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Chapter10:DiseasesoftheAorta
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DiseasesoftheAorta

Introduction
Aorticdiseaseincludesacutelifethreateningconditionssuchasaorticdissection,chronicconditions
suchasaorticatheromathatmayleadtoembolism,andaorticaneurysmsthatcarrytheriskofrupture.
Appropriatesurveillanceandtreatmentofaorticdiseasearecrucialtopreventingcatastrophicvascular
events.

ImagingoftheThoracicAorta
Usingimagingtoscreenasymptomaticpatientsforabnormalitiesofthethoracicaortaisnot
recommended,exceptinpatientswithunderlyingvascularpathology(suchasMarfanorEhlers
Danlossyndrome),abicuspidaorticvalve,orafamilyhistoryofaorticdisease.Abnormalitiesofthe
thoracicaortaaresometimesdiscoveredincidentallyonchestradiographyperformedforother
purposes.Anacuteaorticsyndromemayproduceawideningofthemediastinalsilhouette,
enlargementoftheaorticknob,ordisplacementofthetracheafrommidline(Figure32).Ifan
abnormalityisidentified,additionalnoninvasiveimagingoftheaortamaybeusefultodetermine
aorticcrosssectionalarea,whichmaypredicttheriskofaneurysmruptureordissection.
Echocardiography,CT,andMRIcanbeusedtocrosssectionallyimagetheaorta(Table41).Thereis
substantialvarianceinmeasuredaorticdimensionaccordingtoimagetechniquebasedon
measurementofeitherinternalorexternalaorticdiameter.Caremustbetakentomeasurethe
dimensionperpendiculartothelongaxisoftheaortabecauseobliqueortangentialmeasurementsmay
overestimatethetrueaorticdiameter.
Maximumaorticdiameterataspecificlocationisgenerallyreportedbyimagingresults.Dilation
abovetheupperlimitsfornormalmayrepresentananeurysm,butapatientwithalargerbodysize
mayhavealargerabsoluteaorticdiameter.Themaximumascendingaorticdiametermaybeindexed
tobodysurfacearea(zscore).
Echocardiography,CT,andMRIhavesimilarsensitivityandspecificityfordiagnosisofacute
thoracicaortadisease,althougheachhasadvantagesanddisadvantages.Transthoracic
echocardiography(TTE)andtransesophagealechocardiography(TEE)havetheadvantagesofbeing
performedatthepatient'sbedsideandprovidingresultswithinseveralminutes.However,although
TTEmaybeusefulinidentifyinganintimalflap,enlargedaorticroot,oraorticinsufficiency,itis
limitedinevaluatingthemiddistalascendingaorta,transverseaorta,ordescendingthoracicaorta.
TEEovercomestheshortcomingsofTTEbutrequiresconscioussedation.Theprimaryadvantagesof
TEEcomparedwithCTimagingofsuspectedaorticdissectionincludeitsportabilityintheunstable
patientandlackofcontrastinjection.
Invasiveimagingoftheaortabyangiographyisrarelyindicatedforthediagnosisofacutedisease.It

maybehelpfulindeterminingthelengthofananeurysmandinvolvementofbranchvesselsand
shouldbereservedforpatientsinwhomapercutaneousinterventionisplanned.

KeyPoints
Screeningofasymptomaticpatientsforabnormalitiesofthethoracicaortashouldbereserved
forpatientswithunderlyingvascularpathology(suchasMarfanorEhlersDanlossyndrome),a
bicuspidaorticvalve,orafamilyhistoryofaorticdisease.
Invasiveimagingoftheaortabyangiographyisrarelynecessaryforthediagnosisofacute
diseaseitshouldbereservedforpatientsinwhomapercutaneousinterventionisplanned.

ThoracicAorticAneurysm
RelatedQuestions
Question97
Question117
Thoracicaorticaneurysmsmayinvolvetheaorticroot,ascendingaorta,aorticarch,ordescending
aorta.Aneurysmsoftheaorticrootandascendingaortaaremostcommonandusuallyoccurasa
consequenceofunderlyingmedialdegeneration.Thoracicaorticaneurysmsoftenareasymptomatic
andarefrequentlydetectedincidentallyduringevaluationforanotherproblem.Rarely,thoracicaortic
aneurysmsmaybediscoveredbecauseofcompressivesymptoms,suchashoarseness,stridor,or
dysphagia.Ifruptureoccurs,patientsmayhaveseverechestpain,backpain,suddenshortnessof
breath,orsuddendeath.Adiastolicheartmurmurorsymptomsofheartfailuremayoccurwith
aneurysmaldilatationoftheaorticrootandsubsequentaorticvalveregurgitation.
CausesofthoracicaorticaneurysmsarelistedinTable42.Theseaneurysmstypicallyresultfrom
cysticmedialdegenerationthatleadstolossofsmoothmusclecellsandelasticfiberdegeneration,
resultinginaweakeningoftheaorticwall.Cysticmedialdegenerationoccursnormallywithaging
andisexacerbatedbyhypertension.Inyoungerpatients,aneurysmismostoftenrelatedtoa
connectivetissuedisorder(suchasMarfanorEhlersDanlossyndrome).Bicuspidaorticvalveisan
importantriskfactorforaneurysminvolvingtheaorticrootandascendingthoracicaorta.
Approximately50%ofpatientswithbicuspidaorticvalveshaveenlargementoftheproximalaorta
thatmaybeindependentoftheseverityofaorticvalvedisease.Thevastmajorityofaneurysms
affectingthedescendingthoracicaortaareassociatedwithatherosclerosis.Othercausesofthoracic
aorticaneurysmincludeacquiredinfectionandinflammatoryconditions,suchassyphilis,Takayasu
arteritis,andgiantcellarteritis.
Theleadingcauseofdeathinpatientswiththoracicaorticaneurysmisrupture(60%).Severalstudies
haveshownanincreasingriskofruptureaftertheaneurysmhassurpassed5.0cmindiameter(4.05.0
cminpatientswithgeneticallymediateddisorders),andarapidrateofexpansionisanindependent
riskfactorforrupture.Pregnancyalsoisassociatedwithanincreasedriskofaorticdissection,
particularlyinwomenwithMarfansyndrome,inwhomdissectionmayoccurataorticdiametersthat
aresmallerthanwouldusuallybeconsideredforelectiverepair.
Smallerthoracicaorticaneurysmscanbemedicallymanagedwithaggressivebloodpressurecontrol.
BlockersmaybeofparticularbenefitforreducingtherateofaorticgrowthinpatientswithMarfan
syndrome,althoughtheirbenefitintreatinganeurysmsofotheretiologieshasnotbeenproved.
Losartan,anangiotensinreceptorblocker,hasalsobeenassociatedwithslowerprogressionofaortic
rootdilationinpatientswithMarfansyndrome.
Inanypatientwithathoracicaorticaneurysmthatdoesnotrequireimmediateintervention,regular

surveillanceisimportantforidentifyingthedevelopmentofsignsandsymptoms.Annual
echocardiographyshouldbeperformedtomonitoraorticgrowth.Earlierreevaluationisindicatedfor
changesinsymptomsorphysicalexaminationfindingsandforhemodynamicassessmentrelatedto
pregnancy.Whentheaorticdimensionnearsthethresholdforintervention,patientsshouldbereferred
toanappropriatespecialistforevaluation.Becausepatientswithabicuspidaorticvalvehavean
increasedriskofaorticaneurysmanddissection,thesepatientsshouldundergoechocardiographyof
theaortaannuallyiftheaorticrootorascendingaortadimensionisgreaterthan4.5cm.Inthosewith
anaorticdiameterbetween4.0and4.5cm,theexaminationintervaldependsontherateof
progressionofdilationandthefamilyhistory.
Familialthoracicaorticaneurysmsandaorticdissections(TAAD)isaninheritedautosomaldominant
condition.ScreeningisrecommendedforfirstdegreerelativesofpersonswithTAADonceayearor
atleasteveryfewyears.Ifthemutationisknown,genetictestingcanidentifythoserelativeswho
shouldbescreenedwithaorticimaging.
InpatientswithMarfansyndrome,followupimagingisrecommended6monthsafterdiagnosiswith
annualsurveillancethereafteriftheaorticrootislessthan4.5cmindiameterandotherwisestable.If
theaorticdiameteris4.5cmorgreaterorshowssignificantgrowthovertime,thenmorefrequent
surveillanceissuggested(forexample,twiceyearly).MostpatientswithMarfansyndromepresent
withenlargementoftheascendingaortatherefore,serialexaminationisfocusedmainlyonassessing
thisportionofaorta,andtransthoracicultrasoundisthepreferredimagingmodalityinthesepatients.
Therepairofathoracicaorticaneurysmisoftenrecommendedprophylacticallytopreventthe
morbidityandmortalityassociatedwithaneurysmrupture.Inasymptomaticpatients,electivethoracic
aorticrepairisrecommendediftheaorticrootorascendingaortaisgreaterthan5.5cm(5.56.0cm
forthedescendingaorta)orhasrapidgrowth(>0.5cm/year).Forgeneticallymediateddisorders(such
asMarfansyndrome),alowerthresholdof5.0cm(4.05.0cmincertainpatients)maybeusedfor
repair.Forpatientswithabicuspidaorticvalve,repairisindicatediftheaorticdiameterisgreater
than5.5cmandisreasonableifthediameterisgreaterthan5.0cmandthepatienthasanincreased
riskofdissection(familyhistoryofdissectionorrapidgrowth).
Repairofascendingaorticandaorticarchaneurysmsrequiressurgeryandmayincludeaorticvalve
replacementorrepairinpatientswithsignificantannulardilatationorassociatedaorticvalve
pathology.AconservativeprocedurewherebytheaneurysmisreplacedwithaDacrongraftandthe
aorticvalveispreservedhasgainedwidespreaduse.Iftheaorticvalveneedsreplacementandthe
patienthasadilatedaorticroot,acompositeaorticvalveandaorticrootandascendingaortagraft
replacement(Bentalloperation)maybeperformed.TheBentalloperationincludesreimplantationof
thecoronaryarteriesintotheascendingaorticgraft.Thoracicendovascularaorticrepair(TEVAR)
withstentgraftinghasemergedasapromisingalternativetoopenrepairforaneurysmofthe
descendingthoracicaorta.TEVARhasbeenassociatedwithshorterhospitalstaysandlowerhospital
morbidityandhasthepotentialadvantagesofavoidingthoracotomy,aorticcrossclamping,and
extracorporealsupport.AdverseeventsfollowingTEVARincludestroke,spinalischemia,access
complications,andendoleaks.

KeyPoints
Patientswithathoracicaorticaneurysmshouldundergoannualechocardiographytomonitor
aorticaneurysmgrowth.
Patientswithabicuspidaorticvalveshouldundergoannualechocardiographyoftheaortaifthe
aorticrootorascendingaortadimensionisgreaterthan4.5cm.
Inasymptomaticpatients,electivethoracicaorticrepairisrecommendediftheaorticrootor
ascendingaortaisgreaterthan5.5cm(5.56.0cmforthedescendingaorta)orhasrapidgrowth
(>0.5cm/year)forpatientswithgeneticallymediateddisorders,thethresholdforrepairis
lower.

AcuteAorticSyndromes
Acuteaorticsyndromesincludeaorticdissection,intramuralhematoma,penetratingatherosclerotic
ulcer,andtraumainducedaorticrupture(Figure33).Acuteaorticsyndromesthreatencentralaortic
pressure,criticalorganperfusion,andsurvival.Promptrecognitionanddeliveryofappropriate
medicalandinterventionalcarearecriticaldeterminantsofoutcome.

Pathophysiology
Inaorticdissection,bloodpassesthroughatearintheaorticintima,creatingafalselumenthat
separateslayersoftheaorta.Propagationofthedissectioncanproceedinananterogradeorretrograde
fashionfromtheinitialtear,involvingsidebranchesandcausingcomplicationssuchastamponade,
aorticvalveinsufficiency,ormalperfusionsyndromes.Anintramuralhematomamayresultfrom
ruptureofthevasavasorumormicrotearsintheintima,resultinginacrescentofhematomawithin
themediawithoutidentifiableinterruptionoftheintima.Penetratingatheroscleroticulcersaremost
likelycausedbyatherosclerosiswithsubsequenterosionacrosstheinternalelasticmembraneofthe
aorta,allowingforabloodfilledfalsespacewithinthewalloftheaorta.
TheStanfordclassificationdescribestypeAdissectionsasoriginatingwithintheascendingaortaor
arch,whereastypeBdissectionsoriginatedistaltotheleftsubclavianartery.Thisnomenclaturehas
beengeneralizedtoalloftheacuteaorticsyndromes,althoughmostintramuralhematomasand
penetratingulcersaretypeBlesions.

DiagnosisandEvaluation
Thediagnosisofanacuteaorticsyndromerequiresahighindexofsuspicionbecauseofitslife
threateningcomplications.Theclassicpresentationconsistsofaorticchestpaindescribedassevere
rippingortearingpainthatmayradiatetotheanteriorchestorback,jaw,orabdomen,dependingon
whichsegmentoftheaortaisinvolved.Hypertensionisthemostimportantriskfactorotherrisk
factorsincludesmokingandatherosclerosis.Inthesettingofanacuteaorticdissection,hypertension
andanaorticregurgitationmurmurthatisfaint,shortinduration,andlowinpitchmaybepresent.
Otherfindingsonphysicalexaminationthatmayincreasetheindexofsuspicionincludepulsus
paradoxus,asymmetricbloodpressureintheupperextremities,andanasymmetricpulseexamination.
AlowDdimerlevel(<0.5g/mL[0.5mg/L])suggestsagainstanacuteaorticsyndrome.Inaortic
dissection,chestradiographymaydemonstrateawideningofthemediastinum.Anelectrocardiogram
isoftenabnormal,butnondiagnostic.Clinicalsuspicionshouldbehigh,andCT,MRI,orTEEfor
confirmationofthediagnosisshouldnotbedelayed.

Treatment
RelatedQuestions
Question6
Question24
Question71
Patientswithasuspectedacuteaorticsyndromewhoarenotincardiogenicshockshouldreceive
medicaltherapytocontrolheartrateandbloodpressure.Intravenousblockersshouldbeusedto
targetaheartrateofbelow70/min.Inpatientsrequiringadditionalbloodpressurecontrol,arapidly
titratableantihypertensivemedication,suchassodiumnitroprusside,labetalol,enalaprilat,
hydralazine,ornicardipine,shouldbegivenintravenously,withagoalofdecreasingthemeanarterial
pressuretothelowestlevelthatstillallowsvisceralandcerebralperfusion.

EmergencysurgeryisrecommendedforallpatientswithtypeAaorticdissectionaswellasfortypeA
intramuralhematoma.Anydelaystosurgeryshouldbeavoidedoraddressed,astypeAdissectionhas
averyhighshorttermmortalityrate.Concomitantaorticarchreconstruction,coronaryartery
reimplantation,aorticvalverepairorreplacement,orbranchvesselrepairmayberequireddepending
ontheanatomyandpathologyofthelesion.
UncomplicatedtypeBaorticsyndromesmaybetreatedmedically.TheINSTEADtrial,which
enrolledsubjectswithuncomplicatedchronictypeBdissection,showednodifferenceinclinicalor
aorticoutcomesat2yearsforpatientstreatedwithTEVARversusmedicaltherapyalone.Among
patientswhosurvivedat2years,additionallongtermfollowupoftheINSTEADtrialdemonstrated
thatTEVARinadditiontooptimalmedicaltreatmentwasassociatedwithimprovedlongtermaorta
specificsurvivalanddelayeddiseaseprogression.SurgeryisindicatedforcomplicatedtypeBaortic
dissectiondefinedbyrefractorypainorhypertension,rapidaneurysmalexpansion,rupture,or
malperfusionsyndrome.

KeyPoints
Theclassicpresentationofanacuteaorticsyndromeconsistsofaorticchestpainsevere
rippingortearingpainthatmayradiatetotheanteriorchestorback,jaw,orabdomen,
dependingonwhichsegmentoftheaortaisinvolved.
Findingsthatincreasetheindexofsuspicionforanacuteaorticsyndromeincludepulsus
paradoxus,asymmetricbloodpressureintheupperextremities,andanasymmetricpulse
examination.
TypeAaorticdissectionhasaveryhighshorttermmortalityrate,andemergencysurgeryis
recommendedforallpatientswithoutdelay.

AorticAtheroma
Aorticatheroscleroticplaquesareamanifestationofsystemicatherosclerosis.Aorticatheromamay
bedetectedincidentallyduringimaging(Figure34).Theriskofembolismandstrokeinpatientswith
aorticatheromaissignificantlyincreasedforplaquesthataremobileorprotruding,particularlyifthe
plaqueisgreaterthan4mminsize.Thromboembolismmayalsoresultfromdislodgmentofdebris
fromtheaorticwalloccurringasacomplicationofaninvasivecardiovascularprocedure,suchas
catheterization,intraaorticballoonpumpplacement,orvascularsurgery.
Noncoronaryatheroscleroticdisease,includingaorticatheroma,isacoronaryheartdiseaserisk
equivalentand,therefore,shouldbeaggressivelytreatedtoreducetheriskoffuturecardiovascular
events,includingtreatmentwithantiplateletagentsandstatins.Inanobservationalstudy,statin
therapywasassociatedwitha17%absolutereductioninthromboemboliceventsinpatientswith
aorticatheroma.

KeyPoint
Aorticatheromaisacoronaryheartdiseaseriskequivalent,andpatientsshouldbeaggressively
treatedwithantiplateletagentsandstatinstoreducetheircardiovascularrisk.

AbdominalAorticAneurysm
ScreeningandSurveillance
RelatedQuestions

Question31
Question80
Anabdominalaorticaneurysm(AAA)isconsideredtobepresentwhentheminimumanteroposterior
diameteroftheaortareaches3.0cm.ThemostimportantriskfactorsforAAAareincreasingage,
smoking,andmalesex(menwithAAAoutnumberwomenbyupto6:1).Otherriskfactorsinclude
atherosclerosis,hypertension,andfamilyhistoryofAAA.Basedonrandomizeddata,theU.S.
PreventiveServicesTaskForceguidelinesrecommendaonetimeultrasonographicscreeninginmen
aged65to75yearswhoareactiveorformersmokers(seeMKSAP17GeneralInternalMedicine).
ThesensitivityandspecificityofultrasonographyfordetectionofAAAareexcellent.
AAArupturehasanexceedinglyhighmortalityrate,yetmostAAAsneverrupture.Thus,deciding
whenandinwhomtointerveneandelectivelyrepairanAAAisofmajorimportance.Thestrongest
riskfactorfortheruptureofanAAAismaximalaorticdiameterthismeasurementisthedominant
indicationforrepair.EstimatedannualruptureriskaccordingtoAAAdiameterisshowninTable43.
AfterAAAhasbeenidentified,surveillanceimagingresultsdeterminethetimingofrepair.The
frequencyofsurveillanceisdependentonbaselineaneurysmsizelargeraneurysmsexpandfaster
thansmalleronesandmayrequiremorefrequentsurveillance.IfAAAmaximumdiameteris3.5to
4.4cm,repeatultrasonographyisrecommendedannuallyifmaximumdiameteris4.5to5.4cm,
repeatultrasonographyshouldbeperformedevery6to12months.Electiverepairshouldbe
consideredforAAAof5.5cmindiameter,forthosethatincreaseindiameterbymorethan0.5cm
withina6monthinterval,andforthosethataresymptomatic(tendernessorabdominalorbackpain).
Forwomen,electiverepairmaybeconsideredforanAAAthatreaches5.0cmindiameter.

Treatment
MedicaltherapiesforAAAfocusontargetingmodifiableriskfactorsforAAAandcardiovascular
diseasewiththegoalsofreducinganeurysmexpansionorrupture,reducingmorbidityandmortality
associatedwithrepair,andreducingcardiovascularmorbidityandmortality.Smokingcessationisthe
cornerstoneoftherapyforactivesmokers.
Ifrepairisbeingconsidered,thechoicebetweenopensurgicalorendovascularaneurysmrepair
(EVAR)isindividualizedbasedonthepatient'sage,comorbidities,patientpreference,andaortic
anatomy.RandomizedtrialscomparingopenrepairwithEVARhavefoundsignificantlyimproved
shortterm(30day)morbidityandmortalityforEVAR,butnosignificantdifferencesinlongterm
mortality.Moreover,inthelongterm,EVARwasassociatedwithincreasedcomplicationrates
(includingendovascularleaks,devicemigrationandfailure,andpostimplantationsyndrome)andthe
needforreinterventions.Becauseofthesepotentialcomplications,patientswhohaveundergone
EVARofAAArequirediligentfollowupwithimagingstudiesperformedannuallytoevaluatethe
statusofthegraft.Additionallongtermdatafromprospectiverandomizedtrialsareneededtofully
evaluatethebenefit/riskprofileofopenAAArepairversusEVAR.Inpatientswithseverecomorbid
diseaseconsiderednoteligibleforopensurgicalcorrection,atrialofEVARversusmedicaltherapy
demonstratednodifferenceinallcausemortalityat8years,butgreatercostandincreased
complicationswereassociatedwithendovascularrepair.

KeyPoints
Themostimportantriskfactorsforabdominalaorticaneurysmareincreasingage,smoking,and
malesexonetimeultrasonographicscreeningisrecommendedformenages65to75years
whohaveeversmoked.
Electiverepairshouldbeconsideredforanabdominalaorticaneurysmof5.5cmindiameter,
forthosethatincreaseindiameterbymorethan0.5cmwithina6monthinterval,andforthose

whoaresymptomatic.
Medicaltherapiesforabdominalaorticaneurysmfocusontargetingmodifiableriskfactorsfor
abdominalaorticaneurysmandcardiovasculardiseasesmokingcessationisthecornerstoneof
therapyforactivesmokers.

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Chapter10
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DiseasesoftheAorta
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