倪超-瓣膜病課件

ValvularHeartDiseaseNiChao,M.D.DivisionofCardiology倪超-瓣膜病ObjectivesTounderstandthepathophysiologyofthemajorVHDsTolearnhowtoexaminethepatientTounderstandtheprinciplesoflaboratorydiagnosisTolearnthefundamentalsfortreatmentofcardiacvalveabnormalities倪超-瓣膜病MitralStenosis倪超-瓣膜病EtiologyAlmostalwaystheresultofrheumaticfeverLesscommoncausesCongenitalMSSystemiclupuserythematosusRheumatoidarthritisAtrialmyxomaBacterialendocarditis.倪超-瓣膜病Epidemiology

Rareinindustrializedcountriesinpatients<40Verycommonindevelopingcountries,esp.SouthAsiawithseverediseaseoftenatearlyage(<20)2/3ofallpatientswithMSarefemale.Theonsetofsymptomsisusu.betweenthe3rdand4thdecades.倪超-瓣膜病PathologyThemitralvalvearea(MVA)isnormally4-6cm2inadultAcuterheumaticfevercauses

immune-medi-atedinflammationofthemitralandothervalvesTheleafletsthickenedandthecommissuresfusedalongwiththickeningandshorteningofthechordaetendineae

narrowingofthemitralvalveorifice倪超-瓣膜病倪超-瓣膜病Pathophysiology

MVAreducedto2cm2:increasedleftatrialpressure(LAP)isnecessaryfornormaltrans-mitralflowMVAreducedto1cm2:aLAPof25mmHgisrequired

arisein:Pulmonaryvenouspreesure(PVP)Pulmonarycapillarywedgepressure(PCWP)

exertionaldyspnea倪超-瓣膜病PathophysiologyProgressivedilationoftheLApredisposesmuralthrombiandatrialfibrillationChronicelevationofLAP

pulmonaryhypertension,tricuspidandpulmonaryre-gurgitation

rightheartfailure倪超-瓣膜病PathophysiologyPatientsathighriskareofmuralthrombiover35yearsoldatrialfibrillationwithalowcardiacoutput(CO)havingalargeleftatrialappendage.Atrialfibrillationinupto40%ofpatientsdecreasesCOby20%.倪超-瓣膜病倪超-瓣膜病ClinicalManifestationsHistoriesofrheumaticfever,murmurDyspneaPalpitationsChestpainHemoptysisEdemaThromboembolism倪超-瓣膜病PhysicalExaminationLow-pitcheddiastolicrumbleOpeningsnapS1

,atrialfibrillation,P2

RVheaveElevatedneckveins,hepatomegaly,ascites,pedaledemaCoexistentmurmursThromboembolicevents倪超-瓣膜病ChestX-Ray

LeftatrialenlargementPulmonaryedemaProminenceofpulmonaryarteriesEnlargementofrightventricle倪超-瓣膜病倪超-瓣膜病EKG

Left,rightatrialabnormalitiesatrialfibrillationRightVenticularhypertrophy倪超-瓣膜病Echocardiogram

EnlargedLAMarkedlythickened,oftencalcifiedMVwithverynarrow,"fish-mouth"shapedorificeDelayedLVfillingwithtransmitralgradientindiastole倪超-瓣膜病EchocardiogramRVhypertrophy,RVhypokinesisTVthickness,stenosisorregurgitationPulmonaryhypertensionLVfunctionusuallypreserved倪超-瓣膜病倪超-瓣膜病倪超-瓣膜病SeverityofMitralStenosisMildModerateSevereMVA(cm2)1.5-2.01.0-1.5<1.0MeanPG(mmHg)<1010-20>20倪超-瓣膜病NaturalHistory

Inindustrializedcountries,20-25yearlatentperiodbetweenepisodesofrheumaticfeverandclinicalsignsofMSOncemildsymptomsdevelop,progressiontocompletedisabilityisveryrapid(5years)withoutinterventionTimecoursemorefulminantindevelopingcountries倪超-瓣膜病Management—Medical

SaltreductionDiureticsControlofheartratewithdigoxinAnti-arrhythmicdrugsPreventionofthromboemboliwithadequateanti-coagulants倪超-瓣膜病Management—Surgical

Interventionaltherapyindicatedformitralvalveareaof<1.0cm2Mitralcommisurotomyormitralvalvereplace-mentarecommonsurgicalapproachesForselectedpatients(primarilyyoungwithpureMS),mitralballoonvalvuloplastyisasuccessfuloption倪超-瓣膜病MitralRegurgitation倪超-瓣膜病Etiologies—Acute

Endocarditis(mostoftencausedbyStaphylo-coccusaureus)Papillarymusclerupture(frominfarction)ordysfunction(fromischemia)Chordalrupture(frommyxomatousvalvulardisease)倪超-瓣膜病Etiologies—ChronicRheumaticfeverMitralvalvularprolapseMarfansyndromeCardiomyopathy

倪超-瓣膜病Pathophysiology—Acute

AbruptelevationofLApressureinsettingofLAwithnormalsizeandcomplianceBackflowintopulmonarycirculationwithelevatedPVPandPCWPandpulmonaryedemaDecreasedforwardflowofCO,hypotensionandshockoccuroften倪超-瓣膜病Pathophysiology—Chronic

GradualelevationofLApressurewithdilat-ationofLAandLVIncreasedpreloadandeccentricLVhyper-trophy,LVfunctionfallsElevatedpulmonaryvascularfilling

Pul-monaryhypertension倪超-瓣膜病倪超-瓣膜病倪超-瓣膜病Clinicalmanifestations

DyspneaonexertionParoxysmalnocturnaldyspneaOrthopneaPalpitationsEdema倪超-瓣膜病PhysicalExamination

LaterallydisplacedPMI,RVheaveHolosystolicmurmurS3gallopAtrialfibrillationLoud,palpableP2SignsofendocarditisThromboembolicevents倪超-瓣膜病ChestX-ray

DilatedLV(chronic)Pulmonaryvascularredistribution(chronic)pulmonaryedema(acuteandchronic)倪超-瓣膜病EKG

LeftventricularhypertrophyLeftor/andrightatrialenlargementAtrialfibrillationNonspecificST-TwavechangesIschemicST-TwavechangesMyocardialinfarction倪超-瓣膜病Echocardiogram

DilatedLA,LV,RA,RVDecreasedLVfunctionMitralregurgitationSegmentalwallmotionabnormalities(ifin-farction)Vegetations(ifendocarditis)倪超-瓣膜病SeverityofMitralRegurgitationⅠⅡⅢⅣLength(cm)1.5≥1.5≥3.0≥4.5Area(cm2)1.5≥1.5≥3.0≥4.5IA/LAA(%)<2020-40≥40倪超-瓣膜病ExerciseRadionuclideVentriculography

FallinLVejectionfraction(>5%declineinejectionfraction)indicatingincipientLVdysfunctionDilatedLV,LA,RV,RA倪超-瓣膜病CardiacCatheterization

ElevatedLVEDP,PVP,PCWPandPAPCoronaryarteryocclusions(ifinfarction)倪超-瓣膜病NaturalHistory

TimecourseisvariableforchronicformExtentofLVcavitydilatationisinverselyrelatedtosurvival5yearsurvivalofpatientstreatedmedicallyis45-80%dependingonexerciselimitationAcuteformisassociatedwithmuchhighermortality倪超-瓣膜病Management—Medical

SaltreductionDiureticsDigoxinVasodilators(ACEIs,nitrates,hydralazine)AnticoagulationAnti-arrhythmicsIntra-aorticballoonpump(IABP)倪超-瓣膜病Management—Surgical

Mitralvalverepairorreplacement:mustbeperformedbeforeLVfunctiontooseriouslycompromised倪超-瓣膜病AorticStenosis倪超-瓣膜病Etiologies

Bicuspidaorticvalve(mostcommoncon-genitalanomaly)Calcificationoftri-leafletaorticvalveCongenitalunileafletvalveRheumaticfever(<1%ofpatientswithisolatedaorticvalvedisease-usuallyalsoinvolvesmitralvalve)倪超-瓣膜病Epidemiology

Largestgroupis70-90y/owithdiseaseontri-leafletvalvesNextlargestgroupis45-60y/owithdiseaseonbicuspidaorticvalvesThereisalsoasmallgroupofpatientswithcongenitalAS倪超-瓣膜病倪超-瓣膜病倪超-瓣膜病Pathophysiology

ObstructiontoLVoutflow

theLVhyper-trophiesAadequatecardiacoutputatrestpreservedforyearswithincreasingtrans-aorticgradient倪超-瓣膜病PathophysiologyWhentheAVA<0.7cm2,COmaybenormalatrestbutfailstorisewithexertionduetoobstructiontoLVemptyingfromstenoticvalveTheLVdilateswithlongstandingAS倪超-瓣膜病PathophysiologyProgressiveLVdysfunction

afallinSV,COandtheLV-aorticgradientwhiletheLAP,PCP,PAP,LVDP,RVDPincreaseThedilatationofmitralvalveannulus

MRtheLVmassincreases,diminishedLVcom-pliance,increaseddiastolicstiffness

倪超-瓣膜病ClinicalManifestations

SyncopeAnginaHeartfailureEmboliEndocarditis倪超-瓣膜病PhysicalExam

Loudsystolicmurmur,radiatestotheneckSustainedpointofmaximalimpulseDiminishedanddelayedcarotidupstroke(parvusettardus)PrecordialthrillSingle,softS2valve,S4Aorticejectionsounds(heardafterS1)

倪超-瓣膜病ChestX-ray

CalcificaorticvalveTortuousaortaLAenlargementLVenlargement(late)倪超-瓣膜病EKG

LVhypertrophyLAabnormalityInterventricularconductiondelay倪超-瓣膜病EchocardiogramLVhypertrophyThickened,immobileaorticvalveDilatedaorticroot(post-stenoticdilatation)LV-aorticgradientLAenlargementMR,LVdilatation(late)

倪超-瓣膜病SeverityofAorticStenosisMildModerateSevereMAV(cm2)1-1.50.7-1.0<0.7MeanPG(mmHg)2525-50>50倪超-瓣膜病CardiacCatheterizationLV-aorticgradientCoexistentcoronaryarterydiseasemaybepresentbutisunrelatedtodevelopmentofAS倪超-瓣膜病NaturalHistory

MaybeasymptomaticforyearsdespitesevereobstructionDevelopmentofcertainsymptomsportendsabadprognosisAangina(averagesurvival3years)Syncope(averagesurvival2years)Heartfailureaveragesurvival1.5years)

倪超-瓣膜病Management—Medical

EndocarditisprophylaxisAnti-arrhythmicsNospecificroleformedicaltherapy倪超-瓣膜病Management—SurgicalAorticvalvereplacement:improvedsurvivalandLVfunctioneveninthosewithpre-operativeLVdysfunctionaswellasinoctogenariansAorticballoonvalvuloplasty:purelypalliative,survivaloradequatelong-termbenefitsimprovednotimprovedReservedforptwhocannottoleratesurgery.倪超-瓣膜病AorticRegurgitation倪超-瓣膜病Etiologies—Acute

InfectiveendocarditisTraumaAorticdissection倪超-瓣膜病Etiologies—Chronic

Primaryvalvular

RheumaticfeverBicuspidvalveMarfanEhlers-DanlosAnkylosingspondylitisLupus倪超-瓣膜病Etiologies—Chronic

Aorticrootdisease

SyphilisOsteogenesisimperfectaAorticdissectionBehcetsyndromeReitersyndromeHypertension倪超-瓣膜病Pathophysiology—ChronicTheentireSVejectedintothehighpressureaortainaorticregurgitation(AR)PartoftheSVleaksbackintotheventricleduringdiastoleAnincreaseinLVEDVtomaintaineffectiveforwardflow倪超-瓣膜病Pathophysiology—ChronicLVdilatationoccursasdoesasignificantincreaseinLVstressTheLVmassdramaticallyincreases,oftengreaterthanLVmassinASLVEDPremainrelativelynormaluntillateindiseaseLVEFusuallynormalandmayincreasewithexercise倪超-瓣膜病Pathophysiology—ChronicAfallintheLVEFwithexerciseportendstheonsetofintrinsicLVdysfunctionWithlongstandingAR,LVdysfunctionen-sureswithdecreasedLVEF,SV,COandincreasedLVEDP,LAP,PVPandPCWP倪超-瓣膜病Pathophysiology—Acute

TheventricledoesnothavetimetodilateinresponsetotheincreasedventricularloadWithpartofthestrokevolumeleakingbackintotheventricleindiastole,theeffectiveCOfallsTherapidriseinLVpressureduetoacuteARcausesthemitralvalvetocloseearly倪超-瓣膜病ClinicalManifestationsDyspneaonexertionParoxysmalnocturnaldyspneaOrthopneaPalpitationsAnginaCyanosis/shock(acute)倪超-瓣膜病PhysicalExamination—Chronic

DiastolicdecrescendomurmurDilatedpointofmaximalimpulseRapidlycollapsingpulse(water-hammerorCorriganpulse)Headbobbing(deMussetsign)Capillarypulsations(Quinckepulses)S3倪超-瓣膜病PhysicalExamination—ChronicWideaorticpulsepressurewithsystolichypertensionPistol-shotsoundsofthefemoralarteriesLowpitcheddiastolicrumbleduetonarrowingofmitralvalveorificebyARjet(Austin-Flint)倪超-瓣膜病PhysicalExamination—AcuteNormalpulsepressureSoftorabsentS1SoftdiastolicmurmurCyanosis,shock,vasoconstriction

倪超-瓣膜病ChestX-ray

EnlargedLVTortuous,dilatedaorta(mainlychronic)倪超-瓣膜病EKGLVhypertrophy(chronicbutnotacuteform)InterventricularconductiondelayPRprolongtion倪超-瓣膜病Echocardiogram

Chronicform:dilatedLV;markedLVhyper-trophy,dilatedaorticroot,aorticregurgitation,enlargedLA,thickenedaorticvalveAcuteform:normalLVcavitysize,aorticregurgitati