內(nèi)科學(xué)教學(xué)課件：27 心臟瓣膜疾病

ValvularHeartDisease2012年我國(guó)高血壓

患病率，知曉率，治療率和控制率國(guó)家衛(wèi)計(jì)委疾病預(yù)防控制局，中國(guó)居民營(yíng)養(yǎng)與慢性病狀況報(bào)告(2015年)，人民衛(wèi)生出版社25.2%2012年中國(guó)18歲及以上居民高血壓患病率為25.2%心血管醫(yī)生Keypoints瓣膜損害：狹窄或者關(guān)閉不全我國(guó)，風(fēng)濕熱二尖瓣最常見，主動(dòng)脈瓣最重要診斷靠心超治療靠外科介入也可以主要病因二尖瓣：風(fēng)濕性，感染主動(dòng)脈瓣：二葉式主動(dòng)脈瓣三尖瓣返流：功能性/脫垂，心內(nèi)膜炎肺動(dòng)脈瓣：……

風(fēng)濕熱

RheumaticFever

風(fēng)濕熱（rheumaticfever）

A組乙型溶血性鏈球菌感染后發(fā)生的全身結(jié)締組織的非化膿性炎性疾病,為常見的風(fēng)濕性疾病。主要表現(xiàn)為：心臟炎、關(guān)節(jié)炎、舞蹈病、環(huán)形紅斑及皮下小結(jié)，以心臟損害最為嚴(yán)重和多見，反復(fù)發(fā)作可導(dǎo)致永久性心臟瓣膜病變。A組乙型溶血性鏈球菌咽峽炎的并發(fā)癥約0.3%～3%病例于1～4周后發(fā)生風(fēng)濕熱鏈球菌在咽部存在的時(shí)間致病菌株患兒遺傳學(xué)背景

莢膜：透明質(zhì)酸酶細(xì)胞壁：M蛋白、M相關(guān)蛋白N-乙酰葡糖胺、鼠李糖

細(xì)胞膜：蛋白、脂質(zhì)、糖關(guān)節(jié)心肌心內(nèi)膜下丘腦/尾核心肌A組β鏈球菌相同的抗原性，產(chǎn)生免疫交叉反應(yīng)鏈球菌感染誘導(dǎo)的異常免疫反應(yīng)免疫復(fù)合物致?。号c鏈球菌抗原模擬的自身抗原與鏈球菌抗體形成循環(huán)免疫復(fù)合物，沉積于人體關(guān)節(jié)滑膜、心肌、心內(nèi)膜，激活補(bǔ)體產(chǎn)生炎性病變。細(xì)胞免疫反應(yīng)異常：

T淋巴細(xì)胞對(duì)心肌的毒性作用；淋巴細(xì)胞增殖反應(yīng)降低、自然殺傷細(xì)胞功能增強(qiáng)；扁桃體單核細(xì)胞對(duì)鏈球菌抗原的免疫反應(yīng)異常。

病變過程分急性滲出期、增生期、硬化期，三期可交錯(cuò)存在，持續(xù)約4～6個(gè)月。急性滲出期（1個(gè)月左右）部位：心臟、關(guān)節(jié)、皮膚病理：組織水腫、變性或壞死，炎性細(xì)胞浸潤(rùn)，纖維素及漿液滲出。

增生期（3～4個(gè)月）部位：心肌和心瓣膜，還可分布于肌肉及結(jié)締組織（皮下小結(jié)）病理：風(fēng)濕小體（Aschoffbody)，是風(fēng)濕熱的病理診斷依據(jù)，表明風(fēng)濕活動(dòng)。風(fēng)濕小體模式圖纖維素樣物質(zhì)多核巨噬細(xì)胞淋巴細(xì)胞硬化期（2～3個(gè)月）部位：二尖瓣﹥主動(dòng)脈瓣病理：纖維組織增生和瘢痕形成。以心臟瓣膜損害最突出，在瓣膜的閉鎖線上出現(xiàn)贅生物，使瓣膜增厚。瓣膜贅生物（箭頭處）瓣膜贅生物（箭頭處）一般表現(xiàn)急性患者半數(shù)以上病前1～5周有咽炎、扁桃體炎或猩紅熱感染史。發(fā)熱急性起病….38℃～40℃

，2周后低熱隱匿起病….低熱或無熱關(guān)節(jié)痛、貧血、鼻衄、腹痛心臟炎40%～50%心肌、心內(nèi)膜、心包均可受累。首次風(fēng)濕熱發(fā)作時(shí)，一般于起病1～2周內(nèi)出心臟炎癥狀，需嚴(yán)密觀察。心臟炎/全心炎＝心肌炎﹢心內(nèi)膜炎﹢心包炎心肌炎輕重不一心動(dòng)過速，第一心音減弱；心臟擴(kuò)大，心尖搏動(dòng)彌散，聞及奔馬律；心尖部可聽到Ⅱ/Ⅵ級(jí)收縮期吹風(fēng)樣雜音或主動(dòng)脈瓣區(qū)舒張中期雜音；ECG：P-R間期延長(zhǎng)，ST-T改變；心內(nèi)膜炎二尖瓣區(qū)出現(xiàn)Ⅱ/Ⅵ級(jí)以上全收縮期雜音心尖區(qū)有柔和、短促的舒張中期雜音主動(dòng)脈瓣區(qū)舒張期嘆氣樣雜音反復(fù)發(fā)作后造成永久性瓣膜損害心包炎

心前區(qū)疼痛、呼吸困難及端坐呼吸；心包摩擦音、心音遙遠(yuǎn)、心前區(qū)搏動(dòng)消失；心包填塞的表現(xiàn)：頸靜脈怒張、肝臟腫大；一旦有心包炎表現(xiàn)，提示有嚴(yán)重心臟損害，易發(fā)生心力衰竭；關(guān)節(jié)炎50%～60%特點(diǎn)：為多發(fā)性、游走性大關(guān)節(jié)炎典型表現(xiàn)：有紅、腫、熱、痛和功能障礙,不典型的僅表現(xiàn)關(guān)節(jié)痛；發(fā)病很少超過1個(gè)月不留畸形舞蹈?。?%～10%8～12歲的女孩多見;不自主、突發(fā)、無目的的快速運(yùn)動(dòng)，在興奮和注意力集中時(shí)加劇，睡眠時(shí)消失，可累及全身肌肉，以面部和上肢肌肉為主;鏈感后1～6月發(fā)生，也可為首發(fā)癥狀;自限性，病程平均三個(gè)月。皮膚癥狀環(huán)形紅斑:少見;環(huán)形或半環(huán)形邊界清楚的淡色紅斑，時(shí)隱時(shí)現(xiàn)，可持續(xù)數(shù)周。皮下小結(jié):5%，常伴心臟炎;發(fā)于大關(guān)節(jié)伸面及枕、額、脊突處;直徑0.1～1cm,質(zhì)硬不痛，2～4周消失。其它皮疹:蕁麻疹、結(jié)節(jié)性紅斑、多形紅斑。

環(huán)形紅斑（箭頭處）

一、鏈球菌感染證據(jù)

咽拭子培養(yǎng)ASO↑

抗脫氧核糖核酸酶（anti-DNaseB)

抗鏈球菌激酶（ASK）抗透明質(zhì)酸酶（AH）

二、風(fēng)濕熱活動(dòng)指標(biāo)

血沉增快C-反應(yīng)蛋白和粘蛋白增高白細(xì)胞計(jì)數(shù)增高三、心臟損害依據(jù)X線檢查:嚴(yán)重的出現(xiàn)心胸比例增大。心電圖:常見P-R間期延長(zhǎng)和I°-AVB，可出現(xiàn)ST-T改變及低電壓，心律失常;

超聲心動(dòng)圖:可顯示有無瓣膜增厚、水腫、狹窄和關(guān)閉不全，心臟增大及心包積液;

1992年修訂的Jones診斷標(biāo)準(zhǔn)

主要表現(xiàn)次要表現(xiàn)鏈球菌感染證據(jù)心臟炎發(fā)熱多發(fā)性關(guān)節(jié)炎關(guān)節(jié)痛﹡

咽拭子培養(yǎng)陽(yáng)性舞蹈病血沉增快快速鏈球菌抗原試驗(yàn)陽(yáng)性皮下結(jié)節(jié)CRP陽(yáng)性抗鏈球菌的抗體滴度增高環(huán)形紅斑P-R間期延長(zhǎng)﹡﹡

★2項(xiàng)主要表現(xiàn)，或1項(xiàng)主要指標(biāo)伴2項(xiàng)次要表現(xiàn)者，可診斷為風(fēng)濕熱?！镏饕憩F(xiàn)為關(guān)節(jié)炎者，關(guān)節(jié)痛不再作為次要表現(xiàn)?！镏饕憩F(xiàn)為心臟炎者，P-R間期延長(zhǎng)不再作為次要表現(xiàn)。Jones標(biāo)準(zhǔn)的例外：有鏈球菌感染證據(jù)的前提下，存在以下之一的應(yīng)考慮風(fēng)濕熱:排除其他原因的舞蹈病;無其他原因可解釋的隱匿性心臟炎;以往已確診為風(fēng)濕熱，出現(xiàn)一個(gè)主要表現(xiàn)或幾個(gè)次要表現(xiàn)時(shí)提示風(fēng)濕熱復(fù)發(fā);是否有風(fēng)濕熱活動(dòng)

(以下之一均提示風(fēng)濕熱活動(dòng))

發(fā)熱、乏力、蒼白、脈搏增快伴關(guān)節(jié)癥狀新發(fā)現(xiàn)的雜音心臟進(jìn)行性增大出現(xiàn)充血性心力衰竭ASO持續(xù)升高或CRP陽(yáng)性

清除鏈球菌感染青霉素im或iv2周青霉素過敏改用其它有效抗生素抗風(fēng)濕藥物治療：腎上腺皮質(zhì)激素風(fēng)濕熱心臟型的首選重癥：氫化可的松或甲基強(qiáng)的松強(qiáng)的松2mg/（kg·d）(≤60mg/d),

2～4周減量早期、足量，療程8～12周停藥前用阿司匹林替代，防反跳預(yù)后:1/3的病例死于心臟炎或心臟瓣膜病1/3的病例發(fā)展為風(fēng)濕性心臟瓣膜病1/3的病例痊愈，無后遺癥IntroductionRemarkablechangesintheevaluationandmanagementofpatientswithvalvularheartdiseaseAdvancesinsurgicalapproachesandinterventionalcardiologyprocedureshaveimprovepatient’soutcomesValvularheartdiseaseMitralAorticTricuspidPulmonaryStenosisRegurgitationDiagnosisEtiologyPathophysiologyClinicalmanifestationPhysicalFindingsLaboratoryExaminationDifferentialDiagnosisTreatmentKeyConceptsEchocardiographyremainsthegoldstandardfordiagnosisandfollowuppatientswithvalvularheartdisease.StenoticvalvularlesionscanbemonitoredclinicallyuntilsymptomsappearRegurgitantvalvularlesionsrequirecarefulechocardiographicmonitoringforleftventricularfunctionandmayrequiresurgeryevenifnosymptomsarepresentKeyConceptsMedicaltherapyaimsatcontrolofsymptoms.Surgeryisthetreatmentformostsymptomaticlesionsorforlesionscausingleftventriculardysfunctionevenintheabsenceofsymptoms.MITRALSTENOSIS(MS)ETIOLOGYANDPATHOLOGYRheumaticfeverTwo-thirdsarefemale25%ofallpatientshavepureMS40%havecombinedMSandmitralregurgitation(MR)theincidenceofMSisdecliningAmajorproblemintropicalclimatesanddevelopingcountriesRheumaticfeverresultsinfourformsoffusionleadingtostenosis:CommissuralCuspalChordalCombinedmitralvalvecuspsfuseatthetheiredgesfusionofthechordaetendineaeresultsinthickeningandshorteningofthesestructuresETIOLOGYANDPATHOLOGYCalcificationofthevalveimmobilizestheleafletsandnarrowstheorificeleadtonarrowingofthevalve(fish-mouth)dilatedleftatrium(LA)ThrombusfrequentlyarisefromLAinpatientswithatrialfibrillation(AF)Othercauses:congenital,malignantcarcinoid,SLE,Amyloid,etc.ETIOLOGYANDPATHOLOGYPATHOPHYSIOLOGYmitralvalveorificeis4to6cm2

mild：1.5-2cm2moderate：1-1.5cm2Severe(critical)：1cm2transvalvularpressuregradient,pulmonaryvenousandarterialwedgepressureselevated-exertionaldyspneaTachycardiaaugmentsthetransvalvulargradientandLApressuretheCOisabnormalatrestandmayfailtoriseormayevendeclineduringactivityinpatientswithsevereMSPulmonaryhypertensionCLINICALMANIFESTATIONSMSprogressesNosymptomsDyspnea,coughOrthopneaParoxysmalnocturnaldyspneaHemoptysisruptureofpulmonary-bronchialvenousconnections

SystemicEmbolismmorefrequentinpatientswithAFOtherSymptomsHoarness(Ortnersyndrome)hepatomegaly,edema,ascites,hydrothorax(right-sidedheartfailure)CLINICALMANIFESTATIONSPhysicalFindingsInspectionandPalpationmitralfaciesprominentjugularvenouspulseRVtap-leftsternalborderdiastolicthrill-apxHepatomegaly,ankleedema,ascites,andpleuraleffusioninpatientswithMSandRVfailureAuscultationS1,P2isaccentuatedTheopeningsnap(OS)followsA2low-pitched,rumbling,diastolicmurmurattheapexintheleftlateralrecumbentpositionTheGrahamSteellmurmurofpulmonaryregurgitation(PR)resultsfromdilatationofthepulmonaryvalveringRVoverload-tallPwaveLAenlargement-notched,broadPwaveBiphasicPwaveinV1EchocardiographymostsensitiveandspecificnoninvasivemethodfordiagnosingMSmitralorificesize,thepresenceandseverityofaccompanyingMR,theextentofrestrictionofvalveleafletscardiacchambersize,theLVfunctionX-ray(Roentgenogram)straighteningoftheleftborderofthecardiacsilhouetteprominenceofthemainpulmonaryarteriesenlargedLAKerleyBlines

CardiacCatheterizationandAngiocardiography

notusuallynecessarypositivenoninvasivestresstestsformyocardialischemia,coronaryangiographyisadvisabletodetectpatientswithcriticalcoronaryobstructionsinmales>45yearsofage,females>55yearsofage,oryoungerwithriskfactorsDifferentialDiagnosisMRAR(AustinFlintmurmur)AtrialseptaldefectLeftatrialmyxomaTreatmentPenicillinprophylaxistopreventrheumaticfeverandinfectiveendocarditisrestrictionofsodiumintakeandmaintenancedosesoforaldiureticsDigoxin,BetablockersWarfarinforsystemicand/orpulmonaryembolizationandAFpercutaneousballoonmitralvalvotomy(PBMV)surgicalvalvotomyforsymptomaticpatientswithisolatedMSwhoseorificeis<1.0cm2/m2bodysurfacearea,or<1.7cm2innormal-sizedadultsMitralValvotomyPBMVMitralvalvereplacement(MVR)isnecessaryinpatientswithMSandsignificantassociatedMRMITRALREGURGITATIONETIOLOGYAbnormalmitralleaflets,chordaetendineae,papillarymuscles,andmitralannulusMitralvalveprolapse(MVP),rheumaticheartdisease,infectiveendocarditis,annularcalcification,cardiomyopathy,andischemicheartdiseasePATHOPHYSIOLOGYincreasedLVandLAvolumeelevatedLAandPApressurereducedforwardCOejectionfraction(EF)riseswithlongstandingMR,LVcontractilitybecomesreducedSYMPTOMSFatigueexertionaldyspneaorthopneaAcutesevereMR---acutepulmonaryedemaPHYSICALFINDINGS

S1absentAholosystolicmurmurofgradeIII/IVintensityisthemostcharacteristicauscultatoryfinding,mostprominentattheapexandradiatestotheaxillaMVPofposteriormitralleaflet,theregurgitantjetstrikestheLAwall,thesystolicmurmuristransmittedtothebaseoftheheart"seagull"murmur--rupturedchordaeLABORATORYEXAMINATIONEKGLA,LV,RVenlargementAFECHO:2D,colordopplermostaccuratenoninvasivetechniquefordiagnosisofMRTREATMENTMedicalTreatmentreducingsodiumintakeuseofdiureticsvasodilatorsanddigitalis(ACE)inhibitorsIntravenousnitroprussideornitroglycerinreduceafterloadforpatientswithacuteand/orsevereMRSurgicalTreatmentasymptomaticorlimitedonlyduringstrenuousexertion,LVfunctionsarenormal—nosurgerysevereMRinasymptomaticpatients,orLVdysfunctionisprogressive,withLVEF<60%,and/orend-systoliccavitydimension>45mm—surgeryMVR,mitralvalvuloplasty/annuloplastyMITRALVALVEPROLAPSE(MVP)excessiveorredundantmitralleafletassociatedwithmyxomatousdegenerationTheposteriorleafletisusuallymoreaffectedthantheanteriorthemitralvalveannulusdilatedruptureorredundantchordaetendineaecausemitralregurgitationCLINICALFEATURES

morecommoninfemales,14-30yearsofageabroadspectrumofseveritiesmildprolapse,onlyasystolicclickandmurmurchordalrupture,severeMRarrhythmias,syncope,chestpain,infectiveendocarditis,TIA,suddendeathAuscultationthemid-orlatesystolicclickaftertheS1generatedbythesuddentensingofelongatedchordaeorbytheprolapsingmitralleafletwhenitreachesitsmaximumexcursion.maybefollowedbyahigh-pitched,latesystolicmurmurattheapexLABORATORYEXAMINATIONEKGbiphasicorinvertedTwavesinleadsII,III,andaVF,andprematurecontractionsECHOsystolicdisplacement(intheparasternalview)ofthemitralvalveleafletsbyatleast2mmintotheLAsuperiortotheplaneofthemitralannulusABCDTREATMENTpreventionofinfectiveendocarditisBetablockers,antiarrhythmicagentsAspirinforTIApatientsForsevereMR,mitralvalverepair/replacementAORTICSTENOSISASone-fourthofallpatientswithchronicVHD80%ofadultpatientswithsymptomaticvalvularASaremaleETIOLOGYdegenerativecalcificationoftheaorticcuspscongenitalorrheumaticinflammationPATHOPHYSIOLOGYTheobstructiontoLVoutflowproducesasystolicpressuregradientbetweentheLVandaortaApeaksystolicpressuregradient>50mmHg,oraneffectiveaorticorifice<1.0cm2or<0.6cm2/m2bodysurfacearea,--severeobstructionelevatedLVend-diastolicpressurehypertrophiedLVwalldiminishedcomplianceofLVwallLA,PA,andRVpressuresrisemyocardialischemiaSYMPTOMS

exertionaldyspneaanginapectorissyncopeLVfailureintheadvancedstagesofthediseaseAuscultationanearlysystolicejectionsoundanejection(mid)systolicmurmur,low-pitched,roughandraspingincharacter,andloudestatthebaseoftheheart,inthesecondrightintercostalspace,transmittedupwardalongthecarotidarteries,gradeIII/IVLABORATORYEXAMINATIONEKGmightbenormalLVhypertrophyST-segmentdepressionandT-waveinversionEchocardiographyLVhypertrophyvalvularcalcificationtransaorticvalvulargradientMS,ARCatheterizationCADsuspectedNATURALHISTORYanginapectoris,3years;syncope,3years;dyspnea,2years;congestiveheartfailure,1.5to2yearsTREATMENTMedicineTreatmentstrenuousphysicalactivityshouldbeavoidedinpatientswithsevereAS(<0.5cm2/m2)avoidvolumedepletionstatinsmaybehelpfultoslowprogressionSurgicalTreatmentsevereAS(valvearea<1.0cm2or0.6cm2/m2bodysurfacearea)whoaresymptomaticLVdysfunctionexpandingpoststenoticaorticroot,evenasymptomatic.PercutaneousBalloonAorticValvuloplastyinchildrenandyoungadultswithcongenital,noncalcificASAORTICREGURGITATIONETIOLOGYPrimaryValveDiseasethree-fourthsaremalestwo-thirdsisrheumaticinorigindegenerativecalcification,congenitalorrheumaticthickening,deformity,andshortening,prolapseoftheaorticvalvecuspsPrimaryAorticRootDiseaseaorticroot/annulusdilatationMarfansyndrome,hypertension,dissection,syphilis,spondylitisPATHOPHYSIOLOGYLVEDV

dilatationandeccentrichypertrophyoftheLVelevationoftheLA,PAwedge,PA,andRVpressuresreducedLVEFmyocardialischemiaSYMPTOMSAcute,severeAR--IE,traumapulmonaryedema,cardiogenicshockmaydeveloprapidlyChronic,severeAR--alonglatentperiodpalpitation,exertionaldyspnea,anginaPHYSICALFINDINGSArterialPulse

"water-hammer"(Corrigan'spulse)capillarypulsations(Quincke'spulse)"pistol-shot"("pistol-shot")to-and-fromurmur(Duroziez'ssign)arterialpulsepressureiswidenedPalpationLVimpulseisheavinganddisplacedlaterallyandinferiorlyAdiastolicthrillisoftenpalpablealongtheleftsternalborderAuscultationA2isusuallyabsentahigh-pitched,blowing,decrescendodiastolicmurmur,heardbestinthethirdintercostalspacealongtheleftsternalborderwiththepatientsittingup,leaningforward,andwiththebreathheldAustinFlintmurmursoft,low-pitched,rumblingmiddiastolicdiastolicdisplacementoftheanteriorleafletofthemitralvalvebytheARstreamNothemodynamicallysignificantmitralobstructionLABORATORYEXAMINATIONEKGLVhypertrophyST-segmentdepressionandT-waveinversionleadsI,aVL,V5,andV6("LVstrain")Echocardiogramwallmotionarenormalorevensupernormalrapid,high-frequencyflutteringoftheanteriormitralleafletthickeningandfailureofcoaptationoftheleafletsdilatationoftheaorticannulusColorflowDopplerechocardiographicimagingisverysensitiveinthedetectionofARX-RAYLVenlargement,theapexisdisplaceddownwardandtothelefttheascendingaortaandaorticknobmaybedilatedTREATMENTMedicalTreatmentdigitalis,saltrestriction,diuretics,andvasodilators,especiallyACEinhibitorsCardiacarrhythmiasandinfectionsmustbetreatedpromptlyandvigorously.SurgicalTreatmentoperationshouldbecarriedoutinasymptomaticpatients,whenaleftventricularejectionfraction(LVEF)<55%oraLVend-systolicvolume>55mL/m2.AVR,AVrepair,narrowingtheannulusTRICUSPIDSTENOSISuncommongenerallyrheumaticinoriginmorecommoninfemalesthaninmalesItdoesnotoccurasanisolatedlesionandisusuallyassociatedwithMS,TRPATHOPHYSIOLOGYAdiastolicpressuregradientbetweentheRAandRViselevatedAmeandiastolicpressuregradientof4mmHgissufficienttoresultinsystemicvenouscongestionascitesandedemaSYMPTOMSTScanmaskthehemodynamicandclinicalfeaturesoftheMSAmeliorationofMSsymptomsshouldraisethepossibilitythatTSmaybedevelopingDyspnea,hepatomegaly,ascites,andedemaPHYSICALFINDINGSmarkedhepaticcongestion,distendedjugularveins,jaundice,splenomegalythetricuspidmurmurisgenerallyheardbestalongtheleftlowersternalmargin,augmentedduringinspirationLABORATORYEXAMINATIONEKGRAenlargementincludetall,peakedPwavesinleadII,uprightPwavesinleadV1.ECHOthickenedtricuspidvalve;elevatedtransvalvulargradientTREATMENTintensivesaltrestrictionanddiuretictherapyarerequiredduringthepreoperativeperiodRepair,orreplacementTRICUSPIDREGURGITATION(TR)Mostcommonly,TRisfunctionalandsecondarytomarkeddilatationofthetricuspidannulusItiscommonlyseeninthelatestagesofheartfailurewithseverepulmonaryhypertension,aswellasinischemicheartdisease,cardiomyopathy,andcorpulmonale.InfarctionofRVpapillarymuscles,tricuspidvalveprolapse,carcinoidheartdisease,endomyocardialfibrosis,infectiveendocarditis,andtraumaCongenitalheartdisease:defectsoftheatrioventricularcanal,Ebstein'smalformationSymptomsofrightheartfailureAblowingholosystolicmurmuralongthe