主动脉疾病.ppt

DiseasesoftheAorta,FredWu,M.D.April19,2019,Categoriesofaorticdisease,AneurysmPseudoaneurysmDissectionPenetratingatheroscleroticulcerIntramuralhematomaAtheroscleroticdiseaseCoarctation,Thoracicaorticaneurysms,Aortopathyduetoheritablediseases(20%ofaneurysms)MarfansyndromeEhlers-DanlossyndromeFamilialAnnuloaorticectasiaTurnersyndromeNoonansyndromeOsteogenesisimperfectaBicuspidaorticvalveCoarctationoftheaortaMetabolicdisorders(eg,homocystinuria,familialhypercholesterolemia)Aorticarteritis(Takayasus,giant-cell)TraumaPregnancySyphilis/infectiousCrackcocaineuseIatrogeniccauses(eg,cardiaccatheterization)AtherosclerosisHypertension,MarfanSyndrome,AutosomaldominantinheritanceMissensemutationinthefibrillingeneonchromosome15Prevalenceis1in10,000(25%sporadic)Leadingcauseofprematuredeathisaorticrootaneurysmleadingtodissection(cysticmedialdegeneration),MarfanSyndrome,GhentCriteriaIndexcaseMajorcriteriain2differentorgansystemsandinvolvementinathirdorgansystemFamilyhistoryMajorcriterioninfamily/geneticsand1majorcriterioninanorgansystemandinvolvementinasecondorgansystem,MarfanSyndrome,SkeletalMajor(atleast4items)PectuscarinatumPectusexcavatumrequiringsurgeryDecreaseduppertolowersegmentratioORincreasedarmspamtoheightratioWristandthumbsignsScoliosis20degreesDecreasedelbowextensionPesplanusProtrusioacetabulaeMinorPectusexcavatum(moderate)JointhypermobilityHigharchedpalatewithcrowdingofteethFacialappearanceOcularMajorEctopialentisMinorAbnormallyflatcorneaIncreasedaxiallengthofglobeHypoplasticirisorciliarymuscleSkinandintegumentMinoronlyStriaenotassociatedwithweightchange,pregnancyorstressRecurrentorincisionalhernias,PulmonaryMinoronlySpontaneouspneumothoraxApicalblebsDuraMajoronlyLumbosacralduralectasiaCardiovascularMajorDilatationoftheascendingaortainvolvingatleastthesinusesofValsalvaDissectionoftheascendingaortaMinorMitralvalveprolapsewithorwithoutMRMitralannularcalcificationbeforeage40Dilationordissectionofdescendingaortabeforeage50DilationofthePAwithoutcausebeforeage40Familial/geneticMajoronlyFirstdegreerelativewhomeetscriteriaindependentlyPresenceofmutationinFBN1knowntocauseMFSPresenceofhaplotypearoundFBN1,inheritedbydescent,knowntobeassociatedwithunequivocallydiagnosedMFSinfamily,MarfanSyndrome,SkeletalMajor(atleast4items)PectuscarinatumPectusexcavatumrequiringsurgeryDecreaseduppertolowersegmentratioORincreasedarmspamtoheightratioWristandthumbsignsScoliosis20degreesDecreasedelbowextensionPesplanusProtrusioacetabulaeMinorPectusexcavatum(moderate)JointhypermobilityHigharchedpalatewithcrowdingofteethFacialappearanceOcularMajorEctopialentisMinorAbnormallyflatcorneaIncreasedaxiallengthofglobeHypoplasticirisorciliarymuscleSkinandintegumentMinoronlyStriaenotassociatedwithweightchange,pregnancyorstressRecurrentorincisionalhernias,PulmonaryMinoronlySpontaneouspneumothoraxApicalblebsDuraMajoronlyLumbosacralduralectasiaCardiovascularMajorDilatationoftheascendingaortainvolvingatleastthesinusesofValsalvaDissectionoftheascendingaortaMinorMitralvalveprolapsewithorwithoutMRMitralannularcalcificationbeforeage40Dilationordissectionofdescendingaortabeforeage50DilationofthePAwithoutcausebeforeage40Familial/geneticMajoronlyFirstdegreerelativewhomeetscriteriaindependentlyPresenceofmutationinFBN1knowntocauseMFSPresenceofhaplotypearoundFBN1,inheritedbydescent,knowntobeassociatedwithunequivocallydiagnosedMFSinfamily,FamilialTAASyndromes,Atleast19%ofpatientswiththoracicaorticaneurysms(withoutovertconnectivetissuedisorders)haveafamilyhistoryTendtopresentyoungerthanthosepresentingsporadicallyGeneticallyheterogeneous,Bicuspidaorticvalve,Initially,ascendingthoracicaorticaneurysmsinBAVthoughttobeduetopoststenoticdilatation75%withcysticmedialnecrosisonpathologycomparedwith14%ofthosewithtricuspidaorticvalves,Bicuspidaorticvalve,Nistrietaldemonstrated52%incidenceofaorticdilatationinpatientswithnormallyfunctioningbicuspidaorticvalve,Syphilitic(luetic)aortitis,Latentperiodfrominitialspirochetalinfectionaverages10-30years(range5to40years)Duetodirectinfectionofaorticmedialeadingtoanobliterativeendarteritisofthevasavasorumanddestructionofcollagenandelastictissues(“treebarking”),TurnerSyndrome,Associatedwithbicuspidaorticvalve(30%)andcoarctation.42%ofscreenedpatientsbyonestudywithaorticrootdilatationAorticrootdilatationcanbeseenwithorwithoutBAV,butwhenbotharepresentriskofdissectionisprobablycompoundedScreeningrecommendedevery5yrs,Aorticarteritis,TakayasusarteritisTypicallycausesobliterativeluminalchanges15%ofpatientsmayhaveaorticdilatation/aneurysmsGiant-cellarteritisUpto20%canhavethoracicorabdominalaorticaneurysms,Thoracicaorticaneurysms,Symptoms:MajorityareasymptomaticatdiagnosisChestand/orbackpainHoarsenessCough,dyspnea,stridorDysphagiaSigns:Distendedneckvein(s)AorticregurgitationFixedvocalcordSystemicand/orcerebralembolism,Thoracicaorticaneurysms,DiagnosisChestX-ray(poorsensitivity)WidenedmediastinumEnlargedaorticknobTrachealdeviationCT/MRangiographyAccuratedetectionandsizingofthoracicaorticaneurysmsMRmaybepreferablyforinvolvementofaorticrootTTE/TEETTEgenerallynotrecommendedfordiagnosingandsizingthoracicaorticaneurysmsexceptinpatientswithMarfansyndromeInvasivenatureofTEEpreventsitfrombeingimagingmodalityofchoice,Thoracicaorticaneurysms,NaturalhistoryLongitudinaldatasuggestsmeanincreaseof0.1-0.25cm/yrGreaterfordescendingvs.ascendingGreaterfordissectedvs.nondissectedGreaterforMarfanvs.non-MarfanCriticalsizes(initialaneurysmsize)Ascending:6.0cm;31%riskofruptureDescending:7.0cm;43%riskofruptureYearlyeventrate(6.0cm):14.1%,Daviesetal.AnnThoracSurg2019;73:17-28.,Thoracicaorticaneurysms,SurgicalmanagementAscendingthoracicaorticaneurysms:5.5cmThosewithincreasedoperativerisk:6cmMarfan/BAV:5cmorless(prophylacticreplacementwithaorticvalvesurgeryif4cmorgreater)Descendingthoracicaorticaneurysms:6cmSymptoms;rapidenlargement,Thoracicaorticaneurysms,MedicalmanagementBloodpressureBeta-blockerInrandomizedstudyofadultwithMarfansyndrome,propranololover10yearsresultedinslowerrateofaorticdilatation,feweraorticevents,andlowermortalityOtherantihypertensivesTargetSBP120mmHgorless,70adolescentsandyoungadultswithMFSPropranololvsplaceboSomenonresponders,NEJM1994;330:1335-1341,Thoracicaorticaneurysms,MedicalmanagementAnti-oxidantsSomestudiessuggestbenefitofaddingstatinsand/orangiotensinIItypeIreceptorblockers(noclinicaltrials)ExerciselimitationsNostrenuousisometricexertionAerobicexercisegenerallysafeprovidedthereisnohypertensiveresponsetoexercise(180mmHg)Discontinuetobacco,Thoracicaorticaneurysms,SerialImagingAllpatientsshouldgetatleastonebaselineimagingstudythatincludestheabdominalaortaRepeatimaging6monthsafterinitialdiagnosisDependingonrateofchange,subsequentstudiescanbeperformedat3,6or12monthintervals,Abdominalaorticaneurysms,Abdominalaorticaneurysms,VACooperativeStudyfounda1.3%incidenceofabdominalaorticaneurysmslargerthan4.0cmonscreeningof125,000patientsage50orolderItisestimatedthatonly50%ofAAAsarecurrentlydiagnosedHighmortality65-85%forrupturedabdominalaorticaneurysmsRuptureofabdominalaorticaneurysmscauses15,000deathsperyearintheUS,Lederleetal,ArchInternMed2000;160:1425-30.,Abdominalaorticaneurysms,Riskfactorsforpresenceofabdominalaorticaneurysm,Lederleetal,ArchInternMed2000;160:1425-30.,Abdominalaorticaneurysm,Atherosclerosisbreakdownofcollagen,elastinSmoking,hypertension,hyperlipidemiaConnectivetissuedisordersMarfanSyndromeEhlers-DanlossyndromeVascularinflammationAorticarteritis(Takayasus,giant-cell)BehcetsTuberculosisBrucellosis,salmonellosisTraumaIatrogeniccauses(eg,cardiaccatheterizationorprevioussurgery),Abdominalaorticaneurysms,UnrupturedUsuallyasymptomatic,diagnosedincidentallyAwarenessofheartbeatinabdomenChronicvagueabdominal/backpainDistalembolisationUerterohydronephrosisRupturedComplaintofpainUsuallysuddenonsetOfteninabdomen,butalsolowerbackorflankMayradiatetobuttocks,groin,testiclesorlegMaybeconstant,throbbingorcolickyShockPulsatileabdominalmassSyncopeorlightheadedness,Abdominalaorticaneurysms,PhysicalexamPalpationofsupraumbilicalareaforpulsatilemass3.0-3.9cmSens61%4.0-4.9cmSens69%5.0cmSens82%Imaging(62%ofcasesfoundincidentally)Ultrasoundshouldwebescreening?CT/CTangioMRI/MRAContrastangiography,Abdominalaorticaneurysms,9.4%,10.2%,32.5%,n=52,n=85,n=61,JAMA2019;287:2968,Naturalhistory,Abdominalaorticaneurysms,Surgicalindications:Ruptureorimpendingrupture(90%mortality)Inpatientswiththeclinicaltriadofabdominaland/orbackpain,apulsatileabdominalmass,andhypotension,immediatesurgicalevaluationisindicated.(LevelofEvidence:B)ClassIPatientswithinfrarenalorjuxtarenalAAAsmeasuring5.5cmorlargershouldundergorepairtoeliminatetheriskofrupture.(LevelofEvidence:B)Inpatientswithsymptomatic(e.g.embolization,ureteralcompresion)aorticaneurysms,repairisindicatedregardlessofdiameter.(LevelofEvidence:C),Abdominalaorticaneurysms,Surgicalindications:ClassIIa:RepaircanbebeneficialinpatientswithinfrarenalorjuxtarenalAAAs5.0to5.4cmindiameter(LevelofEvidence:B)RepairisprobablyindicatedinpatientswithsuprarenalortypeIVthoracoabdominalaorticaneurysmslargerthan5.5to6.0cm.(LevelofEvidence:B)ClassIII:InterventionisnotrecommendedforasymptomaticinfrarenalorjuxtarenalAAAsiftheymeasurelessthan5.0cmindiameterinmenorlessthan4.5cmindiameterinwomen.(LevelofEvidence:A)Notmentioned:Rapidexpansion(1.0cmin12months)Inflammatoryofinfectiousetiology,Abdominalaorticaneurysms,Endovascularrepair:EliminatesneedformajortransabdominalsurgeryCanbeperformedunderregionalorlocalanesthesiaMaybeusefulinsettingofseverecardiopulmonarydisease,advancedage,morbidobesityorhistoryofmultipleabdominalsurgeries,Abdominalaorticaneurysms,Endovascularrepair:Patientsneedadequatelengthofrelativelynoralaortabelowtherenalarteriesbecauseofhighriskforproximalattachmentfailure,graftmigration,andendoleakBecauseofinflexibilityofthesegrafts,thesegmentofaortacannotbeseverelyangulated.,Abdominalaorticaneurysms,Endograftindications:ClassI:OpenrepairofinfrarenalAAAsand/orcommoniliacaneurysmsisindicatedinpatientswhoaregoodoraveragesurgicalcandidates(LevelofEvidence:B)Periodiclong-termsurveillanceimagingshouldbeperformedtomonitorforanendoleak,todocumentshrinkageorstabilityoftheexcludedaneurysmsac,andtodeterminetheneedforfurtherinterventioninpatientswhohaveundergoneendovascularrepairofintrarenalaorticand/oriliacaneurysms(LevelofEvidence:B),Abdominalaorticaneurysms,Endograftindications:ClassIIa:Endovascularrepairininfrarenalaorticand/orcommoniliacaneurysmsisreasonableinpatientsathighriskofcomplicationsfromopenoperationsbecauseofcardiopulmonaryorotherassociateddiseases.(LevelofEvidence:B)ClassIIb:Endovascularrepairofinfrarenalaorticand/orcommoniliacaneurysmsmaybeconsideredinpatientsatloworaveragesurgicalrisk.(LevelofEvidence:B),Abdominalaorticaneurysms,Medicaltherapy:ClassIInpatientswithAAAs,bloodpressureandfastingserumlipidvaluesshouldbemonitoredandcontrolledasrecommendedforpatientswithatheroscleroticdisease.(LevelofEvidence:C)Patientswithaneurysmsorafamilyhistoryofaneurysmsshouldbeadvisedtostopsmokingandbeofferedsmokingcessationinterventions,includingbehaviormodification,nicotinereplacement,orbupropion.(LevelofEvidence:B),Abdominalaorticaneurysms,Medicaltherapy:ClassIPerioperativeadministrationofbeta-adrenergicblockingagents,intheabsenceofcontraindications,isindicatedtoreducetheriskofadversecardiaceventsandmortalityinpatientswithcoronaryarterydiseaseundergoingsurgicalrepairofatheroscleroticaorticaneurysms.(LevelofEvidence:A)ClassIIbBeta-adrenergicblockingagentsmaybeconsideredtoreducetherateofaneurysmexpansioninpatientswithaorticaneurysms.(LevelofEvidence:B),Abdominalaorticaneurysms,Surveillance:ClassIPatientswithinfrarenalorjuxtarenalAAAsmeasuring4.0to5.4cmindiametershouldbemonitoredbyultrasoundorCTscansevery6to12monthstodetectexpansion.(LevelofEvidence:A)ClassIIaInpatientswithAAAssmallerthan4.0cmindiameter,monitoringbyultrasoundexaminationevery2to3yearsisreasonable.(LevelofEvidence:B),Abdominalaorticaneurysms,Screening:ClinicalexaminationlackssensitivityEarlydetectionreducesmortalityClassIMen60yearsofageorolderwhoareeitherthesiblingsoroffspringofpatientswithAAAsshouldundergophysicalexaminationandultrasoundscreeningfordetectionofaorticaneurysms.(LevelofEvidence:B)ClassIIaMenwhoare65to75yearsofagewhohaveeversmokedshouldundergoaphysicalexaminationand1-timeultrasoundscreeningfordetectionofAAAs.(LevelofEvidence:B),Abdominalaorticaneurysms,HypertensionandothercardiovascularriskfactorsshouldbetreatedeffectivelydiscontinuetobaccoTreatassociatedcoronaryandcarotiddiseaseAneurysmwithdiameterover3cmshouldbemonitoredevery12months.When5cminmanor4.5cminwoman,shouldbefollowedevery6monthsSurgerywhenaneurysmexceeds5.5cm(UKSmallAneurysmTrial,NEJM2019;348:1895-1901)About1%ofaneurysmswithdiameterof4cmruptureannuallycomparedwith10%ofaneurysms6cmormore.Mortalitywithruptureis90%Elderlybrothersofpatientswithknownaneurysmsshouldbescreened(Salo,Anninternmed2019;130:637-42.,Inflammatorytype2-4%ofallAAAClassictriadofabdominal/backpain,weightloss,andelevatedESRObstructiveuropathyDiagnosis:ultrasound,CT,MRIUnusuallythickenedaneurysmwallShinywhiteperianeurysmalfibrosisIntenseadherencetoadjacentintraabdominalstructuresAccumulationofmacrophagesandcytokinesinaneurysmalaortictissueTreatmentsurgicalgraftingregardlessofsizeRoleofcorticosteroids?,Acuteaorticsyndromes,AorticdissectionPenetratingaorticulcerIntramuralhematoma,Acuteaorticsyndromes,Aorticdissection83-95%PenetratingaorticulcerIntramuralhematoma,5-17%,Acuteaorticsyndromes,9/11/036:10pmAftercollapsingatwork,a54yomaleisbroughttoEDwithcomplaintsofchestpain,nausea,vomitinganddizziness9/11/038:00pmInitiallydiagnosedwithMI,patientisbroughttocathlab.Therea“largeascendingaorticaneurysmwithseveredissection”isfound.9/11/0310:48pmWhileintheoperatingroom,patientarrestsandcannotberesuscitated.,Acuteaorticsyndromes,9/3/04Familyfileslawsuitaccusinghospitalofnegligence,claimingthatthepatientwasmisdiagnosed“atleasttwiceandunderwentimproperandunnecessaryprocedures”duringhishospitalstay.Claimeddamagesareinexcessof$25million3/16/06Partiesreachsettlementfor“alargemonetarysettlement”ofanundisclosedamount.,Aorticdissection,CommonfatalaorticconditionHighmortalityWithmedicaltherapy,1%dieperhourinthefirst24hours50%dieinfirst2weeksCorrectclinicaldiagnosisin15-43%ofcasesDiagnosticdelayof24hoursinupto39%ofcases,Klompasetal,JAMA2019;287:2262-2272.,Aorticdissection,ClinicalpresentationSuddenseverepain-90%Migratingpain31%Tearingpain39%(spec.95%)Nopain4.5%Hypertension49%Diastolicmurmur28%PulsedeficitsorBPdifferential31%Focalneurologicdeficits17%Syncope13%ECGcriteriaforAMI7%,Thereisnodiseasemoreconducivetoclinicalhumilitythananeurysmoftheaorta.SirWilliamOsler,c.1900,Klompasetal,JAMA2019;287:2262-2272.Nallamothyetal,AmJMed2019;113:468-471.,Aorticdissection,Long-standinghypertensionSmoking,dyslipidemia,crackcocaineuseConnectivetissuedisordersMarfansyndromeEhlers-DanlossyndromeBicuspidaorticvalveCoarctationoftheaortaFamilialAnnuloaorticectasiaVascularinflammationAorticarteritis(Takayasus,giant-cell)SyphilisTraumaIatrogeniccauses(eg,cardiaccatheterizationorprevioussurgery),Aorticdissection,IncidenceofmajorriskfactorsinaorticdissectionHypertension50-90%BicuspidAoV9-13%Marfansyndrome2.6-4.9%UnicuspidAoV2.8%Menmostcommonlyaffected(2/3ofcases),meanage63yrsYoungpatients(87%(ClassI)MaybemoreaccuratethanMRIorTEEindetectionofarchvesselinvolvementMRI/MRAHighestsensitivityandspecificity(almost100%)Onlypracticalinstablepatients(ClassIIa)TTE/TEEPreferredmodalityinunstablepatients(ClassI)Sens99%,spec89%Aortography(rare)(ClassIIa)Sens90%,spec95%,EurHeartJ2019;22(18):1642-81.,Aorticdissection,Aorticdissection,JAMA2000;283:897-903.,Aorticdissection,AcuteTypeA(DeBakeyTypeIorII)Surgicalemergencyaimtoavoidtamponadeorrupture(ClassI)ImplantationofacompositegraftwithorwithoutreimplantationofcoronaryarteriesRestorationofaorticvalvecompetenceinpatientswithAI(resuspensionvs.replacement)Withoutsurgery,20%mortalityby24hoursand30%by48hoursOperativemortalityvariesfrom15%to35%,EurHeartJ2019;22(18):1642-81.,Aorticdissection,AcuteTypeB(DeBakeyTypeIII)Medicalmanagement-Instablepatients,surgeryoffersnoprovenbenefit(ClassI)InvasivehemodynamicmonitoringBeta-blockadeArterialvasodilatorstokeepSBP5-6cmTypeB(TypeIII)Surgeryifsymptomsorprogressiveaorticenlargementto6cmClassIIaTypeB(TypeIII)Endovascularstentingifsurgicalindicationan