肥厚型心肌病

肥厚型心肌病从基础研究到临床诊治Valadononry.chAsposeslidesforNET4odientPEvaluationonly.CreatedwithAsposeSlidesforNET4.0dientProfilo71Copyright2019-2019AsposePtyL肥厚型心肌病的病因及流行病学特点第肥厚型心肌病的病理及病理生理改变第二节肥厚型心肌病的临床表现、影像学技术的应用、第兰节诊断及鉴别诊断第凶节·肥厚型心肌病的内外科治疗Valadononry.chAsposeslidesforNET4odientPEvaluationonly.CreatedwithAsposeSlidesforNET4.0dientProfilo71Copyright2019-2019AsposePtyL第一节肥厚型心肌病的病因及流行病学特点病因肥厚型心肌病是一种传统意义上的单基因遗传疾病≥至少25个基因,超过1400个位点的突变与肥厚型心肌病的发病有关这些基因多为编码肌小节结构蛋白的基因Valadononry.chAsposeslidesforNET4odientPEvaluationonly.CreatedwithAsposeSlidesforNET4.0dientProfilo71Copyright2019-2019AsposePtyL致病基因其他编码β肌球蛋白基因突变35%编码TNNT2、TNNT3基因突编码心脏变球蛋白经5%0%Valadononry.chAsposeslidesforNET4odientPEvaluationonly.CreatedwithAsposeSlidesforNET4.0dientProfilo71Copyright2019-2019AsposePtyL流行病学特点发病率约为0.2%年死亡率>约为1.3%年猝死率约为0.9%Valadononry.chAsposeslidesforNET4odientPEvaluationonly.CreatedwithAsposeSlidesforNET4.0dientProfilo71Copyright2019-2019AsposePtyL我国肥厚型心肌病发病率为流行病学特点0.16%,据此推算,我国大约有200万患者,PrevalenceofIdiopathic/HypertrophicCardiomyopathyinChina:APopulation-BasedEchocardiographicAnalysisof8080AdultsYubaoZou,MD,PhD,LeiSong,*MD,PhD,ZhiminWang,*MD,AiqunMa,MD,PhD,TangweiLiu,MD,PhD,HuiminGu,MD,SailanLu,MD,PengzhuWu,MD,YingZhang,tMDLiShen,tMD,YulingCai,MD,YisongZhen,fPhD,YanlingLiu,MD,RutaiHui,MD,PhDPURPOSE:TodeterminetheprevalenceofhypertrophicRESULTS:Ofthe4064menand4016weHODS:Thisepforhypertrophiccardiomyopathy.TheFebruary2019,usingamusiteinage.andsex-adiustedprevalencewasabout80per100,000CONCLUSION:Hypertrophiccardiomyopathyestimatedprevalence,thereleast1echocardiography,thatwasnotcausedbyanyknowncardiacmillioncasesinChina.AmJMed.2019;116:14-18.200systemicdiscaseExcerptaMedicaInc.AmJMed2019;116:14-18Valadononry.chAsposeslidesforNET4odientPEvaluationonly.CreatedwithAsposeSlidesforNET4.0dientProfilo71Copyright2019-2019AsposePtyL流行病学特点Valadononry.chAsposeslidesforNET4odientPEvaluationonly.CreatedwithAsposeSlidesforNET4.0dientProfilo71Copyright2019-2019AsposePtyL第二节肥厚型心肌病的病理及病理生理改变大体病理改变NormalheartHeartwithHypertropcardiomyopathy心脏的大体形态方面表现为心脏重量增加、心室壁增厚、左心室腔明显变小、左房扩大②MavoFoundatonforMValadononry.chAsposeslidesforNET4odientPEvaluationonly.CreatedwithAsposeSlidesforNET4.0dientProfilo71Copyright2019-2019AsposePtyL组织病理改变心肌细胞12-3肥大心肌细胞排列紊乱心肌纤维化Valadononry.chAsposeslidesforNET4odientPEvaluationonly.CreatedwithAsposeSlidesforNET4.0dientProfilo71Copyright2019-2019AsposePtyL组织病理改变正常心肌组HCM惠者心肌组织织Valad