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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
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