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时间:2018-10-13
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1、PathologyofPulmonaryHypertensionWolterJ.MooiDepartmentofPathology,VUmcAmsterdamWJ.Mooi@VUmc.nlPulmonaryarterialhypertension(WHOdefinition)Sustainedelevationofpulmonaryarterialpressuretomorethan25mmHgatrest,ortomorethan30mmHgwithexerciseMeanpulmonarywedgepressureandleftventricularend-diastolicpress
2、urelessthan15mmHgWHOClassificationofPulmonaryHypertensionGroupI:PulmonaryarterialhypertensionIdiopathic(primary)FamilialAssociatedwithsignificantvenousorcapillaryinvolvementPersistentpulmonaryhypertensionofthenewbornGroupII:PulmonaryvenoushypertensionLeft-sidedartialorventricularheartdiseaseLeft-si
3、dedvalvularheartdiseaseGroupIII:PulmonaryhypertensionassociatedwithhypoxemiaGroupIV:Pulmonaryhypertensionduetochronicthromboticdisease,embolicdisease,orbothGroupV:MiscellaneousWHOClassificationofPulmonaryHypertension:criticismsPulmonaryarterialhypertensionisnotlimitedtoGroupI(thisisinappropriatelys
4、uggestedbyitsdesignationas‘pulmonaryarterialhypertension’)ItmaybeassociatedwithpulmonaryvenoushypertensionItmaybeposttrombotic(GroupIV)orhypoxic(GroupIII)Thegroup‘miscellaneous’includes:compressionofpulmonaryveins,whichshouldbeinGroupII(pulmonaryvenoushypertension)Mainhistologicalpatternsofpulmonar
5、yhypertensivevasculardiseaseCongestivevasculopathyBackwardfailureofleftsideofheartObstructionofpulopmarylargeveinsHypoxicpulmonaryvasculopathyCOPD,highaltitudedwellers,alevoarhypoventialtiondisorders,&cPostthromboticpulmonaryvasculopathyChronicrecurrentthromboembolism,primarythrombosisPlexogenicpul
6、monaryarteriopathyPosttricuspidLRshunts;associatedwithanorexigens,HIV,portalhypertension,idiopathicPulmonaryvascularocclusivediseaseMiscellaneousraredisordersCongestivepulmonaryvasculopathyVeins:IntimalfibrosisMedialhypertrophy,‘arterialization’InterstitialfibrosisHaemosiderosisArteries:Intimalfibr
7、osisMedialhypertrophyAdventitialthickeningHypoxicpulmonaryvasculopathyArteriesandarterioles:Medialhypertrophy(accentuatedinsmallerbranches)MuscularizationofarteriolesLongitudinalsmoothmusclebundlesMildintim
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