心肌病詳解演示文稿

心肌病詳解演示文稿現(xiàn)在是1頁(yè)\一共有85頁(yè)\編輯于星期二（優(yōu)選）心肌病現(xiàn)在是2頁(yè)\一共有85頁(yè)\編輯于星期二ClassificationDilatedcardiomyopathy

(DCM)Hypertrophiccardiomyopathy

(HCM)

Restrictivecardiomyopathy

(RCM)Arrhythmogenicrightventricularcardiomyopathy(ARVC)SpecificcardiomyopathyUnclassifiedcardiomyopathy

WHO/ISFC

Circulation1996;93:841現(xiàn)在是3頁(yè)\一共有85頁(yè)\編輯于星期二DilatedCardiomyopathy(DCM)

現(xiàn)在是4頁(yè)\一共有85頁(yè)\編輯于星期二Pathology

dilatedcardiomyopathy(fromBeckerandAnderson)現(xiàn)在是5頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是6頁(yè)\一共有85頁(yè)\編輯于星期二ClinicalFindings-LowCardiacOutputfatigue,headache,hypotension-PulmonaryCongestion -Exertionaldyspnea -orthopneaparoxysmalnocturnaldyspnea-SystemicCongestionEdemaAscitesWeightgain現(xiàn)在是7頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是8頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是9頁(yè)\一共有85頁(yè)\編輯于星期二INVESTIGATION現(xiàn)在是10頁(yè)\一共有85頁(yè)\編輯于星期二Examination

a．X-Rayb．ECG

c．Ultrasoniccardiogramd．Cardiaccatheterizatione．Examinationofradionucide現(xiàn)在是11頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是12頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是13頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是14頁(yè)\一共有85頁(yè)\編輯于星期二DiagosisClinical

cardiacenlargement、arrhythmia、heartfailureUltrasoniccardiogramAmplificationofcardiacchamberDiffuseweakenedmotionofventricularwall

現(xiàn)在是15頁(yè)\一共有85頁(yè)\編輯于星期二Shouldexclude:AllkindsofheartdiseaseswithclearpathogenyDifferentialdiagnosis現(xiàn)在是16頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是17頁(yè)\一共有85頁(yè)\編輯于星期二

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

現(xiàn)在是18頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是19頁(yè)\一共有85頁(yè)\編輯于星期二CRT:CardiacResynchronizationTherapy1.ImprovedhemodynamicsIncreasedCOReducedLVfillingpressuresReducedsympatheticactivity2.ReverseLVDecreasedLVES/EDvolumesIncreasedLVEF現(xiàn)在是20頁(yè)\一共有85頁(yè)\編輯于星期二

Hypertrophiccardoiomyopathy(HCM）

現(xiàn)在是21頁(yè)\一共有85頁(yè)\編輯于星期二ObstructivehypertrophiccardoiomyopathyNonobstructivehypertrophiccardoiomyopathy

Classification

ofHCM現(xiàn)在是22頁(yè)\一共有85頁(yè)\編輯于星期二

Pathology

Asymmetryinterventricularseptumhypertrophy

(90%)Symmetryleftventriclehypertrophy(5%)Asmmetricseptalhypertrophy,ASH）(3%)現(xiàn)在是23頁(yè)\一共有85頁(yè)\編輯于星期二HypertrophicCardiomyopathy現(xiàn)在是24頁(yè)\一共有85頁(yè)\編輯于星期二hypertrophyofcardiacmusclecell,

Pathology現(xiàn)在是25頁(yè)\一共有85頁(yè)\編輯于星期二

Pathology現(xiàn)在是26頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是27頁(yè)\一共有85頁(yè)\編輯于星期二Clinicalsituation-

Symptom

Absenceofsymptoms(>50%)

Commonsymptom:

chestpain

dyspnoea

syncope

suddendeath

現(xiàn)在是28頁(yè)\一共有85頁(yè)\編輯于星期二Pathophysiology現(xiàn)在是29頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是30頁(yè)\一共有85頁(yè)\編輯于星期二Nonobstructive

Noevidentsign,heartenlarge

Obstructive:

systolicmurmuratthelowerleftofsternum;

systolicmurmuinapexof50%patients.Clinicalsituation-Sign現(xiàn)在是31頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是32頁(yè)\一共有85頁(yè)\編輯于星期二Examination現(xiàn)在是33頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是34頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是35頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是36頁(yè)\一共有85頁(yè)\編輯于星期二

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

V4-V6HypertrophyoftheleftventricleAlterationofST-T,TwaveinvertedArrhythmiaECG現(xiàn)在是37頁(yè)\一共有85頁(yè)\編輯于星期二Ultrasoniccardiogram

Thicknessofinterventricularseptum>1.3cmLeftventricularoutflowtract(LVOT)narrowDiastolicdysfunctionSystolicanteriormotion,(SAM)--HOCM現(xiàn)在是38頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是39頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是40頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是41頁(yè)\一共有85頁(yè)\編輯于星期二MechanisimofSAM現(xiàn)在是42頁(yè)\一共有85頁(yè)\編輯于星期二Diagosis

Heredityoffamily

symptomsPhysicalinspectionUltrasoniccardiogram現(xiàn)在是43頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是44頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是45頁(yè)\一共有85頁(yè)\編輯于星期二DifferentialdiagnosisChestpain

,ECGQwave、ST-Tchange——CoronaryheartdiseaseHighvoltageinECG,

Ultrasoniccardiogram--HypertensionChestpain,murmur

–Aorticstenosis現(xiàn)在是46頁(yè)\一共有85頁(yè)\編輯于星期二

Drugsshouldbeavoidedandcarefullyused：Digitalis(exceptAforcontractioninsufficiency)Diureticβ-receptorblockerManagement現(xiàn)在是47頁(yè)\一共有85頁(yè)\編輯于星期二managementβ-blockerCalciumantagonistAmiodarone現(xiàn)在是48頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是49頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是50頁(yè)\一共有85頁(yè)\編輯于星期二室間隔部分心肌切除術(shù)室間隔心肌剝離擴(kuò)大術(shù)

Management

No-drug-treatmentAdaptation：Medicationdifferenceorinefficacy；Differencepressure

inoutflowtractofleftventricle≥50mmHg現(xiàn)在是51頁(yè)\一共有85頁(yè)\編輯于星期二

經(jīng)皮間隔心肌消融術(shù)(TransluminalSeptalMyocardialAblation，PTSMA)

Sigwart于1995年首次應(yīng)用于臨床。ManagementNo-drug-treatment現(xiàn)在是52頁(yè)\一共有85頁(yè)\編輯于星期二TransluminalSeptalMyocardialAblation，PTSMA現(xiàn)在是53頁(yè)\一共有85頁(yè)\編輯于星期二PTSMA現(xiàn)在是54頁(yè)\一共有85頁(yè)\編輯于星期二PTSMA現(xiàn)在是55頁(yè)\一共有85頁(yè)\編輯于星期二ACC/ESCExpertConsensusDocumentonHypertrophicCardiomyopathyJACC2003;42:1現(xiàn)在是56頁(yè)\一共有85頁(yè)\編輯于星期二pacingObstructivehypertrophiccardoiomyopathy

Management

No-drug-treatment現(xiàn)在是57頁(yè)\一共有85頁(yè)\編輯于星期二ThepacingofObstructivehypertrophiccardoiomyopathy現(xiàn)在是58頁(yè)\一共有85頁(yè)\編輯于星期二ThepacingofObstructivehypertrophiccardoiomyopathy現(xiàn)在是59頁(yè)\一共有85頁(yè)\編輯于星期二

highriskpatients（arrhythmia）埋藏式心臟復(fù)律除顫器（AICD）

Management

No-drug-treatment現(xiàn)在是60頁(yè)\一共有85頁(yè)\編輯于星期二RiskFactorsforSuddenDeathinHCMMajorPossibleinIindividualPatientsCardiacarrest(ventricularfibrillation)SpontaneoussustainedventriculartachycardiaFamilyhistoryofprematuresuddendeathUnexplainedSyncopeLVthicknessgreaterthanorequalto30mmAbnormalexercisebloodpressureNonsustainedventriculartachycardia(Holter)AtrialfibrillationMyocardialischemiaLVoutflowobstructionHigh-riskmutationIntense(competitive)physicalexertion*SeetextfordetailsHCM=hypertrophiccardoiomyopathyLV=leftventricular.現(xiàn)在是61頁(yè)\一共有85頁(yè)\編輯于星期二致心律失常性右室心肌病

（arrhythmogenicrightventricularcardiomyopathy,ARVC）

（arrhythmogenicrightventriculardysplasia,ARVD）

（ARVD/C）現(xiàn)在是62頁(yè)\一共有85頁(yè)\編輯于星期二

CharacteristicHereditydiseaseCardiacmusclesinrightventriculararereplacedbyfibrinsandfatsClinicrepresent：

rightheartenlarge

rightheartfailure

and/orventriculartachycardia

suddendeath現(xiàn)在是63頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是64頁(yè)\一共有85頁(yè)\編輯于星期二ExamineECG:LBBB–typeVTorventriclepremuturebeatfrequentlyUCG:

rightventriclelarge，contractionweak，ventricularwallthin，Electric-physiology:

slow

conductionofexcitationthroughrightventricle現(xiàn)在是65頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是66頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是67頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是68頁(yè)\一共有85頁(yè)\編輯于星期二DiagosisanddifferentialdiagnosisSymptomofrightheartfailureventriclepremuturebeat（LBBB)LargerightventricleTwaveconvertiononchestleadDiagosis現(xiàn)在是69頁(yè)\一共有85頁(yè)\編輯于星期二Therapeutics

NOspeciallyeffectivetreatment

Aimatrightheartfailrueandarrhythmia

ventriculartachycardia

repeatly:

radiofrequencycatheterablation

implantablecardioverterdefibrillator現(xiàn)在是70頁(yè)\一共有85頁(yè)\編輯于星期二RestrictiveCardiomyopathy現(xiàn)在是71頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是72頁(yè)\一共有85頁(yè)\編輯于星期二ClinicalManifestationsSymptomsofrightandleftheartfailureJugularVenousPulseprominentxandydescentsEcho-DopplerabnormalmitralinflowpatternprominentEwave(rapiddiastolicfilling)reduceddecelerationtime(LApressure)現(xiàn)在是73頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是74頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是75頁(yè)\一共有85頁(yè)\編輯于星期二現(xiàn)在是76頁(yè)\一共有85頁(yè)\編輯于星期二TreatmentNosatisfactorymedicaltherapyDrugtherapymustbeusedwithcautiondiureticsforextremelyhighfillingprssuresvasodilatorsmaydecreasefillingpressure?Calciumchannelblockerstoimprovediastoliccompliance現(xiàn)在是77頁(yè)\一共有85頁(yè)\編輯于星期二

SpecificCardiomyopathies

Diseaseofmyocardiumwithdefinitudepathogenyorhascorrelationwiththesystemdisease.

特異性心肌病

specificcardiomyopathies現(xiàn)在是78頁(yè)\一共有85頁(yè)\編輯于星期二Classofs