二尖瓣病理生理学课件

Epidemiology2019intheUK:6471firsttimevalvereplacementsofwhich28%MVRNumbersincreasingEpidemiology2019intheUK:1MitralAnnulus:fibro-muscularskeletonAnchorsbaseofvalveleafletsLeaflets:conntissue+muscle+vessels/nervesAnterior(aortic):larger;1/3ofannulusPosterior(mural):2/3ofannulusAnatomyMitralAnnulus:fibro-muscular2AnatomyPapillarymuscles:AnterolateralPosteromedialChordaetendinae1st,2nd,3rdorderApprox25majorchordaltrunks>100attachmentstoleafletsNoconsensusontimingofmuscleactivitywithcardiaccycleAnatomyPapillarymuscles:3AnnulardynamicsAnnularsizeIncreasesinlatesystole(maximumindiastole)Contractsinpre-systole(minimuminmidsystole)AnnularshapeMoreeccentricinsystoleAnnularpositionMovesuptowardsLAindiastoleMovesdowntowardsLVapexinsystoleAnnulardynamicsAnnularsize4LeafletdynamicsOpeningStartsincenter,movingtoedgesFlappingofedgesatmax.openingClosing (beginsinlatediastole)Bulgingatbase/annularattachmentLeafletascendstowardsLABulging‘rolls’fromannulustoedgeLeafletdynamicsOpening5MitralStenosis(MS)Aetiology: RheumaticMale:femaleratiois1:2-3Acquiredearly(<20yo?)Butclinicallymanifest10-40yrslaterDefiniteHxofRFin50-60%Pancarditis: mitralvalveinvolvedin90% mitralvalvealonein40%MitralStenosis(MS)Aetiology:6MitralStenosis(MS)Rheumaticdisease:Leafletthickening,calcification,retractionPeriannularcalcification(restrictedmovement)Commisuralfusion:‘fish-mouthing’Chordalthickening,shortening,fusionPapillarymuscleinflammationAlso: calcific congenital neoplastic thrombotic infective iatrogenicMitralStenosis(MS)Rheumatic7MS:HemodynamicsDiastolicAVpressuregradientLApressuremayriseto15-20mmHgatrestGradientriseswithexerciseSmallervalveareahighergradientInflowobstruction:idurationofLVfillingHencereducedcardiacoutputExacerbatedbyhheartrate(e.g.AF)MS:HemodynamicsDiastolicAVp8MS:CardiacadaptationsLVessentiallynormalBut?associateddiseasesaffectingLVLAhypertrophyDisorganizedmusclefibres

abnormalconduction/refractoryperiods atrialfibrillation(30-50%,espolder)MaygiveLAmuralthrombiMS:CardiacadaptationsLVesse9MS:PulmonarychangesInseverechronicMS:PulmonaryHTLApressure>30mmHg:pulmtransudation

reducedlungcompliancePulmartsystolicpressure>60mmHg

impedesRVemptying rightheartfailureUltimatelyirreversiblepulmvascularchangesMS:PulmonarychangesInsevere10MS:NaturalhistoryProgressivelife-longdiseaseLonglatencySymptoms:Lowcardiacoutput:dyspnoea,fatiguePulmonarycongestion/HT(orthopnea,PND) rightheartfailure

hemoptysisAtrialfibrillation/Thromboembolism‘Cardiaccachexia’MS:NaturalhistoryProgressive11MS:NaturalhistoryOnsetofsymptomstodisability:10years10yearsurvival:Asymptomatic(NYHAclassI)80%(progression)Symptomatic(NYHAclassIII)20%Causesofdeath:CHF60-70%Systemicembolism20-30%Pulmonaryembolism10%Infection1-5%MS:NaturalhistoryOnsetofsy12MS:InvestigationsCXR:LAenlargement,pulmcongestionECG:LAenlargement(notchedPinII,V1)atrialarrhythmias?RVHEcho:valvearea,LA/LVdimensionsDoppler:measurespressuregradientsTOE:bettermitral/LAvisualizationCardiaccatheter:notessentialAssocddisease;LVventriculography&pressuresMS:InvestigationsCXR:LAenla13MS:MedicaltherapyPharmacologicalTxofmildheartfailure,bronchitis,arrhythmias,hemoptysisEndocarditisprophylaxisAnticoagulation:HxofAF/thromboembolismBalloon(oropen)ValvuloplastyMS:MedicaltherapyPharmacolog14MS:IndicationsforsurgerySymptomatic(NYHAclassIII-IV):MVR

hlong-termsurvival10yearsurvival: 0-20%90%(89%at15yrs)

hfunctionalcapacityValvearea<1-1.5cm2 (normal4-6

cm2)SystemicemboliMS:IndicationsforsurgerySym15MS:IndicationsforsurgeryClassI-II:controversialRiskofSCDifasymptomatic:negligibleSurvivalnotimprovedbyMVR?roleofvalvotomy(pulmonaryHT,AF)MVRindicatedwhen:Valvearea<1.5cm2 PulmonaryHT(higherrisk)Thromboembolism ?PregnancyplannedSymptomprogression>NYHAclassII+MS:IndicationsforsurgeryCla16MitralRegurgitation(MR)AetiologymorediversethanMSMyxomatousdegenerationLeadingcauseinWest(30-70%)DefectivefibroelastictissuefloppyvalveMostasymptomaticComplicatedbyannulardilatation,chordalrupture,endocarditisRheumaticdiseasenextmostcommonMitralRegurgitation(MR)Aetio17MR:CarpentierclassificationNormalleafletmotionAnnular/ventriculardilatationLeafletdisease/perforation MR:CarpentierclassificationN18MR:CarpentierclassificationExcessiveleafletmotion(prolapse)Chordal/papillarymuscleelongationorruptureMR:CarpentierclassificationE19MR:CarpentierclassificationRestrictedleaflet/chordalmotione.g.fibrosis,calcification,retraction MR:CarpentierclassificationR20MR:AetiologyMitralAnnulusMyxomatousdegenerationSenilecalcificationFunctionaldilatation(e.g.myocarditis)RingabscessMarfan’sMR:AetiologyMitralAnnulus21MR:AetiologyMitralleafletsRheumaticdisease,endocarditis (1-30%)UnknownwhysomedevelopMS,othersMRFibrocalcificleafletthickening(withoutfusion)Chordaeshortened,annulusdilatedalso:congenital,connectivetissuediseaseMR:AetiologyMitralleaflets22MR:AetiologyChordaeIschaemiaMyxomatousInfectiveConnectivetissueTraumaIdiopathicMR:AetiologyChordae23MR:AetiologyPapillarymuscle

(10-25%)Dysfunction/ruptureIHD/MI:muscle&annularinjuryfrankrupturerare,usuallyfatalesp.PosteromedialmuscleAlso:abscess,sarcoid/amyloid,myocarditisMalalignmente.g.LVaneurysm,dilatation,myopathyMR:AetiologyPapillarymuscle24MR:HemodynamicsAcute: JLApressure,pulmoedemaChronic:LA/PVcompliance:ipulmcongestnRegurgitantvolumedependson:MitralorificesizeLV-LApressuregradientHeartrateMedicalTxaimstocontrolabovefactorsesp.decreaseafterloadtoreduceLVdilatationMR:HemodynamicsAcute: JLApr25MR:CardiacadaptationsLV:hpreload,iafterloadLVdilated,morespherical,thinnedIncreasedSV(O2consumptionnotmarkedlyh)ButdecompensationcangraduallyoccurLA:hsizeinchronicMRhcomplianceLessthromboembloism,AFthanMSMR:CardiacadaptationsLV:hp26MR:SymptomsAcute:pulmonarycongestion&oedemaChronic:maybeprolongedasymptomaticphaseRiskofendocarditisCongestiveheartfailure&fatigueRightheartfailureMR:SymptomsAcute:pulmonaryc27MR:InvestigationsCXR:LA/LVenlargementECG:normal;LVH,?AF/arrhythmiasEcho:leafletmorphology&functionChamberdimensions,LVfunctionDopplercolourmappingCardiaccatheter:assesscoronaries,LVMRI:Dx,LVvolumes,regurgitantfractionMR:InvestigationsCXR:LA/LVe28MR:MedicaltherapyMainstay:Afterloadreduction

iregurgitantvolumeipulmcongestion

iLVvolumeimitralorificebut:ongoingLVvolumeoverload10%classI-IIprogresstoIII-IVperyearClassII-IIIsurvivalonmedicalMx:5year:50%10year:25%MR:MedicaltherapyMainstay:A29MR:NaturalhistoryVariableaetiologydifficulttopredictDifficulttoidentifythoseprogressingtoirreversibleLVdamagePrognosticindicators:LVfunctionDegreeofregurgitationUnderlyingaetiology(esp.CAD)MR:NaturalhistoryVariableae30MR:IndicationsforsurgeryAcute:muscle/chordalrupturewithshockImmediateMVRChronic,NYHAclassII-IV:MVRAimforsurgerybeforeirreversibleLVchangeLVdimensionisapredictorofoutcomeMR:IndicationsforsurgeryAcu31MR:IndicationsforsurgeryChronic,asymptomatic:surveillanceSurgeryifLVsystolicdysfunctionorhLVsizealso:?ifAForpulmonaryHTdevelopMVRforMRlesssuccessfulthanforMSTendencyforlatepresentation&OTHenceirreversibleLVdamagemorelikelyMR:IndicationsforsurgeryChr32ENDEND33二尖瓣病理生理学课件34Epidemiology2019intheUK:6471firsttimevalvereplacementsofwhich28%MVRNumbersincreasingEpidemiology2019intheUK:35MitralAnnulus:fibro-muscularskeletonAnchorsbaseofvalveleafletsLeaflets:conntissue+muscle+vessels/nervesAnterior(aortic):larger;1/3ofannulusPosterior(mural):2/3ofannulusAnatomyMitralAnnulus:fibro-muscular36AnatomyPapillarymuscles:AnterolateralPosteromedialChordaetendinae1st,2nd,3rdorderApprox25majorchordaltrunks>100attachmentstoleafletsNoconsensusontimingofmuscleactivitywithcardiaccycleAnatomyPapillarymuscles:37AnnulardynamicsAnnularsizeIncreasesinlatesystole(maximumindiastole)Contractsinpre-systole(minimuminmidsystole)AnnularshapeMoreeccentricinsystoleAnnularpositionMovesuptowardsLAindiastoleMovesdowntowardsLVapexinsystoleAnnulardynamicsAnnularsize38LeafletdynamicsOpeningStartsincenter,movingtoedgesFlappingofedgesatmax.openingClosing (beginsinlatediastole)Bulgingatbase/annularattachmentLeafletascendstowardsLABulging‘rolls’fromannulustoedgeLeafletdynamicsOpening39MitralStenosis(MS)Aetiology: RheumaticMale:femaleratiois1:2-3Acquiredearly(<20yo?)Butclinicallymanifest10-40yrslaterDefiniteHxofRFin50-60%Pancarditis: mitralvalveinvolvedin90% mitralvalvealonein40%MitralStenosis(MS)Aetiology:40MitralStenosis(MS)Rheumaticdisease:Leafletthickening,calcification,retractionPeriannularcalcification(restrictedmovement)Commisuralfusion:‘fish-mouthing’Chordalthickening,shortening,fusionPapillarymuscleinflammationAlso: calcific congenital neoplastic thrombotic infective iatrogenicMitralStenosis(MS)Rheumatic41MS:HemodynamicsDiastolicAVpressuregradientLApressuremayriseto15-20mmHgatrestGradientriseswithexerciseSmallervalveareahighergradientInflowobstruction:idurationofLVfillingHencereducedcardiacoutputExacerbatedbyhheartrate(e.g.AF)MS:HemodynamicsDiastolicAVp42MS:CardiacadaptationsLVessentiallynormalBut?associateddiseasesaffectingLVLAhypertrophyDisorganizedmusclefibres

abnormalconduction/refractoryperiods atrialfibrillation(30-50%,espolder)MaygiveLAmuralthrombiMS:CardiacadaptationsLVesse43MS:PulmonarychangesInseverechronicMS:PulmonaryHTLApressure>30mmHg:pulmtransudation

reducedlungcompliancePulmartsystolicpressure>60mmHg

impedesRVemptying rightheartfailureUltimatelyirreversiblepulmvascularchangesMS:PulmonarychangesInsevere44MS:NaturalhistoryProgressivelife-longdiseaseLonglatencySymptoms:Lowcardiacoutput:dyspnoea,fatiguePulmonarycongestion/HT(orthopnea,PND) rightheartfailure

hemoptysisAtrialfibrillation/Thromboembolism‘Cardiaccachexia’MS:NaturalhistoryProgressive45MS:NaturalhistoryOnsetofsymptomstodisability:10years10yearsurvival:Asymptomatic(NYHAclassI)80%(progression)Symptomatic(NYHAclassIII)20%Causesofdeath:CHF60-70%Systemicembolism20-30%Pulmonaryembolism10%Infection1-5%MS:NaturalhistoryOnsetofsy46MS:InvestigationsCXR:LAenlargement,pulmcongestionECG:LAenlargement(notchedPinII,V1)atrialarrhythmias?RVHEcho:valvearea,LA/LVdimensionsDoppler:measurespressuregradientsTOE:bettermitral/LAvisualizationCardiaccatheter:notessentialAssocddisease;LVventriculography&pressuresMS:InvestigationsCXR:LAenla47MS:MedicaltherapyPharmacologicalTxofmildheartfailure,bronchitis,arrhythmias,hemoptysisEndocarditisprophylaxisAnticoagulation:HxofAF/thromboembolismBalloon(oropen)ValvuloplastyMS:MedicaltherapyPharmacolog48MS:IndicationsforsurgerySymptomatic(NYHAclassIII-IV):MVR

hlong-termsurvival10yearsurvival: 0-20%90%(89%at15yrs)

hfunctionalcapacityValvearea<1-1.5cm2 (normal4-6

cm2)SystemicemboliMS:IndicationsforsurgerySym49MS:IndicationsforsurgeryClassI-II:controversialRiskofSCDifasymptomatic:negligibleSurvivalnotimprovedbyMVR?roleofvalvotomy(pulmonaryHT,AF)MVRindicatedwhen:Valvearea<1.5cm2 PulmonaryHT(higherrisk)Thromboembolism ?PregnancyplannedSymptomprogression>NYHAclassII+MS:IndicationsforsurgeryCla50MitralRegurgitation(MR)AetiologymorediversethanMSMyxomatousdegenerationLeadingcauseinWest(30-70%)DefectivefibroelastictissuefloppyvalveMostasymptomaticComplicatedbyannulardilatation,chordalrupture,endocarditisRheumaticdiseasenextmostcommonMitralRegurgitation(MR)Aetio51MR:CarpentierclassificationNormalleafletmotionAnnular/ventriculardilatationLeafletdisease/perforation MR:CarpentierclassificationN52MR:CarpentierclassificationExcessiveleafletmotion(prolapse)Chordal/papillarymuscleelongationorruptureMR:CarpentierclassificationE53MR:CarpentierclassificationRestrictedleaflet/chordalmotione.g.fibrosis,calcification,retraction MR:CarpentierclassificationR54MR:AetiologyMitralAnnulusMyxomatousdegenerationSenilecalcificationFunctionaldilatation(e.g.myocarditis)RingabscessMarfan’sMR:AetiologyMitralAnnulus55MR:AetiologyMitralleafletsRheumaticdisease,endocarditis (1-30%)UnknownwhysomedevelopMS,othersMRFibrocalcificleafletthickening(withoutfusion)Chordaeshortened,annulusdilatedalso:congenital,connectivetissuediseaseMR:AetiologyMitralleaflets56MR:AetiologyChordaeIschaemiaMyxomatousInfectiveConnectivetissueTraumaIdiopathicMR:AetiologyChordae57MR:AetiologyPapillarymuscle

(10-25%)Dysfunction/ruptureIHD/MI:muscle&annularinjuryfrankrupturerare,usuallyfatalesp.PosteromedialmuscleAlso:abscess,sarcoid/amyloid,myocarditisMalalignmente.g.LVaneurysm,dilatation,myopathyMR:AetiologyPapillarymuscle58MR:HemodynamicsAcute: JLApressure,pulmoedemaChronic:LA/PVcompliance:ipulmcongestnRegurgitantvolumedependson:MitralorificesizeLV-LApressuregradientHeartrateMedicalTxaimstocontrolabovefactorsesp.decreaseafterloadtoreduceLVdilatationMR:HemodynamicsAcute: JLApr59MR:CardiacadaptationsLV:hpreload,iafterloadLVdilated,morespherical,thinnedIncreasedSV(O2consumptionnotmarkedlyh)ButdecompensationcangraduallyoccurLA:hsizeinchronicMRhcomplianceLessthromboembloism,AFthanMSMR:Cardiacada