廖玉华-阜外心肌病诊治进展(英文)

Advancementofdiagnosisandtherapyincardiomyopathies心肌病诊治进展LIAOYu-HuaInstituteofCardiology,UnionHospital,TongjiMedicalCollegeofHuazhongUniversityofScienceandTechnology,Wuhan,ChinaContemporaryDefinitionsandClassificationoftheCardiomyopathies（2006AHA）•Cardiomyopathiesareaheterogeneousgroupofdiseasesofthemyocardiumassociatedwithmechanicaland/orelectricaldysfunctionthatusually(butnotinvariably)exhibitinappropriateventricularhypertrophyordilatationandareduetoavarietyofcausesthatfrequentlyaregenetic.•Cardiomyopathieseitherareconfinedtotheheartorarepartofgeneralizedsystemicdisorders,oftenleadingtocardiovasculardeathorprogressiveheartfailure–relateddisability.MaronBJ,etal.Circulation，2006，113:1807-1816Classification•Cardiomyopathiescanbemosteffectivelyclassifiedasprimary:genetic,mixed(geneticandnongenetic),acquired;andsecondary1.Primarycardiomyopathiesarethosesolelyorpredominantlyconfinedtoheartmuscleandarerelativelyfewinnumber2.Secondarycardiomyopathiesshowpathologicalmyocardialinvolvementaspartofalargenumberandvarietyofgeneralizedsystemic(multiorgan)disordersMaronBJ,etal.Circulation，2006，113:1807-1816MaronBJ,etal.Circulation，2006，113:1807-1816DilatedCardiomyopathy•DilatedformsofcardiomyopathyarecharacterizedbyventricularchamberenlargementandsystolicdysfunctionwithnormalLVwallthickness;usuallydiagnosisismadewith2-dimensionalechocardiography•DCMisacommonandlargelyirreversibleformofheartmusclediseasewithanestimatedprevalenceof1:2500;itisthethirdmostcommoncauseofheartfailureandthemostfrequentcauseofhearttransplantationMaronBJ,etal.Circulation，2006，113:1807-1816DCMphenotypewithgeneticoccurrenc•About20%to35%ofDCMcaseshavebeenreportedasfamilial,althoughwithincompleteandage-dependentpenetrance,andlinkedtoadiversegroupof20lociandgenes.•DCMisalsocausedbyanumberofmutationsinothergenesencodingcytoskeletal/sarcolemmal,nuclearenvelope,sarcomere,andtranscriptionalcoactivatorproteins.ThemostcommonoftheseprobablyisthelaminA/Cgene,alsoassociatedwithconductionsystemdisease,whichencodesanuclearenvelopeintermediatefilamentprotein.MaronBJ,etal.Circulation，2006，113:1807-1816DCMphenotypewithsporadicoccurrence•Infectiousagents,particularlyviruses(coxsackievirus,adenovirus,parvovirus,HIV);bacterial;fungalrickettsial;myobacterial;andparasitic•Othercausesincludetoxins;chronicexcessiveconsumptionofalcohol;chemotherapeuticagents;metalsandothercompounds;autoimmuneandsystemicdisorders;pheochromocytoma;neuromusculardisorderssuchasDuchenne/BeckerandEmery-Dreifussmusculardystrophies;andmitochondrial,metabolic,endocrine,andnutritionaldisordersMaronBJ,etal.Circulation，2006，113:1807-1816•Criteria①leftventricularend-diastolicdimension（LVEDd）2.7cm/m2②leftventricularejectionfraction（LVEF）45%and/orfactionalshortening25%•Exclusion：①hypertension,②CHD,③long-termoverdosedrinkingalcohol,④persistencesupraventriculararrhythmia,⑤systemicdisease,⑥pericardialdisease,⑦congenitalheartdisease,⑧pneumocardialdiseaseDiagnosticcriteriaofidiopathicdilatedcardiomyopathyManolioTA,etal.AmJCardiol，1992，69：1459–66①diagnosticcriteriaofdilatedcardiomyopathy②Thediagnosisoffamilialdilatedcardiomyopathyismade:1.inthepresenceoftwoormoreaffectedindividualsinasinglefamily2.orinthepresenceofafirst-degreerelativeofadilatedcardiomyopathypatient,withwelldocumentedunexplainedsuddendeathat35yearsofageDiagnosisoffamilialdilatedcardiomyopathyMestroniL,etal.EuroHeartJ，1999，20:93–102①diagnosticcriteriaofdilatedcardiomyopathy②immunologicmarkers—anti-heartantibodiesaremainmarkers1including:anti-ANTAb、anti-ß1-receptorAb、anti-MHCAb、anti-M2-receptorAbSecondarymarkersincluding：•persistentviralinfection•Th2cellpredominance•cytokines•genetypeofhumamleucocyteantigenDiagnosisofimmunedilatedcardiomyopathy1苑海涛，廖玉华等.临床心血管病杂志，2000,16:313-315AnalysisofautoantibodyindilatedcardiomyopathyANT31*(64.6%)4(8.3%)64.6%91.7%β126*(54.2%)4(8.3%)54.2%91.7%M220*(41.7%)3(6.3%)41.7%93.7%MHC23*(47.9%)2(4.2%)47.9%95.8%positiveofautoantibodyinDCMAHAinDCMPeptidesDCMgroup(n=48)HDgroup(n=48)sensitivityspecificityn(%)n(%)ComparedwithHDgroup*p0.005苑海涛,廖玉华.临床心血管病杂志,2000,16:313ImmunopathogenesisinDCMVoltage-gatingCa-channelsCa-overloadCelldamageCardiacdilationReceptor-gatingCa-channelsVirusinfectionandautoimmunityresponseDCMTreatmentofDCM1、Conventionaltreatmentofheartfailure2、Preventembolism3、PreventSCD4、Improvecardiacmetabolism5、Cardiacresynchronizationtherapy6、Cardiactransplantation7、ImmunologictherapyinDCM1．β-receptorblocker•Toinhibittheanti-β1-Abmediatedmyocardiualdamage,especiallyinearlystage•Especiallysuitforthepatientswithtachycardiaorventriculararrhythmias,orwithanti-β1-Abpatients•Dose：metroprolol6.25mgBidto12.5～100mgBid,slowlytitrateBlockedautoantibodiesresponse(1)•MDCtrialPatients345,MGn=177andPGn=16816-75yearsofageMetoprolol10mg/day→100~150mg/daytherapyofHF：digitalis、diuretics、ACEIFollowingup18monthsMetoprololisindicatedtoimprovequanlityoflifeinheartfailurewithDCMβ-receptorblockerclinicaltrialinDCMWaagsteinF，etal.Lancet，1993，342:1441-462．Diltiazem•FrequeceofAnti-ANTantibodyispositiveabout60%-95%•Mechanism：Anti-ANTantibo