廖玉华阜外心肌病诊治进展英.ppt
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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 1Contemporary Definitions and Classification of the Cardiomyopathies(2006AHA2006AHA)Cardiomyopathies
2、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 causes that frequently are genetic.Cardiomyopathies either are conf
3、ined 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 2ClassificationCardiomyopathies can be most effectively classified as primary:genetic,mixed(genetic and n
4、ongenetic),acquired;and secondary1.Primary cardiomyopathies are those solely or predominantly confined to heart muscle and are relatively few in number2.Secondary cardiomyopathies show pathological myocardial involvement as part of a large number and variety of generalized systemic(multiorgan)disord
5、ersMaron BJ,et al.Circulation,2006,113:1807-1816 3Maron BJ,et al.Circulation,2006,113:1807-1816 4Dilated CardiomyopathyDilated forms of cardiomyopathy are characterized by ventricular chamber enlargement and systolic dysfunction with normal LV wall thickness;usually diagnosis is made with 2-dimensio
6、nal echocardiographyDCM 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 transplantationMaron BJ,et al.Circulation,2006,113:1807-1816 5DCM phenotype with ge
7、netic occurrencAbout 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 encoding cytoskeletal/sarcolemmal,nuclear envelope,sarcomere,a
8、nd 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,2006,113:1807-1816 6DCM phenotype with sporadic occurrenceInfectiou
9、s agents,particularly viruses(coxsackievirus,adenovirus,parvovirus,HIV);bacterial;fungal rickettsial;myobacterial;and parasitic Other causes include toxins;chronic excessive consumption of alcohol;chemotherapeutic agents;metals and other compounds;autoimmune and systemic disorders;pheochromocytoma;n
10、euromuscular disorders such as Duchenne/Becker and Emery-Dreifuss muscular dystrophies;and mitochondrial,metabolic,endocrine,and nutritional disordersMaron BJ,et al.Circulation,2006,113:1807-1816 7Criteria left ventricular end-diastolic dimension(LVEDd)2.7cm/m2 left ventricular ejection fraction(LVE
11、F)45%and/or factional shortening 25%Exclusion:hypertension,CHD,long-term overdose drinking alcohol,persistence supraventricular arrhythmia,systemic disease,pericardial disease,congenital heart disease,pneumocardial disease Diagnostic criteria of idiopathic dilated cardiomyopathyManolio TA,et al.Am J
12、 Cardiol,1992,69:145966 8 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 docum
13、ented unexplained sudden death at 35 years of ageDiagnosis of familial dilated cardiomyopathyMestroni L,et al.Euro Heart J,1999,20:93102 9 diagnostic criteria of dilated cardiomyopathy immunologic markers anti-heart antibodies are main markers1 including:anti-ANT Ab、anti-1-receptor Ab、anti-MHC Ab、an
14、ti-M2-receptor Ab Secondary markers including:persistent viral infectionTh2 cell predominancecytokines genetype of humam leucocyte antigenDiagnosis of immunedilated cardiomyopathy 1 苑海涛,廖玉华等.临床心血管病杂志,2000,16:313-31510Analysis of autoantibody in dilated cardiomyopathyANT 31*(64.6%)4(8.3%)64.6%91.7%1
15、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)sensitivity specificity n (%)n (%)Compared with HD group *p0.005苑海涛,廖玉华.临床心血管病杂志,2000,16:31311Immunopathogenesis in DCMVoltage-gati
16、ng Ca-channelsVoltage-gating Ca-channelsCa-overload Ca-overload Cell damageCell damageCardiac dilation Cardiac dilation Receptor-gating Ca-Receptor-gating Ca-channelschannelsVirus infection and autoimmunity Virus infection and autoimmunity responseresponseDCMA An nt ti i-A ANNT T-A Ab bA An nt ti i-
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