心肌病的分类（英文）.ppt

The4thChina-JapanCardiovascularForumNewClassificationsofCardiomyopathiesAkiraMatsumori,MD,PhD,FACC,FAHA,FESCPresident,InternationalSocietyofCardiomyopathiesandHeartFailureSecretary,WorldHeartFederationChairman,ScientificAdvisoryBoardTreasurer,APSCKyotoUniversityGraduateSchoolofMedicineKyoto,JapanOctober23,2008,Beijing

NormalDilatedHypertrophicRestrictiveArrhythmogenicRightVentricularSystoleDiastoleClassificationofCardiomyopathies

PrimaryCardiomyopathies(predominantlyinvolvingtheheart)GeneticMixed*AcquiredHCMARVC/DLVNCConductionDefectsMitochondrialmyopathiesDCMRestrictive(non-hypertrophiedandnon-dilated)Inflammatory(myocarditis)Stress-provoked（”tako-tsubo”）PeripartumTachycardia-inducedInfantsofinsulin-dependentDiabeticmothersCirculation2006;113:1807-1816IonChannelDisordersLQTSBrugadaSQTSCPVTAsianSUNDS*PredominantlynongeneticPRKAG2DanonGlycogenStorage

SecondaryCardiomyopathiesInfiltrativeStorageToxicityEndomyocardialInflammatory(granulomatous)EndocrineCardiofacialNeuromuscular/neurologicalNutritionaldeficiencyAutoimmune/collagenElectrolyteimbalanceConsequenceofCancertherapyCirculation2006;113:1807-1816

GeneMutationsAssociatedwithMultiplePhenotypesofCardiomyopathies?-MyosinheavychainCardiactroponinT?-TropomyosinCardiacmyosinbindingProteinCCardiactroponinIActinTitinDesminMuscleLIMproteinTelethoninDesmoplakinPlakoglobinGenePhenotypesHCMDCMRCMARVC/DSarcomereproteinsZ-discproteinDesmosomeproteinIntermediatefilaments

HypertrophicCardiomyopathy?Abouthalfofcasesshowfamilialoccurrence.?AbouthalfoffamilialHCMhavegenemutationsofsarcomereproteins(~25%oftotalHCM).?Specific(Secondary)cardiomyopathiesoftenshowHCMphenotype.Storage:Fabry’sdiseaseInflammatory:Sarcoidosis HCVcardiomyopathy

CoxsackieBvirusAdenovirusHepatitisCvirusDilatedCardiomyopathyMyocarditisViralInfectionandPhenotypesofCardiomyop