主動脈疾病-英文主動脈疾病-英文1DiseasesoftheaortaFredWU,MDApril19,2006Diseasesoftheaorta2CategoriesofaorticdiseaseAneurysmaPseudoaneurysmDissectionaPenetratingatheroscleroticulceraIntramuralhematomaAtheroscleroticdiseaseCoarctationCategoriesofaorticdisease3主動脈疾病-英文課件4ThoracicaorticaneurysmsAortopathyduetoheritablediseases(20%ofaneurysms)MarfansyndromeEhlers-DanlossyndromeFamilialAnnuloaorticectasiaaTurnersyndromeNoonansyndromeOsteogenesisimperfectaBicuspidaorticvalveCoarctationoftheaortaMetabolicdisorders(eg,homocystinuria,familialhypercholesterolemiaAorticarteritis(Takayasu's,giant-cell)TraumaPrenanSyphilis/infectiousCrackcocaineuseIatrogeniccauses(eg,cardiaccatheterization)AtherosclerosisHypertensionThoracicaorticaneurysms5MarfanSyndromeAutosomaldominantinheritanceMissensemutationinthefibrillingeneonchromosome15aPrevalenceis1in10,000(25%sporadaLeadingcauseofprematuredeathisaorticrootaneurysmleadingtodissection(cysticmedialdegeneration)MarfanSyndrome6MarfanSyndromeaGhentCriteriaIndexcaseaMajorcriteriain2differentorgansystemsandinvolvementinathirdorgansystemaFamilyhistoryaMajorcriterioninfamily/geneticsand1majorcriterioninanorgansystemandinvolvementinasecondorgansystemMarfanSyndrome7MarfanSyndromeSkeletalMajor(atleast4itemsMinoronlyPectuscannatumSpontaneouspneumothoraxLumbosacralduralectDecreasedelbowextensionCardiovascularProtrusioacetabularMinorDilatationoftheascendingaortavalsalvaDissectionoftheascendingaoMinorgharchedpalatewithcrowdingofMitralvalveprolapsewithorwithoutMFacialappearanceMitralannularcalcificationbeforeageOcularMajorDilationofthePAwithoutcausebeforeEctopialentisAbnormallyflatcornejoronlystdegreerelativewhoHypoplasticirisorciliarymuscleSkinandintegumentmutationinFBNlknowntoMinoronlyStriaenotassoclatedwithweightchange,pregnancyorstressMFSinfamilyRecurrentorincisionalherniasMarfanSyndrome8MarfanSyndromeSkeletalPulmonaryaMajor(atleast4items)MinoroWristandthumbCardiovascularnPesplanusacetabularDilatationoftheascendingaortaMinorvalsalvacum(moderate)DissectionoftheascendingMinorHigharchedpaMitralvalveprolapsewithorwithoutMaFacalMitralannularcalcificationbeforeageOcularofthePAwithoutcausebeforeMinorAbnormallyflatcorneaSkinandaMinoronlyStiaenotasMarfanSyndrome9FamilialTAASyndromesaAtleast19%ofpatientswiththoracicaorticaneurysms(withoutovertconnectivetissuedisorders)haveafamilyhistoryaTendtopresentyoungerthanthosepresentingsporadicallyaGeneticallyheterogeneousFamilialTAASyndromes10BicuspidaorticvalveaInitially,ascendingthoracicaorticaneurysmsinBAVthoughttobeduetopoststenoticdilatationa75%withcysticmedialnecrosisonpathologycomparedwith14%ofthosewithtricuspidaorticvalvesBicuspidaorticvalve11主動脈疾病-英文課件12主動脈疾病-英文課件13主動脈疾病-英文課件14主動脈疾病-英文課件15主動脈疾病-英文課件16主動脈疾病-英文課件17主動脈疾病-英文課件18主動脈疾病-英文課件19主動脈疾病-英文課件20主動脈疾病-英文課件21主動脈疾病-英文課件22主動脈疾病-英文課件23主動脈疾病-英文課件24主動脈疾病-英文課件25主動脈疾病-英文課件26主動脈疾病-英文課件27主動脈疾病-英文課件28主動脈疾病-英文課件29主動脈疾病-英文課件30主動脈疾病-英文課件31主動脈疾病-英文課件32主動脈疾病-英文課件33主動脈疾病-英文課件34主動脈疾病-英文課件35主動脈疾病-英文課件36主動脈疾病-英文課件37主動脈疾病-英文課件38主動脈疾病-英文課件39主動脈疾病-英文課件40主動脈疾病-英文課件41主動脈疾病-英文課件42主動脈疾病-英文課件43主動脈疾病-英文課件44主動脈疾病-英文課件45主動脈疾病-英文課件46主動脈疾病-英文課件47主動脈疾病-英文課件48主動脈疾病-英文課件49主動脈疾病-英文課件50主動脈疾病-英文課件51主動脈疾病-英文課件52主動脈疾病-英文課件53主動脈疾病-英文課件54主動脈疾病-英文課件55主動脈疾病-英文課件56主動脈疾病-英文課件57主動脈疾病-英文課件58主動脈疾病-英文課件59主動脈疾病-英文課件60主動脈疾病-英文課件61主動脈疾病-英文課件62主動脈疾病-英文課件63主動脈疾病-英文課件64主動脈疾病-英文課件65主動脈疾病-英文課件66主動脈疾病-英文課件67主動脈疾病-英文課件68主動脈疾病-英文課件69主動脈疾病-英文課件70主動脈疾病-英文課件71主動脈疾病-英文課件72主動脈疾病-英文課件73主動脈疾病-英文課件74謝謝你的閱讀知識就是財富豐富你的人生71、既然我已經(jīng)踏上這條道路，那么，任何東西都不應(yīng)妨礙我沿著這條路走下去。——康德

72、家庭成為快樂的種子在外也不致成為障礙物但在旅行之際卻是夜間的伴侶?！魅_

73、堅(jiān)持意志偉大的事業(yè)需要始終不渝的精神?！鼱柼?/p>

74、路漫漫其修道遠(yuǎn)，吾將上下而求索。——屈原

75、內(nèi)外相應(yīng)，言行相稱?！n非謝謝你的閱讀知識就是財富71、既然我已經(jīng)踏上這條道路，那么，75主動脈疾病-英文主動脈疾病-英文76DiseasesoftheaortaFredWU,MDApril19,2006Diseasesoftheaorta77CategoriesofaorticdiseaseAneurysmaPseudoaneurysmDissectionaPenetratingatheroscleroticulceraIntramuralhematomaAtheroscleroticdiseaseCoarctationCategoriesofaorticdisease78主動脈疾病-英文課件79ThoracicaorticaneurysmsAortopathyduetoheritablediseases(20%ofaneurysms)MarfansyndromeEhlers-DanlossyndromeFamilialAnnuloaorticectasiaaTurnersyndromeNoonansyndromeOsteogenesisimperfectaBicuspidaorticvalveCoarctationoftheaortaMetabolicdisorders(eg,homocystinuria,familialhypercholesterolemiaAorticarteritis(Takayasu's,giant-cell)TraumaPrenanSyphilis/infectiousCrackcocaineuseIatrogeniccauses(eg,cardiaccatheterization)AtherosclerosisHypertensionThoracicaorticaneurysms80MarfanSyndromeAutosomaldominantinheritanceMissensemutationinthefibrillingeneonchromosome15aPrevalenceis1in10,000(25%sporadaLeadingcauseofprematuredeathisaorticrootaneurysmleadingtodissection(cysticmedialdegeneration)MarfanSyndrome81MarfanSyndromeaGhentCriteriaIndexcaseaMajorcriteriain2differentorgansystemsandinvolvementinathirdorgansystemaFamilyhistoryaMajorcriterioninfamily/geneticsand1majorcriterioninanorgansystemandinvolvementinasecondorgansystemMarfanSyndrome82MarfanSyndromeSkeletalMajor(atleast4itemsMinoronlyPectuscannatumSpontaneouspneumothoraxLumbosacralduralectDecreasedelbowextensionCardiovascularProtrusioacetabularMinorDilatationoftheascendingaortavalsalvaDissectionoftheascendingaoMinorgharchedpalatewithcrowdingofMitralvalveprolapsewithorwithoutMFacialappearanceMitralannularcalcificationbeforeageOcularMajorDilationofthePAwithoutcausebeforeEctopialentisAbnormallyflatcornejoronlystdegreerelativewhoHypoplasticirisorciliarymuscleSkinandintegumentmutationinFBNlknowntoMinoronlyStriaenotassoclatedwithweightchange,pregnancyorstressMFSinfamilyRecurrentorincisionalherniasMarfanSyndrome83MarfanSyndromeSkeletalPulmonaryaMajor(atleast4items)MinoroWristandthumbCardiovascularnPesplanusacetabularDilatationoftheascendingaortaMinorvalsalvacum(moderate)DissectionoftheascendingMinorHigharchedpaMitralvalveprolapsewithorwithoutMaFacalMitralannularcalcificationbeforeageOcularofthePAwithoutcausebeforeMinorAbnormallyflatcorneaSkinandaMinoronlyStiaenotasMarfanSyndrome84FamilialTAASyndromesaAtleast19%ofpatientswiththoracicaorticaneurysms(withoutovertconnectivetissuedisorders)haveafamilyhistoryaTendtopresentyoungerthanthosepresentingsporadicallyaGeneticallyheterogeneousFamilialTAASyndromes85BicuspidaorticvalveaInitially,ascendingthoracicaorticaneurysmsinBAVthoughttobeduetopoststenoticdilatationa75%withcysticmedialnecrosisonpathologycomparedwith14%ofthosewithtricuspidaorticvalvesBicuspidaorticvalve86主動脈疾病-英文課件87主動脈疾病-英文課件88主動脈疾病-英文課件89主動脈疾病-英文課件90主動脈疾病-英文課件91主動脈疾病-英文課件92主動脈疾病-英文課件93主動脈疾病-英文課件94主動脈疾病-英文課件95主動脈疾病-英文課件96主動脈疾病-英文課件97主動脈疾病-英文課件98主動脈疾病-英文課件99主動脈疾病-英文課件100主動脈疾病-英文課件101主動脈疾病-英文課件102主動脈疾病-英文課件103主動脈疾病-英文課件104主動脈疾病-英文課件105主動脈疾病-英文課件106主動脈疾病-英文課件107主動脈疾病-英文課件108主動脈疾病-英文