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1、心肌病心内科心肌病心内科CaseCase AA 3636 yearyear oldold manman comescomes toto youryour officeofficecomplainingcomplaining ofof threethree monthsmonths ofof progressiveprogressivefatiguefatigue andand dyspneadyspnea onon exertion.exertion.SeveralSeveraltimestimes inin thethe pastpast monthmonth hehe hashas awa
2、kenedawakenedfromfrom sleepsleep withwith severesevere breathlessnessbreathlessness andandfeltfelt a a needneed toto sitsit upup inin orderordertoto breath.breath.HeHedeniesanychestpain.deniesanychestpain.HeHe hashas nono pastpast medicalmedical historyhistory ofof heartheartdisease,disease,hyperten
3、sionhypertensionorordiabetes.diabetes.HisHisfamilyfamilyhistoryhistoryisisnegativenegativeforforheartheartdisease.disease.HeHedoesdoesnotnotsmokesmokeandanddrinksdrinksalcoholalcoholonlyonlyrarely.rarely.HeHetakesnomedications.takesnomedications.n nMicroscopicexamination:Myocardial cell may be hyper
4、trophy,denaturation,fibrosis or necrosis.PathologyPathophysiologyPumplessbloodforthebloodforthebodysneedsbodysneedsHeartfailureMyocardialchangesinvolveconductionsystemArrhythmiaMuralthrombusfalloffEmbolismClinicalManifestation n nSymptoms:NosymptomintheearlystageFatigue,dyspnea,orthopneaAnorexia,ede
5、maPalpitation,arrhythmia(Af,VT)Embolism(brain,lung,lowerextremity)Suddendeath(VT,embolism,bradycardia)ClinicalManifestation n nSigns:CardiacdullnessextendstoleftanddownwardsRales,pleuraleffusionJugularvenousdistention,hepatomegaly,edemaGalloprhythm,S3,S4Systolicmurmurovertheapexarea(mitralinsufficie
6、ncy)AccessoryExaminationn nChestX-rayEnlargementoftheheartSignsofpulmonarycongestionAccessoryExaminationn nECGAtrialfibrillationConductionblockST-TchangesLow-voltageQRSPathologicQwaveVentricularprematureortachycardiaAccessoryExaminationAccessoryExaminationn nEchocardiographyDilatedleftventricleorbot
7、hventriclesRelativemitral/tricuspidinsufficiencyduetodilatedleft/rightventricleVentricularwallsnotthickPoorlycontractileleftventricleEF50%AccessoryExaminationn nCoronaryangiography:Usuallynormaln nCardiacbiopsy:Fibrosisormyocardialcelluarhypertrophy,denaturationornecrosisExcludeotherheartdiseasesDil
8、atedheartArrhythmiaHeartfailureEcho:dilated&poorlycontractileventricleCoronary/Rheu-matic/HypertensiveheartdiseaseDiagnosisSpecificcardiomyopathy EXCLUDEDiagnosisDCMIschemiccardiomyopathyICMAgeHistoryOfteninmiddleage,40yearsoldMyocarditis,NohistoryofanginaRiskfactorsofCHD,historyofanginaormyocardial
9、infarctionDifferentialdiagnosisDCMICMEchoOftenfourchambersdilated,theweakmovementofwholeheart,muralthrombusinsomepatientsOftenonlyleftventricledilated,segmentalmovementabnormality,ventricularaneurysminsomepatients Angio-graphyNormalMultivesseldiseaseDifferentialdiagnosisTreatmentn nGeneraltreatmentD
10、ecreasedactivityandbedrestSaltrestrictionStopalcoholNutritionalsupportTreatmentn nTreatmentofheartfailureDigitalisDiureticsVasodilatorsACEI(angiotensinconvertingenzymeinhibitors)ARB(angiotensinreceptorblockers)Treatmentn nAntiarrhythmictreatmentBetablockers(startwithlow-dose,increasedosegradually)AmiodaronePacemakerimplantationICD(implantablecardioverterdefibrillator)Treatmentn nCRT(CardiacResynchronizationTherapy)n nSurgery:hearttransplantationQuestionsn nWhatareechocardiographicfindingsinDCM?n nHowtodistinguishbetweenDCMandICM?n nHowtotreatDCM?