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VALVULARHEARTDISEASE..VALVULARHEARTDISEASE..1LearningobjectivesDescribetheetiologyandclinicalsymptomsofacuterheumaticfever.Describethepathologyandnaturalhistoryofacuterheumaticfeverandrheumaticheartdisease.DescribeanAschoffbody.Describewhichpartsoftheheartareaffectedinacuterheumaticfever.Describethelong-termconsequencesofrheumaticfever.Describethepathologyandnaturalhistoryofchronicrheumaticheartdisease,andknowwhichvalvesaremostofteninvolved.Describethepathologyofpostrheumaticmitralstenosis.Enlistthecomponentsofvegetation...LearningobjectivesDescribeth2Rheumaticfeverand

Rheumaticheartdisease..Rheumaticfeverand

Rheumatic3RheumaticfeverRheumaticfeverisanacuteimmunologicallymediatedmultisysteminflammatorydiseasethatoccursafewweeksfollowinganepisodeofgroupAstreptococcalpharyngitis.Acuterheumaticcarditis,duringactivephaseMayprogresstoChronicrheumaticheartdisease...RheumaticfeverRheumaticfever4EtiopathogenesisAcuterheumaticfeverisahypersensitivityreactioninducedbygroupAstreptococci.AntibodiesagainstMproteinsofcertainstrainsofstreptococcicrossreactwithantigensinheart,jointsandothertissues.GeneticsusceptibilityissuggestedAutoimmuneresponsetoselfantigensalsosuggested.Chronicsequelaearearesultofprogressivefibrosis(healingprocess)andbloodturbulanceinvalvularareas..EtiopathogenesisAcuterheumati5PathogenesisvegetationsAschoffbody,myocardiumFibrinouspericarditisCROSSREACTIONS..PathogenesisvegetationsAschoff6Morphology ACUTERH.FEVER--PancarditisPericarditis-serofibrinous/BreadandbuttertypeMyocarditisAschoffbodiesEndocarditisVerrucousvegetations(1-2mm)atlinesofclosureofvalvesFibrinoidnecrosisalongcuspsandteninouscordsMacCallumplaquesinleftatrium(Subendocardialthickeningsduetoregurgitantjets)..Morphology ACUTERH.FEVER--7vegetationsAschoffbody,myocardiumFibrinouspericarditis..vegetationsAschoffbody,Fibrin8CarditisAschoffbodiesAschoff/AnitschowcellsAschoffgiantcell..CarditisAschoffbodiesAschoff/9ChronicdiseaserheumaticaorticstenosisWithfusedcommisuresrheumaticmitralvalve,..Chronicdiseaserheumaticaorti10MorphologyofchronicRHDMitralvalveismostoftenaffectedwithrheumaticheartdisease,followedbymitralandaortictogether,thenaorticalone,thenmitral,aortic,andtricuspidtogether.Mitralstenosis(99%cases)Fishmouth/buttonholestenosis

MicroscopyFibrosis/scarringNeovascularization..MorphologyofchronicRHDMitra11Mitralvalveasseenfromaboveintheleftatrium.Typical"fishmouth"shapewithchronicrheumaticscarring.Mitralvalveismostoftenaffectedwithrheumaticheartdisease,followedbymitralandaortictogether,thenaorticalone,thenmitral,aortic,andtricuspidtogether...Mitralvalveasseenfromabov12ClinicalfeaturesofARF

ThemajorclinicalmanifestationsofARF:migratorypolyarthritiscarditis,subcutaneousnodules,erythemamarginatum,andSydenhamchorea. MinormanifestationsofARF:Fever,arthralgias,Increasedbloodlevelsofacutephasereactantsetc...ClinicalfeaturesofARF Them13DIAGNOSIS

Jonescriteria:EvidenceofprecedinggroupAstrept.InfectionPresenceoftwomajororonemajorandtwominormanifestations..DIAGNOSIS Jonescriteria:..14ClinicalfeaturesofARFAge:5-15butmaybeinadultsTime:10daysto6wksafterpharyngitis3%ofptseffectedPrognosisof1stattackgood...ClinicalfeaturesofARFAge:515Clinicalfeaturesofchronicrheumaticcarditis

Valvulardiseaseanditssequelae(yrslater)MurmursCardiachypertrophy,dilatation,heartfailureArrythmiasespAtrialfibrillationThromboemboliccomplicationsInfectiveendocarditis..Clinicalfeaturesofchronicr16Inthedilatedatriumwithastenoticmitralvalve,thebloodstagnates.Hence,stasisisafactorinthrombogenesis....Inthedilatedatriumwithas17Infectiveendocarditis..Infectiveendocarditis..18Infectionofheartvalveormuralendocardiumbyamicrobeleadingtoformationofbulkyfriablevegetationsanddestructionofunderlyingtissue.Vegetationsarecomposedofthromboticdebrisandorganisms..Infectionofheartvalveormu19ClassificationAcuteVirulentorganismNormalvalveNecrotizingulcerative,invasiveinfectionMaybefatalSubacuteLessvirulentorganismDamagedvalveTreatablewithantibiotics..ClassificationAcute..20Etiopathogenesis

ORGANISMS MostcasesarebacterialalthoughchlamydiaeandricketiaalsoimplicatedDamagedvalves:Strept.Viridans(50-60%)Normal/damaged:Staph.Aureus(10-20%)I/Vdrugabusers:Staph.AureusProstheticvalves:StaphepidermidisOthersareenterococci,HACEKCulturenegative:10%..Etiopathogenesis ORGANISMS.21Etiopathogenesis PREDISPOSEDVALVESRHD(previously)MyxomatousmitralvalveDegenerativecalcificvalvularstenosisProstheticvalves HOSTFACTORSImmunodeficiency,immunosupressionMalignancyDiabetesAlcoholI/Vdrugabuse..Etiopathogenesis PREDISPOSED22ClinicalfeaturesSplinterhgsPetechiaeJanewaylesionsOsler’snodes..ClinicalfeaturesSplinterhgsP23Diagnosis..Diagnosis..24....25morphologyThemorevirulentbacteriacausingtheacutebacterialformofinfectiveendocarditiscanleadtoseriousdestruction,asshownhereintheaorticvalve.Irregularreddishtanvegetationsoverlievalvecuspsthatarebeingdestroyed.Portionsofthevegetationcanbreakoffandbecomesepticemboli...morphologyThemorevirulentba26Hereisavalvewithinfectiveendocarditis.Thebluebacterialcoloniesonthelowerleftareextendingintothepinkconnectivetissueofthevalve.Valvesarerelativelyavascular,sohighdoseantibiotictherapyisneededtoeradicatetheinfection...Hereisavalvewithinfective27VALVULARHEARTDISEASE..VALVULARHEARTDISEASE..28LearningobjectivesDescribetheetiologyandclinicalsymptomsofacuterheumaticfever.Describethepathologyandnaturalhistoryofacuterheumaticfeverandrheumaticheartdisease.DescribeanAschoffbody.Describewhichpartsoftheheartareaffectedinacuterheumaticfever.Describethelong-termconsequencesofrheumaticfever.Describethepathologyandnaturalhistoryofchronicrheumaticheartdisease,andknowwhichvalvesaremostofteninvolved.Describethepathologyofpostrheumaticmitralstenosis.Enlistthecomponentsofvegetation...LearningobjectivesDescribeth29Rheumaticfeverand

Rheumaticheartdisease..Rheumaticfeverand

Rheumatic30RheumaticfeverRheumaticfeverisanacuteimmunologicallymediatedmultisysteminflammatorydiseasethatoccursafewweeksfollowinganepisodeofgroupAstreptococcalpharyngitis.Acuterheumaticcarditis,duringactivephaseMayprogresstoChronicrheumaticheartdisease...RheumaticfeverRheumaticfever31EtiopathogenesisAcuterheumaticfeverisahypersensitivityreactioninducedbygroupAstreptococci.AntibodiesagainstMproteinsofcertainstrainsofstreptococcicrossreactwithantigensinheart,jointsandothertissues.GeneticsusceptibilityissuggestedAutoimmuneresponsetoselfantigensalsosuggested.Chronicsequelaearearesultofprogressivefibrosis(healingprocess)andbloodturbulanceinvalvularareas..EtiopathogenesisAcuterheumati32PathogenesisvegetationsAschoffbody,myocardiumFibrinouspericarditisCROSSREACTIONS..PathogenesisvegetationsAschoff33Morphology ACUTERH.FEVER--PancarditisPericarditis-serofibrinous/BreadandbuttertypeMyocarditisAschoffbodiesEndocarditisVerrucousvegetations(1-2mm)atlinesofclosureofvalvesFibrinoidnecrosisalongcuspsandteninouscordsMacCallumplaquesinleftatrium(Subendocardialthickeningsduetoregurgitantjets)..Morphology ACUTERH.FEVER--34vegetationsAschoffbody,myocardiumFibrinouspericarditis..vegetationsAschoffbody,Fibrin35CarditisAschoffbodiesAschoff/AnitschowcellsAschoffgiantcell..CarditisAschoffbodiesAschoff/36ChronicdiseaserheumaticaorticstenosisWithfusedcommisuresrheumaticmitralvalve,..Chronicdiseaserheumaticaorti37MorphologyofchronicRHDMitralvalveismostoftenaffectedwithrheumaticheartdisease,followedbymitralandaortictogether,thenaorticalone,thenmitral,aortic,andtricuspidtogether.Mitralstenosis(99%cases)Fishmouth/buttonholestenosis

MicroscopyFibrosis/scarringNeovascularization..MorphologyofchronicRHDMitra38Mitralvalveasseenfromaboveintheleftatrium.Typical"fishmouth"shapewithchronicrheumaticscarring.Mitralvalveismostoftenaffectedwithrheumaticheartdisease,followedbymitralandaortictogether,thenaorticalone,thenmitral,aortic,andtricuspidtogether...Mitralvalveasseenfromabov39ClinicalfeaturesofARF

ThemajorclinicalmanifestationsofARF:migratorypolyarthritiscarditis,subcutaneousnodules,erythemamarginatum,andSydenhamchorea. MinormanifestationsofARF:Fever,arthralgias,Increasedbloodlevelsofacutephasereactantsetc...ClinicalfeaturesofARF Them40DIAGNOSIS

Jonescriteria:EvidenceofprecedinggroupAstrept.InfectionPresenceoftwomajororonemajorandtwominormanifestations..DIAGNOSIS Jonescriteria:..41ClinicalfeaturesofARFAge:5-15butmaybeinadultsTime:10daysto6wksafterpharyngitis3%ofptseffectedPrognosisof1stattackgood...ClinicalfeaturesofARFAge:542Clinicalfeaturesofchronicrheumaticcarditis

Valvulardiseaseanditssequelae(yrslater)MurmursCardiachypertrophy,dilatation,heartfailureArrythmiasespAtrialfibrillationThromboemboliccomplicationsInfectiveendocarditis..Clinicalfeaturesofchronicr43Inthedilatedatriumwithastenoticmitralvalve,thebloodstagnates.Hence,stasisisafactorinthrombogenesis....Inthedilatedatriumwithas44Infectiveendocarditis..Infectiveendocarditis..45Infectionofheartvalveormuralendocardiumbyamicrobeleadingtoformationofbulkyfriablevegetationsanddestructionofunderlyingtissue.Vegetationsarecomposedofthromboticdebrisandorganisms..Infectionofheartvalveormu46ClassificationAcuteVirulentorganismNormalvalveNecrotizingulcerative,invasiveinfectionMaybefatalSubacuteLessvirulentorganismDamagedvalveTreatablewithantibiotics..ClassificationAcute..47Etiopathogenesis

ORGANISMS MostcasesarebacterialalthoughchlamydiaeandricketiaalsoimplicatedDamagedvalves:Strept.Viridans(50-60%)Normal/damaged:Staph.Aureus(10-20%)I/Vdruga

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