BritishHeartJournal19763810531057Ventriculararrhythmiasinsyndrome - PDF document

BritishHeartJournal,1976,38,1053-1057.VentriculararrhythmiasinsyndromeofballoondeformityofmitralvalveDefinitionofpossiblehighriskgroupRonaldW.F.Campbell,.MichaelG.Godman,GarrickI.Fiddler,RobertM.Marquis,andDesmondG.JulianFromtheDepartmentofCardiology,RoyalInfirmary,Edinburgh;andtheDepartmentofCardiology,RoyalHospitalforSickChildren,EdinburghTwentypatientsclinicallyidentifiedashavingballoondeformityofthemitralvalvewerestudiedtoassesstheincidenceofventriculararrhythmias.Echocardiographyandphonocardiographywereusedtoconfirmthenatureofthemitralvalvelesion.Continuous24-hourelectrocardiogramswereobtainedfromallpatientsandanalysedbyacomputerand2observers.Onepatienthadventricularfibrillationand3patientshadventriculartachycardia.Therewasahighincidenceofotherlesssevereformsofventriculararrhythmis.EightpatientshadinferolateralSTandTwaveabnormalityontherestingelectrocardiogram,andweredescribedashavingtheauscultatory-electrocardiographicvariantoftheballoonmntralvalvesyndrome.Theoccurrenceofseriousventriculararrhythmias(ventricularfibrillationandtachycardia)wass4igficantlymorefrequentinthisgroup.Thisraisesthepossibilitythattherestingelectrocardiogrammayidentifythosepatientswithballoondeformityofthemitralvalvewhoareatriskfromsuddendeath.Balloondeformityofthemitralvalvehasbeendetectedwithincreasingfrequencysincethecharac-teristicmid-systolicclickwasfirstdescribedbyCufferandBarbillon(1887).Inmostpatientstheballoondeformityhaslittlehaemodynamicconse-quencebuttheconditionhasbeenassociatedwithventricularandsupraventriculararrhythmias(HancockandCohn,1966;Kreismanetal.,1971;Goochetal.,1972;Sloman,Wong,andWalker,1972;Jeresaty,1973;Rizzonetal.,1973;DeMariaetal.,1974;Gulottaetal.,1974;Schaal,Fontana,andWooley,1974;Winkleetal.,1975),andsuddendeathhasbeenreportedin9patients(HancockandCohn,1966;Barlowetal.,1968;Trentetal.,1970;Jeresaty,1973;MarshallandSchappell,1974;Rakowskietal.,1975).Theincidenceofthelesionisnotknownaccuratelybutinarecentnecropsyseriestheabnormalitywasdetectedin0'33percentofthepopulationstudied(Rizzonetal.,1973),andclinicalreportshavesuggestedincidencesfrom6to10percentReceived9February1976.'Presentaddress:Box3165,DukeUniversityMedicalCenter,Durham,NorthCarolina,27710,U.S.A.(Markiewiczetal.,1975;Brown,Kloster,andDeMots,1975;Procaccietal.,1975).Reportsoftheconditionareconfusedbytheheterogeneityofpathologieswhichcancausemechanicaldysfunctionofthemitralvalveleaflets.Theincidenceofar-rhythmiasmayvaryineachgroupanditispossiblethatsuddenarrhythmicdeathismorecommoninaspecificpopulation.Theobjectofthecurrentstudywastoassessthetypeandsignificanceofrhythmdisordersinpatientswithidiopathicballoondeformityofthemitralvalve.PatientsandmethodsClinicalinvestigationThesubjectswere20patientsattendingthede-partmentofcardiologyintheRoyalInfirmaryandRoyalHospitalforSickChildrenofEdinburghwhohadtheclinicalfeaturesofballoondeformityofthemitralvalvewithoutcoexistingcardiovasculardisease.Therewere13femaleand7malepatientswhoseagesrangedfrom12to61years.Initiallythediagnosiswasmadeonthepresenceofamid-systolicclickorlatesystolicmurmurorboth.The 1054Campbell,Godman,Fiddler,Marquis,andJulianpatientswereexaminedby12-leadelectrocardio-graphy,phonocardiography,posteroanteriorchestx-rayfilm,echocardiography,and24-hourcon-tinuousambulatoryelectrocardiographicmonitor-ing.ThelatterrecordingswereobtainedusingtheOxfordInstrumentsMiniatureAnalogueTapeRe-cordingSystemwhichallowedallpatientstounder-takeallnormalactivitieswithoutrestriction.TheelectrocardiographicleadusedwasamodifiedV5.24-hourelectrocardiographicanalysisThetaperecordingswerereplayedat60timesrealspeedandprocessedby

aNeilsonArrhythmiaComputer(Neilson,1974)programmedtodetectventricularectopicbeats.Thisproducedachartofthe24-hourventricularectopicbeatactivity.Thecomputeralso'froze'arrhythmiasinasolid-statememoryfromwhichtheycouldbewrittenoutinrealtimeonstandardelectrocardiographicpaper.Duringtheprocessing,therecordingswereobservedbytwoindependentobserverswhoscoredarrhythmiasandensuredaccuracyofthecomputerprogrammeifbackgroundnoiselevelsorelectricalaxisshiftnecessitatedmodification.Thetapere-cordingswererepeatedifbecauseofpoorqualitylessthan22outof24hourswerecapableofanalysis.Noattemptwasmadetoanalysetherecordingsofatrialectopicbeatactivitybutthesystemidentifiedsupraventriculartachycardia.ArrhythmiadefinitionsVentriculartachycardiawasdefinedas3ormoreconsecutiveventricularectopicbeatsatarategreaterthan100/min.R-on-Tphenomenonwasdefinedasaventricularectopicbeatfallingwithin85percentoftheprevailingQTinterval.CoupledventricularectopicbeatswerethoseoccurringinpairswithR-Rlessthan400ms.Ventricularectopicbeatsweredefinedasfrequentwhentheyoccurredmoreoftenthan30anhour.Auscultatory-electrocardiographicvariantRoutine12-leadelectrocardiogramswerecarefullyexaminedforinferolateralSTandTwaveabnor-malitiesandagroupofpatientswiththesechangeswasdescribedastheauscultatory-electrocardio-graphicvariantoftheballoondeformityofthemitralvalve(HumphriesandMcKusick,1962;BarlowandBosman,1966;Slomanetal.,1969;Liedtkeetal.,1973;PocockandBarlow,1970;Ehlersetal.,1970;Stannardetal.,1967).ResultsClinicalpresentationOfthe6patientswhowereasymptomatic,5hadbeenreferredbecauseauscultatoryabnormalitieshadbeenfoundonroutineexamination;inonepatient,ventricularectopicbeatswerenotedonanelectrocardiogram.Oftheremaining14patients,12hadcomplainedofsymptomssuggestiveofanarrhythmia,namelysyncope,palpitation,ordizzi-ness;7ofthe14alsocomplainedofill-definedchestpain.Therecordeddurationofsymptomsorsignsrangedfromafewdaysto30years.RoutineelectrocardiogramIn9ofthe20patients,theroutine12-leadelectro-cardiogramwasnormal.In3,varyingdegreesofrightbundle-branchblockwerepresent.Intheremaining8,non-specificSTandTwavechangeswerepresentintheinferolateralleads;thesepatientsconstitutedthegroupalreadydefinedastheauscultatory-electrocardiographicvariantoftheballoondeformityofthemitralvalve.Therewasnoapparentdifferenceinageorsexdistributioninthisgroupcomparedwiththeotherpatients,butcomplaintsoflassitude,breathlessness,andchestpainweremorecommon.Posteroanteriorchestx-rayfilmOfthe20patients,8showedminimalcardio-megalyonaroutinechestx-rayfilm(cardiothoracicratio�50%).FIG.1Typicalechocardiogramandsimultaneousphonocardiogram.60-.44w--4-Mot.,,-111!w ArrhythmiasinBMV105512'80Q._-oE4z'30/h'30/hformFIG.2Numbersofpatients(total16)showingeachtypeofventriculararrhythmiaon24-hourcontinuouselectrocardiogram.PhonocardiographyThecombinedfeaturesofamid-systolicclickandlatesystolicmurmurwereconfirmedin18of19patients.Thephonocardiogramwasrepeatedlyun-satisfactoryinonepatient;anotherhadonlyalatesystolicmurmur:echocardiographyconfirmedmitralvalveprolapseinbothpatients.Severalattemptswererequiredwithsomepatientsbeforeasatisfactoryrecordwasobtained.EchocardiographyThisshowedprolapseofoneorbothmitralvalveleafletsin17patients.In3patientsthemitralechogramwasnormal,butamidsystolicclickandlatesystolicmurmurwereconfirmedbyphono-cardiography.Fig.1showsatypicaltracingwiththesimultaneousphonocardiogram.4-vbr3]IVI0.0.2a_a_______-_EI0VEBsVEBsCoupledMulti-R-on-TVTVF'30/h'30/hformFIG.3'Mostsevere'ventriculararrhythmiashownin16patientson24-hourcontinuouselectrocardio-gram.The'severity'ofarrhythmiahasbeenarbitrarilygradedfrominfrequentVEB

stoVF.Shadedareasrepresentpatientswiththeauscultatory-electrocardio-graphicvariant.AmbulatoryelectrocardiographicmonitoringOnlyonepatienthadnoarrhythmiarecordedduringthe24hours.Supraventriculararrhythmiasalonewerefoundin3patients,whichin2wereatarateof160/minwhilethethird,agirlof12,de-velopedatrialflutterwith1:1atrioventricularconductionandaventricularrateof300/min.Theremaining16patientsallshowedventriculararrhythmiasofvaryingtype.Fig.2showsthenumbersofpatientswithspecificarrhythmiasonthe24-hourrecording.Multiformventricularectopicbeatswerethemostcommonformofventriculararrhythmia.Wehavenotattemptedtodocumentthenumbersofepisodesofeacharrhyth-miaineachpatientasonrepeatedrecordingsthesefigureswerevariable.Fig.3showstheresultswhentheventriculararrhythmiasarearbitrarilygradedin'severity'frominfrequentventricularectopicbeatstoventricularfibrillation.Eachpatientisclassifiedonthebasisofthemost'severe'ar-rhythmiadetectedontheir24-hourelectrocardio-gram.Ventricularfibrillationwasdetectedinonepatient.Hertaperecorderhadbeenequippedwithanalarmdevicewhich,whenpressed,pro-ducedapulseonaparalleltapetrack.ThetracingofthiseventisshowninFig.4.Thisarrhythmiaproducedsyncopebutfortunatelywasself-ter-minating.Threepatientsshowedventriculartachycardiaandin4theR-on-Tphenomenonwasthemost'severe'arrhythmiadetected.Relationbetweenventriculararrhythmiasandresting12-leadelectrocardiogramInFig.3thehatchedareasrepresentthosepatientswiththeauscultatory-electrocardiographicvariantoftheballoonmitralvalveaspreviouslydefined.Thusventricularfibrillationandventriculartachy-cardiawereseenonlyinpatientswiththisabnor-malityontheirelectrocardiogram.Thisdifferenceisstatisticallysignificant(P0'05)(Z2+Yatescorrec-tion).Thisaccountsfor4ofthe8patientsofthisgroup.TwoothershadR-on-Tphenomenon,onehadmultiformventricularectopicbeats,andthere-mainingpatienthadonlyasupraventriculartachy-cardiaatarateof160/min.Thehighincidenceof'serious'ventriculararrhythmiasinthosepatientshavinginferolateralSTandTwavechangesontherestingelectrocardiogramsuggestsanassociationbetweentheseobservations.DiscussionContinuous24-hourelectrocardiographicmonitor-ingof20patientswiththeclinicalfeaturesofapparentidiopathicballoondeformityofthemitral 1056Campbell,Godman,Fiddler,Marquis,andJulianJ..etenepeFIGAVentricularfibrillationdetectedinonepatient.Patientpressedthealarmbuttonasshown.valvehasshownthattheincidenceofarrhythmias,andinparticularof'serious'formsofventriculararrhythmia,maybehigherthanhasbeenwidelyrecognizedinthepast.Thecoexistenceofrhythmdisordersandmechanicaldysfunctionofthemitralvalveintheconditionhasbeenattributedtoamyocardialdisorder(Scampardonisetal.,1973;Gulottaetal.,1974)possiblysecondarytoaninitiallyabnormalmitralvalveapparatus(Nutteretal.,1975).Whetherornotthenon-specificSTandTwavechangesaretakenasevidenceofadisorderinthemyocardium,itispossiblethattheymayidentifyagroupofpatientswhoareathighriskfromarrhythmias.Insupportofthissuggestionarethe11reportedpatientswithballoondeformityofthemitralvalvewhodiedsuddenlyorhadventricularfibrillation,andforwhomelectrocardio-graphicdataareavailable:8hadtheauscultatory-electrocardiographicvariantofthesyndromeand3showedQTprolongation(HancockandCohn,1966;Barlowetal.,1968;Trentetal.,1970;Jeresaty,1973;MarshallandShappell,1974;Rakowskietal.,1975).Theseresultsandthepreviousreportsofsuddendeathsuggestthatitisimportanttodiagnosethiscondition.Unfortunatelytherecognitionofthesyndromeismadedifficultbythevariabilityofsymptomsandsigns.Oftenthepresentingcom-plaintsofvaguechestpain,lassitude,andlight-headednesssuggestafunctionaldiagnosis.Theauscultatoryfeaturesofamidsys

tolicclickorlatesystolicmurmurorbothmaynotbeconstantlypresentinanindividualanditisoftendifficulttoobtainanechocardiographicrecordoftheposteriorleafletofthemitralvalve.Leftventricularcine-angiographycarriesasmallbutdefiniterisk.Forthesereasonstheauscultatorysignsremainthesimplestandmostusefuldiagnosticfeature.Theexpenseandpersonnelinvolvedinlong-termambulatorymonitoringprohibititsuseinanun-definedpopulation,butourresultssuggestthatitispossiblethatthe12-leadelectrocardiogramcandefinea'highrisk'groupforwhomthistechniquecouldbeofvalue.Allen,Harris,andLeatham(1974)reportagoodprognosisfortheconditionofballoondeformityofthemitralvalvebuttheirexclusionofpatientswithelectrocardiographicabnormalitiesmayhavebiasedtheresultsinfavourofthoselesslikelytodeveloparrhythmias.Incontrast,ourexperienceconfirmsthatventriculararrhythmiasareapartofthesyn-dromeofballoondeformityofthemitralvalveandsuggeststhatallpatientswithanabnormalrestingelectrocardiogrambyvirtueofinferolateralSTandTwavechangesshouldbeinvestigatedbylong-termambulatorymonitoringevenintheabsenceofsymptoms.Inthe20patientsstudiedallepisodesofventriculartachycardiaandfibrillationwereseeninindividualswithsuchelectrocardiographicabnor-malities.Itisnotyetknownwhetherventricularar-rhythmiasassociatedwithballoondeformityofthemitralvalvearewelltoleratedoriftheycarrythelong-termriskofsuddendeath.Atpresentitisourpolicytotreatthosepatientswhoaresymptomaticbecauseoftheirarrhythmiasandthoseinwhomwehavedetectedventriculartachycardiaorfibrillation.Thechoiceofantiarrhythmicagentrequirescareasitislikelythatlong-termmedicationwillbeneces-sary.Somepatientswillrespondtobeta-adrenergicblockingdrugsbutothersareresistant(Goochetal.,1972),andinthissituation,theorallyactiveantiar-rhythmicdrug,mexiletine(Talbotetal.,1973;Campbelletal.,1973,1975)giventhreetimesdailyhasbeenwelltoleratedandeffectiveinabolishing ArrhythmiasinBMV1057ventriculartachycardiaandfibrillationinour4patientswiththeserhythmdisorders.Ambulatoryelectrocardiographicmonitoringhasprovedusefulinevaluatingtheeffectoftherapeuticinterventionandhasallowedustousetheminimaldrugdosageconsistentwitharrhtyhmiacontrol.Itisclearlyim-portanttoestablishwhetherlong-termanti-arrhythmictherapyinthesepatientscanreducetheriskofsuddenarrhythmicdeath.MexiletinewassuppliedbyBoehringerIngelheim,SouthernIndustrialEstate,Bracknell,Berkshire,England.ReferencesAllen,H.,Harris,A.,andLeatham,A.(1974).Significanceandprognosisofanisolatedlatesystolicmurmur:a9-to22-yearfollow-up.BritishHeartJournal,36,525.Barlow,J.B.,andBosman,C.K.(1966).Aneurysmalpro-trusionoftheposteriorleafletofthemitralvalve.Anauscultatory-electrocardiographicsyndrome.AmericanHeartJournal,71,166.Barlow,J.B.,Bosman,C.K.,Pocock,W.A.,andMarchand,P.(1968).Latesystolicmurmursandnon-ejection('mid-late')systolicclicks.Ananalysisof90patients.BritishHeartJournal,30,203.Brown,0.R.,Kloster,F.E.,andDeMots,H.(1975).Incidenceofmitralvalveprolapseintheasymptomaticnormal.Circulation,52,Suppl.II,77.Campbell,N.P.S.,Chaturvedi,N.C.,Kelly,J.G.,Strong,J.E.,Shanks,R.G.,andPantridge,J.F.(1973).Mexile-tine(Ko1173)inthemanagementofventriculardys-rhythmias.Lancet,2,404.Campbell,R.W.F.,Talbot,R.G.,Dolder,M.A.,Murray,A.,Prescott,L.F.,andJulian,D.G.(1975).Comparisonofprocainamideandmexiletineinthepreventionofventriculararrhythmiasafteracutemyocardialinfarction.Lancet,1,1257.Cuffer,andBarbillon(1887).Nouvellesrecherchessurlebruitdegalopcardiaque.ArchivesGeneralsdeMedecine,1,129and301DeMaria,A.N.,Amsterdam,E.A.,Vismara,L.A.,Markson,W.,Brocchini,R.,andMason,D.T.(1974).Thevariablespectrumofrhythmdisturbancesinthemitralvalveprolapsesyndrome

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