心肌疾病.ppt

Cardiomyopathyandmyocarditis,TangQizhuTel:88041911-6604Email:qizhutyahoo,RenminHospitalofWuhanUniversity,Difinition,CardiomyopathyisaseriousdiseaseinwhichtheheartmusclebecomesinflamedanddoesntworkaswellasitshouldItcanbeclassifiedasprimaryorsecondary.Primarycardiomyopathycantbeattributedtoaspecificcause,suchashighbloodpressure,heartvalvedisease,arterydiseasesorcongenitalheartdefects.Secondarycardiomyopathyisduetospecificcauses,Clasification(2019WHO/ISFC),Therearefourmaintypes:DilatedcardiomyopathyHypertrophiccardiomyopathyRestrictivecardiomyopathyArrhythmogenicrightventriculardysplasia,Dilatedcardiomyopathy,Thistypeofcardiomyopathyischaracterizedbyadilatationandimpairedcontractionoftheleftorbothventricles,Importances,Theincidenceandprevalenceofcardiomyopathyappearstobeincreasing.Thereportedincidenceis0.013-0.084%CardiomyopathyisanimportantcauseofmorbidityandmortalityamongtheworldsagingpopulationSex:men/womenis2.5/1Age:Allagegroupsareaffected.However,studiessuggestthat50%ofpatientswithnewonsetofdiseaseareyoungerthan2years,CAUSES,Cardiomyopathieshavemanycauses,includingnutritionaldeficiencies,depositsintheheartmuscleassociatedwithmedicalconditions,anemia,stress,viralinfections,alcoholism,coronaryarterydisease,andothersThereisnoidentifiablecause,althoughitissuspectedtobeanendstageofmyocarditis,SYMPTOMS,Symptomsoftendevelopgraduallyandusuallyincludesymptomsofrightheartfailureand/orleftheartfailureBecausethebodycompensatesfordilatedcardiomyopathy,thediseasemayhavenosymptomsinitially.Astheconditionworsens,theheartmayperformnormallywhenapersonisresting,butmaycausesymptomsduringperiodsofexerciseorpsychologicalstress,symptoms,fatigueshortnessofbreathonexertion,orthopnea(breathingdifficultywhenlyingdown),wakingupatnightshortofbreathswellingoftheanklesexcessiveurinationatnightirregularheartbeat(palpitations-afeelingofracingorskippingoftheheart)decreasedurineoutput(maynotincludeatnight),DIAGNOSIS,AphysiciandiagnosesdilatedcardiomyopathyafteraphysicalexaminationcheckforshortnessofbreathduringexerciseandweaknessThephysicianmayalsohearrales,orwetcrackles,throughastethoscope,indicatingfluidinthelungs,NeckandLungs,Jugularvenousdistention(asanestimateofcentralvenouspressure)HepatojugularrefluxLargecvwave(observedwithtricuspidregurgitation)Crackles(pulmonaryrales)Signsofpleuraleffusionmaybenoted,Inspectionandpalpation,Palpateforheaves,shiftedpointofmaximalimpulse,andcardiomegaly(broadanddisplacedpointofmaximalimpulse,rightventricularheave).Thenormalapicalimpulseshouldbeapproximatelythesizeofaquarterandshouldbelocatedinone(fourthorfifth)intercostalspace.Theapicalimpulseisnormallywithin10cmofthemidsternalline,Auscultation,Murmurs(withappropriatemaneuvers),tachycardia,S2atthebase(paradoxicalsplitting,prominentP2),S3,andS4maybenotedAnirregularlyirregularrhythm(atrialfibrillation)maybenotedGallopsarealmostalwayspresentinpersonswithDCM,Abdomenandothers,Percussionandpalpationofthelivermayrevealhepatomegalyduetoelevatedvenouspressure,infiltrativedisease,hepatojugularreflux,orascitesObserveforcardiaccachexia,peripheraledema,cyanosis,andclubbing,TESTSFORDETECTION,Toconfirmthediagnosis,thephysicianmayordertests,including:Bloodtests;Chestxray;Echocardiogram;Electrocardiography(ECG)Cardiaccatheterizationandangiography,X-ray,Thisverylargehearthasagloboidshapebecauseallofthechambersaredilated.Itfeltveryflabby,andthemyocardiumwaspoorlycontractile,DopplerEcho,Markeddilationoftheleftventriclewithglobalhypokinesiaisthehallmarkofthedisease.Leftventricularejectionfraction50%.Leftventricularwallsarethinandareasofdyskinesismaybeobserved.Theleftatriumisalsodilated.varyingdegreesofmitralregurgitation,Electrocardiogram,ECGchangesareusuallynonspecificLowvoltagecomplexesPresenceofQwavesandinversionofTwavesinleadsI,II,aVL,andV4throughV6(anterolateralinfarctionpattern)Significantarrhythmia,Myocardialbiopsy,Thenumberofbiopsyspecimenscollectedshouldbelimitedtotheminimumrequired(usually4-8)Myocytehypertrophyandfibrosiswithoutlymphocyticinfiltrate,TREATMENTS,Hospitalizationmayberequiredofpatientswhensymptomsofdilatedcardiomyopathyaresevere.Treatmentfordilatedcardiomyopathyisfocusedonreliefofsymptoms,justasisforothertypesofcardiomyopathy,andisessentiallythesameasthetreatmentofheartfailure,Medications,Medicationsmayincludedigitalis,Angiotensinconvertingenzyme(ACE)inhibitors,Anticoagulants,Beta-blockers,Calciumchannelblockers,Vasodilators,diuretics,nutritionalsupplements,orothercardiacmedications,SurgicalCare,Ahearttransplantmaybeconsideredifheartfunctionisverypoor,Dietandothers,Alow-saltdietmayberecommendedtopatientsandfluidintakemayberestrictedinsomecases.PhysicalactivitymayberestrictedassymptomsprogressSmokingandalcoholcessationrecommendationsmaybegiven,becausethesehabitsmakesymptomofdilatedcardiomyopathyworse,COMPLICATIONS,CongestiveheartfailureCardiacarrhythmias,includinglethalarrhythmiasPulmonary(lung)edemaTotalfailureofthehearttofunction(circulatorycollapse)Sideeffectsofmedications,includinglowbloodpressure(hypotension),lightheadedness,fainting,lupusreaction,headache,GIupset,anddigitalistoxicity,HYPERTROPHICCARDIOMYOPATHY,Hypertrophiccardiomyopathyisaprimarycardiacdiseasecharacterizedbyaninappropriatemyocardialhypertrophy,whichoccursintheabsenceofanobvioushemodynamicloadsuchasaorticstenosisorsystemichypertension,HISTORYANDOTHERNAMES,HypertrophicCardiomyopathywasfirstrecognisedinthelate1950s.TheconditionhasbeenknownbyanumberofnamesincludingHypertrophicObstructiveCardiomyopathy(HOCM),IdiopathicHypertrophicSub-aorticStenosis(IHSS)andMuscularSub-aorticStenosis.ThegeneraltermHypertrophicCardiomyopathy(HCM)isnowmostwidelyused,THECAUSE,ThecauseofHypertrophicCardiomyopathyisnotyetknown.Inthemajorityofcasestheconditionisinherited.Inothersthereiseithernoevidenceofinheritanceorthereisinsufficientinformationabouttheindividualsfamilytoassessinheritance.Inaffectedfamiliestheconditionusuallypassesfromonegenerationtothenextandgenerationsarenotskipped,AFamilyTree,NEWDISCOVERIES,Recentlyresearchhasidentifiedabnormalitiesinatleast6relatedgenesthatareimportantinthedevelopmentofheartmusclecells.Inapproximately50-60%offamilies,affectedindividualsarefoundtohaveamutationinthegeneformyosin,troponinT,alphatropomyosin,cardiacmyosinbindingprotein-C,ortheessentialandregulatorylightchains.Theseareimportantproteinsforthecontractionoftheheart,pathology,anexcessivethickeningofthemuscle.Abnormalmyocardialfiberarrangementwhichinterfereswiththeproperdiastolicsequenceofrelaxation.Abnormalmyocardialcalciumionhandlingresultinginhighcellularcalciumionconcentrationintheregionofmyofibrillarcontractileproteinsanddelayedre-uptakeofcalciumionbythesarcoplasmicreticulumresultinginprolongedrelaxation.Ischemia.Myocardialfibrosis,LEFTVENTRICULAROUTFLOWTRACT(LVOT)OBSTRUCTION,Approximately25%ofpatientswithhypertrophiccardiomyopathymanifestvariabledegreeofLVOTobstruction.Insome,theobstructionorgradientisabsentatrestbutthiscanbeprovokedbyexerciseorotherphysiologicorpharmacologicmeans,mechanismofLVOTobstructionAsymmetricSeptalHypertrophy,mechanismofLVOTobstructionAsymmetricSeptalHypertrophywithObstruction,ThemechanismofLVOTobstruction,ThethickupperseptumprotrudesintheLVOTreducingtheorificesize.BloodflowingthroughthisnarrowedLVOTcausesaVenturi(suction)effectwhichdrawstheanteriorleafletofthemitralvalvetowardstheseptum,furtherreducingtheLVOTorificesize.Simultaneously,asaresultofsystolicanteriormovementofthemitralvalve(SAM),thereisfunctionalmitralregurgitation,symptom,dyspnea:ShortnessofbreathExercisecapacitymaybelimitedbybreathlessnessandfatigueChestpainpainmayoccuratrestorduringsleepandmaypersistPalpitationSyncopy/dizzinessSuddendeath,PHYSICALEXAMINATION,Thesystolicmurmurischaracteristicallycrescendo-decrescendoandstartsalittleaftertheonsetofS1.Itisbestheardattheleftlowersternalborderandradiatestothebase,butunlikesystolicmurmurofvalvularaorticstenosis,itdoesnotradiatetothecarotids.,murmur,Theintensityofthemurmurcanbevariedbyvariousphysiologicandpharmacologicinterventions.Tthemurmur(andgradient)canbeintensifiedbydecreasingpreload(decreasevenousreturn)byabruptassumptionofuprightpostureorbyValsalvasmaneuver.Themurmurintensitycanbediminishedbyincreasingafterload(squatting),orbyincreasingpreload(legraisinginsupineposition)Themurmurofmitralregurgitationisprominentintheapicalregionandshowsthesamedirectionalchangesinresponsetotheinterventionsnotedabove,ElectrocardiogramorECG,Mainfeaturesincludeleftventricularhypertrophy(LVH)patternandthepresenceofprominentQwaveswhichresembleQwavesofmyocardialinfarction(pseudo-infarctionpattern).Ambulatoryelectrocardiogrammayrevealatrialandventriculararrhythmiasincludingnon-sustainedventriculartachycardia(NSVT),EchocardiogramorECHO,includehypertrophicwalls,ASH,andseptalwalltoposteriorwallratioof1.3:1.InpatientswithHOCM,thereispresenceofSAM.Leftventricularchamberisnormalorsmall;leftatriummaybeenlarged.ColorDopplerstudiesprovideadditionalhemodynamicinformation,CardiacCatheterisation,showsagradientbetweenthemainleftventricularchamberandthesubaorticchamberproximaltotheobstruction.Thispullbacktracingdifferentiatesobstructivehypertrophiccardiomyopathyfromaorticstenosis,Genetictesting,Genetictestingmayplayanimportantroleinhelpingdoctorsruleoutotherdiagnoses.Forexample,inpatientswhohaveasmallincreaseinwallthickness(liketrainedathleteswithventricularhypertrophy),andsomepatientswithconstanthighbloodpressurewhoarethoughttohaveHCM,TREATMENT,DrugTreatmentDrugtreatmentormedicationisprimarilygivenwhenapersonhassomeorallofthesymptomsdescribedearlierAlcoholSeptalAblationAcatheterizationisperformed,alcoholisinjectedintotheseptumthroughasmallcoronaryartery.ThiscausesacontrolledmyocardialinfarctionPacemakerdualchamberedpacingwouldrelieveobstruction,eliminatemostsymptomsandthinthewallsoftheheartSurgeryremovingasmallamountofmusclefromtheventricularwall,DrugTreatment,Beta-BlockersThesedrugsreduceheartrate,decreasemyocardialcontractility,prolongdiastolicfillingtimeandpossessanti-arrhythmicpropertiesespeciallyagainstexercise-inducedarrhythmiasCalciumAntagonistsAnti-ArrhythmicDrugsAnticoagulantsDiureticsAntibiotics,DualChamberPacemaker,ItisbelievedthatpacemakertherapyaltersthesequenceofcontractionwhichreducesLVOTgradientandimprovessymptomsLongtermbenefits,however,arequestionable,Restrictivecardiomyopathy,Itisadisorderoftheheartmuscleinwhichthewallsoftheventriclesbecomestiff,butnotnecessarilythickened,suchthattheyresistnormalfillingwithblood.Thereisnocure.Treatmentaimstoamelioratesymptomscausedbythebackupofbloodintothelungsandveinsoftheneckandliver,Symptoms,PatientswithRCMpresentwithsignsand/orsymptomsofheartfailuresuchasfatigue,shortnessofbreath,tissueswelling(oedema)andabdominalenlargementUptoathirdofpatientsmaypresentwithanemboliccomplication.Abnormalheartrhythmsandpalpitationsarecommonwhatevertheunderlyingcauseofthedisease,diagnosis,ThediagnosisofRCMisusuallybasedaphysicalexamination,anelectrocardiogram(ECG)andanechocardiogram.Magneticresonanceimaging(MRI)canprovideadditionalinformationaboutthestructureoftheheart.Insomepatientsaprecisediagnosismayrequirecatheterisationofthehearttomeasurepressuresandtoperformabiopsyoftheheartmuscle(removalandmicroscopicexaminationofaspecimen),whichmayenablethedoctortoidentifytheinfiltratingsubstance,Treatment,AstheonsetofsymptomsinRCMisoftenveryinsidious,thediagnosisofRCMmaybemadelateinthecourseofthedisease.ThereisnospecifictreatmentforRCM,andtheaimsofmedicaltherapyaretoimprovesymptomsofheartfailure,treatcardiacrhythmdisturbanceandtopreventthromboembolism.Pacemakersareindicatedforpatientswithslowheartratesorheartblock,Arrhythmogenicrightventriculardysplasia,ARVDisararetypeofcardiomyopathyinwhichthemuscletissueinthelower-rightchamberoftheheart(rightventricle)dies.ThedeadmuscletissueisreplacedbyfatPatientscandevelopdangerousarrhythmiasorevengointocardiacarrest,particularlywhenunderphysicaloremotionalstress.StudieshaveshownthatARVDisasignificantcauseofsuddencardiacdeathamongyoungathletes,Names,ArrhythmogenicRightVentricularCardiomyopathy(ARVC)Eventhoughthisdiseasehasbeenrecognisedonlyrecently,Thesearenotformalguidelinesforassessmentortreatment,Musclecellsbecomedisorganisedandarereplacedbyfibrousandfattytissue,Patchyinvolvementoftherightventriclemayleadtooneormoresitesofabnormalelectricalactivity,SYMPTOMSofARVC,Palpitationlight-headednessfatigueblackoutorcollapseor