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Rheumaticheartdisease DigitalPathologyCollection Case142010 Ref.II:iv:2&II:iv:5 UniversityofCapeTown Case1 Rheumaticvegetationsonmitralvalve UniversityofCapeTown Pathology: Acuterheumaticendocarditis • Thisheartlesionwasan autopsyfindinginagirlof16 years. • Thereisarowofsmall,firm, greyishvegetationsalong thecontactlineofthemitral valvecusps.Inplacesthe vegetationshavefusedto formacontinuousridge. UniversityofCapeTown • Thevegetationshave occurredatasiteofminor trauma,wherethevalve cuspscomeintocontact eachtimethevalvecloses i.e.onthecontactline(not thefreeedgeofthevalve). UniversityofCapeTown • Therheumaticvegetationsorverrucaearisedueto inflammatoryerosionofthevalvesurface,followedbyplatelet andfibrinprecipitation. • Theyarethecardinalfeatureofrheumaticendocarditis. • Butendocarditisisjustonecomponentofthepancarditisthat occursinacuterheumaticfever,i.e.thereareinflammatory changesinallthreelayersoftheheart,endocarditis, myocarditisandpericarditis. UniversityofCapeTown Anatomy • Asidefromthevisiblerheumaticvegetations,theanatomyof theheartandthemitralvalveisnormalatthisstage. • Thehearthasbeenopenedinaverticalplanethroughtheleft atriumandventricle. • Themitralvalveringhasbeenopenedandflattenedout horizontally. UniversityofCapeTown Leftatrium Mitralvalve LV LV UniversityofCapeTown • Inthenextslide,noteinparticularthenormaldelicate chordaetendineaeandthepapillarymuscles. UniversityofCapeTown Chordaetendineae Papillarymuscles UniversityofCapeTown Chronicrheumaticheartdisease • Inchronicrheumaticheartdisease(RHD)theinflammatory lesionsintheheartundergoorganisationandfibrosis. • Recurrentepisodesofrheumaticfevercausecumulativedamage. • Inparticular,thevalvesbecomescarredanddistorted,affecting theirfunction. • Clinicalsignsofthevalvularandothercardiacdamagemaytake yearsordecadestoappear. • ThemitralvalveisalmostalwaysaffectedbyRHD,withor withoutaorticvalveabnormality.Morerarelythereareright sidedvalveabnormalities. (Thinkaboutwhytheleftsidedvalvesare moresusceptibletodamage.) UniversityofCapeTown UniversityofCapeTown Mitralstenosis • 99%ofcasesofmitralstenosisareattributabletoRHD. • Inasignificantnumberofcasestheacutephaseofrheumatic fevergoesunnoticed. • Thispatientwasawhitemanaged38years.Hehadno previoushistoryofrheumaticfever. UniversityofCapeTown Clinicalfindings • Hepresentedwithswellingoftheabdomenforonemonth. • Hehadalsonoticedbreathlessnessandpalpitations.On examinationhewasorthopnoeic. • Therewasoedemaofthelegsandscrotum. • Hispulsewasveryrapidandtotallyirregular. • Theheartwasenlargedwithanearlydiastolicmurmurin mitralarea. • Theliverwasgreatlyenlarged. • Signsofcardiacfailureworsenedandthepatientdiedasa consequence. UniversityofCapeTown Pathology • Across-sectionoftheleftsideoftheheart,viewingtheleft atriumfromabove. Aorta LA UniversityofCapeTown • Themitralvalve curtainsare adhesed, thickened, distortedand calcified,resulting infunnelstenosis. • Theorificeis narrowedtoa typical“fishmouth” or"buttonhole”. • ". UniversityofCapeTown PhotobyCindySeigle,CreativeCommonsBY-NC-SA UniversityofCapeTown • Hereisthetransected leftventricle,viewing theabnormalmitral valvefrombelow. • Thechordae tendineaearealso thickenedand retracted(shortened) byfibrosis. UniversityofCapeTown TheresultsofchronicRHD • Theconsequencesofvalvedamageareheartmurmurs,cardiac hypertrophyand/ordilatationandcardiacfailure. • Othercomplicationsarearrhythmias,thromboembolicevents (suchasstroke)andinfectiveendocarditis. UniversityofCapeTown Comment • Notethatour2casesdate fromthe1920’sand1930’s. • Atthistimethelinkbetween streptococcalsorethroat andrheumaticfeverwas recognisedbutnotfully understood. • Penicillinprophylaxisto prevent(recurrent)attacksof rheumaticfeveronlycame intouseafterWorldWarII. Imageusedwithpermissionfromthe DepartmentofMicrobiology,UCT UniversityofCapeTown Streptococci α-haemolytic β-haemolytic GroupAStreptococcuspyogenes rheumaticfever,scarletfever,glomerulonephritis ImageusedwithpermissionfromtheDepartmentofMicrobiology,UCT UniversityofCapeTown • Life-savingvalvereplacementsurgerywasonlydevelopedin the1960’s. UCTprostheticmitralvalve PM239/1968 Starr-Edwardsmitralvalve prosthesis PM239/1978 • Currently,approximately150rheumaticvalvesarereplaced eachyearbythecardiothoracicsurgeryunitatGroote SchuurHospital. UniversityofCapeTown Links&references • Rheumaticheartdiseaseisstillveryimportantindeveloping countriessuchasSouthAfrica,andevenincrowded, economicallydepressedurbanareasintheWesternworld.See thisWHOmapsummarisingtheepidemiologyhttp://www.who.int/ cardiovascular_diseases/en/cvd_atlas_02_rheumaticHD.pdf UniversityofCapeTown AselectionofcasesfromtheDigitalPathologyCollectionbythe DepartmentofPathology UniversityofCapeTown islicensedundera CreativeCommonsAttribution-NonCommercial-ShareAlike4.0InternationalLicence ThefullDigitalPathologyCollectionisaccessibleat www.digitalpathology.uct.ac.za IfyouwouldliketouseanimageorotheritemfromoursitethatisnotlabelledwiththeCreative CommonsLicenceLogo,pleasecontactthecuratorforpermission. UniversityofCapeTown