资源描述：
《廖玉华-阜外心肌病诊治进展(英文).ppt》由会员上传分享，免费在线阅读，更多相关内容在教育资源-天天文库。
1、Advancementofdiagnosisandtherapyincardiomyopathies心肌病诊治进展LIAOYu-HuaInstituteofCardiology,UnionHospital,TongjiMedicalCollegeofHuazhongUniversityofScienceandTechnology,Wuhan,ChinaContemporaryDefinitionsandClassificationoftheCardiomyopathies（2006AHA）Cardiomyopathiesareaheterogeneousgroupofdise
2、asesofthemyocardiumassociatedwithmechanicaland/orelectricaldysfunctionthatusually(butnotinvariably)exhibitinappropriateventricularhypertrophyordilatationandareduetoavarietyofcausesthatfrequentlyaregenetic.Cardiomyopathieseitherareconfinedtotheheartorarepartofgeneralizedsystemicdisorders,oft
3、enleadingtocardiovasculardeathorprogressiveheartfailure–relateddisability.MaronBJ,etal.Circulation，2006，113:1807-1816ClassificationCardiomyopathiescanbemosteffectivelyclassifiedasprimary:genetic,mixed(geneticandnongenetic),acquired;andsecondaryPrimarycardiomyopathiesarethosesolelyorpredomin
4、antlyconfinedtoheartmuscleandarerelativelyfewinnumberSecondarycardiomyopathiesshowpathologicalmyocardialinvolvementaspartofalargenumberandvarietyofgeneralizedsystemic(multiorgan)disordersMaronBJ,etal.Circulation，2006，113:1807-1816MaronBJ,etal.Circulation，2006，113:1807-1816DilatedCardiomyopa
5、thyDilatedformsofcardiomyopathyarecharacterizedbyventricularchamberenlargementandsystolicdysfunctionwithnormalLVwallthickness;usuallydiagnosisismadewith2-dimensionalechocardiographyDCMisacommonandlargelyirreversibleformofheartmusclediseasewithanestimatedprevalenceof1:2500;itisthethirdmostco
6、mmoncauseofheartfailureandthemostfrequentcauseofhearttransplantationMaronBJ,etal.Circulation，2006，113:1807-1816DCMphenotypewithgeneticoccurrencAbout20%to35%ofDCMcaseshavebeenreportedasfamilial,althoughwithincompleteandage-dependentpenetrance,andlinkedtoadiversegroupof20lociandgenes.DCMisals
7、ocausedbyanumberofmutationsinothergenesencodingcytoskeletal/sarcolemmal,nuclearenvelope,sarcomere,andtranscriptionalcoactivatorproteins.ThemostcommonoftheseprobablyisthelaminA/Cgene,alsoassociatedwithconductionsystemdisease,whichencodesanuclearenvelopein
