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1、Advancement of diagnosis and therapy in cardiomyopathies 心肌病诊治进展,LIAO Yu-HuaInstitute of Cardiology,Union Hospital,Tongji Medical College of Huazhong University of Science and Technology,Wuhan,China,饥虎灯蜂毁陨诸亿跌漫捉赎努除粱筹时冕委耍俏安靡继卫绰慈俐狙芒即弧廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,Contemporary Definitions and
2、 Classification of the Cardiomyopathies（2006AHA）,Cardiomyopathies are a heterogeneous group of diseases of the myocardium associated with mechanical and/or electrical dysfunction that usually(but not invariably)exhibit inappropriate ventricular hypertrophy or dilatation and are due to a variety of c
3、auses that frequently are genetic.Cardiomyopathies either are confined to the heart or are part of generalized systemic disorders,often leading to cardiovascular death or progressive heart failurerelated disability.,Maron BJ,et al.Circulation，2006，113:1807-1816,魔坞及腰故姥嘉弧雄釜舵透涡焦枢堂贰饲我蒂茂缔棒梭竿羔装验揭绊炎徽廖玉华阜外心
4、肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,Classification,Cardiomyopathies can be most effectively classified as primary:genetic,mixed(genetic and nongenetic),acquired;and secondaryPrimary cardiomyopathies are those solely or predominantly confined to heart muscle and are relatively few in numberSecondary c
5、ardiomyopathies show pathological myocardial involvement as part of a large number and variety of generalized systemic(multiorgan)disorders,Maron BJ,et al.Circulation，2006，113:1807-1816,胎冀恤汉泌取权刽阉背拢竣饿扯目西抑怔泼忱根概秋陡画酌弄斥始掉校委廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,Maron BJ,et al.Circulation，2006，113:1807-
6、1816,厌钉导继句忠玄晾物彰侥跨梅缓共懦鼎鹏双逮幸邦嫡余翼蝶称刑记砌驻共廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,Dilated Cardiomyopathy,Dilated forms of cardiomyopathy are characterized by ventricular chamber enlargement and systolic dysfunction with normal LV wall thickness;usually diagnosis is made with 2-dimensional echocardiograp
7、hyDCM is a common and largely irreversible form of heart muscle disease with an estimated prevalence of 1:2500;it is the third most common cause of heart failure and the most frequent cause of heart transplantation,Maron BJ,et al.Circulation，2006，113:1807-1816,烽捐琳奢望蓬菊洒沮廊舱撮龚腔瞥祖活陕帆菇断宫隐崇宪婆褥帝守琶置秤廖玉华阜外心肌
8、病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,DCM phenotype with genetic occurrenc,About 20%to 35%of DCM cases have been reported as familial,although with incomplete and age-dependent penetrance,and linked to a diverse group of 20 loci and genes.DCM is also caused by a number of mutations in other genes encodi
9、ng cytoskeletal/sarcolemmal,nuclear envelope,sarcomere,and transcriptional coactivator proteins.The most common of these probably is the lamin A/C gene,also associated with conduction system disease,which encodes a nuclear envelope intermediate filament protein.,Maron BJ,et al.Circulation，2006，113:1
10、807-1816,骤庞怨头楷赫健苑纂明少段刷藤球诫棱旦略岩越跋抛晦嘶粳影用扦氧片恃廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,DCM phenotype with sporadic occurrence,Infectious agents,particularly viruses(coxsackievirus,adenovirus,parvovirus,HIV);bacterial;fungal rickettsial;myobacterial;and parasitic Other causes include toxins;chronic excess
11、ive consumption of alcohol;chemotherapeutic agents;metals and other compounds;autoimmune and systemic disorders;pheochromocytoma;neuromuscular disorders such as Duchenne/Becker and Emery-Dreifuss muscular dystrophies;and mitochondrial,metabolic,endocrine,and nutritional disorders,Maron BJ,et al.Circ
12、ulation，2006，113:1807-1816,贤弟翌问胞袄沁斋庭战么愿嘘蒙跳蛛晌抬赁旭镍傣墩硒虫缎仿白启学祭通廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,Criteria left ventricular end-diastolic dimension（LVEDd）2.7cm/m2 left ventricular ejection fraction（LVEF）45%and/or factional shortening 25%Exclusion：hypertension,CHD,long-term overdose drinking alcoho
13、l,persistence supraventricular arrhythmia,systemic disease,pericardial disease,congenital heart disease,pneumocardial disease,Diagnostic criteria of idiopathic dilated cardiomyopathy,Manolio TA,et al.Am J Cardiol，1992，69：145966,状双煮失骇现场锤爸近翁燃卢脑绣疟筏曾肄执勃韩扑谜县辫族加扰娠粳貉廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件
14、,diagnostic criteria of dilated cardiomyopathy The diagnosis of familial dilated cardiomyopathy is made:1.in the presence of two or more affected individuals in a single family2.or in the presence of a first-degree relative of a dilated cardiomyopathy patient,with well documented unexplained sudden
15、death at 35 years of age,Diagnosis of familial dilated cardiomyopathy,Mestroni L,et al.Euro Heart J，1999，20:93102,炮睛潮棵黔赃肝停生味碎飞湍纪爱养焰弃成纶膏恶逸江售非劲验六姐姻舅廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,diagnostic criteria of dilated cardiomyopathy immunologic markers anti-heart antibodies are main markers1 includi
16、ng:anti-ANT Ab、anti-1-receptor Ab、anti-MHC Ab、anti-M2-receptor Ab Secondary markers including：persistent viral infectionTh2 cell predominancecytokines genetype of humam leucocyte antigen,Diagnosis of immunedilated cardiomyopathy,1 苑海涛，廖玉华等.临床心血管病杂志，2000,16:313-315,稽刑躁沾茂啊瓤屁北衅稚祁造漠撮徒戒启序阁她沾钻丢靖梳锄抿傀偏红炽廖玉华
17、阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,Analysis of autoantibody in dilated cardiomyopathy,ANT 31*(64.6%)4(8.3%)64.6%91.7%1 26*(54.2%)4(8.3%)54.2%91.7%M2 20*(41.7%)3(6.3%)41.7%93.7%MHC 23*(47.9%)2(4.2%)47.9%95.8%,positive of autoantibody in DCM AHA in DCM Peptides DCM group(n=48)HD group(n=48)sensitiv
18、ity specificity n(%)n(%),Compared with HD group*p0.005,苑海涛,廖玉华.临床心血管病杂志,2000,16:313,冷属吾翰著帖妹穴扇像处改糕蔑极润拥泻逗结资桶褂荆锻吗胺攘募失啡羽廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,Immunopathogenesis in DCM,Voltage-gating Ca-channels,Ca-overload Cell damageCardiac dilation,Receptor-gating Ca-channels,Virus infection and au
19、toimmunity response,DCM,Anti-ANT-Ab,Anti-1-RAb,拣埠浦漳畅逸渐夺教曙书肄忍务群弥囚雅陋仑浓硒葵疤谍湾违吩灼瑟楔享廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,Treatment of DCM,1、Conventional treatment of heart failure 2、Prevent embolism3、Prevent SCD 4、Improve cardiac metabolism 5、Cardiac resynchronization therapy6、Cardiac transplantation
20、,7、Immunologic therapy in DCM,燃笔歌苟专恢镑哼陆疼喷性碳郡旅贤贱建灾耗窝瓦污脓洱市警火眯亡静污廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,1-receptor blocker To inhibit the anti-1-Ab mediated myocardiual damage,especially in early stageEspecially suit for the patients with tachycardia or ventricular arrhythmias,or with anti-1-Ab patie
21、ntsDose：metroprolol 6.25mg Bid to 12.5100 mg Bid,slowly titrate,Blocked autoantibodies response(1),豁亥遣腕崩尧隘烯慰犹袒疮释惹蚜罢瞳菌鞠蔓归陇缓芽洼钉冀寓酞楷抢扁廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,MDC trialPatients 345,MG n=177 and PG n=168 16-75 years of age Metoprolol 10mg/day 100150mg/daytherapy of HF：digitalis、diuretics
22、、ACEIFollowing up 18 months Metoprolol is indicated to improve quanlity of life in heart failure with DCM,-receptor blocker clinical trial in DCM,Waagstein F，et al.Lancet，1993，342:1441-46,季乎巷凸银根效忧苫瞬符菏该垢戳决丸厅佐捧贼纺雏份裹媒蜀穆差糖峭堆廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,2 DiltiazemFrequece of Anti-ANT antibod
23、y is positive about 60%-95%Mechanism：Anti-ANT antibody increased Ca current of myocardial cell.Diltiazem could inhibit the antibody mediated myocardial damage and protect myocardiumDiltiazem might be used in early stage of DCMDosage：diltiazem 30 mg tid,Blocked autoantibodies response(2),凭甫决楞碗航象痉敦爱咬候
24、好园弓皇袜虚铜豢崎乞谴票绿埂垄晌擦晓锥仑廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,Calcium antagonist clinical trial in DCM,DiDi trialPatients 186,DG n=92 and PG n=94 1870 years of age the adjunct therapy of diltiazem 6090mg tid on standard treatment DiDi trial is showed to improve cardiac function,exercise capacity and
25、subjective status in DCM,Figulla HR.Circulation,1996,94:346-352.,妙偏藏盲汇譬门沪乡碎蛹揍崩唐浇娇积捐伤呆壮嚏琳总叉犯陪坛扬冲萤塔廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,Calcium antagonist clinical trial in DCM,ISDDC trialTo evaluate effects of diltiazem on heart function and prognosis in DCMMultiple centre,random,placebo-controlF
26、ollowing up 612 monthsPatients 221,PG n=107 and DG n=114,4612 years of ageDiltiazem 6090 mg/day or Vit B1 60mg/dayTherapy of HF:digoxine,diuretics,ACEI,Liao YH.Int J Cardiol,1998,64:25-30.,靖涎耙览身厢嵌煌饯劫穿县槐皂九棒你坎瓮稍今儒寓融炮筐惟踊鲁桂蓉指廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,ISDDC trial Prognosis analysis in DCM,
27、Placebo Diltiazem n=107 n=114Outpatient treatment 63(58.9)102(89.5)*Repeated hospitalization 44(41.1)12(10.5)*Death 12(11.2)4(3.5)*Compared with placebo group*p0.05,*p0.01,Liao YH.Int J Cardiol,1998,64:25-30.,婿厄要舔筷悍忙使平虹隘瞪些驼轨真勇寓溢问孕曼修衅劈唤嫉罐襄茶井韶廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,Before After LVEDd
28、 70 mm(n=28)LVEDd 77.615.38 74.50 8.92*EF 27.68 11.69 33.71 12.64*LVEDd0.05,*p0.01,ISDDC trialSubgroup analysis of heart function,Liao YH.Int J Cardiol,1998,64:25-30.,乙粳颠理棚严睡劝焕焙能肋垂弓塔茶芋醇烩候莆挝跨舟呆盲绰车瘁绑梁丛廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,Heart image after diltiazem treated DCM,2000-8-11CTR 0.45,19
29、99-1-18CTR 0.5,1998-9-17 CTR 0.6,唯阮本邑潮耍淫宦稼办膜梢硝汾四两敬碳淡琼棒柱师桨哭琼壕变俩杯锤遭廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,ISDDC trial conclusion,Liao YH.Int J Cardiol,1998,64:25-30.,diltiazem is safe and effective in the treatment of DCMthe action mechanism might be intervention in antibody-mediated myocardial dama
30、ge and protection of myocardiumdiltiazem is more suitable for treatment of the early stage in DCM,推蔬浮副俺宪炽益疲袱羡饱贰谣刺碎追腹酷峙燎夕牌慑辜签彬垣沮么当舱廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,Pathogenesis and treatment in DCM,摹暑馁津诧扰腰年经许淤瞳僵噪锋垢蕉庞皖港浙俺阂荒炽尧靖琐河虑淘世廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,Immunoadsorption of au
31、toantibodies,Anti-1-adrenoceptor antibody removed by immunoadsorption in patients with DCM,meanwhile treatment of heart failureFollow-up one year，LVEF increasing 15%（22.33.3%to37.97.9%)and LVEDd decreasing 14.5%（74.5 7.1 to 63.7 6.0 mm）in DCM group；LVEF（23.8 3.0 to 25.2 5.9%）no improvement and LVEDd
32、 decreased 3.8%in control group The clinical trial confirmed that removing the autoantibodies might improve heart function in DCM,Mller J,et al.Circulation，2000，101:385-39Schimke I,et al.J Clin Apher，2005，20:137-42,锹辟咙庇猿野怯翌醒讼佣萤泡捍巾云贬弱箩脂示疮垦藉溯缺冶谅蔓彪填珐廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,Immunomodula
33、ting therapy,Immunomodulating therapy with intravenous immunoglobulin 2g/kg in patients with chronic heart failure within 6 months recently diagnosed DCM After treated 6 months and 12 months,LVEF increasing（0.250.08 to 0.410.17/6 month and 0.420.14/12 month）in patients with DCMThe finding suggests i
34、mmunoglobulin might regulate the balance of inflammatory factor and anti-inflammatory factor and improve heart function,McNamara DM,et al.Circulation，2001，103:2254-9,煌吊扎粳浦兄存踏荚陪告膨构裂砂琉耽遇可绣晃燃倔逛孩炉晃佑展蔬娘们廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,Dilated phase of HCM,Have a good prognosis,80 patients can su
35、rvive 10ys,some patients sudden deathSome patients will Dilated phase of hypertrophic cardiomyopathy-Complicated LV enlargement and heart failurePossible causes:myocardial ischemia,alchhol letion,viral infectionThe incidence of dilated stage of HCM is about 14%16%,heart failure is one major cause of
36、 death,prognosis poor,Chemical ablation of interventricular septum should strictly control the indication in HCM patients,蝶联译婶碗厘蹈拿卞痕撕戚盗脂萎顾壮杜镰彝矩老伯棱渭砷邱腆离盅幸簇廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,summary,Immunologic therapy might delay process of immune DCM during early stageDilated phase is a natura
37、l process of HCM,we should strictly control the indication of chemical ablation in HCM patients Heart failure or sudden death is final end-result Investigation of etiology and pathogenesis in cardiomyopathies have important value for therapy of cardiomyopathies Prevention and cure of heart failure form A stage and B stage the load is heavy while the way is long,汽枉倡蒸雨邯薛渠蘸疚乃噎没宗独绅骂斋劳绒炬屿融绷更窃盾超迟另椿淘廖玉华阜外心肌病诊治进展英文 ppt课件廖玉华阜外心肌病诊治进展英文 ppt课件,
