主动脉疾病英文.pptx

DiseasesoftheAortaFredWu,M.D.FredWu,M.D.April19,2006April19,2006Categoriesofaorticdiseasen nAneurysmn nPseudoaneurysmn nDissectionn nPenetratingatheroscleroticulcern nIntramuralhematoman nAtheroscleroticdiseasen nCoarctationThoracicaorticaneurysmsnAortopathyduetoheritablediseases(20%ofaneurysms)nMarfansyndromenEhlers-DanlossyndromenFamilialAnnuloaorticectasianTurnersyndromenNoonansyndromenOsteogenesisimperfectanBicuspidaorticvalvenCoarctationoftheaortanMetabolicdisorders(eg,homocystinuria,familialhypercholesterolemia)nAorticarteritis(Takayasus,giant-cell)nTraumanPregnancynSyphilis/infectiousnCrackcocaineusenIatrogeniccauses(eg,cardiaccatheterization)nAtherosclerosisnHypertensionMarfanSyndromen nAutosomaldominantAutosomaldominantinheritanceinheritancen nMissensemutationintheMissensemutationinthefibrillingeneonfibrillingeneonchromosome15chromosome15n nPrevalenceis1in10,000Prevalenceis1in10000(25%sporadic)(25%sporadic)n nLeadingcauseofLeadingcauseofprematuredeathisaorticprematuredeathisaorticrootaneurysmleadingtorootaneurysmleadingtodissection(cysticmedialdissection(cysticmedialdegeneration)degeneration)MarfanSyndromen nGhentCriterian nIndexcaseIndexcasen nMajorcriteriain2differentorgansystemsMajorcriteriain2differentorgansystemsandinvolvementinathirdorgansystemandinvolvementinathirdorgansystemn nFamilyhistoryFamilyhistoryn nMajorcriterioninfamily/geneticsand1Majorcriterioninfamily/geneticsand1majorcriterioninanorgansystemandmajorcriterioninanorgansystemandinvolvementinasecondorgansysteminvolvementinasecondorgansystemMarfanSyndromen nSkeletalSkeletaln nMajor(atleast4items)Major(atleast4items)n nPectuscarinatumPectuscarinatumn nPectusexcavatumrequiringsurgeryPectusexcavatumrequiringsurgeryn nDecreaseduppertolowersegmentratioDecreaseduppertolowersegmentratioORincreasedarmspamtoheightratioORincreasedarmspamtoheightration nWristandthumbsignsWristandthumbsignsn nScoliosis20degreesScoliosis20degreesn nDecreasedelbowextensionDecreasedelbowextensionn nPesplanusPesplanusn nProtrusioacetabulaeProtrusioacetabulaen nMinorMinorn nPectusexcavatum(moderate)Pectusexcavatum(moderate)n nJointhypermobilityJointhypermobilityn nHigharchedpalatewithcrowdingofHigharchedpalatewithcrowdingofteethteethn nFacialappearanceFacialappearancen nOcularOcularn nMajorMajorn nEctopialentisEctopialentisn nMinorMinorn nAbnormallyflatcorneaAbnormallyflatcornean nIncreasedaxiallengthofglobeIncreasedaxiallengthofgloben nHypoplasticirisorciliarymuscleHypoplasticirisorciliarymusclen nSkin and integumentSkin and integumentn nMinoronlyMinoronlyn nStriaenotassociatedwithweightStriaenotassociatedwithweightchange,pregnancyorstresschange,pregnancyorstressn nRecurrentorincisionalherniasRecurrentorincisionalherniasn nPulmonaryPulmonaryn nMinoronlyMinoronlyn nSpontaneouspneumothoraxSpontaneouspneumothoraxn nApicalblebsApicalblebsn nDuraDuran nMajoronlyMajoronlyn nLumbosacralduralectasiaLumbosacralduralectasian nCardiovascularCardiovascularn nMajorMajorn nDilatationoftheascendingaortaDilatationoftheascendingaortainvolvingatleastthesinusesofValsalvainvolvingatleastthesinusesofValsalvan nDissectionoftheascendingaortaDissectionoftheascendingaortan nMinorMinorn nMitralvalveprolapsewithorwithoutMRMitralvalveprolapsewithorwithoutMRn nMitralannularcalcificationbeforeage40Mitralannularcalcificationbeforeage40n nDilationordissectionofdescendingDilationordissectionofdescendingaortabeforeage50aortabeforeage50n nDilationofthePAwithoutcausebeforeDilationofthePAwithoutcausebeforeage40age40n nFamilial/geneticFamilial/geneticn nMajoronlyMajoronlyn nFirstdegreerelativewhomeetscriteriaFirstdegreerelativewhomeetscriteriaindependentlyindependentlyn nPresenceofmutationinFBN1knowntoPresenceofmutationinFBN1knowntocauseMFScauseMFSn nPresenceofhaplotypearoundFBN1,PresenceofhaplotypearoundFBN1,inheritedbydescent,knowntobeinheritedbydescent,knowntobeassociatedwithunequivocallydiagnosedassociatedwithunequivocallydiagnosedMFSinfamilyMFSinfamilyMarfanSyndromen nSkeletalSkeletaln nMajor(atleast4items)Major(atleast4items)n nPectuscarinatumPectuscarinatumn nPectusexcavatumrequiringsurgeryPectusexcavatumrequiringsurgeryn nDecreaseduppertolowersegmentratioDecreaseduppertolowersegmentratioORincreasedarmspamtoheightratioORincreasedarmspamtoheightration nWristandthumbsignsWristandthumbsignsn nScoliosis20degreesScoliosis20degreesn nDecreasedelbowextensionDecreasedelbowextensionn nPesplanusPesplanusn nProtrusioacetabulaeProtrusioacetabulaen nMinorMinorn nPectusexcavatum(moderate)Pectusexcavatum(moderate)n nJointhypermobilityJointhypermobilityn nHigharchedpalatewithcrowdingofHigharchedpalatewithcrowdingofteethteethn nFacialappearanceFacialappearancen nOcularOcularn nMajorMajorn nEctopialentisEctopialentisn nMinorMinorn nAbnormallyflatcorneaAbnormallyflatcornean nIncreasedaxiallengthofglobeIncreasedaxiallengthofgloben nHypoplasticirisorciliarymuscleHypoplasticirisorciliarymusclen nSkin and integumentSkin and integumentn nMinoronlyMinoronlyn nStriaenotassociatedwithweightStriaenotassociatedwithweightchange,pregnancyorstresschange,pregnancyorstressn nRecurrentorincisionalherniasRecurrentorincisionalherniasn nPulmonaryPulmonaryn nMinoronlyMinoronlyn nSpontaneouspneumothoraxSpontaneouspneumothoraxn nApicalblebsApicalblebsn nDuraDuran nMajoronlyMajoronlyn nLumbosacralduralectasiaLumbosacralduralectasian nCardiovascularCardiovascularn nMajorMajorn nDilatationoftheascendingaortaDilatationoftheascendingaortainvolvingatleastthesinusesofValsalvainvolvingatleastthesinusesofValsalvan nDissectionoftheascendingaortaDissectionoftheascendingaortan nMinorMinorn nMitralvalveprolapsewithorwithoutMRMitralvalveprolapsewithorwithoutMRn nMitralannularcalcificationbeforeage40Mitralannularcalcificationbeforeage40n nDilationordissectionofdescendingDilationordissectionofdescendingaortabeforeage50aortabeforeage50n nDilationofthePAwithoutcausebeforeDilationofthePAwithoutcausebeforeage40age40n nFamilial/geneticFamilial/geneticn nMajoronlyMajoronlyn nFirstdegreerelativewhomeetscriteriaFirstdegreerelativewhomeetscriteriaindependentlyindependentlyn nPresenceofmutationinFBN1knowntoPresenceofmutationinFBN1knowntocauseMFScauseMFSn nPresenceofhaplotypearoundFBN1,PresenceofhaplotypearoundFBN1,inheritedbydescent,knowntobeinheritedbydescent,knowntobeassociatedwithunequivocallydiagnosedassociatedwithunequivocallydiagnosedMFSinfamilyMFSinfamilyFamilialTAASyndromesn nAtleast19%ofpatientswiththoracicaorticaneurysms(withoutovertconnectivetissuedisorders)haveafamilyhistoryn nTendtopresentyoungerthanthosepresentingsporadicallyn nGeneticallyheterogeneousBicuspidaorticvalven nInitially,ascendingthoracicaorticaneurysmsinBAVthoughttobeduetopoststenoticdilatationn n75%withcysticmedialnecrosisonpathologycomparedwith14%ofthosewithtricuspidaorticvalvesBicuspidaorticvalven nNistrietaldemonstrated52%incidenceofaorticdilatationinpatientswithnormallyfunctioningbicuspidaorticvalveSyphilitic(luetic)aortitisn nLatentperiodfrominitialspirochetalinfectionaverages10-30years(range5to40years)n nDuetodirectinfectionofaorticmedialeadingtoanobliterativeendarteritisofthevasavasorumanddestructionofcollagenandelastictissues(“treebarking”)TurnerSyndromen nAssociatedwithbicuspidaorticvalve(30%)andcoarctation.n n42%ofscreenedpatientsbyonestudywithaorticrootdilatationn nAorticrootdilatationcanbeseenwithorwithoutBAV,butwhenbotharepresentriskofdissectionisprobablycompoundedn nScreeningrecommendedevery5yrsAorticarteritisn nTakayasusarteritisn nTypicallycausesobliterativeluminalTypicallycausesobliterativeluminalchangeschangesn n15%ofpatientsmayhaveaortic15%ofpatientsmayhaveaorticdilatation/aneurysmsdilatation/aneurysmsn nGiant-cellarteritisn nUpto20%canhavethoracicorUpto20%canhavethoracicorabdominalaorticaneurysmsabdominalaorticaneurysmsThoracicaorticaneurysmsn nSymptoms:Symptoms:n nMajorityareasymptomaticatdiagnosisMajorityareasymptomaticatdiagnosisn nChestand/orbackpainChestand/orbackpainn nHoarsenessHoarsenessn nCough,dyspnea,stridorCough,dyspnea,stridorn nDysphagiaDysphagian nSigns:Signs:n nDistendedneckvein(s)Distendedneckvein(s)n nAorticregurgitationAorticregurgitationn nFixedvocalcordFixedvocalcordn nSystemicand/orcerebralembolismSystemicand/orcerebralembolismThoracicaorticaneurysmsn nDiagnosisDiagnosisn nChestX-ray(poorsensitivity)ChestX-ray(poorsensitivity)n nWidenedmediastinumWidenedmediastinumn nEnlargedaorticknobEnlargedaorticknobn nTrachealdeviationTrachealdeviationn nCT/MRangiographyCT/MRangiographyn nAccuratedetectionandsizingofthoracicaorticAccuratedetectionandsizingofthoracicaorticaneurysmsaneurysmsn nMRmaybepreferablyforinvolvementofaorticrootMRmaybepreferablyforinvolvementofaorticrootn nTTE/TEETTE/TEEn nTTEgenerallynotrecommendedfordiagnosingandTTEgenerallynotrecommendedfordiagnosingandsizingthoracicaorticaneurysmsexceptinpatientssizingthoracicaorticaneurysmsexceptinpatientswithMarfansyndromewithMarfansyndromen nInvasivenatureofTEEpreventsitfrombeingInvasivenatureofTEEpreventsitfrombeingimagingmodalityofchoiceimagingmodalityofchoiceThoracicaorticaneurysmsn nNaturalhistoryn nLongitudinaldatasuggestsmeanLongitudinaldatasuggestsmeanincreaseof0.1-0.25cm/yrincreaseof0.1-0.25cm/yrn nGreaterfordescendingvs.ascendingGreaterfordescendingvs.ascendingn nGreaterfordissectedvs.nondissectedGreaterfordissectedvs.nondissectedn nGreaterforMarfanvs.non-MarfanGreaterforMarfanvs.non-Marfann nCriticalsizes(initialaneurysmsize)Criticalsizes(initialaneurysmsize)n nAscending:6.0cm;31%riskofruptureAscending:6.0cm;31%riskofrupturen nDescending:7.0cm;43%riskofruptureDescending:7.0cm;43%riskofrupturen nYearlyeventrate(6.0cm):14.1%Yearlyeventrate(6.0cm):14.1%Daviesetal.AnnThoracSurg2002;73:17-28.Thoracicaorticaneurysmsn nSurgicalmanagementn nAscendingthoracicaorticaneurysms:Ascendingthoracicaorticaneurysms:5.5cm5.5cmn nThosewithincreasedoperativerisk:6cmThosewithincreasedoperativerisk:6cmn nMarfan/BAV:5cmorless(prophylacticMarfan/BAV:5cmorless(prophylacticreplacementwithaorticvalvesurgeryifreplacementwithaorticvalvesurgeryif4cmorgreater)4cmorgreater)n nDescendingthoracicaorticaneurysms:Descendingthoracicaorticaneurysms:6cm6cmn nSymptoms;rapidenlargementSymptoms;rapidenlargementThoracicaorticaneurysmsn nMedicalmanagementn nBloodpressureBloodpressuren nBeta-blockerBeta-blockern nInrandomizedstudyofadultwithMarfanInrandomizedstudyofadultwithMarfansyndrome,propranololover10yearsresultedinsyndrome,propranololover10yearsresultedinslowerrateofaorticdilatation,feweraorticslowerrateofaorticdilatation,feweraorticevents,andlowermortalityevents,andlowermortalityn nOtherantihypertensivesOtherantihypertensivesn nTargetSBP120mmHgorlessTargetSBP120mmHgorlessn n70adolescentsand70adolescentsandyoungadultswithyoungadultswithMFSMFSn nPropranololvsPropranololvsplaceboplacebon nSomeSomenonrespondersnonrespondersNEJM 1994;330:1335-1341Thoracicaorticaneurysmsn nMedicalmanagementn nAnti-oxidantsAnti-oxidantsn nSomestudiessuggestbenefitofaddingSomestudiessuggestbenefitofaddingstatinsand/orangiotensinIItypeIreceptorstatinsand/orangiotensinIItypeIreceptorblockers(noclinicaltrials)blockers(noclinicaltrials)n nExerciselimitationsExerciselimitationsn nNostrenuousisometricexertionNostrenuousisometricexertionn nAerobicexercisegenerallysafeprovidedAerobicexercisegenerallysafeprovidedthereisnohypertensiveresponsetothereisnohypertensiveresponsetoexercise(180mmHg)exercise(180mmHg)n nDiscontinuetobaccoDiscontinuetobaccoThoracicaorticaneurysmsn nSerialImagingn nAllpatientsshouldgetatleastoneAllpatientsshouldgetatleastonebaselineimagingstudythatincludesthebaselineimagingstudythatincludestheabdominalaortaabdominalaortan nRepeatimaging6monthsafterinitialRepeatimaging6monthsafterinitialdiagnosisdiagnosisn nDependingonrateofchange,Dependingonrateofchange,subsequentstudiescanbeperformedatsubsequentstudiescanbeperformedat3,6or12monthintervals3,6or12monthintervalsAbdominalaorticaneurysmsAbdominalaorticaneurysmsn nVACooperativeStudyfounda1.3%incidenceofVACooperativeStudyfounda1.3%incidenceofabdominalaorticaneurysmslargerthan4.0cmonabdominalaorticaneurysmslargerthan4.0cmonscreeningof125,000patientsage50orolderscreeningof125,000patientsage50oroldern nItisestimatedthatonly50%ofAAAsareItisestimatedthatonly50%ofAAAsarecurrentlydiagnosedcurrentlydiagnosedn nHighmortality65-85%forrupturedabdominalHighmortality65-85%forrupturedabdominalaorticaneurysmsaorticaneurysmsn nRuptureofabdominalaorticaneurysmscausesRuptureofabdominalaorticaneurysmscauses15,000deathsperyearintheUS15,000deathsperyearintheUSLederleetal,ArchInternMed2000;160:1425-30.AbdominalaorticaneurysmsRiskfactorRiskfactorORORSmokinghistorySmokinghistory5.175.17FamilyhistoryFamilyhistory1.91.9Increasedage,per7yearsIncreasedage,per7years1.71.7EstablishedatherosclerosisEstablishedatherosclerosis1.61.6BlackraceBlackrace0.590.59DiabetesmellitusDiabetesmellitus0.500.50FemalegenderFemalegender0.180.18RiskfactorsforpresenceofabdominalaorticaneurysmLederleetal,ArchInternMed2000;160:1425-30.Abdominalaorticaneurysmn nAtherosclerosisbreakdownofcollagen,elastinAtherosclerosisbreakdownofcollagen,elastinn nSmoking,hypertension,hyperlipidemiaSmoking,hypertension,hyperlipidemian nConnectivetissuedisordersConnectivetissuedisordersn nMarfanSyndromeMarfanSyndromen nEhlers-DanlossyndromeEhlers-Danlossyndromen nVascularinflammationVascularinflammationn nAorticarteritis(Takayasus,giant-cell)Aorticarteritis(Takayasus,giant-cell)n nBehcetsBehcetsn nTuberculosisTuberculosisn nBrucellosis,salmonellosisBrucellosis,salmonellosisn nTraumaTrauman nIatrogeniccauses(eg,cardiaccatheterizationorIatrogeniccauses(eg,cardiaccatheterizationorprevioussurgery)previoussurgery)Abdominalaorticaneurysmsn nUnrupturedUnrupturedn nUsuallyasymptomatic,diagnosedincidentallyUsuallyasymptomatic,diagnosedincidentallyn nAwarenessofheartbeatinabdomenAwarenessofheartbeatinabdomenn nChronicvagueabdominal/backpainChronicvagueabdominal/backpainn nDistalembolisationDistalembolisationn nUerterohydronephrosisUerterohydronephrosisn nRupturedRupturedn nComplaintofpainComplaintofpainn nUsuallysuddenonsetUsuallysuddenonsetn nOfteninabdomen,butalsolowerbackorflankOfteninabdomen,butalsolowerbackorflankn nMayradiatetobuttocks,groin,testiclesorlegMayradiatetobuttocks,groin,testiclesorlegn nMaybeconstant,throbbingorcolickyMaybeconstant,throbbingorcolickyn nShockShockn nPulsatileabdominalmassPulsatileabdominalmassn nSyncopeorlightheadednessSyncopeorlightheadednessAbdominalaorticaneurysmsn nPhysicalexamPalpationofsupraumbilicalPhysicalexamPalpationofsupraumbilicalareaforpulsatilemassareaforpulsatilemassn n3.0-3.9cm3.0-3.9cmSens61%Sens61%n n4.0-4.9cm4.0-4.9cmSens69%Sens69%n n 5.0cm5.0cmSens82%Sens82%n nImaging(62%ofcasesfoundincidentally)Imaging(62%ofcasesfoundincidentally)n nUltrasoundshouldwebescreening?Ultrasoundshouldwebescreening?n nCT/CTangioCT/CTangion nMRI/MRAMRI/MRAn nContrastangiographyContrastangiographyAbdominalaorticaneurysms9.4%10.2%32.5%n=52n=85n=61JAMA2002;287:2968JAMA2002;287:2968NaturalhistoryNaturalhistoryAbdominalaorticaneurysmsn nSurgicalindications:Surgicalindications:n nRuptureorimpendingrupture(90%mortality)Ruptureorimpendingrupture(90%mort