内科学教学课件：27 心脏瓣膜疾病

ValvularHeartDisease2012年我国高血压

患病率，知晓率，治疗率和控制率国家卫计委疾病预防控制局，中国居民营养与慢性病状况报告(2015年)，人民卫生出版社25.2%2012年中国18岁及以上居民高血压患病率为25.2%心血管医生Keypoints瓣膜损害：狭窄或者关闭不全我国，风湿热二尖瓣最常见，主动脉瓣最重要诊断靠心超治疗靠外科介入也可以主要病因二尖瓣：风湿性，感染主动脉瓣：二叶式主动脉瓣三尖瓣返流：功能性/脱垂，心内膜炎肺动脉瓣：……

风湿热

RheumaticFever

风湿热（rheumaticfever）

A组乙型溶血性链球菌感染后发生的全身结缔组织的非化脓性炎性疾病,为常见的风湿性疾病。主要表现为：心脏炎、关节炎、舞蹈病、环形红斑及皮下小结，以心脏损害最为严重和多见，反复发作可导致永久性心脏瓣膜病变。A组乙型溶血性链球菌咽峡炎的并发症约0.3%～3%病例于1～4周后发生风湿热链球菌在咽部存在的时间致病菌株患儿遗传学背景

荚膜：透明质酸酶细胞壁：M蛋白、M相关蛋白N-乙酰葡糖胺、鼠李糖

细胞膜：蛋白、脂质、糖关节心肌心内膜下丘脑/尾核心肌A组β链球菌相同的抗原性，产生免疫交叉反应链球菌感染诱导的异常免疫反应免疫复合物致病：与链球菌抗原模拟的自身抗原与链球菌抗体形成循环免疫复合物，沉积于人体关节滑膜、心肌、心内膜，激活补体产生炎性病变。细胞免疫反应异常：

T淋巴细胞对心肌的毒性作用；淋巴细胞增殖反应降低、自然杀伤细胞功能增强；扁桃体单核细胞对链球菌抗原的免疫反应异常。

病变过程分急性渗出期、增生期、硬化期，三期可交错存在，持续约4～6个月。急性渗出期（1个月左右）部位：心脏、关节、皮肤病理：组织水肿、变性或坏死，炎性细胞浸润，纤维素及浆液渗出。

增生期（3～4个月）部位：心肌和心瓣膜，还可分布于肌肉及结缔组织（皮下小结）病理：风湿小体（Aschoffbody)，是风湿热的病理诊断依据，表明风湿活动。风湿小体模式图纤维素样物质多核巨噬细胞淋巴细胞硬化期（2～3个月）部位：二尖瓣﹥主动脉瓣病理：纤维组织增生和瘢痕形成。以心脏瓣膜损害最突出，在瓣膜的闭锁线上出现赘生物，使瓣膜增厚。瓣膜赘生物（箭头处）瓣膜赘生物（箭头处）一般表现急性患者半数以上病前1～5周有咽炎、扁桃体炎或猩红热感染史。发热急性起病….38℃～40℃

，2周后低热隐匿起病….低热或无热关节痛、贫血、鼻衄、腹痛心脏炎40%～50%心肌、心内膜、心包均可受累。首次风湿热发作时，一般于起病1～2周内出心脏炎症状，需严密观察。心脏炎/全心炎＝心肌炎﹢心内膜炎﹢心包炎心肌炎轻重不一心动过速，第一心音减弱；心脏扩大，心尖搏动弥散，闻及奔马律；心尖部可听到Ⅱ/Ⅵ级收缩期吹风样杂音或主动脉瓣区舒张中期杂音；ECG：P-R间期延长，ST-T改变；心内膜炎二尖瓣区出现Ⅱ/Ⅵ级以上全收缩期杂音心尖区有柔和、短促的舒张中期杂音主动脉瓣区舒张期叹气样杂音反复发作后造成永久性瓣膜损害心包炎

心前区疼痛、呼吸困难及端坐呼吸；心包摩擦音、心音遥远、心前区搏动消失；心包填塞的表现：颈静脉怒张、肝脏肿大；一旦有心包炎表现，提示有严重心脏损害，易发生心力衰竭；关节炎50%～60%特点：为多发性、游走性大关节炎典型表现：有红、肿、热、痛和功能障碍,不典型的仅表现关节痛；发病很少超过1个月不留畸形舞蹈病：3%～10%8～12岁的女孩多见;不自主、突发、无目的的快速运动，在兴奋和注意力集中时加剧，睡眠时消失，可累及全身肌肉，以面部和上肢肌肉为主;链感后1～6月发生，也可为首发症状;自限性，病程平均三个月。皮肤症状环形红斑:少见;环形或半环形边界清楚的淡色红斑，时隐时现，可持续数周。皮下小结:5%，常伴心脏炎;发于大关节伸面及枕、额、脊突处;直径0.1～1cm,质硬不痛，2～4周消失。其它皮疹:荨麻疹、结节性红斑、多形红斑。

环形红斑（箭头处）

一、链球菌感染证据

咽拭子培养ASO↑

抗脱氧核糖核酸酶（anti-DNaseB)

抗链球菌激酶（ASK）抗透明质酸酶（AH）

二、风湿热活动指标

血沉增快C-反应蛋白和粘蛋白增高白细胞计数增高三、心脏损害依据X线检查:严重的出现心胸比例增大。心电图:常见P-R间期延长和I°-AVB，可出现ST-T改变及低电压，心律失常;

超声心动图:可显示有无瓣膜增厚、水肿、狭窄和关闭不全，心脏增大及心包积液;

1992年修订的Jones诊断标准

主要表现次要表现链球菌感染证据心脏炎发热多发性关节炎关节痛﹡

咽拭子培养阳性舞蹈病血沉增快快速链球菌抗原试验阳性皮下结节CRP阳性抗链球菌的抗体滴度增高环形红斑P-R间期延长﹡﹡

★2项主要表现，或1项主要指标伴2项次要表现者，可诊断为风湿热。★主要表现为关节炎者，关节痛不再作为次要表现。★主要表现为心脏炎者，P-R间期延长不再作为次要表现。Jones标准的例外：有链球菌感染证据的前提下，存在以下之一的应考虑风湿热:排除其他原因的舞蹈病;无其他原因可解释的隐匿性心脏炎;以往已确诊为风湿热，出现一个主要表现或几个次要表现时提示风湿热复发;是否有风湿热活动

(以下之一均提示风湿热活动)

发热、乏力、苍白、脉搏增快伴关节症状新发现的杂音心脏进行性增大出现充血性心力衰竭ASO持续升高或CRP阳性

清除链球菌感染青霉素im或iv2周青霉素过敏改用其它有效抗生素抗风湿药物治疗：肾上腺皮质激素风湿热心脏型的首选重症：氢化可的松或甲基强的松强的松2mg/（kg·d）(≤60mg/d),

2～4周减量早期、足量，疗程8～12周停药前用阿司匹林替代，防反跳预后:1/3的病例死于心脏炎或心脏瓣膜病1/3的病例发展为风湿性心脏瓣膜病1/3的病例痊愈，无后遗症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