心肌病的分类英文课件幻灯PPT

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1、 The 4th China-Japan Cardiovascular Forum New Classifications of Cardiomyopathies Akira Matsumori, MD, PhD, FACC, FAHA, FESCPresident, International Society of Cardiomyopathies and Heart FailureSecretary, World Heart FederationChairman, Scientific Advisory Board & Treasurer, APSCKyoto University Gra

2、duate School of MedicineKyoto , JapanOctober 23, 2008, BeijingNormalDilatedHypertrophicRestrictiveArrhythmogenicRight VentricularSystoleDiastoleClassification of CardiomyopathiesPrimary Cardiomyopathies(predominantly involving the heart)GeneticMixed*AcquiredHCMARVC/DLVNCConductionDefectsMitochondria

3、lmyopathiesDCMRestrictive(non-hypertrophiedand non-dilated)Inflammatory(myocarditis)Stress-provoked（ （” ”tako-tsubo”） ）PeripartumTachycardia-inducedInfants of insulin-dependentDiabetic mothersCirculation 2006; 113:1807-1816Ion Channel DisordersLQTSBrugadaSQTSCPVTAsian SUNDS*Predominantly nongeneticP

4、RKAG2DanonGlycogenStorageSecondary CardiomyopathiesInfiltrativeStorageToxicityEndomyocardialInflammatory (granulomatous)EndocrineCardiofacialNeuromuscular/ neurologicalNutritional deficiencyAutoimmune/ collagenElectrolyte imbalanceConsequence of Cancer therapyCirculation 2006; 113:1807-1816Gene Muta

5、tions Associated with Multiple Phenotypes of Cardiomyopathies -Myosin heavy chainCardiac troponin T -TropomyosinCardiac myosin bindingProtein CCardiac troponin I ActinTitinDesminMuscle LIM proteinTelethoninDesmoplakinPlakoglobinGenePhenotypesHCMDCMRCMARVC/DSarcomere proteinsZ-disc proteinDesmosome p

6、roteinIntermediate filamentsHypertrophic 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) cardiomyopathies often show HCM phenotype. Storage: Fabrys disease Inflammatory: Sarcoid

7、osis HCV cardiomyopathyCoxsackie B virusAdenovirusHepatitis C virusDilated Cardiomyopathy Myocarditis Viral Infection and Phenotypes of CardiomyopathiesCirculation 1995; 92: 2519-2525Biochem Biophys Res Commun 1996; 222: 678-682Lab Inv. 2000; 80: 1137-1142Hypertrophic CardiomyopathyViral Infection o

8、f the HeartDiffuseCHF/DCMSystolic HFRegionalAneurysmSubendocardialRCMDiastolic HFARVC/DLV AneurysmRandomHypertrophy/HCMVirusReceptorMyocyteFibroblastCompleterecoveryDiffusehypokinesisRegionalabnormalitySubendocardiallesionsIncreasedwall thicknessUnclassifiedabnormalityViral MyocarditisCHF/DCMSystoli

9、c HFHCMRCMARVC/DDiastolic HFHepatitis C Virus LV AneurysmARVCHCMDCMMyocarditisMatsumori A Circ Res 2005;96:144-147Hypertrophic Obstructive Cardiomyopathy Associated with HCV InfectionHECoreA-2Immunohistochemical Staining of HCV Core Antigen in the Heart of a Patient with HCM Apical Hypertrophic Card

10、iomyopathy Associated with HCV InfectionEndomyocardial Biopsy in Patients with HCM with HCV InfectionA Patient with HCM, Hepatitis and Nephritis Associated with HCV InfectionBiopsy Finding in a Patient with Hypertropic Cardiomyopathy,Hepatitis and NephritisKidneyHeartLiverAcute HepatitisChronic Hepa

11、titisLiver CirrhosisMyocardialFibrosisDCMAcute MyocarditisChronicInflammationHepaticFailureHCCHCM85%20%6%4%HCV HepatitisHCV CardiomyopathiesAn Atypical Variant of Fabrys Disease in Men with Left Ventricular Hypertrophy. Nakao S et al NEJM 1995;333:288-293 Fabrys disease is an X-linked recessive diso

12、rder 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 mmLVPWT 11 mmLVEDD 58 mmLVESD 45 mmLVEF 45%UCG201 Tl ScintigraphyIncreased uptake at IVS

13、and anterior wallIncreased LV cavity compared to those of1.5 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;64:229-236IVST 14mmLVPWT 12mmDisarrangement of bizzare-shapedmyocardial fibers with hypertrophy and interstitial

14、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 of HCM. ASH: 2 cases, APH: 1 caseDepar tment of Cardiovascular

15、 Medicine, Kyoto University LV Aneurysm in a Patient with HCV CardiomyopathyVT , Hepatitis C (IFN Rx), Lymphadenopathy FH: Hepatitis C, HCC in 2 brothers UCG: IVS 16mm, LVPW 13mm, LVDd 47mm, LVDs 37mm, EF 36%RAOEDESMT 52 MDetection of HCV RNA in Heart Tissues from Patients with ARVCnPositive nFreque

16、ncyWHF Council ofCardiomyopathiesNational CardiovascularCenter, Japan63922433.0%66.7%44.4%TotalImmunohistochemical Staining of HCV Core Protein in the Heart from Patients with ARVC/DGenetic Background of the Host Influences the Phenotype of CardiomyopathiesHLA and HCMHLA-DRW4 antigen linkage in pati

17、ents 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;43:445-449HL-A and Hypertrophic Cardiomyopathy Matsumori A et al. Am Heart J 1979;97:428-431 Frequencies

18、of DPB1 Alleles in PatientsWith HCV-Associated Cardiomyopathy and ControlsBoth DPB1*0401 and DPB*0901 was significantly associated with HCV-HCM (* P 0.05), whereas none of DPB1 allele demonstrated significant association with HCV-DCMShichi D, Matsumori A, et al. Int J Immunogenet 2008;35:37-43 DPB1a

19、lleleHCV-HCM(2n=76)HCV-DCM(2n=42)Control(2n=264)*0201 0.145 0.143 0.205*03010.053 0.095 0.057*04010.079* 0.024 0.023*05010.368 0.381 0.413*06010.013 0.004*09010.184* 0.119 0.095Association with Polymorphic of DP -Chain in HCV-HCMShichi D, Matsumori A, et al. Int J Immunogenet 2008;35:37-43 PositionA

20、mino acidresidueCases (n=38)+ -DP 8DP 9DP 11DP 36DP 55DP 57DP 76LeuPheGlyAlaAlaGluMetControls (n=132)+ -OR (95% CI)PPc 6 6 63131 6 6129129129 7 7129129 3 3 3125125 3 3323232 7 73232 0.12(0.03-0.52) 0.12(0.03-0.52) 0.12(0.03-0.52)4.03(1.32-12.35)4.03(1.32-12.35) 0.12(0.03-0.52) 0.12(0.03-0.52)0.0040.

21、0040.0040.0170.0170.0040.0040.0080.0120.0080.0340.0510.0080.012(Pockets)(6)(9)(6)(9)(9)(-)(4)The polymorphic residues from DPB1 alleles located in P9 pockets (at position 36A and 55A) showed positive associations with HCV-HCM. In contrast, five polymorphic residues showed significant negative associ

22、ations: 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 associations composed of DPB1*0401. The Susceptible Gene Mapping for HCV-DCM and HCMwith Microsatellite Markers througho

23、ut the HLA regionOdds RatioCorrected PSusceptibility 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 DPB1 alleles.The candidate genes may encode molecules involved in the immunity and inflammation.Shichi D et alTis

24、sue Antigen2005:66:200HCMDCMHLA and HCV Infection Our study provides new and suggestive information on the immunological involvement of DPB1 gene in the HCV-HCM development. The polymorphic amino acids residues by which the DP chain adopt specificity pocket appear to influence on disease-susceptibil

25、ity 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 each clinical outcome may arise from distinct pathogenic conditions on the basis of differential HLA-mediated immune re

26、sponses.Etiology of HCMHCMGeneticInflammatorySarcomereStorageVirusUnknownEtiology of DCMDCMGeneticInflammatorySarcomereStorageVirusUnknownEtiology of ARVC/DARVC/DGeneticInflammatoryVirusUnknownDefinition and Classifications of CardiomyopathiesI.Etiological ClassificationA.GeneticB.InfectiousC.Nutrit

27、ionalD.UnknownII. Anatomical (Structural) ClassificationA.Dilated a. LV b. RVB.Hypertrophica. Septum b. Diffuse c. Free wall d. ApexIII. Physiological (mechanical) ClassificationA. Systolic failure/dysfunctionB. Diastolic failure/dysfunctionC. BothD. Normal functionIV. Electrical ClassificationA.Ion

28、 channel disordersB.Conduction system diseaseC.OthersDefinition and Classifications of CardiomyopathiesISFC ClassificationProposed ClassificationDCM (with viral infection)DCM (with gene mutation)HCM (with gene mutation and outflow obstruction)Apical HCM (with unknown causes)Apical HCM (with HCV infe

29、ction)ARVC/D (with gene mutation)ARVC/D (with HCV infection)Long QT SyndromeI B, II A a, III AI A, II A a, III AI A, II B a, III B I D, II B d, III BI B, II B d, III BI A, II A b, III AI B, II A b, III AI A, ，IV AI.EtiologicalA.GeneticB.InfectionsC.NutritionalD.UnknownII. Anatomical (Structural)A.Dilated a. LV b. RVB.Hypertrophica. Septum b. Diffuse c. Free wall d. ApexIII. Physiological (mechanical)A. Systolic failure/dysfunctionB. Diastolic failure/dysfunctionC.Both D. Normal FunctionIV. Electrical ClassificationA.Ion channel disordersB.Conduction system diseaseC.Others