousClinicalFeaturesReportedinPatientsWithAcuteThoracicAorticDissection Table5 Dataarederivedfromametaanalysisinvolving16differentstudiesand1,553patients. AdaptedwithpermissionfromKlompasM.Doesthispatienthaveanacutethoracicaorticdissection?JAMA2002287:226272.

Biomarkers
AlowDdimer(<500ng/ml)discoveredatthetimeofpresentationsuggestsagainst acuteaorticdissection.Conversely,elevatedcirculatinglevelsofsmoothmuscle myosinheavychainprotein(>2.5g/L)raisesclinicalindexofsuspicionforacute typeAaorticdissection.Theroleoftheseandotherbiomarkers,suchasCreactive proteinandsolubleelastinfragmentforaorticdissectiondiagnosisorpatientrisk stratification,continuestoevolve(Figure5).

Figure5

CirculatingLevelofAorticBiomarkersAfterAcuteDissection Figure5 ReproducedwithpermissionfromTrimarchiS,SangiorgiG,SangX,etal.Insearchofbloodtestsforthoracicaorticdiseases.AnnThoracSurg 201090:173542.

DiagnosticImaging
Thechestroentgenographisabnormalinthemajorityofaorticdissectionpatients, butaloneisnotsufficientforestablishinganaccuratediagnosis.Commonfindings includewideningofthemediastinumandangulationoftheaorticborder.Similarly, theelectrocardiogram(ECG)isoftenabnormal,butnondiagnostic.Importantly, althoughpresentin<4%ofpatients,thepresenceofnewQwavesorSTsegment elevationmustbeinterpretedwithcautiontoavoiderroneousfibrinolytic/reperfusion therapy. TransesophagealEchocardiography TEEisaneffective,easilyassembledimagingmodalityfordiagnosingaortic dissection(Figure6).Whencombinedwithsurfaceechocardiography,thesensitivity andspecificityofthesetestsapproaches99%and89%,respectively.27 IdentifyingthefalselumenfromthetruelumenmaybedifficultwithBmodeimaging alone,andincasesforwhichthefalselumenislarge,thisdistinctionshouldnotbe madeaccordingtovesseldiameteronly.ContinuouswaveDopplerinterrogation maybeusedinthesecasestomeasurethevelocitytimeintegralofbloodflow withineachlumen,thusprovidinganestimationofintraluminalpressureto distinguishbetweenthetrueandfalselumens.Thedistalascendingaorta,anterior aspectoftheaorticarch,andregionsoftheaortaanteriortothecartilaginous tracheaareoftenobstructedfromviewwithTEE. ComputedTomographicAngiography Thediagnosticaccuracyof64sliceCTAapproaches100%foraorticdissection (Figure7).16,28Contrastenhancementallowsthoroughanatomicalcharacterization ofthedissectionplane,includingdetailedcharacterizationofdistalbranchvessel involvement.AdditionalbenefitsofCTAincluderapidimageacquisitionandthe availabilityofemergencydepartmentscannersatmosthospitals. LimitationstoCTAincludepatientexposuretoradiationandcontrast.Testaccuracy maybeinfluencedadverselybymotionartifact,androutineCTAdoesnotassessleft ventricularoraorticvalvefunction.CTAhasbecomethefirstimagingmodalityof choiceforsuspectedacuteaorticdissectionworldwide. MagneticResonanceImaging MRimaging/angiography(MRA)offerssuperiorspatialresolutionforgeometricand volumetricanalysisofaorticdissections,andenhancessensitivityfordetectingIMH andPAU.Inmostcases,weightedimagingprotocolsalsoallowfordistinction betweenathrombosedversuspatentfalselumen.IncontrasttoCTA,MRAprovides informationonleftventricularandaorticvalvefunction,aswellasquantifyingaortic regurgitation. InvasiveAortography Limiteddiagnosticsensitivity,contrastinducednephropathy,andtheavailabilityof othermoreaccurate,butlessinvasivediagnostictestshaveresultedina substantialdeclineintheapplicationofinvasiveaortographyasafirstlinetestfor thediagnosisofaorticdissection.Thisstrategyremainsanoptioninspecific circumstances,suchastoevaluateaorticdissectioninpatientsmisdirectedtothe cardiaccatheterizationlaboratoryforevaluationofpresumedischemiccoronary disease.Undertheseclinicalcircumstances,intravascularultrasound(IVUS)may alsobeutilized.ThediagnosticaccuracyofIVUSisnearly100%foraorticdissection, andthisstrategyisparticularlyusefulfordefiningcircumferentialextentofdisease aswellaslocatingdissectionentrypoints. Coronaryangiographyisnotindicatedinthegeneralmanagementofunstable patientswithacuteaorticdissection.Ontheotherhand,coronaryangiographyprior toelectiverepairofchronicstabletypeAdissectionsmaybeconsidered,particularly forpatientswithaknownhistoryofischemicheartdisease.16
Figure7

Figure6

TransesophagealEchocardiographicCaptureofAorticDissection Figure6 Anascendingaorticdissectionvisualizedbytransesophagealechocardiographydemonstratesacommunication(arrow)throughthedissection flapthatjoinsthetruelumen(TL)andfalselumen(FL).*,aorticvalveleaflets. ReproducedwithpermissionfromMeredithEL,etal:Echocardiographyintheemergencyassessmentofacuteaorticsyndromes.EurJEcho 10:i31,2009

PlanarComputedTomographicAngiogramandThreeDimensionalReconstructedImagesofStanfordTypeAAorticDissection Figure7 In(A),coronalCTAimagedelineatesthedissectionplanethatseparatesthetruelumen(TL)fromfalselumen(FL).In(B)threedimensional reconstructionimaginginthesamepatientprovidesenhancedspatialresolutionpostsurgicalrepairoftheaorticdissectionandthesurrounding anatomicstructures.Inthiscase,theaorticdissectionextendsfromtheaorticroottotheinnominateandleftsubclavianarteries,continues throughtheaorticarchandintothedescendingaorta,withterminationnearthebifurcationoftheleftcommoniliacartery. ReproducedwithpermissionfromMaronBAandOGaraPT.Pathophysiology,ClinicalEvaluation,andMedicalManagementofAorticDissection. InVascularMedicine:ACompaniontoBraunwald'sHeartDisease(Creager,Beckman,Loscalzo)2ndEd.Inpress.

ClinicalManagementofAorticDissection
Theclinicalmanagementofacuteaorticdissectionisperformedmethodicallyand expeditiously,oftencombiningmedicalandsurgicaltherapies.Theclinical indication(s)fortheuseofendovascularaorticstentgraftsfortypeBdissection continuestoevolve.Oneproposedpathwayalgorithmforthemanagementof patientswithacuteaorticdissectionisprovidedinFigure8. MedicalTherapy Medicaltherapywithintravenous(IV)agentstocontrolheartrateandbloodpressure isthecornerstoneoftheinitialtherapeuticstrategyforpatientswithtypeAortypeB aorticdissection.Exceptionstothisincludepatientswithcardiogenicshockand profoundsystemichypotension,typicallyduetotypeAaorticdissectioncomplicated byaorticruptureorcardiactamponade.Inthiscase,volumeresuscitationis indicated,butfunctionsasatemporizingmeasure.Emergentsurgerywithoutdelay forbedsidepericardiocentesisisadvised. Shortactingadrenergicreceptorantagonistsarepreferredfirstline pharmacotherapeuticagents,asthesedrugsreducetherateofpressure development(i.e.,changeinpressuredividedbychangeintime[dP/dT])by decreasingbothleftventricularcontractilityandheartrate(Table6).Thetargetheart rateintheacutephaseofmanagementis60bpm. Inpatientsrequiringadditionaltherapytoachieveatargetbloodpressureof110mm Hg,theIVadministrationofshortactingdirectvasodilatorsisrecommended,such asnitroprusside,labetalol,enalaprilat,hydralazine,ornicardipine.29Other supportivemeasuresmaybenecessarytoalleviatehypoxemia,patientdiscomfort, andanxiety,whichcollectivelymayexacerbatedissectionpathophysiology. Surgery Theindicationsforsurgicalrepairofacuteandchronicaorticdissectionareoutlined inTable7.
Figure10

Figure8

Table6

Table7

Figure9

EmergencysurgeryisrecommendedforallpatientswithacutetypeAaortic dissection,aswellasforpatientswithtypeAIMH.Thepotentialforaorticarch reconstruction,coronaryarteryreimplantation,aorticvalverepairorreplacement, andbranchvesselrepairisdependentonaorticdissectionanatomyandassessed ineachpatient.SurgicalrepairofchronicstabletypeAaorticdissectionis recommendedinthepresenceofsignificantaorticvalvedysfunction(regurgitation), leftventricularcavitydilation,orleftventricularsystolicdysfunction.Likewise,surgery totreattypeAaorticaneurysmisindicatedforamaximaldimension5.5cmor4.5 cminMFSpatients,oraccelerateddilationatarateof1cm/y.1 Surgerytypicallyconsistsofresectionandreplacementofthedissectedascending aortausingaGelweaveinterpositiongraft,althoughaorticvalveresuspensionor useofacompositevalvegraftconduitwithreimplantationofthecoronaryarteries mayberequired,dependingontheextentofrootinvolvementandthemechanismof anyassociatedaorticregurgitation.Techniquesformanagingcomplexarch dissectionsandbranchvesselinvolvementwithbifurcatedgraftsarebeyondthe scopeofthismodule.Cannulationforcardiopulmonarybypassisusuallyperformed viatherightaxillaryartery,andaperiodofhypothermiccirculatoryarrestmaybe necessaryforcompletionofthedistalanastomosis. SurgeryisalsoindicatedforcomplicatedtypeBaorticdissectiondefinedby refractorypainorhypertension,rapidaneurysmalexpansion,rupture,or malperfusionsyndrome(i.e.,endorganischemia).Dissectionlocationwithina previouslyaneurysmalaorticsegmentisananatomicalindicationforsurgicalrepair. Considerationofaggressivesurgicalrepairmightalsobegiventopatientswith MFS.Surgerytotreatchronicdescendingthoracicaorticaneurysmisindicatedfora maximaldimension5.5cm.Lowersizethresholdsmaybeappropriateforpatients withconnectivetissuedisordersandinthepresenceofacceleratedgrowthatarate of1cm/y.

EndovascularRepair Thoracicendovascularstentgrafting(thoracicendovascularaorticrepair[TEVAR]), percutaneousfenestration,andbranchvesselstentinghavebeenevaluatedin patientswithacute(orchronic)aorticsyndromes(Figure9).TEVARhasgained increasingacceptanceasthetreatmentofchoiceforcomplicatedtypeBdissection, aswellasforanatomicallyappropriateaneurysmdisease,althoughrandomized prospectivetrialdataarelacking. Inobservationalseries,morbidityandmortalityratesarelowerforTEVARcompared withopensurgery,andsimilartothosereportedformedicaltherapyinpatientswith uncomplicatedtypeBdissection(InternationalRegistryofAcuteAorticDissection [IRAD]).Acomprehensivelongtermassessmentofdevicerelatedcomplications, suchasratesofendoleakandstentmigration,isneeded.Itseemsunlikelythata randomizedtrialofTEVARcomparedwithsurgeryforcomplicatedacutetypeB dissectionwillbeperformed. TheINSTEAD(IvestigationofStentGraftsinAorticDissection)trialenrolledpatients withuncomplicated,chronictypeBdissection,andshowednodifferenceinclinical oraorticendpointsoutto2yearsforpatientstreatedwithTEVARversusthose managedmedically(Figure10).30AnatomicalconsiderationsforTEVARare reviewedelsewhere. TheSocietyofThoracicSurgeonshasassignedaClassIArecommendationtothe useofthoracicstentgraftsforrepairofacutecomplicatedtypeBaorticdissection, andaClassICrecommendationforacutetraumatictranssection.31TEVARisalso recommendedformanagementofcomplicatedPAUdiseasenotresponsiveto medicaltherapyorwithsignsofthreatenedrupture.

OneProposedManagementPathwayforAcuteAorticDissection Figure8 Instep1,alowindexofclinicalsuspicionforacuteaorticdissectionshouldpromptearlydiagnostictestingwhilemedicaltherapyisinitiated.Step 2involvesthedeterminationofascendingaorticinvolvement,whichinfluencessignificantlytheimportanceofemergentsurgicalconsultation.In step3,patientswithtypeAaorticdissectionarereferredforsurgeryandpatientswithcomplicatedtypeBaorticdissectionarereferredfor endovasculartherapyorsurgery.PatientswithuncomplicatedtypeBaorticdissectionarecontinuedonmedicaltherapyandmonitoredfor changesinclinicalstatus.Instep4,acareplanisestablishedthatemphasizestheimportanceoflongtermmedicaltherapy,radiologic surveillance,andlifestylemodificationstodecreasetheriskofpostdissectioncomplications.Longtermmedicaltherapyshouldinclude receptorantagonistsandangiotensinreceptorblockersorangiotensinconvertingenzymeinhibitorstoachievearestingheartrate(HR)of60 bpmandbloodpressure(BP)of120/80mmHg,respectively. CTA=computedtomographyangiographyECG=electrocardiogramTEE=transesophagealechocardiographyTEVAR=thoracicendovascular aorticrepair. LegendadaptedwithpermissionfromMaronBA,OGaraPT.Pathophysiology,clinicalevaluation,andmedicalmanagementofaorticdissection. In:CreagerMA,BeckmanJA,LoscalzoJ.VascularMedicine:ACompaniontoBraunwaldsHeartDisease.2nded.Philadelphia:Saunders2012. ImageadaptedwithpermissionfromHiratzkaLF,BakrisGL,BeckmanJA,etal.2010ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelinesforthediagnosisandmanagementofpatientswiththoracicaorticdisease.AReportoftheAmericanCollegeofCardiology Foundation/AmericanHeartAssociationTaskForceonPracticeGuidelines,AmericanAssociationforThoracicSurgery,AmericanCollegeof Radiology,AmericanStrokeAssociation,SocietyofCardiovascularAnesthesiologists,SocietyforCardiovascularAngiographyand Interventions,SocietyofInterventionalRadiology,SocietyofThoracicSurgeons,andSocietyforVascularMedicine.JAmCollCardiol 201055:e27e129.

IntravenousBetaAdrenergicReceptorAntagonistsfortheManagementofAcuteAorticDissection Table6 ReproducedwithpermissionfromMaronBA,OGaraPT.Pathophysiology,clinicalevaluation,andmedicalmanagementofaorticdissection.In: CreagerMA,BeckmanJA,LoscalzoJ.VascularMedicine:ACompaniontoBraunwaldsHeartDisease.2nded.Philadelphia:Saunders2012.

SurgicalIndicationsforAcuteandChronicAorticDissection Table7 AdaptedwithpermissionfromHiratzkaLF,BakrisGL,BeckmanJA,etal.2010ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVMGuidelines forthediagnosisandmanagementofpatientswiththoracicaorticdisease.AReportoftheAmericanCollegeofCardiologyFoundation/American HeartAssociationTaskForceonPracticeGuidelines,AmericanAssociationforThoracicSurgery,AmericanCollegeofRadiology,American StrokeAssociation,SocietyofCardiovascularAnesthesiologists,SocietyforCardiovascularAngiographyandInterventions,Societyof InterventionalRadiology,SocietyofThoracicSurgeons,andSocietyforVascularMedicine.JAmCollCardiol201055:e27e129.

EndovascularStentGraftinTypeBDissection Figure9 CartoondemonstratingthetypicalfeaturesoftypeBdissectionwithflowinboththetrueandtheexpandedfalselumen,resultingfromamajor proximalentrytear(left)planesAtoDwerefolloweduplongitudinallyineverypatient.Astentgraftwasplacedtoscaffoldthedissectedaorta andtosealtheentrytothefalselumen,resultinginreconstructionofthetruelumenwithsubsequentfalselumenthrombosis(right).Levelswere definedas(A)atthesinotubularjunction,(B)atthecenterofthearchbetweentruncusbrachiocephalicusandleftcommoncarotidartery,(C)at thelevelofthemaximumaorticdiameter,and(D)atthehiatus. ReproducedwithpermissionfromNienaberCA,RousseauH,EggebrechtH,etal.RandomizedcomparisonorstrategiesfortypeBaortic dissection:theINvestigationofSTEntGraftsinAorticDissection(INSTEAD)trial.Circulation2009120:251928.

RandomizedComparisonofStrategiesforTypeBAorticDissection Figure10 PanelA:KaplanMeierestimatesof2yearoverallcumulativesurvivalrateinoptimalmedicaltherapy(OMT)versusthoracicendovascularaortic repair(TEVAR)p=0.15bylogranktest.PanelB:KaplanMeierestimatesof2yearaortarelatedsurvivalrateinbothgroupsp=0.44bylog ranktest.PanelC:KaplanMeierestimatesof2yearcumulativefreedomfromcombinedendpointofprogressionandadverseevents.The combinedendpointconsistedofrelateddeath,conversion,andancillaryinterventions(includingasecondstentgraftprocedure,access revision,andperipheralinterventions).Endovascularinterventions(conversiontoTEVARinthecontrolgrouporadditionalTEVARinthestent graftgroup)areanintegralpartofthecombinedendpointofprogressiveaorticpathology.Therewasnodifferencebetweengroups(logrank testp=0.65).Pat.atriskindicatespatientsatrisk. ReproducedwithpermissionfromNienaberCA,RousseauH,EggebrechtH,etal.RandomizedcomparisonorstrategiesfortypeBaortic dissection:theINvestigationofSTEntGraftsinAorticDissection(INSTEAD)trial.Circulation2009120:251928.

PrognosisandRecommendationsforAorticDissectionAftercare
Theestimated1yearsurvivalratefortypeAaorticdissectionis60%,althoughthisiscontingentuponpromptsurgical repair.Hospitalmortalityratesstillapproach25%inmanyseries.The1monthsurvivalrateforuncomplicatedtypeB aorticdissectionis90%.27Manyofthesepatientsareselfselected. FactorsassociatedwithworseprognosisinacutetypeBaorticdissectionincludeadvancedage,malperfusion syndrome,falselumenpartialthrombosis,andsurgicaltreatment.Surgicaloutcomesareworsebecauseofunavoidable adverseselectionbias.Historically,patientswithtypeBdissectionreferredforsurgeryhavehadagreaterburdenof dissectionrelatedcomplicationsandantecedentcomorbidities.Patientswithamaximalthoracicaorticdiameterof4.0 cmandapatentfalselumenhaveanincreasedlikelihoodofdevelopingasubsequentaorticaneurysmaldisease.16,30 Reoperationforlate,longtermaorticcomplicationsisrequiredinuptoonethirdofsurvivors. Routineclinicalevaluationatshorttimeintervalsandaggressivemedicaltherapytomaintainatargetbloodpressureof 130/80mmHgandheartrateof60bpmarecentraltothemanagementofacuteaorticsyndromepatientsfollowing hospitaldischarge.Betareceptorantagonistsarethemainstayoflongtermtherapyotherantihypertensiveagentsare commonlyrequired.ObservationaldatasuggestasalutarysurvivalbenefitofbetareceptorblockersinsurvivorsoftypeA dissectionandforcalciumchannelblockersforsurvivorsoftypeBdissection.32Intheabsenceofacontraindication, statintherapyistypicallyinitiated,owingtothecontributionofatherosclerosistoaorticdissectioninsomepatients. Toassessforpathologicalchangesinaorticanatomy,patientsshouldundergoserialimagingoftheentireaortaat1,3, 6,and12monthsfollowinghospitaldischarge.33Patientsshouldbeadvisedagainstengaginginstrenuousexercise, particularlythosethatinvolvestrainingorliftingofheavyweights.Theseactivitiesareassociatedwithincreasedaortic wallstrainand/ortorsionstress.

KeyPoints
MutationsintheFBN1andCOL3AgenescauseMFSandvascularEDS,respectively,whicharetwokeygenetic riskfactorsforaorticaneurysmand/ordissection. Acquiredriskfactorsforaorticdissectionincludesystemichypertension,tobaccouse,andthedevelopmentof variousautoimmunediseases.Aorticrupturemostcommonlyoccursfollowingamotorvehiclecollision. Severeandabruptonsetofchestpainisthemostcommonsymptominacuteaorticdissection. Rapiddiagnosisofaorticdissectioniscriticaltoafavorableoutcome. TEE,CTA,andMRAareeffectiveimagingmodalitiesfordiagnosisofacuteaorticdissection.CTAhasbecomethe mostwidelyusedinitialtesttodiagnosesuspectedaorticdissection. Aninitialtreatmentstrategythataimstodecreaseaorticwallstrainbycontrollingheartrateandleftventricular contractility(dP/dT)withanIVshortactingadrenergicreceptorantagonistisadvisedinacuteaorticdissection. AcutetypeAaorticdissectionisasurgicalemergency. MedicaltherapyisthepreferredfirstlinetreatmentstrategyforpatientswithuncomplicatedtypeBaortic dissection. SurgeryorTEVARmayberequiredinthemanagementofunstableorcomplicatedacutetypeBaorticdissection, suchasinthosewithmalperfusionsyndromeorearlyexpansion.Inmostcenters,TEVARhasreplacedsurgery forthisindication. Partialthrombosisofthefalselumen,falselumenpatency,increasedage,andmalperfusionsyndromearepoor prognosticsignsinacuteaorticdissection. Serialimagingoftheentireaortaisindicatedinfollowupforaorticdissectionpatientssurvivingtohospital discharge.

References
1. HiratzkaLF,BakrisGL,BeckmanJA,etal.2010ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVMGuidelinesfor thediagnosisandmanagementofpatientswiththoracicaorticdisease.AReportoftheAmericanCollegeof CardiologyFoundation/AmericanHeartAssociationTaskForceonPracticeGuidelines,AmericanAssociationfor ThoracicSurgery,AmericanCollegeofRadiology,AmericanStrokeAssociation,SocietyofCardiovascular Anesthesiologists,SocietyforCardiovascularAngiographyandInterventions,SocietyofInterventionalRadiology, SocietyofThoracicSurgeons,andSocietyforVascularMedicine.JAmCollCardiol201055:e27e129. 2. DailyPO,TruebloodHW,StinsonEB,WuerfleinRD,ShumwayNE.Managementofacuteaorticdissections.Ann ThoracSurg197010:23747. 3. DeBakeyME,BeallACJr,CooleyDA,etal.Dissectinganeurysmsoftheaorta.SurgClinNorthAm196646:1045 55. 4. ChuaM,IbrahimI,NeoX,SorokinV,ShenL,OoiSB.AcuteaorticdissectionintheED:riskfactorsandpredictors formisseddiagnosis.AmJEmergMed2012Feb3:[Epubaheadofprint]. 5. HaganPG,NienaberCA,IsselbacherEM,etal.TheInternationalRegistryofAcuteAorticDissection(IRAD):new insightsintoanolddisease.JAMA2000283:897903. 6. OlssonC,ThelinS,SthleE,EkbomA,GranathF.Thoracicaorticaneurysmanddissection:increasing prevalenceandimprovedoutcomesreportedinanationwidepopulationbasedstudyofmorethan14,000cases from1987to2002.Circulation2006114:26118. 7. KeaneMG,PyeritzRE.MedicalmanagementofMarfansyndrome.Circulation2008117:280213. 8. LoeysBL,DietzHC,BravermanAC,etal.TherevisedGhentnosologyfortheMarfansyndrome.JMedGenet 201047:47685. 9. GuoD,HashamS,KuangSQ,etal.Familialthoracicaorticaneurysmsanddissections:geneticheterogeneity withamajorlocusmappingto5q1314.Circulation2001103:24618. 10. VaughanCJ,CaseyM,HeJ,etal.Identificationofachromosome11q23.2q24locusforfamilialaorticaneurysm disease,ageneticallyheterogeneousdisorder.Circulation2001103:246975. 11. MilewiczDM,RegaladoES,GuoDC.Treatmentguidelinesforthoracicaorticaneurysmsanddissectionsbased ontheunderlyingcausativegene.JThoracCardiovascSurg2010140(6Suppl):S24discussionS4551. 12. LumYW,BrookeBS,BlackJH.ContemporarymanagementofvascularEhlersDanlossyndrome.CurrOpin Cardiol201126:494501. 13. PepinM,SchwarzeU,SupertiFurgaA,ByersPH.ClinicalandgeneticfeaturesofEhlersDanlossyndrometypeIV, thevasculartype.NEnglJMed2000342:67380. 14. SorrellVL,PanczykE,AlpertJS.Anewdisease:bicuspidaorticvalveaortopathysyndrome.AmJMed 2012125:3223. 15. MichelenaHI,KhannaAD,MahoneyD,etal.Incidenceofaorticcomplicationsinpatientswithbicuspidaortic valves.JAMA2011306:110412. 16. MaronBA,O'GaraPT.Pathophysiology,clinicalevaluation,andmedicalmanagementofaorticdissection.In: CreagerMA,BeckmanJA,LoscalzoJ.VascularMedicine:ACompaniontoBraunwald'sHeartDisease.2nded. Philadelphia:Saunders2012. 17. BravermanAC.Acuteaorticdissection:clinicianupdate.Circulation2010122:1848. 18. KetenciB,EncY,OzayB,etal.PerioperativetypeIaorticdissectionduringconventionalcoronaryarterybypass surgery:riskfactorsandmanagement.HeartSurgForum200811:E2316. 19. ElefteriadesJA.Naturalhistoryofthoracicaorticaneurysms:indicationsforsurgery,andsurgicalversus nonsurgicalrisks.AnnThoracSurg200274:S187780. 20. PapeLA,TsaiTT,IsselbacherEM,etal.Aorticdiameter?5.5cmisnotagoodpredictoroftypeAaorticdissection: observationsfromtheInternationalRegistryofAcuteAorticDissection(IRAD).Circulation2007116:11207. 21. TsaiTT,EvangelistaA,NienaberCA,etal.PartialthrombosisofthefalselumeninpatientswithacutetypeB aorticdissection.NEnglJMed2007357:34959. 22. KangDH,SongJK,SongMG,etal.Clinicalandechocardiographicoutcomesofaorticintramuralhemorrhage comparedwithacuteaorticdissection.AmJCardiol199881:2026. 23. NienaberCA,vonKodolitschY,PetersenB,etal.Intramuralhemorrhageofthethoracicaorta.Diagnosticand therapeuticimplications.Circulation199592:146572. 24. EvangelistaA,DominguezR,SebastiaC,etal.Longtermfollowupofaorticintramuralhematoma:predictorsof outcome.Circulation2003108:58389. 25. RogersAM,HermannLK,BooherAM,etal.Sensitivityoftheaorticdissectiondetectionriskscore,anovel guidelinebasedtoolforidentificationofacuteaorticdissectionatinitialpresentation:resultsfromthe internationalregistryofacuteaorticdissection.Circulation2011123:22138. 26. BossoneE,RampoldiV,NienaberCA,etal.Usefulnessofpulsedeficittopredictinhospitalcomplicationsand mortalityinpatientswithacutetypeAaorticdissection.AmJCardiol200289:8515. 27. ErbelR,AlfonsoF,BoileauC,etal.,onbehalfoftheTaskForceonAorticDissection,EuropeanSocietyof Cardiology.Diagnosisandmanagementofaorticdissection.EurHeartJ200122:164281. 28. MacuraKJ,SzarfG,FishmanEK,BluemkeDA.Roleofcomputedtomographyandmagneticresonanceimaging inassessmentofacuteaorticsyndromes.SeminUltrasoundCTMR200324:23254.

29. KimKH,MoonIS,ParkJS,KohYB,AhnH.NicardipinehydrochlorideinjectablephaseIVopenlabelclinicaltrial: studyontheantihypertensiveeffectandsafetyofnicardipineforacuteaorticdissection.JIntMedRes 200230:33745. 30. NienaberCA,RousseauH,EggebrechtH,etal.RandomizedcomparisonorstrategiesfortypeBaortic dissection:theINvestigationofSTEntGraftsinAorticDissection(INSTEAD)trial.Circulation2009120:251928. 31. SvenssonLG,KouchoukosNT,MillerDC,etal.Expertconsensusdocumentonthetreatmentofdescending thoracicaorticdiseaseusingendovascularstentgrafts.AnnThoracSurg200882:S1. 32. SuzukiT,IsselbacherEM,NienaberCA,etal.Typeselectivebenefitsofmedicationsintreatmentofaortic dissection(fromtheInternationalRegistryofAcuteAorticDissection[IRAD]).AmJCardiol2012109:1227. 33. YehCH,ChenMC,WuYC,WangYC,ChuJJ,LinPJ.Riskfactorsfordescendingaorticaneurysmformationin mediumtermfollowupofpatientswithtypeAaorticdissection.Chest2003124:98995.

AdditionalReading
1. JondeauG,DetaintD,TubachF,etal.AorticeventrateintheMarfanpopulation:acohortstudy.Circulation 2012125:22632. 2. BosnerRS,RanasingheAM,LoubaniM,etal.Evidence,lackofevidence,controversy,anddebateintheprovision andperformanceofthesurgeryofacutetypeAaorticdissection.JAmCollCardiol201158:245574. 3. HarrisKM,StraussCE,EagleKA,etal.CorrelatesofdelayedrecognitionandtreatmentofacutetypeAaortic dissection:theInternationalRegistryofAcuteAorticDissection(IRAD).Circulation2011124:19118. 4. MilewiczDM.Stoppingakiller:improvingthediagnosis,treatment,andpreventionofacuteascendingaortic dissections.Circulation2011124:19024.

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Chapter8Exam
Visittheonlineversionoftheproducttoseethecorrectanswerandcommentary. 1. Youdiagnoseda78yearoldfemalepatientwithadescendingTAAthatwas6.5cm indiameter.Sheunderwentsuccessfulthoracicendovascularstentgraftrepairof theaneurysm.Inadditiontotreatingherriskfactors,whichofthefollowingimaging techniquesshouldbeusedtoimageheraorta? A. Achestradiograph(chestXray)every6months. B. ACTAannually. C. Anechocardiogramannually. D. Anabdominalultrasoundevery6months. E. Nofollowupimagingisnecessary.

2. Youcarefora67yearoldmanwithhypertensionandhyperlipidemia,whowas recentlydiagnosedwitha5.7cmAAA.Youhaverecommendedaorticrepair,andhe tellsyouhepreferstohaveitrepairedwithanendovascularstentgraftbecausehe hasreadthatitisbothlessinvasiveandsaferthananopenrepair.Youwouldliketo helphimtomakeaninformeddecisionabouthowhisaneurysmshouldbe repaired.WhichofthefollowingwouldyoutellhimregardingEVARcomparedwith openrepairforthetreatmentofAAAs? A. EVARisassociatedwithlowershorttermmortality,butequivalentlongterm mortality. B. EVARisassociatedwithfewerendoleaks. C. EVARisassociatedwithbothlowershorttermandlongtermmortality. D. EVARisassociatedwithlowershorttermmortalityandfewerlongterm repeatinterventions. E. EVARisassociatedwithanequivalentshorttermmortality,butalowerlong termmortality.

3.A65yearoldmanpresentstotheemergencydepartmentwithabdominal discomfortthatbegan2daysprior,butresolvedovertheprevious4hours.Hisblood pressureis125/82mmHgandheartrateis72bpmandregular.Physical examinationrevealsmildabdominaltendernessonpalpationandstrong, symmetricfemoral,popliteal,anddorsalispedispulses.Magneticresonance imagingoftheabdomenrevealsatwochannel4cminlengthdescendingaortic dissectionthatterminateswithin1cmproximaltotheoriginofthesplenicartery. Thereiscompletethrombosisofthefalselumenwithoutevidenceof communicationbetweenthetrueandfalselumen. Whichofthefollowingstatementsmostappropriatelycharacterizesthepatients clinicalcondition? A. ThisisahighriskStanfordtypeAdissectionduetocompletethrombosis

withinthefalselumen. B. ThisisahighriskStanfordtypeBdissectionduetocompletethrombosis withinthefalselumen. C. ThisisalowriskStanfordtypeAdissectionduetocompletethrombosis withinthefalselumen. D. ThisisalowriskStanfordtypeBdissectioncompletethrombosiswithinthe falselumen.

4.A31yearoldwomanis6dayspostpartumandevaluatedintheemergency departmentforacomplaintofsevereanteriorchestpain.Sheappearsanxiousand complainsofchestdiscomfortthatbegantheprevioushour,whichisassociated withnausea.Shedeniesshortnessofbreath.Herrightupperextremityandleft upperextremitybloodpressureis150/65and161/67mmHg,respectively.The patientsECGreveals2mmSTsegmentelevationinleadsII,III,andaVFandis otherwisesignificantforsinustachycardiaatarateof115bpm.Plain roentgenogramrevealsmildcardiomegalywithoutevidenceofmediastinalwidening orpulmonaryedema. Whichofthefollowingisthenextmostappropriatestepinthepatients management? A. InitiatetherapywithIVesmololtitratedtoaheartrateof60bpmandrequest anemergencycontrastCTscanofthechestandabdomen. B. InitiatetherapywithIVunfractionatedheparin,includingaloadingbolus,and performanemergencytransthoracicechocardiogramtoassessrightventricular function. C. InitiatetherapywithIVunfractionatedheparin,includingaloadingbolus, aspirin325mgbymouth,0.5mgnitroglycerintabletsublingual,andactivatethe cardiaccatheterizationlaboratory. D. Prepareforemergencypericardiocentesis.

PleasevisittheonlineversiontoengageinthisExam. 1.ThecorrectanswerisB.Evenaftersuccessfuldeploymentofanendograft,endoleaksoccur inapproximately10%ofpatientsat30days.Also,evenifnotpresentearlypostprocedure,they mayalsoappearlateandpressurizetheaneurysmsac,leavingitatriskforrupture.Therefore, theaneurysmsacneedstobefollowedwithannualsurveillanceCTAstoidentifythepresenceof endoleaks,aswellastoassessstabilizationoftheaneurysmsacandtoexcludethe appearanceofaneurysmselsewhere. Chestradiographyistoocrudetopermitdetectionofaneurysmgrowthorchangesinthestatus oftheaneurysmssac.Echocardiographyisveryusefultoexaminetheaorticroot,ascending thoracicaorta,andaorticarch,buttypicallyimagesthedescendingthoracicaortapoorly,soit wouldnotbehelpfulinthissetting.Anabdominalultrasoundimagestheabdominalaortawell, butnotthethoracicaorta. 2.ThecorrectanswerisA.Inrandomizedprospectivetrials,EVARisassociatedwithlower shorttermmortalitythanopenrepair,butby2yearsfollowingaorticrepair,themortalityis equivalent.EVARisassociatedwithmorelongtermcomplicationsthat,inturn,resultintheneed formorerepeatinterventions.EndoleaksareacomplicationthatmayariseafterEVARwhen thereispersistentbloodflowintotheaneurysmsacendoleaksdonotoccurwithopenaortic repair.

3.ThecorrectanswerisD.OptionDisthecorrectanswerfortworeasons.First,comparedto partialthrombosisorfreeflowingbloodinthefalselumenofadissection,completethrombosis isassociatedwithamorefavorableoutcome.Therefore,intheabsenceofotherclinicalfeatures suggestiveofhemodynamiccompromise,thispatientsriskprofileisrelativelylow.Second,the aorticdissectionis4cminlengthandterminatesneartheoriginofthesplenicarterythus, ascendingaorticarchinvolvementisnotpresent.ThecorrectStanfordaorticdissection classificationfordissectionsthatdonotinvolvetheascendingaortaistypeB. OptionsA,B,andCareincorrectbecausethesechoicesdonotaccuratelycharacterizeeitherthe appropriateStanfordaorticdissectiontypeorclinicalriskprofile. References
1. TsaiTT,EvangelistaA,NienaberCA,etal.Partialthrombosisofthefalselumeninpatientswithacute typeBaorticdissection.NEnglJMed2007357:34959.

4.ThecorrectanswerisA.OptionAisthecorrectanswerbecausetheclinicalinformation providedismostconsistentwithacuteaorticdissection.Althoughacuteaorticdissectionis overalluncommonasacomplicationofpregnancy,nearly50%ofalldissectionsinwomen youngerthan40yearsofageoccurintheperipartumtimeperiod.Therefore,alowclinicalindex ofsuspicionforaorticdissectionisnecessaryinthemanagementofanywomaninthe peripartumperiodpresentingwithsymptomsorclinicalsignsthatmaybeconsistentwithaortic dissection(suchasseverechestpaininthispatient).Theinitialclinicalmanagementof suspectedaorticdissectioninvolvesshortactingIVadrenergicreceptorantagonisttherapyand adiagnosticimagingstudy,suchasaCTscanofthechestandabdomen. OptionsBandCdescribetheappropriatemanagementforpulmonaryembolismand spontaneouscoronarydissection,respectively.Despitetheassociationofpulmonaryembolism withpregnancy,intheabsenceofshortnessofbreath,hemodynamicallysignificantpulmonary embolismisunlikelyandmaybeevaluatedfurther,ifnecessary,pendingexclusionofaortic dissection.Cardiaccatheterizationtoevaluateforacutecoronarysyndromeorspontaneous aorticdissectionmaybetemptinggiventhepresenceofchestpainandSTsegmentelevations onECG.However,typeAaorticdissectioncaninvolvetherightcoronaryarterytoproducesigns ofinferiormyocardialischemia.Owingtothepotentiallycatastrophicconsequencesof anticoagulationandinstrumentationoftheaortainpatientswithaorticdissection,itiscriticalto considerthepossibilitythataorticdissectionispresenteveninthepresenceof electrocardiographicfeaturesofmyocardialinjury.Inthispatient,hypertension(ratherthan hypotension),severechestpain,andbloodpressuredissymmetrybetweentheupper extremitiessuggestsaorticdissection. OptionDisincorrectbecausethereisnoclinicalevidencetosupportthepresenceofcardiac tamponade.Furthermore,hemopericardiumduetoacute,proximalaorticdissectionrequires surgicaltreatment.

References
1. AzizF,PenupoluS,AlokA,DoddiS,AbedM.Peripartumacuteaorticdissection:acasereportand reviewoftheliterature.JThoracDis20113:6567. 2. BravermanAC.Acuteaorticdissection:clinicianupdate.Circulation2010122:1848.