《心肌病的分类（英文）-课件，幻灯，PPT.ppt》由会员分享，可在线阅读，更多相关《心肌病的分类（英文）-课件，幻灯，PPT.ppt（37页珍藏版）》请在三一文库上搜索。
1、,The 4th China-Japan Cardiovascular Forum New Classifications of Cardiomyopathies,Akira Matsumori, MD, PhD, FACC, FAHA, FESC President, International Society of Cardiomyopathies and Heart Failure Secretary, World Heart Federation Chairman, Scientific Advisory Board & Treasurer, APSC Kyoto University
2、 Graduate School of Medicine Kyoto , Japan,October 23, 2008, Beijing,Normal,Dilated,Hypertrophic,Restrictive,Arrhythmogenic Right Ventricular,Systole,Diastole,Classification of Cardiomyopathies,Primary Cardiomyopathies (predominantly involving the heart),Genetic,Mixed*,Acquired,HCM ARVC/D LVNC Condu
3、ction Defects Mitochondrial myopathies,DCM Restrictive (non-hypertrophied and non-dilated),Inflammatory (myocarditis) Stress-provoked （”tako-tsubo”） Peripartum Tachycardia -induced Infants of insulin- dependent Diabetic mothers,Circulation 2006; 113:1807-1816,Ion Channel Disorders,LQTS,Brugada,SQTS,
4、CPVT,Asian SUNDS,*Predominantly nongenetic,PRKAG2,Danon,Glycogen Storage,Secondary Cardiomyopathies,Infiltrative Storage Toxicity Endomyocardial Inflammatory (granulomatous) Endocrine Cardiofacial Neuromuscular/ neurological Nutritional deficiency Autoimmune/ collagen Electrolyte imbalance Consequen
5、ce of Cancer therapy,Circulation 2006; 113:1807-1816,Gene Mutations Associated with Multiple Phenotypes of Cardiomyopathies,-Myosin heavy chain Cardiac troponin T -Tropomyosin Cardiac myosin binding Protein C Cardiac troponin I Actin Titin Desmin Muscle LIM protein Telethonin Desmoplakin Plakoglobin
6、,Gene,Phenotypes,HCM,DCM,RCM,ARVC/D,Sarcomere proteins,Z-disc protein,Desmosome protein,Intermediate filaments,Hypertrophic Cardiomyopathy,About half of cases show familial occurrence. About half of familial HCM have gene mutations of sarcomere proteins (25% of total HCM). Specific (Secondary) cardi
7、omyopathies often show HCM phenotype. Storage: Fabrys disease Inflammatory: Sarcoidosis HCV cardiomyopathy,Coxsackie B virus Adenovirus Hepatitis C virus,Dilated Cardiomyopathy,Myocarditis,Viral Infection and Phenotypes of Cardiomyopathies,Circulation 1995; 92: 2519-2525 Biochem Biophys Res Commun 1
8、996; 222: 678-682 Lab Inv. 2000; 80: 1137-1142,Hypertrophic Cardiomyopathy,Viral Infection of the Heart,Diffuse CHF/DCM,Systolic HF,Regional Aneurysm,Subendocardial RCM,Diastolic HF,ARVC/D LV Aneurysm,Random Hypertrophy/HCM,Complete recovery,Diffuse hypokinesis,Regional abnormality,Subendocardial le
9、sions,Increased wall thickness,Unclassified abnormality,Viral Myocarditis,CHF/DCM Systolic HF,HCM,RCM,ARVC/D,Diastolic HF,Hepatitis C Virus,L,V,Aneurysm,ARVC,HCM,DCM,Myocarditis,Matsumori A Circ Res 2005;96:144-147,Hypertrophic Obstructive Cardiomyopathy Associated with HCV Infection,HE,CoreA-2,Apic
10、al Hypertrophic Cardiomyopathy Associated with HCV Infection,Endomyocardial Biopsy in Patients with HCM with HCV Infection,A Patient with HCM, Hepatitis and Nephritis Associated with HCV Infection,Biopsy Finding in a Patient with Hypertropic Cardiomyopathy, Hepatitis and Nephritis,Kidney,Heart,Liver
11、,Acute Hepatitis,Chronic Hepatitis,Liver Cirrhosis,Myocardial Fibrosis,DCM,Acute Myocarditis,Chronic Inflammation,Hepatic Failure,HCC,HCM,85%,20%,6%,4%,HCV Hepatitis,HCV Cardiomyopathies,An Atypical Variant of Fabrys Disease in Men with Left Ventricular Hypertrophy.,Nakao S et al NEJM 1995;333:288-2
12、93,Fabrys disease is an X-linked recessive disorder that results from a deficiency of -galactosidase. Seven of the 230 patients (3%) of HCM.,Fabrys Disease Presented as HCM,Alpha-galactosidase activity 0.7 n moles/hr/ml (Normal 4.8-17.6 n moles/hr/ml),IVST 20 mm LVPWT 11 mm LVEDD 58 mm LVESD 45 mm L
13、VEF 45% UCG,201 Tl Scintigraphy,Increased uptake at IVS and anterior wall Increased LV cavity compared to those of 1.5 yr before.,Heart Disease in Friedreichs Ataxia Observation of a Case for Half a Century.,Kawai C, Kato S et al Jpn Circ J 2000;64:229-236,IVST 14mm LVPWT 12mm,Disarrangement of bizz
14、are-shaped myocardial fibers with hypertrophy and interstitial fibrosis,Hypertrophic Cardiomyopathy as a Manifestation of Cardiac Sarcoidosis.,Matsumori A et al Jpn Circ J 2000;64:679-683,Six of 82 (7.3%) patients with sarcoidosis have echocardiographic abnormality. Four of 82(4.8%) showed phenotype
15、 of HCM. ASH: 2 cases, APH: 1 case,Depar,tment of Cardiovascular,Medicine, Kyoto University,LV Aneurysm in a Patient with HCV Cardiomyopathy,VT, Hepatitis C (IFN Rx), Lymphadenopathy,FH: Hepatitis C, HCC in 2 brothers,UCG: IVS 16mm, LVPW 13mm, LVDd 47mm, LVDs 37mm, EF 36%,RAO,ED,ES,MT,52,M,Detection
16、 of HCV RNA in Heart Tissues from Patients with ARVC,n,Positive n,Frequency,WHF Council of Cardiomyopathies National Cardiovascular Center, Japan,6 3 9,2 2 4,33.0% 66.7% 44.4%,Total,Immunohistochemical Staining of HCV Core Protein in the Heart from Patients with ARVC/D,Genetic Background of the Host
17、 Influences the Phenotype of Cardiomyopathies,HLA and HCM,HLA-DRW4 antigen linkage in patients with hypertrophic obstructive cardiomyopathy Matsumori A et al. Am Heart J. 1981;101:14-16. HLA in hypertrophic cardiomyopathy and rheumatic heart disease Matsumori A et al. Jpn Circ J 1979;43:445-449 HL-A
18、 and Hypertrophic Cardiomyopathy Matsumori A et al. Am Heart J 1979;97:428-431,Frequencies of DPB1 Alleles in Patients With HCV-Associated Cardiomyopathy and Controls,Both DPB1*0401 and DPB*0901 was significantly associated with HCV-HCM (* P0.05), whereas none of DPB1 allele demonstrated significant
19、 association with HCV-DCM,Shichi D, Matsumori A, et al. Int J Immunogenet 2008;35:37-43,Association with Polymorphic of DP-Chain in HCV-HCM,Shichi D, Matsumori A, et al. Int J Immunogenet 2008;35:37-43,The polymorphic residues from DPB1 alleles located in P9 pockets (at position 36A and 55A) showed
20、positive associations with HCV-HCM. In contrast, five polymorphic residues showed significant negative associations: 76M in P4 pockets; 8L and 11G in P6 pockets; 9F in P9 pockets and 57E adjacent to P9 pocket. Quite interestingly, all the residues showing significant positive or negative association
21、s composed of DPB1*0401.,The Susceptible Gene Mapping for HCV-DCM and HCM with Microsatellite Markers throughout the HLA region,Odds Ratio,Corrected P,Susceptibility to HCV-DCM was mapped at the locus spanning from NFKBlL1 to BAT1 loci within the HLA class III subregion. HCV-HCM was associated with
22、DPB1 alleles. The candidate genes may encode molecules involved in the immunity and inflammation.,Shichi D et al Tissue Antigen 2005:66:200,HCM,DCM,HLA and HCV Infection,Our study provides new and suggestive information on the immunological involvement of DPB1 gene in the HCV-HCM development. The po
23、lymorphic amino acids residues by which the DP chain adopt specificity pocket appear to influence on disease-susceptibility at the allelic manner level. The existence of different risk alleles among HCV-related diseases including chronic liver disease, asymptomatic carrier and HCV- DCM suggests that
24、 each clinical outcome may arise from distinct pathogenic conditions on the basis of differential HLA-mediated immune responses.,Etiology of HCM,HCM,Genetic,Inflammatory,Sarcomere,Storage,Virus,Unknown,Etiology of DCM,DCM,Genetic,Inflammatory,Sarcomere,Storage,Virus,Unknown,Etiology of ARVC/D,ARVC/D
25、,Genetic,Inflammatory,Virus,Unknown,Definition and Classifications of Cardiomyopathies,Etiological Classification A. Genetic B. Infectious C. Nutritional D. Unknown II. Anatomical (Structural) Classification A. Dilated a. LV b. RV B. Hypertrophic a. Septum b. Diffuse c. Free wall d. Apex III. Physio
26、logical (mechanical) Classification A. Systolic failure/dysfunction B. Diastolic failure/dysfunction C. Both D. Normal function IV. Electrical Classification Ion channel disorders Conduction system disease Others,Definition and Classifications of Cardiomyopathies,ISFC Classification,Proposed Classif
27、ication,DCM (with viral infection) DCM (with gene mutation) HCM (with gene mutation and outflow obstruction) Apical HCM (with unknown causes) Apical HCM (with HCV infection) ARVC/D (with gene mutation) ARVC/D (with HCV infection) Long QT Syndrome,I B, II A a, III A I A, II A a, III A I A, II B a, II
28、I B I D, II B d, III B I B, II B d, III B I A, II A b, III A I B, II A b, III A I A, ，IV A,Etiological A. Genetic B. Infections C. Nutritional D. Unknown II. Anatomical (Structural) A. Dilated a. LV b. RV B. Hypertrophic a. Septum b. Diffuse c. Free wall d. Apex III. Physiological (mechanical) A. Systolic failure/dysfunction B. Diastolic failure/dysfunction Both D. Normal Function IV. Electrical Classification Ion channel disorders Conduction system disease Others,