心肌病的分类英文幻灯.pptxVIP

心肌病的分类英文幻灯第1页/共38页 NormalDilatedHypertrophicRestrictiveArrhythmogenicRight VentricularSystoleDiastoleClassification of Cardiomyopathies第2页/共38页 Primary Cardiomyopathies(predominantly involving the heart)GeneticMixed*AcquiredHCMARVC/DLVNCConductionDefectsMitochondrialmyopathiesDCMRestrictive(non-hypertrophiedand non-dilated)Inflammatory(myocarditis)Stress-provoked（”tako-tsubo”）PeripartumTachycardia-inducedInfants of insulin-dependentDiabetic mothersCirculation 2006; 113:1807-1816Ion Channel DisordersLQTSBrugadaSQTSCPVTAsian SUNDS*Predominantly nongeneticPRKAG2DanonGlycogenStorage第3页/共38页 Secondary CardiomyopathiesInfiltrativeStorageToxicityEndomyocardialInflammatory (granulomatous)EndocrineCardiofacialNeuromuscular/ neurologicalNutritional deficiencyAutoimmune/ collagenElectrolyte imbalanceConsequence of Cancer therapyCirculation 2006; 113:1807-1816第4页/共38页 Gene Mutations Associated with Multiple Phenotypes of Cardiomyopathies?-Myosin heavy chainCardiac troponin T?-TropomyosinCardiac myosin bindingProtein CCardiac troponin I ActinTitinDesminMuscle LIM proteinTelethoninDesmoplakinPlakoglobinGenePhenotypesHCMDCMRCMARVC/DSarcomere proteinsZ-disc proteinDesmosome proteinIntermediate filaments第5页/共38页 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) cardiomyopathies often show HCM phenotype. Storage: Fabry’s disease Inflammatory: Sarcoidosis HCV cardiomyopathy第6页/共38页 Coxsackie 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 Cardiomyopathy第7页/共38页 Viral Infection of the HeartDiffuseCHF/DCMSystolic HFRegionalAneurysmSubendocardialRCMDiastolic HFARVC/DLV AneurysmRandomHypertrophy/HCMVirusReceptorMyocyteFibroblast第8页/共38页 Completereco