心脏瓣膜病-(2).ppt

VALVULAR HAERT DISEASE （心脏瓣膜病心脏瓣膜病）北京同仁医院心脏中心北京同仁医院心脏中心李洁李洁Rheumatic Fever(风风 湿湿 热热)Rheumaticfeverisadiffuseinflammatorydiseaseofconnectivetissueorcollagenvasculardisease,primarilyinvolvingheart,bloodvessels,joints,SubcuttissueandCNSUsuallyoccursinpeoplebetween6and15yearsold DefinitionPathogenesis（发病机制）（发病机制）lGroup A hemolytic streptococcal(A组乙型溶血性链球组乙型溶血性链球菌菌)infectionprecedesARFby2-6weekslAntibodiesmadeagainstgroupAstrepcross-reactwithhumantissue:heartvalveandconnectivetissuelHostimmuneresponsesmayplayaroleindeterminingwhogetsARFfollowinginfectionPathology(病理病理)lThree periods:acuteexudativeperiod：inflammatoryedema,(急性渗出期)inflammatorycellinfiltrationproliferativeperiod：Aschoffbodymarkofrheumatic（增生期）feveractivescleroticperiod：proliferationoffibrinotictissue,（硬化期）formationofscarlAschoffAschoff小体，多发生在血管周围的间质中，胶原纤维素样坏死，周围小体，多发生在血管周围的间质中，胶原纤维素样坏死，周围炎性细胞浸润炎性细胞浸润Clinical Features（临床表现）（临床表现）Group A hemolytic streptococcal(A组乙型溶血性链球菌组乙型溶血性链球菌)infectionprecedesARFby2-6weeks1.Arthritis（关节炎）（关节炎）Painful,Flittingmigratorypolyarthritis,involvingmajorjoints,Excellentresponseofsalicylates（水杨酸）,notprogresstochronicdiseaseClinical Features（临床表现）（临床表现）2.Carditis:Endocarditis：valve lesionMyocarditis：tachycardias,heartfailurePericarditis：arrthythmia(prolongedPRinterval)Cardiomegaly,Pericardialfrictionrubs3.Sydenham Chorea(舞蹈病舞蹈病)表现：肢体或躯干无目的，不自主的快速运动，注意力集中时加重，入睡后消失。Usuallyalatemanifestation:monthsafterinfectionOftentheonlymanifestationofARF4 Cutaneous lesion:Erythema marginatum（环形红斑）（环形红斑）:Reddishpinkborder,palecenter,roundorirregularshape Subcutaneous nodules（皮下结节）（皮下结节）:Mostcommon:alongextensorsurfacesofjointClinical Features（临床表现）（临床表现）Laboratory Findings（实验室检查）（实验室检查）lESR,C-reactiveprotien（CRP）lASOtitre500U(Peakvalueattainedat3w,thencomesdowntonormalby6weeks)lAnti-DNAse160250UlCK,CK-MBDiagnosis:modifiedJonescriteria1992lThepresenceof2majorcriteriaor1majorcriteriaand2minorcriteriaindicatesacuterheumaticfever,ifsupportedbyevidenceofgroupAstreptococcalinfectionlEvidence of Previous InfectionPositivethroatCultureAntibodytitrehistoryDiagnosis:modifiedJonescriteria1992The major criteria(CANCER)C:CarditisA:ArthritisN:Nodules(sub-cutaneous)C:ChoreaER:ERythemaMarginatumThe minor criteriaFeverArthralgia(关节痛)LaboratoryFindings:ESR,CRPECGabnormalities:aprolongedPRintervalTreatment(治疗治疗)lStep I-primaryprevention(eradicationofstreptococci)lStep II-antiinflammatorytreatment(aspirin,steroids)lStep III-supportivemanagementmanagementofcomplicationslStep IV-secondaryprevention(preventionofrecurrentattacks)Agent Dose Mode DurationPenicillin 1200 000 U bid im 10 d For individuals allergic to penicillinErythromycin:250mg 4 times daily Oral 10 d STEP I:Primary Prevention of RFStep II:Anti inflammatory treatmentlAnti-strep infection:Penicillin1200000U/dorErythromycinfor23weekslAnti-reumatic fever treatment:Arthritis：Aspirin:34g/d,tid.usuallyexcellentresponse,ifpoorresponse,diagnosisinquestionCarditis:prednisolone(泼尼松):4060mg/d,tidfor2weeks.lBedrestlTreatmentofcongestivecardiacfailure:-digitalis,diureticslTreatmentofchorea:-diazepamStep III:Supportive management Agent DoseModeBenzathine penicillin G1 200 000 U every 4 weeks*IntramuscularorPenicillin V250 mg twice daily OralFor individuals allergic to penicillin and sulfadiazineErythromycin250 mg twice daily Oral*In high-risk situations,administration every 3 weeks is justified and recommendedSTEP IV:Secondary Prevention of Rheumatic Fever Mitral Stenosis （二尖瓣狭窄二尖瓣狭窄）Etiology(病因)Rheumatic fever:2/3 are female.25%ofpatientshavepureMS;40%havecombinedMSandMR.Calcification Systemic lupus erythematous（SLE）病理生理病理生理(1)Area of mitral valve oriface（MVA）NormalMitral stenosis mild moderate severe MVA(cm2)46 2 1.5 11.5 1.0病理生理病理生理(2)LA pressurePulmonary pressureRV pressuresLVRVRALAPALung心率加快心率加快舒舒张张期期缩缩短短左房向左室血液左房向左室血液回流回流 左房压左房压临床表现Dyspnea:istheresultofreducedcomplianceofthelungs.Exertionaldyspnea（劳力性呼吸困难）Orthopnea（端坐呼吸）Paroxysmalnocturnaldyspnea（夜间阵发性呼吸困难）Acutepulmonaryedema（急性肺水肿）临床表现临床表现 Hemoptysis(咯血咯血)Suddenhemorrhage:ruptureofbronchialveinsPink,frothysputum：acutepulmonaryedemaCough:Hoarseness(Ortner syndrome)体格检查 Mitralfacies（二尖瓣面容）:pinkish-purplepatchesonthecheeks.体格检查杂音Thediastolicmurmur：low-pitched,rumblingmurmur,bestheardattheapex.Itisaguidetotheseverityofmitralnarrowing.Silentmitralstenosis:severeMS体格检查体格检查-听诊听诊ReflectMVleafletsareflexible：AccentuatedS1Openingsnap(OS):bestheardattheapexandL3-4Pulmonaryhypertension：AccentuatedP2Dilationofthepulmonaryartery:SystolicpulmonicejectionmurmurGrahamSteellmurmur:high-pitcheddiastolicmurmur 辅助检查辅助检查-心电图心电图左房大(二尖瓣型P波)：P波宽度0.12s；V1导联终末负向波前后位前后位:心影右缘扩大，左心耳扩大（左心缘局部膨隆）心影右缘扩大，左心耳扩大（左心缘局部膨隆）辅助检查：辅助检查：X线（线（1）食管左心房段有明显压迹食管左心房段有明显压迹 肺淤血肺淤血辅助检查：辅助检查：X线（线（2）辅助检查辅助检查-超声心动图超声心动图Echocardiography:M-modeUCG:theposteriorleafletmovesanteriorlyTwo-dimensionalUCG:RevealsrestrictedmotionoftheMV.Determinmitralorificesizeandwhetherissuitableforballoonmitralvalvuloplasty前叶活动双峰消失，前叶活动双峰消失，形成城墙样改变形成城墙样改变前后叶同向运动前后叶同向运动M型：二尖瓣前后叶反型：二尖瓣前后叶反向运动，前叶活动双峰向运动，前叶活动双峰 诊断诊断Low-pitched,rumblingmurmurattheapexECGXraysEchocardiography鉴别诊断鉴别诊断Relativelymitralstenosis:见于二尖瓣血流增加的疾病Leftatrialmyxoma(左房粘液瘤):themurmurvarieswithpositionAustin-flintmurmur:severeaorticregurgitation并发症并发症Atrialfibrillation(房颤):rapidventricularratesmayinduceacutepulmonaryedemaAcutepulmonaryedema:severeMSRight-sidedheartfailure:Embolism（栓塞）:体循环栓塞。80%合并房颤.pulmonaryinfection:常见常见LVRVRALAPALungMANAGEMENT（治疗）（治疗）药物治疗药物治疗Acute pulmonary edema（急性肺水肿）（急性肺水肿）Reducepulmonaryvenouspressure:sedation（镇静）oxygen（吸氧）assumptionoftheuprightposture（端坐位）aggressivediuresis（积极利尿）venousvasodilator（扩张静脉）：硝酸酯类注意：禁用扩张动脉为主的药物药物治疗药物治疗lAtrial fibrillation(房颤房颤)RatecontrolinAf:reducetheventricularrate(70bpmatrest)Digitalis/Betablockers/非二氢吡啶类CCBCardioversion:drugorelectricalcardioversionAnticoagulation:华法令介入治疗介入治疗球囊二尖瓣成形术球囊二尖瓣成形术介入治疗介入治疗球囊二尖瓣成形术球囊二尖瓣成形术 适应症适应症:1:ModeratetoseverepureMS2:Leafletpliablewithoutcalcification,subvalvularthickness,andthrombosisinleftatrial3:CardiacfunctionclassII-III4:MildMRwithoutLVdilatiation禁忌症：左房血栓和重度二尖瓣关闭不全手术治疗手术治疗-二尖瓣置换二尖瓣置换 适应症：适应症：Moderatetoseveresymptoms(ClassII)MVorifice1.0cm2,HistoryofsystemicembolismPrognosis 预后不良:PatientswithsymptomaticMS,AFCHFwithdilationoftheheartHistoryofsystemicembolismMitral Insufficiency(MI)（二尖瓣关闭不全二尖瓣关闭不全）Etiology（病因）（病因）InfectiveendocarditisInflammatory:rheumaticheartdisease,SLECoronaryarterydisease(AMI)Degenerative:marfansyndrome,myxomatous病理病理valveleaflets:shortening,rigidity,andretractionofthemitralvalvemitralannulus:dilatationorcalcification chordaeChordaetotheposteriorleafletrupturemorefrequentlypapillarymusclesTheposteriorpapillarymusclebecomesischemicandinfarctedmorefrequently病理生理病理生理(1)l Chronic MI LVEDV LA(LV)enlarge（FRANK-STARLING）LV outputLVEDP norm or LAP norm or LVRVRAPALungLALA病理生理病理生理(2)Acute MILVoverloadLVEDPLAPdilationofLVislimitedpulmonarycongestionpulmonaryhypertentionrightsided-heartfailure 临床表现临床表现 Chronic MI:Theseverityofthesymptomsdependsonitsseverity,rateofprogression,andthepresenceofassociateddisease.Acute MIMildMI:exertionaldyspneaSevereMI:acuteleft-sidedheartfailure,acuteedema听诊-Acute MISystolicmurmurisdecrescendo,low-pitchedandendbeforetheS2AcutesevereMI:accompaniedbyS3attheapexandashortdiastolicrumblingmurmur听诊-Chronic MI心音心音:S1S2分裂:resultsfromtheshorteningofleftventricularejectionandearliershutofaorticvalve杂音杂音:Systolic murmur at apex:Thepansystolicmurmurswithlatesystolicaccentuation,radiatetotheaxilla（腋下）andleftinfrascapulararea（肩胛区）Physical ExaminationChronic MI:Rheumaticfever:radiatetothesternumortheaorticareamayoccurwithabnormalitiesoftheposteriorleaflet.ProlapseoftheMV:beginsinmid-tolatesystolicclicks ThemurmurofpapillarymuscledysfunctionduetoCADisvariable,oftendisappearswhenischemiaisrelieved.辅助检查辅助检查ECG:Chronic MI:leftatrialandLVenlargementatrialfibrillation.Acute MI:sinustachycardiaX Ray:Chronic MI:EnlargementofLVandLA Acute MI:leftsided-heartfailure(pulmonarycongestiveandinterstitialedema）辅助检查辅助检查-UCGUCG：收缩期左心室-左心房返流束诊断和鉴别诊断诊断和鉴别诊断 Diagnosis:Systolicmurmurattheapex,accompaniedbyLAandLVenlargement.Differential Diagnosis:1.Relativemitralregurgitation:Thesoft,mid-tolatemurmurissecondarytoLVdilatationwhichleafletsofMVarenormal.Differential DiagnosisDifferential Diagnosis:2.Ventricularseptaldefect:aroughholosystolicmurmur,loudestattheleftsternalborderaccompaniedbyathrill.3.Aorticstenosis:ejectionsoundatthebaseoftheheart,transmittedalongthecarotidvesselsandtotheapex4.Tricuspidregurgitation:holosystolicmurmur,alongtheleftsternalborder,augmentedbyinspirationComplicationsHeartfailure:occursearlyinacuteMI;lateinchronicMISystemicembolism:lessthaninMSInfectiveendocarditis:morethaninMSMANAGEMENT （治疗）（治疗）Acute MIMedical Treatment:stabilizethecondition.Emergencyorelectivesurgicaltreatment.Chronic MIMedical Treatment1.Prophylaxisforrheumaticfeverandforinfectiveendocarditis.2.Chronicheartfailure:useageofdigitalisdiureticsACEI3.Anticoagulanttherapy:wafarinChronic MISurgical TreatmentIndication(1)Mildsymptomaticpatientsfollowingmedicaltreatmentbutleftventricularfunctionprogressivelydeteriorates(2)FunctionalclassII,accompaniedwithheartdilatation,LVESV30ml/m2,(3)FunctionalclassIIIIV,aftertheconditionisstabilizedbymedicaltreatmentAortic Stenosis(主动脉瓣狭窄主动脉瓣狭窄)Etiology and PathologyRheumatic AS:adhesionsandfusionsofthecommissuresandcusps,leadingtoretractionandstiffeningoftheleaflets Degenerative calcific AS:thecuspsareimmobilizedbyadepositofcalciumCongenital aortic stenosis:Unicuspidvalve:producesevereobstructionininfancy.Bicuspid:commen.Tricuspid:.病理生理（病理生理（1）Normalaorticorificeis3.04.0cm2.Aorticvalveorifice 50 mm Hg may besymptomatic.病理生理（病理生理（2）ASLVafterloadconcentricLVhypertrophyLVcomplianceLVEDPpulmonary congestion Myocardial ischemia:O2consumption：hypertrophiedLVmasssystolicpressureincreasedprolongationofejectionO2supplycoronaryperfusion临床表现临床表现 Exertionaldyspnea（劳力性呼吸困难）:latesymptomsinAS.Anginapectoris(心绞痛):occursinabout2/3ofpatients Syncope（晕厥）:mostoccursduringexertion.(1)Systemicvaso-dilatation.(2)LVfunctiondecreased,resultedinCO(3)Severearrhythmias:Physical ExaminationCardiac sound:S2:ParadoxicalsplittingSystolic ejection murmur:Transmitealongthecarotidvesselsandtotheapex.themoreseverethestenosis,thelongerthedurationofthemurmurPhysical Examinationothers:Relative aortic valve regurgitation:duetopost-stenoticdilatationoftheascendingaorta.Themurmurismildearlydiastolicdecrecendomurmur,heardbestatthe3rdto4thleftintercostalspace.The arterial pulserisesslowlyandissmallandsustained.Systolic and pulse pressuresarebothreduced.Laboratory ExaminationECG:LVhypertrophyandST-Tsegmentdepression.Laboratory ExaminationX Ray:Theheartisusuallyofnormalsizeorslightlyenlarged.Poststenoticdilatationoftheascendingaortamaybeseen.Laboratory ExaminationEchocardiography:Two-dimensionalUCG:determinetheseverityofthestenosis.DiagnosisSystolicejectionmurmur+UCG Etiology:ASwithregurgitation:rheumaticheartdiseaseDegenerative(senile)calcificAS:morecommomineldersDifferential DiagnosisCongenitalsupravalvularaorticstenosis:themurmurisloudestatthe1stto3rdrightintercostalspace,withoutsystolicejectionmurmurandregurgitantmurmurCongenitalsubaorticstenosis:accompaniedbyMR,withoutsystolicejectionmurmur,S4isaudible.Obstructivehypertrophiccardiomyopathy:themurmuristheloudestbetweentheleftinferiorborderandtheapexwithoutsystolicejectionmurmurComplicationsCardiac suddern death(SCD):about10%20%.Patientsusuallyexperiencesyncope,anginapectorisandheartfailurebeforeSCD.Heart failure:Arrhythmias:Atrialfibrillationdecreaseheartoutput,resultinsyncopeandacutepulmonaryedema.ManagementMedical Treatment Prophylaxisforrheumaticfeverandforinfectiveendocarditis.Arrhythmias:AFmaycauseanginaandheartfailure,shouldbetreatedpromptlyandshouldbepreventrdbyprophylaxiswhenprematureatrialcontractionsarefrequent.ManagementAnginapectoris:treatmentedwithNitratesandCCB.Leftheartfailure:thetreatmentissimilarwithheartfailure Attention:avoidusingofintensivediuresisandvasodilator(positionalhypotension).ManagementSurgical Treatment:Reciprocalsyncope,anginapectorisHistoryofsignificantheartfailureAsymptomaticpatientswithprogressiveLVhypertrophyand/orLVdysfunction(gradientpressure50mmHg)Aorticvalveorifice0.8cm2or4550mm)carriedoutatintervalsof3to6months.Acute AIlMedicaltreatmentisthetransitionmanagement.l Promptsurgicalinterventionisindicated.lInhemodynamicallystablepatientswithacuteAIsecondarytoactiveinfectiveendocarditis,operationmaybedeferredtoallow5to7daysofintensiveantibiotictherapy.PrognosislApproximately75%ofpatientssurvivefor5yearsand50%for10yearsafterdiagnosislOncethepatientbecomessymptomatic,theconditionoftendeteriorates rapidly,and SCD may occur,usually inpreviouslysymptomaticpatients.lWithoutsurgicaltreatment,deathusuallyoccurswithin4yearsafterthedevelopmentofanginaandwithin2yearsaftertheonsetofheartfailure.