石大内科学课件03循环系统疾病-10心肌病

Cardiomyopathy心肌病Definition “Aprimarydisorderoftheheartmusclethatcausesabnormalmyocardialperformanceandisnottheresultofdiseaseordysfunctionofothercardiacstructures…myocardialinfarction,systemichypertension,valvular

stenosisorregurgitation”心肌病特点总结病因不明心肌功能障碍与特异性心肌病(原因已知)鉴别

WHOClassification

(1995年根据病理生理学)1）扩张型心肌病(Dilated,DCM).2)肥厚型心肌病(Hypertrophic

HCM).3）限制型心肌病(Restrictive,RCM).4）致心律失常型右室心肌病(ARVD/C)

另有不定型的心肌病特异性心肌病---Specificheartmuscledisease(secondary)感染性:细菌,病毒,支原体,真菌等代谢性:甲亢,甲低,糖尿病,脚气病全身性:SLE,内分湿性关节炎,硬皮病遗传性，家族性:假性肥大型肌萎缩过敏性及中毒性:酒精,磺胺,阿霉素扩张型心肌病IdiopathicDilatedCardiomyopathyIDCIDC-DefinitionadiseaseofunknownetiologythatprincipallyaffectsthemyocardiumLVdilatationandsystolicdysfunctionpathologyincreasedheartsizeandweightventriculardilatation,normalwallthicknessheartdysfunctionoutofportiontofibrosis

病因

1）病毒感染；2）基因及自身免疫（发现本病与组织相容性有关）；3）遗传，代谢，中毒等。IncidenceandPrognosis3-10casesper100,00020,000newcasesperyearintheU.S.A.deathfromprogressivepumpfailure

1-year 25%

2-year 35-40%

5-year 40-80%（现存活率可达７０％）stabilizationobservedin20-50%ofpatientcompleterecoveryisrareIdiopathicDilatedCardiomyopathy

ObservedSurvivalof104PatientsYearsAmJCardiol1981;47:525病理心腔扩张

室壁变薄：弥漫性纤维斑痕形成附壁血栓心肌细胞肥大，变性，纤维化临床表现Symptomsofheartfailurepulmonarycongestion(leftHF)

dyspnea(rest,exertional,nocturnal),orthpneasystemiccongestion(rightHF)

edema,nausea,abdominalpain,nocturia(起病隐匿，发病即表现为心功能不全，严重心衰)lowcardiacoutput：fatigueandweakness低血压（hypotension）心动过速（tachycardia）

呼吸急促（tachypnea）猝死（suddendeath）心律失常（cardiacarrythmia)扩张型心肌病四大特点：心力衰竭心律失常猝死栓塞体征心脏扩大Ｓ３或Ｓ４心音奔马律收缩期杂音心律失常（cardiacarrythmia)CardiacImagingChestradiogramElectrocardiogram24-hourambulatoryECG(Holter)lightheadedness,palpitation,syncopeRadionuclideventriculographyCardiaccatheterizationage>40,ischemichistory,highriskprofile,abnormalECG心胸比例50%Chestradiogram超声心动图示室壁运动弥漫性减弱，射血分数小于正常值。图注：左室长轴切面显示左房左室扩大二尖瓣开放幅度减少ECGEndomyocardialBiopsy

心内膜心肌活检：病理检查无特异性，但有助于特异性心肌病和急性心肌炎诊断。

ManagementofDCM限制活动

(Limitactivitybasedonfunctionalstatus)限制钠盐saltrestrictionofa(5gNaCl)die限水(fluidrestrictionforsignificantlowNa+)针对心衰ACEinhibitorsdiureticsdigoxin,Carvedilol(B-blockade)nitratecombination心脏移植抗心律失常治疗双腔或多腔起搏TransvenousApproachStandardpacingleadsinRAandRVSpeciallydesignedleftheartleadplacedinaleftventricularcardiacveinviathecoronarysinusAchievingCardiacResynchronization(CRT)

MechanicalGoal:PaceRightandLeftVentriclesCardiacResynchronizationSystemCardiacResynchronizationTherapy(CRT)Cardiacresynchronization,inassociationwithanoptimizedAVdelay,improveshemodynamicperformancebyforcingtheleftventricletocompletecontractionandbeginrelaxationearlier,allowinganincreaseinventricularfillingtime.Coordinateactivationoftheventriclesandseptum.ECGdepictingcardiacresynchronizationECGdepictingIVCD肥厚型心肌病Hypertrophic

CardiomyopathyHCMHypertrophic

CardiomyopathyFirstdescribedbytheFrenchandGermansaround1900uncommonwithoccurrenceof0.02to0.2%ahypertrophiedandnon-dilatedleftventricleintheabsenceofanotherdisease

smallLVcavity,asymmetricalseptalhypertrophy(ASH),systolicanteriormotionofthemitralvalveleaflet(SAM)65%35%10%/hcmFamilialHCMFirstreportedbySeidmanetalin1989occursasautosomaldominantin50%5differentgenesonatleast4chromosomewithover3dozenmutationschromosome14(myosin)chromosome1(troponinT)chromosome15(tropomyosin)chromosome11(?)

图注：心脏体积明显增大，室壁增厚，尤以左室壁及室间隔为著（正常0.8--1.2cm）。图注：8个月小男孩，尸解发现心脏明显大于手拳，肉柱及乳头肌粗大并竖立于心腔。组织学表现为弥漫性心肌纤维增粗，横纹不清，并可见胞浆内红染细颗粒。病理生理

1）左室流出道梗阻。2）舒张功能异常。3）心肌缺血。

Myocardialischemia

musclemass,fillingpressure,O2demand

vasodilatorreserve,capillarydensity

abnormalintramuralcoronaryarteries

systoliccompressionofarteries临床表现：1）呼吸困难:dyspneain90%

2）心前区疼痛:anginapectorisin75%3）乏力，头晕及晕厥:fatigue,dizziness,syncope4）心慌:palpitation,5）心衰及猝死体征心脏轻度增大第四心音胸骨左缘３－４肋间及心尖部喷射性收缩期杂音辅助检查胸片心电图心脏超声心动图心导管检查心内膜活检室间隔厚度／后壁＞1.3;室间隔流出道向左室突出；SAM现象左室肥厚老损；IIIIIavF

avLV4V5病理性Ｑ波Managementbeta-adrenergicblockerscalciumantagonistdisopyramideamiodarone,sotololDDDpacingmyotomy-myectomy心肌消融NaturalHistoryannualmortality1.4%SCD0.7%,是HCM主要死因HF0.5%中风0.2％RiskFactorsforSCDYoungage(<30years)“Malignant”familyhistoryofsuddendeathGenemutationspronetoSCD(ex.Arg403Gln)AbortedsuddencardiacdeathSustainedVTorSVTRecurrentsyncopeintheyoungNonsustainedVT(HolterMonitoring)Bradyarrhythmias(occultconductiondisease)BrHeartJ1994;72:S13RecommendationsforAthleticActivityAvoidmostcompetitivesports(whetherornotsymptomsand/oroutflowgradientarepresent)Low-riskolderpatients(>30yrs)mayparticipateinathleticactivityifallofthefollowingareabsentRestrictiveCardiomyopathies

RCMHallmark:abnormaldiastolicfunctionrigidventricularwallwithimpairedventricularfillingbearsomefunctionalresemblancetoconstrictivepericarditisimportanceliesinitsdifferentiationfromoperableconstrictivepericarditisClinicalManifestationsSymptomsofrightandleftheartfailureJugularVenousPulseprominentxandydescentsEcho-DopplerabnormalmitralinflowpatternprominentEwave(rapiddiastolicfilling)reduceddecelerationtime(LApressure)内膜及内膜下纤维增生，心内膜硬化心肌炎A、感染性疾病病程中发生的心肌炎B、过敏或变态反应所致的心肌炎。C、化学，物理或药物致的心肌炎。图注：亲心病毒引起的原发性心肌炎症，最常见的为柯萨奇病毒。主要病变为心肌细胞的弥漫性变性坏死，单核