心肌病详解演示文稿

心肌病详解演示文稿当前第1页\共有85页\编于星期三\23点（优选）心肌病当前第2页\共有85页\编于星期三\23点ClassificationDilatedcardiomyopathy

(DCM)Hypertrophiccardiomyopathy

(HCM)

Restrictivecardiomyopathy

(RCM)Arrhythmogenicrightventricularcardiomyopathy(ARVC)SpecificcardiomyopathyUnclassifiedcardiomyopathy

WHO/ISFC

Circulation1996;93:841当前第3页\共有85页\编于星期三\23点DilatedCardiomyopathy(DCM)

当前第4页\共有85页\编于星期三\23点Pathology

dilatedcardiomyopathy(fromBeckerandAnderson)当前第5页\共有85页\编于星期三\23点当前第6页\共有85页\编于星期三\23点ClinicalFindings-LowCardiacOutputfatigue,headache,hypotension-PulmonaryCongestion -Exertionaldyspnea -orthopneaparoxysmalnocturnaldyspnea-SystemicCongestionEdemaAscitesWeightgain当前第7页\共有85页\编于星期三\23点当前第8页\共有85页\编于星期三\23点当前第9页\共有85页\编于星期三\23点INVESTIGATION当前第10页\共有85页\编于星期三\23点Examination

a．X-Rayb．ECG

c．Ultrasoniccardiogramd．Cardiaccatheterizatione．Examinationofradionucide当前第11页\共有85页\编于星期三\23点当前第12页\共有85页\编于星期三\23点当前第13页\共有85页\编于星期三\23点当前第14页\共有85页\编于星期三\23点DiagosisClinical

cardiacenlargement、arrhythmia、heartfailureUltrasoniccardiogramAmplificationofcardiacchamberDiffuseweakenedmotionofventricularwall

当前第15页\共有85页\编于星期三\23点Shouldexclude:AllkindsofheartdiseaseswithclearpathogenyDifferentialdiagnosis当前第16页\共有85页\编于星期三\23点当前第17页\共有85页\编于星期三\23点

Management1.Heartfailure:digoxin、diuretic、ACEI、β-blocker2.Preventtheembolism:LVEF<30%、Aforhavethehistoryofembolism:Warfarin（INR2.0-3.0）3.Preventionofsuddendeath4.Hearttransplantation

当前第18页\共有85页\编于星期三\23点当前第19页\共有85页\编于星期三\23点CRT:CardiacResynchronizationTherapy1.ImprovedhemodynamicsIncreasedCOReducedLVfillingpressuresReducedsympatheticactivity2.ReverseLVDecreasedLVES/EDvolumesIncreasedLVEF当前第20页\共有85页\编于星期三\23点

Hypertrophiccardoiomyopathy(HCM）

当前第21页\共有85页\编于星期三\23点ObstructivehypertrophiccardoiomyopathyNonobstructivehypertrophiccardoiomyopathy

Classification

ofHCM当前第22页\共有85页\编于星期三\23点

Pathology

Asymmetryinterventricularseptumhypertrophy

(90%)Symmetryleftventriclehypertrophy(5%)Asmmetricseptalhypertrophy,ASH）(3%)当前第23页\共有85页\编于星期三\23点HypertrophicCardiomyopathy当前第24页\共有85页\编于星期三\23点hypertrophyofcardiacmusclecell,

Pathology当前第25页\共有85页\编于星期三\23点

Pathology当前第26页\共有85页\编于星期三\23点当前第27页\共有85页\编于星期三\23点Clinicalsituation-

Symptom

Absenceofsymptoms(>50%)

Commonsymptom:

chestpain

dyspnoea

syncope

suddendeath

当前第28页\共有85页\编于星期三\23点Pathophysiology当前第29页\共有85页\编于星期三\23点当前第30页\共有85页\编于星期三\23点Nonobstructive

Noevidentsign,heartenlarge

Obstructive:

systolicmurmuratthelowerleftofsternum;

systolicmurmuinapexof50%patients.Clinicalsituation-Sign当前第31页\共有85页\编于星期三\23点当前第32页\共有85页\编于星期三\23点Examination当前第33页\共有85页\编于星期三\23点当前第34页\共有85页\编于星期三\23点当前第35页\共有85页\编于星期三\23点当前第36页\共有85页\编于星期三\23点

AbnormalQwave（30-50%）inleadII、III、AVFand

V4-V6HypertrophyoftheleftventricleAlterationofST-T,TwaveinvertedArrhythmiaECG当前第37页\共有85页\编于星期三\23点Ultrasoniccardiogram

Thicknessofinterventricularseptum>1.3cmLeftventricularoutflowtract(LVOT)narrowDiastolicdysfunctionSystolicanteriormotion,(SAM)--HOCM当前第38页\共有85页\编于星期三\23点当前第39页\共有85页\编于星期三\23点当前第40页\共有85页\编于星期三\23点当前第41页\共有85页\编于星期三\23点MechanisimofSAM当前第42页\共有85页\编于星期三\23点Diagosis

Heredityoffamily

symptomsPhysicalinspectionUltrasoniccardiogram当前第43页\共有85页\编于星期三\23点当前第44页\共有85页\编于星期三\23点当前第45页\共有85页\编于星期三\23点DifferentialdiagnosisChestpain

,ECGQwave、ST-Tchange——CoronaryheartdiseaseHighvoltageinECG,

Ultrasoniccardiogram--HypertensionChestpain,murmur

–Aorticstenosis当前第46页\共有85页\编于星期三\23点

Drugsshouldbeavoidedandcarefullyused：Digitalis(exceptAforcontractioninsufficiency)Diureticβ-receptorblockerManagement当前第47页\共有85页\编于星期三\23点managementβ-blockerCalciumantagonistAmiodarone当前第48页\共有85页\编于星期三\23点当前第49页\共有85页\编于星期三\23点当前第50页\共有85页\编于星期三\23点室间隔部分心肌切除术室间隔心肌剥离扩大术

Management

No-drug-treatmentAdaptation：Medicationdifferenceorinefficacy；Differencepressure

inoutflowtractofleftventricle≥50mmHg当前第51页\共有85页\编于星期三\23点

经皮间隔心肌消融术(TransluminalSeptalMyocardialAblation，PTSMA)

Sigwart于1995年首次应用于临床。ManagementNo-drug-treatment当前第52页\共有85页\编于星期三\23点TransluminalSeptalMyocardialAblation，PTSMA当前第53页\共有85页\编于星期三\23点PTSMA当前第54页\共有85页\编于星期三\23点PTSMA当前第55页\共有85页\编于星期三\23点ACC/ESCExpertConsensusDocumentonHypertrophicCardiomyopathyJACC2003;42:1当前第56页\共有85页\编于星期三\23点pacingObstructivehypertrophiccardoiomyopathy

Management

No-drug-treatment当前第57页\共有85页\编于星期三\23点ThepacingofObstructivehypertrophiccardoiomyopathy当前第58页\共有85页\编于星期三\23点ThepacingofObstructivehypertrophiccardoiomyopathy当前第59页\共有85页\编于星期三\23点

highriskpatients（arrhythmia）埋藏式心脏复律除颤器（AICD）

Management

No-drug-treatment当前第60页\共有85页\编于星期三\23点RiskFactorsforSuddenDeathinHCMMajorPossibleinIindividualPatientsCardiacarrest(ventricularfibrillation)SpontaneoussustainedventriculartachycardiaFamilyhistoryofprematuresuddendeathUnexplainedSyncopeLVthicknessgreaterthanorequalto30mmAbnormalexercisebloodpressureNonsustainedventriculartachycardia(Holter)AtrialfibrillationMyocardialischemiaLVoutflowobstructionHigh-riskmutationIntense(competitive)physicalexertion*SeetextfordetailsHCM=hypertrophiccardoiomyopathyLV=leftventricular.当前第61页\共有85页\编于星期三\23点致心律失常性右室心肌病

（arrhythmogenicrightventricularcardiomyopathy,ARVC）

（arrhythmogenicrightventriculardysplasia,ARVD）

（ARVD/C）当前第62页\共有85页\编于星期三\23点

CharacteristicHereditydiseaseCardiacmusclesinrightventriculararereplacedbyfibrinsandfatsClinicrepresent：

rightheartenlarge

rightheartfailure

and/orventriculartachycardia

suddendeath当前第63页\共有85页\编于星期三\23点当前第64页\共有85页\编于星期三\23点ExamineECG:LBBB–typeVTorventriclepremuturebeatfrequentlyUCG:

rightventriclelarge，contractionweak，ventricularwallthin，Electric-physiology:

slow

conductionofexcitationthroughrightventricle当前第65页\共有85页\编于星期三\23点当前第66页\共有85页\编于星期三\23点当前第67页\共有85页\编于星期三\23点当前第68页\共有85页\编于星期三\23点DiagosisanddifferentialdiagnosisSymptomofrightheartfailureventriclepremuturebeat（LBBB)LargerightventricleTwaveconvertiononchestleadDiagosis当前第69页\共有85页\编于星期三\23点Therapeutics

NOspeciallyeffectivetreatment

Aimatrightheartfailrueandarrhythmia

ventriculartachycardia

repeatly:

radiofrequencycatheterablation

implantablecardioverterdefibrillator当前第70页\共有85页\编于星期三\23点RestrictiveCardiomyopathy当前第71页\共有85页\编于星期三\23点当前第72页\共有85页\编于星期三\23点ClinicalManifestationsSymptomsofrightandleftheartfailureJugularVenousPulseprominentxandydescentsEcho-DopplerabnormalmitralinflowpatternprominentEwave(rapiddiastolicfilling)reduceddecelerationtime(LApressure)当前第73页\共有85页\编于星期三\23点当前第74页\共有85页\编于星期三\23点当前第75页\共有85页\编于星期三\23点当前第76页\共有85页\编于星期三\23点TreatmentNosatisfactorymedicaltherapyDrugtherapymustbeusedwithcautiondiureticsforextremelyhighfillingprssuresvasodilatorsmaydecreasefillingpressure?Calciumchannelblockerstoimprovediastoliccompliance当前第77页\共有85页\编于星期三\23点

SpecificCardiomyopathies

Diseaseofmyocardiumwithdefinitudepathogenyorhascorrelationwiththesystemdisease.

特异性心肌病

specificcardiomyopathies当前第78页\共有85页\编于星期三\23点Classo