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MitralStenosis

MitralStenosis
Author:ClaudiaDima,MD,FACCChiefEditor:RichardALange,MD,MBAmore...
Updated:Nov6,2014

Background
Mitralstenosis(MS)ischaracterizedbyobstructiontoleftventricularinflowatthelevelofmitralvalvedueto
structuralabnormalityofthemitralvalveapparatus.Themostcommoncauseofmitralstenosisisrheumaticfever.
TheassociationofatrialseptaldefectwithrheumaticmitralstenosisiscalledLutembachersyndrome.
Stenosisofthemitralvalvetypicallyoccursdecadesaftertheepisodeofacuterheumaticcarditis.Acuteinsultleads
toformationofmultipleinflammatoryfoci(Aschoffbodies,perivascularmononuclearinfiltrate)intheendocardium
andmyocardium.Smallvegetationsalongtheborderofthevalvesmayalsobeobserved.Withtime,thevalve
apparatusbecomesthickened,calcified,andcontracted,andcommissuraladhesionoccurs,ultimatelyresultingin
stenosis.
Whethertheprogressionofvalvedamageisduetohemodynamicinjuryofthealreadyaffectedvalveapparatusorto
thechronicinflammatorynatureoftherheumaticprocessisunclear.

Othercauses
Other,lesscommonetiologiesformitralstenosisincludemalignantcarcinoiddisease,systemiclupus
erythematosus,rheumatoidarthritis,mucopolysaccharidosesoftheHunterHurlerphenotype,Fabrydisease,
Whippledisease,andmethysergidetherapy.Congenitalmitralstenosiscanalsooccur.
Anumberofconditionscansimulatethephysiologyofmitralstenosis:severenonrheumaticmitralannular
calcification,infectiveendocarditiswithlargevegetation,leftatrialmyxoma,ballvalvethrombus,andcortriatriatum.
Indeed,astudybyIwatakietalindicatedthatinpatientswithdegenerativeaorticstenosis,calcificextensiontothe
mitralvalve,causingmitralannular/leafletcalcification,canresultinnonrheumaticmitralstenosis.Usingrealtime
threedimensional(3D)transesophagealechocardiographyin101patientswithdegenerativeaorticstenosisand26
controlsubjects,theinvestigatorsfoundanaveragedecreaseof45%intheeffectivemitralannularareaofthe
patientswithdegenerativeaorticstenosis,aswellasasignificantreductioninthemaximalanteriorandposterior
leafletopeningangle.Consequently,asignificantdecreaseinthemitralvalveareainthesepatientswasfound,with
anareaoflessthan1.5cm2detectedin24ofthem(24%).[1]

Pathophysiology
Thenormalmitralvalveorificeareaisapproximately46cm2.Astheorificesizedecreases,thepressuregradient
acrossthemitralvalveincreasestomaintainadequateflow.
Patientswillnotexperiencevalverelatedsymptomsuntilthevalveareais22.5cm2orless,atwhichpointmoderate
exerciseortachycardiamayresultinexertionaldyspneafromtheincreasedtransmitralgradientandleftatrial
pressure.
Severemitralstenosisoccurswithavalveareaoflessthan1cm2.Asthevalveprogressivelynarrows,theresting
diastolicmitralvalvegradient,andhenceleftatrialpressure,increases.Thisleadstotransudationoffluidintothe
lunginterstitiumanddyspneaatrestorwithminimalexertion.Hemoptysismayoccurifthebronchialveinsrupture
andleftatrialdilatationincreasestheriskforatrialfibrillationandsubsequentthromboembolism.
Pulmonaryhypertensionmaydevelopasaresultof(1)retrogradetransmissionofleftatrialpressure,(2)pulmonary
arteriolarconstriction,(3)interstitialedema,or(4)obliterativechangesinthepulmonaryvascularbed(intimal
hyperplasiaandmedialhypertrophy).Aspulmonaryarterialpressureincreases,rightventriculardilationandtricuspid
regurgitationmaydevelop,leadingtoelevatedjugularvenouspressure,livercongestion,ascites,andpedaledema.
Leftventricularenddiastolicpressureandcardiacoutputareusuallynormalinthepersonwithisolatedmitral
stenosis.Astheseverityofstenosisincreases,thecardiacoutputbecomessubnormalatrestandfailstoincrease
duringexercise.Approximatelyonethirdofpatientswithrheumaticmitralstenosishavedepressedleftventricular
systolicfunctionasaresultofchronicrheumaticmyocarditis.Thepresenceofconcomitantmitralregurgitation,
systemichypertension,aorticstenosis,ormyocardialinfarctioncanalsoadverselyaffectleftventricularfunctionand
cardiacoutput.

Epidemiology
Frequency
UnitedStates
Theprevalenceofrheumaticdiseaseindevelopednationsissteadilydecliningwithanestimatedincidenceof1in
100,000.
International
TheprevalenceofrheumaticdiseaseishigherindevelopingnationsthanintheUnitedStates.[2]InIndia,for
example,theprevalenceisapproximately100150casesper100,000,andinAfricatheprevalenceis35casesper
100,000.

Mortality/Morbidity
Mitralstenosisisaprogressivediseaseconsistingofaslow,stablecourseintheearlyyearsfollowedbyan
acceleratedcourselaterinlife.Typically,thereisalatentperiodof2040yearsfromtheoccurrenceofrheumatic
fevertotheonsetofsymptoms.Oncesymptomsdevelop,itisalmostadecadebeforetheybecomedisabling.In
somegeographicareas,mitralstenosisprogressesmorerapidly,presumablyduetoeitheramoresevererheumatic
insultorrepeatedepisodesofrheumaticcarditisduetonewstreptococcalinfections,whichresultsinsevere

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symptomaticmitralstenosisinthelateteensandearly20s.
Intheasymptomaticorminimallysymptomaticpatient,survivalisgreaterthan80%at10years.Whenlimiting
symptomsoccur,10yearsurvivalislessthan15%inthepatientwithuntreatedmitralstenosis.Whensevere
pulmonaryhypertensiondevelops,meansurvivalislessthan3years.Most(60%)patientswithsevereuntreated
mitralstenosisdieofprogressivepulmonaryorsystemiccongestion,butothersmaysuffersystemicembolism(20
30%),pulmonaryembolism(10%),orinfection(15%).

Sex
Twothirdsofallpatientswithrheumaticmitralstenosisarefemale.

Age
Theonsetofsymptomsusuallyoccursbetweenthethirdandfourthdecadeoflife.

ContributorInformationandDisclosures
Author
ClaudiaDima,MD,FACCInterventionalCardiology
Disclosure:Nothingtodisclose.
Coauthor(s)
KennethBDesser,MDClinicalProfessor,DirectorofCardiologyFellowship,BannerGoodSamaritanMedical
Center,Phoenix,Arizona
Disclosure:Nothingtodisclose.
SpecialtyEditorBoard
LMichaelPrisant,MD,FACC,FAHACardiologist,EmeritusProfessorofMedicine,MedicalCollegeofGeorgia,
GeorgiaRegentsUniversity
LMichaelPrisant,MD,FACC,FAHAisamemberofthefollowingmedicalsocieties:AmericanCollegeof
Cardiology,AmericanCollegeofChestPhysicians,AmericanCollegeofClinicalPharmacology,AmericanCollege
ofForensicExaminers,AmericanCollegeofPhysicians,AmericanHeartAssociation,andAmericanMedical
Association
Disclosure:BoehringerIngelheimHonorariaSpeakingandteaching
FranciscoTalavera,PharmD,PhDAdjunctAssistantProfessor,UniversityofNebraskaMedicalCenterCollege
ofPharmacyEditorinChief,MedscapeDrugReference
Disclosure:MedscapeSalaryEmployment
StevenJCompton,MD,FACC,FACP,FHRSDirectorofCardiacElectrophysiology,AlaskaHeartInstitute,
ProvidenceandAlaskaRegionalHospitals
StevenJCompton,MD,FACC,FACP,FHRSisamemberofthefollowingmedicalsocieties:AlaskaState
MedicalAssociation,AmericanCollegeofCardiology,AmericanCollegeofPhysicians,AmericanHeart
Association,AmericanMedicalAssociation,andHeartRhythmSociety
Disclosure:Nothingtodisclose.
AmerSuleman,MDPrivatePractice
AmerSuleman,MDisamemberofthefollowingmedicalsocieties:AmericanCollegeofPhysicians,American
HeartAssociation,AmericanInstituteofStress,AmericanSocietyofHypertension,FederationofAmerican
SocietiesforExperimentalBiology,RoyalSocietyofMedicine,andSocietyofCardiacAngiographyand
Interventions
Disclosure:Nothingtodisclose.
ChiefEditor
RichardALange,MD,MBAPresident,TexasTechUniversityHealthSciencesCenter,Dean,PaulLFoster
SchoolofMedicine
RichardALange,MD,MBAisamemberofthefollowingmedicalsocieties:AlphaOmegaAlpha,American
CollegeofCardiology,AmericanHeartAssociation,andAssociationofSubspecialtyProfessors
Disclosure:Nothingtodisclose.
AdditionalContributors
TheauthorsandeditorsofMedscapeDrugs&Diseasesgratefullyacknowledgethecontributionsofprevious
authorsHolgerPSalazar,MD,SenthilNachimuthu,MD,FACP,andKiruthikaBalasundaram,MBBS,tothe
developmentandwritingofthisarticle.

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