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1、1Cardiomyopathy周晓莉重医附一院心内科2016.102DefinitionDiseaseofthemyocardiumassociatedwithcardiacdysfunction.nottheresultofdiseaseordysfunctionofothercardiacstructures…myocardialinfarction,systemichypertension,valvularstenosisorregurgitation”3Classification(WHO1995)acceptedworld-w
2、ideprimaryDilatedCardiomyopathy(DCM)HypertrophicCardiomyopathy(HCM)RestrictiveCardiomyopathy(RCM)ArrhythmogenicrightventricularCardiomyopathy(ARVC)UnclassifiedCardiomyopathySpecificDilatedCardiomyopathy,DCMDilatedcardiomyopathyisaprogressivediseaseofheartmusclethatischar
3、acterizedbyventricularchamberenlargementandcontractiledysfunctionwithnormalleftventricular(LV)wallthickness.Therightventriclemayalsobedilatedanddysfunctional.5EtiologyInheriteddiseaseInfections,viral…ToxinsNutritionalInfiltrativeMetabolic……geneticmutationsthecurrent5(LMN
4、A,MYH7,TNNT2,SCN5A,MYBPC3)novelmutations(TTN,BAG3)infamilialdilatedcardiomyopathy.Findingaspecificcauseforanindividualcasemaybedifficult.6EpidemiologyThetrueincidenceofcardiomyopathiesisunknown.19casesper100,000inChina,recentlyreported.3-10casesper100,000;20,000newcasesp
5、eryearintheU.S.A.7PathologyIncreasedheartsizeandweightVentriculardilatation,normalwallthicknessFibrosisThrombosis8Fig1DilatedCardiomyopathyDilatedheart,wallthickness9Fig2DilatedCardiomyopathyMyocardialfibrosis10Fig3DilatedCardiomyopathyThrombosis11PathophysiologyEarlycom
6、pensatorymechanismsleadtofurthermyocardialinjury,dysfunction,andgeometricremodelingNeurohormonalactivationCirculatingcytokinesasmediatorsofmyocardialinjury12ClinicalmanifestationOccursinanyage,esp.in30－50yearsold.Men2timesmorefrequentthanwomen.Theincidenceisabout4-8per10
7、0000person-yearsandtheprevalence36.5per100000individuals.Symptomsmaybegradual.13IdiopathicDilatedCardiomyopathyObservedSurvivalof104PatientsYearsAmJCardiol1981;47:52514Symptomsheartfailurepulmonarycongestion(leftHF)dyspnea(exertional,atrest,paroxysmalnocturnaldyspnea,ort
8、hopnea)systemiccongestion(rightHF)edema,nausea,abdominalpain,nocturialowcardiacoutputfatigueandweakness
