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23.TaggartNW,MinahanM,CabalkaAK,CettaF,UsmaniK,RingelRE.Immediateoutcomesofcoveredstentplacementfortreatment

orpreventionofaorticwallinjuryassociatedwithcoarctationoftheaorta(COASTII).JACCCardiovascInterv.2016;9(5):484-493. doi:10.1016/j.jcin.2015.11.038.

24.GoldenAB,HellenbrandWE.Coarctationoftheaorta:stentinginchildrenandadults.CatheterCardiovascInterv.2007;69(2):289-299.

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25.ForbesTJ,KimDW,DuW,etal.Comparisonofsurgical,stent,andballoonangioplastytreatmentofnativecoarctationoftheaorta:an

observationalstudybytheCCISC(CongenitalCardiovascularInterventionalStudyConsortium).JAmCollCardiol.2011;58(25):2664-

2674.doi:10.1016/j.jacc.2011.08.053.

26.LockJE,BassJL,AmplatzK,FuhrmanBP,Castaneda-ZunigaW.Balloondilationangioplastyofaorticcoarctationsininfantsand

children.Circulation.1983;68(1):109-116.doi:10.1161/01.CIR.68.1.109.

27.TorokRD.Coarctationoftheaorta:managementfrominfancytoadulthood.WorldJCardiol.2015;7(11):765.doi:10.4330/wjc.v7.i11.765.

28.CowleyCG,OrsmondGS,FeolaP,McQuillanL,ShaddyRE.Long-term,randomizedcomparisonofballoonangioplastyandsurgery

fornativecoarctationoftheaortainchildhood.Circulation.2005;111(25):3453-3456.doi:10.1161/CIRCULATIONAHA.104.510198.

29.HarrisKC,DuW,CowleyCG,ForbesTJ,KimDW.Aprospectiveobservationalmulticenterstudyofballoonangioplastyforthe

treatmentofnativeandrecurrentcoarctationoftheaorta.CatheterCardiovascInterv.2014;83(7):1116-1123.doi:10.1002/ccd.25284.

30.FawzyME,FathalaA,OsmanA,etal.Twenty-twoyearsoffollow-upresultsofballoonangioplastyfordiscreetnativecoarctationof

theaortainadolescentsandadults.AmHeartJ.2008;156(5):910-917.doi:10.1016/j.ahj.2008.06.037.

31.MullinsCE.Dilationofcoarctationoftheaorta-nativeandre/residualcoarctation.In:CardiacCatheterizationinCongenital

HeartDisease.1sted.:BlackwellPublishing;2006:454-471.

https://t.me/med1917

32.HolzerR,QureshiS,GhasemiA,etal.Stentingofaorticcoarctation:Acute,intermediate,andlong-termresultsofaprospectivemulti-

institutionalregistry-Congenitalcardiovascularinterventionalstudyconsortium(CCISC).CatheterCardiovascInterv.2010;76(4):553-563. doi:10.1002/ccd.22587.

33.KischeS,D’AnconaG,StoeckichtY,OrtakJ,ElsässerA,InceH.Percutaneoustreatmentofadultisthmicaorticcoarctationacute

andlong-termclinicalandimagingoutcomewithaself-expandableuncoverednitinolstent.CircCardiovascInterv.2015;8(1):1-10. doi:10.1161/CIRCINTERVENTIONS.114.001799.

34.MeadowsJ,MinahanM,McElhinneyDB,McEnaneyK,RingelR.Intermediateoutcomesintheprospective,multicentercoarctation

oftheaortastenttrial(COAST).Circulation.2015;131(19):1656-1664.doi:10.1161/CIRCULATIONAHA.114.013937.

35.OliverJM,GallegoP,GonzalezA,ArocaA,BretM,MesaJM.Riskfactorsforaorticcomplicationsinadultswithcoarctationofthe

aorta.JAmCollCardiol.2004;44(8):1641-1647.doi:10.1016/j.jacc.2004.07.037.

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BlackwellPublishing;2006:642-660.

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resultsinacohortof22patients.EurJCardio-thoracicSurg.2010;37(2):322-327.doi:10.1016/j.ejcts.2009.04.071.

C H A P T E R  5 B

EndovascularManagementoftheAscending AortaandtheAorticArch

CamiloA.VelasquezMD

YoungErbenMD

MohammadA.ZafarMD

ChandniPatelMD

AymanSaeyeldinMD

AntonA.Gryaznov

BulatZiganshinMD,PhD

JohnA.ElefteriadesMD,PhD(hon)

KeyPoints

■Thoracicaorticaneurysmsareusuallyasymptomaticandnoteasilydetectableuntilanacute

andoftencatastrophiccomplicationoccurs.

■Diameters greater than 6 cm increase the risk of death and complication threatening to

producedeath.Recently,evidencehasshownhingepointsat5.25cmand5.75cm,suggesting aleftwardshiftintheaorticdiameteratwhichinterventionshouldberecommended.

■Theimagingmodalitiesusedfordiagnosingpathologyoftheascendingaortaarecomputed

tomography,magneticresonanceimaging,andechocardiography.

■TypeAacuteaorticdissectionisasurgicalemergencyrequiringimmediateconsultationand

intervention.

■Open surgical intervention withthe replacementofthe ascendingaortaor the aortic arch

withagraftisthegoldstandardforthetreatmentofthoracicaorticpathology.

■High-riskpatientswhoareunabletoundergoopensurgicalrepair mayfairlybemanaged

https://t.me/med1917

withmedicaltherapywithadequateoutcomes.

■Endovascular management of aortic pathology is an alternative for high-risk patients in

whomtheopenapproachisprohibited.

■Therearenospecificapproveddevicesfortheendovascularmanagementoftheascending

aortaandtheaorticarch.

I.Introduction

A.Thoracicaorticdiseasesarevirulent,oftencapableofleadingtodeathofthepatient.

1,2

Generally,the

thoracicaortaisasilentorganthatonlybecomessymptomaticwhenacatastrophiceventsuchasdeathor a major complicationthat threatens toproducedeath occurs.1According to datafrom theCenters for DiseaseControlandPrevention,fromtheyears1999to2015,aorticaneurysmwasthe19thleadingcause ofdeathinallagesandthe16thcauseofdeathinpatientsolderthan65years.3Approximately,10,000 aorticdeaths peryearhave beenreported,withadecreaseinincidencefrom15,807deathsin1999to 9988deathsby2015.

1,3

B.Theaortaitselfisconsideredanactiveorganwithmechanicalpropertiesandanintrinsicandcomplex

biology.Diseasesoftheaortaarecategorizedbasedonlocation:aorticroot,ascendingaorta,aorticarch, anddescendingaorta.Thediseasesaffectingtheaortaareseparatedintwodistinctentitiesatthelevelof the ligamentum arteriosum: above the ligament (ascending aorta and aortic arch), the disease is nonarteriosclerotic in nature, whereas below the ligament (descending aorta and abdominal aorta), arteriosclerosisisabundant.

C.Thoracic endovascularaorticrepair(TEVAR)hasbeenshown to reduceperioperative mortality

and morbidity with a sustained benefit during follow-up. However, recent studies of endovascular approaches to aneurysmsinthe abdominal and thoracic aorta show a seriously disturbingtendency to endoleak by 5 years.

4-6

 In recent years, TEVAR has become an accepted treatment for patients with

suitableanatomyinthedescendingthoracicaorta.

7,8

a. However,anendovascularapproachforrepairoftheascendingaortaandaorticarchpathologyis

troublesome.Anatomicandphysiologicchallengesareformidablefortheadequatedeploymentof endovascularsystems.Fundamentalproblemsabound.Proximalgraftfixationclosetoboththeaortic valveandthecoronaryostiaisdifficultanddangerous.Distallandingzonesmayimpingeonthe innominateartery.Theseareexamplesofthecomplexityofendovasculartechniquesappliedinthe “high-rent”regionproximaltotheligamentumarteriosum.1Additionally,thehemodynamicforces experiencedintheascendingaortacanbeanobstacleforaccurategraftdeployment.

D.Therearenocurrentsocietalguidelinesforendovascularmanagementofascendingaorticaneurysm.

Infact,severalcasereportsconstitutethebulkofthecurrentliterature.9Therefore,thischapteraimsto providethereaderwiththecurrentstateoftheartintheemergingendovasculartreatmentoftheascending aorta andthe aortic arch. The pathologies amenable tointervention will be discussed, andthe current devicesandtechniquesavailablefortheascendingandarchsegmentswillbedescribed.

II.AorticPathologyfortheEndovascularTreatmentofthe AscendingAorta

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A.CurrentManagementandTreatments

In recent years, interest in the management of aortic diseases with endovascular devices has seen an expansion beyond the treatment of the abdominal aorta to an increased attention to more proximal segmentsofthethoracicaorta.Theascendingaortaandtheaorticarchbecametheultimatefrontiersfor utilizationofendovasculartechniques.

8,10

Additionally,withtheincreasedsafetyofopenaorticsurgery,

11,12

theendovascular approachhasbeenreserved forpatientswho posea prohibitiveriskforanopen

procedure,orasalastresortinemergentconditionsinwhichanopensurgicalapproachisnotfeasible andsolemedicalmanagementcanbeexpectedtoleadtodecreasedsurvival.10Endovascularmanagement oftheascendingaortaandaorticarchcanbeappliedinhigh-riskpatientswiththefollowingconditions: type A aortic dissection, aortic pseudoaneurysm,penetrating aortic ulcer (PAU), intramural hematoma (IMH),ascendingaorticaneurysm,andascendingaorticrupture.

8,10

B.ThoracicAorticDissection

1. Comorbidities that are associated with an increase in the wall stress (hypertension, weightlifting,

coarctation,cocaineuse) or aortic media abnormalities (Marfan disease, Loeys-Dietz, Ehlers-Danlos, bicuspidaorticvalve,familialaorticaneurysm,steroidtreatment)canpredisposetothedevelopmentof aorticdissection(Table5B.1).

TABLE5B.1

RiskFactorsAssociatedWiththeDevelopmentofAorticDissection

ConditionsAssociatedWithIncrease dAorticWallStress

■Uncontrolledhypertension

■Pheochromocytoma

■Cocaineandotherstimulants

■WeightliftingandValsalvamaneuvers

■Trauma

■Decelerationortorsionalinjury

■Coarctationoftheaorta

ConditionsAssociatedWithAorticMe diaAbnormalities

Genetic

■Marfansyndrome

■Ehlers-Danlossyndrome,vascularform(typeIV)

■Bicuspidaorticvalve

■Turnersyndrome

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■Loeys-Dietzsyndrome

■Familialthoracicaorticaneurysmanddissectionsyndrome

InflammatoryVasculitis

■Takayasuarteritis

■Giantcellarteritis

■Bechetarteritis

Others

■Pregnancy

■Polycystickidneydisease

■Chroniccorticosteroidandimmunosuppressionagentadministration

■Infectionsinvolvingtheaorticwall

2.Dependingontheseverityanddegreeofdissection,multipleorgansystemscanbeaffected,including

cardiovascular,pulmonary,renal,neurologic,gastrointestinal,andperipheralvascular(Table5B.2).

TABLE5B.2

ComplicationbyOrganSysteminPatientsWithAorticDissection

Cardiovascular

Cardiacarrest Syncope Aorticregurgitation Congestiveheartfailure Coronaryischemia Myocardialinfarction Cardiactamponade Pericarditis

Pulmonary

Pleuraleffusion Hemothorax Hemoptysis(aortotrachealorbronchialfistula)

Renal

Acuterenalfailure Renovascularhypertension Renalischemiaorinfarction

Neurologic

Stroke Transientischemicattack

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Paraparesisorparaplegia Encephalopathy Coma Spinalcordsyndrome Ischemicneuropathy

Gastrointestinal

Mesentericischemiaorinfarction Pancreatitis Hemorrhage(aortoentericfistula)

Peripheralvascular

Upperorlowerextremityischemia

Systemic

Fever

3.Overall,typeAdissectionsaretreatedemergentlywithoperativerepair.Thisisincontrasttopatients

withtypeBdissections,whoareinitiallytreatedconservativelywithanti-impulsetherapy,withsurgery being reserved for patients with complications.

15,16

 The indications for surgical, endovascular, and

medicaltherapyareasfollows(Table5B.3):

TABLE5B.3

IndicationsforSurgicalInterventioninPatientsWithAorticDissection

2,13

SurgicalTherapy

■AcutetypeAdissection

■Retrogradedissectionintotheascendingaorta

Endovascularand/orSurgicalTherapy

■EndovasculartherapyinacutetypeAdissectionforpatientswithprohibitiveriskforsurgicaltherapy

■AcutetypeBdissectioncomplicatedby

■Visceralischemia

■Limbischemia

■Ruptureorimpendingrupture

■Aneurysmaldilatation

■Refractorypain

Me dicalTherapy

■UncomplicatedtypeBaorticdissection

■Uncomplicatedisolatedarchdissection

a. AcutetypeAaorticdissectionsposesubstantialriskofaorticrupture,aorticregurgitationwithheart

failure,stroke,cardiactamponade,andvisceralischemia.

b. IntheIRADregistry,patientswithtypeAaorticdissectionwhoweremedicallymanagedhada

mortalityrateof58%comparedwith26%inthosewhounderwentsurgery.

c. Thegoalofrepairistoexcise/obliteratetheproximalentrytear,preventpericardialrupture,prevent

ortreatcoronaryostialdissection,correctaorticvalveregurgitation,restoreflowintothetrue lumen,correctmalperfusion,and,ifpossible,obliteratethedistalfalsechannel.

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4.Iftheaorticvalveisunabletoberepaired,aorticvalvereplacementisoftenrequired.Therefore,the

emergenttreatmentoftypeAdissectionconsistsinthereplacementoftheascendingaorta,oftentogether withreplacementoftheaorticvalveandthedissectedaorticarch.1Inpatientsinwhomopenrepairisnot feasible, endovascular techniques have been applied. These involve placement of an endograft in the ascendingaortaandthe aorticarch.However,such applicationsare investigational in nature andoften carriedoutonacompassionateusebasis.

C.ThoracicAorticAneurysm

1.Thoracicaorticaneurysms(TAAs)aredefinedasenlargementsoftheaortagreaterthan1.5timesits

normal size.1 We often use a diameter greater than 4 cm as the definition for TAA. True aneurysms involvethethree layersoftheaortic wallwithoutlosingcontinuity;however,inherentweaknessofthe aorticwallpredisposestodiameterexpansionandrupture.

13,14,18

Ontheotherhand,falseaneurysms,or

pseudoaneurysms,occurwhenthereisalossofcontinuityintheaorticwallitself,withbleedingthatis containedbythe adventitiaorthesurroundingperivascular tissues. Pseudoaneurysmsgenerallyposean increasedriskofrupturecomparedwithtrueaneurysms.

2. The pathophysiology of aortic dilatation is generally attributed to cystic medial degeneration and

inflammatorychangeswithintheaorticwall.Incysticmedialdegeneration,thereisadisruptionandloss ofelastic fibers with increased depositionofproteoglycansinthe medial layers.During inflammatory changes,thereisashifttowardexcessivedegradationoftheextracellularmatrix,overridingitssynthesis, thusadverselyaffectingthedelicatehomeostasisthatnormallyexistsbetweenthevascularsmoothmuscle cellsandtheextracellularproteinsinthemediallayeroftheaorta.

1,19,20

Theactivityoftheproteolytic

enzymes such as matrix metalloproteinases (MMPs) plays a major pathophysiologic role in aortic aneurysmformation. MMPs, especiallythe MMP-2andMMP-9 subtypes, degradetheelastin, fibrillin, andcollagen in themedial layeroftheaorticwall. Normally,MMPs areregulatedby thepresenceof tissueinhibitorsofmetalloproteinases(TIMPs),butinaneurysmalpatients,thebalancebetweenMMPs andTIMPsisshifted towardanincrease inproteolysis, correlating withthe observed degradation and thinning seen in the aortic wall.

1,21

 Additionally, inflammatory conditions such as Takayasu arteritis,

rheumatoidarthritis,andgiantcellarteritis,amongothers,canleadtothedevelopmentofTAAs,further exemplifyingtheinflammatoryroleinaneurysmformation.

1.AnatomicalCategorizationofAscendingThoracicAorticAneurysms:

TAAscanbecategorizedinthreegeneralclassesaccordingtothepatternofaorticrootinvolvement(see

Fig.5B.1).

a. Supracoronaryaneurysm: Theaorticannulusandtheshortsegmentofaortabetweentheannulus

andthecoronaryorificesareofnormalsize,withsupracoronarydilatationoftheascendingaorta.

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FIGURE5B.1 Threecommonpatternsofascendingaorticaneurysmdisease:supracoronary,annulo-aortic

ectasia,andtubular(seetextfordiscussion).

Reproducedwithpermissionfrom

ElefteriadesJA.Thoracicaorticaneurysm:readingtheenemy’splaybook.CurrProblCardiol.2008;33(5):203-277.

b. Marfanoidaneurysm:Alsotermedannuloaorticectasia,thistypeofaneurysminvolvesadilatation

oftheaorticannulusandtheproximalportionoftheaorta.

c. Tubularaneurysm:Inthiscategory,theaorticannulusandtheproximalaortaaresomewhatbutnot

markedly dilated, with a uniform caliber throughout the ascending aorta conferring a “tubular” appearance.

i. Onrareoccasions,onemayalsoseeasaccularaneurysm,whichprotrudeslikeasackfromthe

aorticlumen,involvingonlyasmallportionoftheaorticwalllengthandcircumference.

ii. Thesepatternsofanatomicenlargementareimportantbecausesurgicaltherapyispredicatedon

theprecisepatternofenlargement.

2.Long-termComplicationsofThoracicAorticAneurysms

a. GrowthofTAAspredisposesthepatientinthelongtermtosufferaorticdissectionorrupture.

Intheascendingaorta,rupturerarelyoccurswithoutaorticdissection.

i.Workdonetounveilthenaturalhistoryofascendingaorticaneurysmshasrevealed diameter“hingepoints”atwhichriskofruptureanddissectionincreasesdramatically.

ii.Traditionally,inthecaseofascendingaorticaneurysms,thehingepointhasbeen6cm, where31%ofthepatientswillhavesufferedadissectionorrupturebythetimetheaneurysm reachesthispoint

1,22-24

(seeFig.5B.2).

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FIGURE5B.2 Notethe“hingepoint”at6cmdiameter,atwhichthenaturalriskofanascending

aneurysmincreasesdramatically.

Reproducedwithpermissionfrom

ElefteriadesJA.Naturalhistoryofthoracicaorticaneurysms:indicationsforsurgery,andsurgicalversusnonsurgicalrisks.Ann

ThoracSurg.2002;74(5):S1877-S1880;discussionS1892-S1898.

iii.Currentdata,moregranularasthenumberofstudiedpatientshasgrown,haveshown hingepointsat5.75cmandagainat5.25cmsuggestingthatcriteriaforinterventionshouldbe movedleftwardtosmallersizes.

b.Clinicalfeaturesofanacuteaorticevent(ruptureordissection)dependonthelocationof thepathology.Generally,ascendingaorticdissectionsproducetearing,severe,substernal pain.Generally,descendingaorticaneurysmsproducesevereinterscapularpain,whichoften radiatesandprogressescaudallydownthebody.

3.CriteriaforSurgicalIntervention

a. The generally accepted criterion for intervention in patients with chronic ascending thoracic

aneurysms is a diameter of the ascending aorta greater than 5.5 cm. For institutions with large experience,whichcandeliveroperationatlow risk,5.0cmisanacceptedcriterion. However,a criticalpointforemphasisisthatdimensionalcriteriaapplyforasymptomaticaneurysmsonly.

b. Symptomatic aneurysms require operation regardless of size, as pain in an aneurysm portends

rupture.Itissaidthatrapidgrowthofthethoracicaorta,atorabove0.5cm/y,requiresoperation.In matteroffact,suchrapidgrowthisextremelyrareinthethoracicaorta.

c. Putative growth at this rate is usually due to measurement error (oblique measurements or

comparison on noncorresponding aortic segments). Furthermore, connective tissue diseases and familialTAAsmayrequireamoreaggressivesurgicalapproach,becausedissections canoccurat quitesmallsizes.

d. Weoftenoperateevenbefore5cmforpatientswithMarfansyndrome,Loeys-Dietz,Ehlers-Danlos,

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andotherinheritedaortopathies(seeFig.5B.3).26InTurnersyndrome,wheredissectioncanoccur suddenly at small sizes, surgery canbe considered when the ascending aorta reaches 3.5 cm or greater

1,13

(Table5B.4).

FIGURE5B.3 Simplifiedschematicillustrationofascendingaortadimensionsforprophylacticsurgicalintervention

dividedbygenecategory:ECMgenes,SMCcontractileunitandmetabolismgenes,andTGF-βsignalingpathway

genes.ECM,extracellularmatrix;LDS,Loeys-Dietzsyndrome;MFS,Marfansyndrome;SMC,smoothmusclecell;

EDS,Ehlers-Danlossyndrome.

Reproducedwithpermissionfrom

BrownsteinAJ,ZiganshinBA,KuivaniemiH,BodySC,BaleAE,ElefteriadesJA.Genesassociatedwiththoracicaorticaneurysmanddissection:an

updateandclinicalimplications.Aorta(Stamford).2017;5(1):11-20.

Table5B.4

IndicationsforSurgeryAccordingtoAscendingAorticDiameter

Asce ndingaorticdiameteratwhichsurgeryisrecommended

TAA(noothercoe xistingconditions) 5.5cm BAV,Marfan,familialTAA 5cm Loe ys-Die tz 4.4-4.6cmbyCTorMRI;4.2cmbyTEE Turnersyndrome >3.5cm

4.SurgicalProcedures

a. Surgicaltreatmentforascendingaorticaneurysmsusuallyinvolvesresectionandgrafting+/−aortic

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