肥厚梗阻型心肌病酒精消融-fellow-course-20140827

肥厚型心肌病的介入治療

HypertrophicCardiomyopathy&InvasiveTherapies廣東省人民醫(yī)院董豪堅NomenclatureIHSS(idiopathichypertrophicsubaorticstenosis)HOCM(hypertrophicobstructivecardiomyopathy)HCM(hypertrophiccardiomyopathy)Massiveleftventricularhypertrophy,mainlyconfinedtotheseptum

HistopathologyshowingsignificantmyofiberdisarrayandinterstitialfibrosisCellResearch.2003;13(1):10.PathologyEchocardiographyinHCMLVOutflowObstructioninHCMPhysiologicalconsequencesofobstructionElevatedintraventricularpressuresProlongationofventricularrelaxationIncreasedmyocardialwallstressIncreasedoxygendemandDecreaseinforwardcardiacoutput

SAMConditionsthatincreaseintraventriculargradientinHOCMDefinitionofHCMAdiseasestatecharacterizedbyunexplainedLVhypertrophyassociatedwithnondilatedventricularchambersintheabsenceofanothercardiacorsystemicdiseasethatitselfwouldbecapableofproducingthemagnitudeofhypertrophyevidentinagivenpatientMaximalLVwallthickness≥15mm,particularlyinthepresenceofothercompellinginformation(e.g.,familyhistoryofHCM),basedonechocardiography.Wallthicknessof13to14mmconsideredborderlineCaveat:patientswhoaregenotypepositivemaybephenotypicallynegativewithoutoverthypertrophy.ClinicalPresentationDyspneaonexertion(90%),orthopneaAngina(70-80%)Syncope(20%),Presyncope(50%)outflowobstructionworsenswithincreasedcontractilityduringexertionalactivitiesSuddencardiacdeathHCMismostcommoncauseofSCDinyoungpeople,includingathletesMaronMSetal.NEJM.2003;348:295.DifferentialdiagnosisFabrydiseaseDanondiseaseNoonansyndromePompediseaseHypertensiveheartdiseaseAthlete’sheartDiseaseswithLVhypertrophyDifferentialdiagnosishypertensiveheartdiseaseandthephysiologicremodelingassociatedwithathletictraining(“athlete’sheart”)ThelikelihoodofHCMcanbedeterminedbyidentificationofadiagnosticsarcomeremutationorinferredbymarkedLVthickness>25mmand/orLVOTobstructionwithsystolicanteriormotion(SAM)andmitral-septalcontact.TheimportantdistinctionbetweenpathologicLVhypertrophy(i.e.,HCM)andphysiologicLVhypertrophy(i.e.,athlete’sheart)isimpactedbytherecognitionthatathleticconditioningcanproduceLV,rightventricular,andleftatrial(LA)chamberenlargement,ventricularseptalthickening,andevenaorticenlargement(26)butisoftenresolvedbynoninvasivemarkers,includingsarcomericmutationsorfamilyhistoryofHCM,LVcavitydimension(ifenlarged,favoringathlete’sheart),diastolicfunction,patternofLVhypertrophy(ifunusuallocationornoncontiguous,favoringHCM),orshortdeconditioningperiodsinwhichadecreaseinwallthicknesswouldfavorathlete’sheart(22–26).metabolicorinfiltrativestoragedisorderswithLVhypertrophyinbabies,olderchildren,andyoungadultscanmimicclinicallydiagnosedHCMmitochondrialdisease(27,28),Fabrydisease(29),orstoragediseases-2-regulatorysubunitoftheadenosinemonophosphate(AMP)-activatedproteinkinase(PRKAG2)ortheX-linkedlysosome-associatedmembraneproteingene(LAMP2;Danondisease)Noonansyndrome(withcraniofacialandcongenitalheartmalformations,aswellasLVhypertrophyfrommutationsingenesoftheRAS[RatSarcoma]pathway[14,15]),distinctcardiomyopathiessuchasPompedisease(alsoaglycogenstoragediseaseII,withskeletalmuscleweaknessandcardiomyopathybecauseofdeficiencyof1,4glycosidase[acidmaltase])hypertrophiccardiomyopathyandtheacronymHCMremainaclinicaldiagnosislimitedtothosepatientsinwhom1)overtdiseaseexpression(withLVhypertrophy)appearstobeconfinedtotheheartand2)thedefinitivemutationiseitheroneofageneencodingproteinsofthecardiacsarcomereoralternativelywhenthegenotypeisunresolvedusingcurrentgenetictesting.ComplicationsAbsenceofComplicationsSCDduetounpredictableventriculartachyarrhythmias,mostcommonlyinyoungasymptomaticpatients<35yearsofage(includingcompetitiveathletes)Heartfailurecharacterizedbyexertionaldyspnea(withorwithoutchestpain)thatmaybeprogressivedespitepreservedsystolicfunctionandsinusrhythm,orinasmallproportionofpatients,heartfailuremayprogresstotheendstagewithLVremodelingandsystolicdysfunctioncausedbyextensivemyocardialscarringAF,eitherparoxysmalorchronic,alsoassociatedwithvariousdegreesofheartfailure(60)andanincreasedriskofsystemicthromboembolismandbothfatalandnonfatalstroke.ComplicationsSuddenCardiacDeathinHCMMostfrequentinyoungadults<30-35yearsoldPrimaryVF/VTTendtodieduringorjustfollowingvigorousphysicalactivityOftenis1stclinicalmanifestationofdiseaseHCMismostcommoncauseofSCDamongyoungcompetitiveathletesJAmCollCardiol.2003;42(9):1693.ManagementofHCMAsymptomaticpatientsPharmacologicSymptomaticpatientsPharmacologicInvasiveSeptumreductionSurgicalseptalmyectomyAlcoholseptalablationPacemakerDDDICDHearttransplatationAsymptomaticPatientsLow-intensityaerobicexerciseBetablockade&calciumchannelblockersAvoidpurevasodilatorsorhigh-dosediureticsSymptomaticPatientsBeta-blockingdrugstotitratethedosetoarestingheartrateof<60to65bpmVerapamiltherapystartinginlowdosesandtitratingupto480mg/dDisopyramidewithB/VinobstructiveptsOraldiureticswithB/Vinnon-obstructiveptsSymptomaticPatientsInvasiveTherapiesSeptalreductiontherapyshouldbeperformedonlybyexperiencedoperators*inthecontextofacomprehensiveHCMclinicalprogramandonlyforthetreatmentofeligiblepatientswithseveredrug-refractorysymptomsandLVOTobstruction.?*Experiencedoperatorsaredefinedasanindividualoperatorwithacumulativecasevolumeofatleast20proceduresoranindividualoperatorwhoisworkinginadedicatedHCMprogramwithacumulativetotalofatleast50procedures(Section)?Eligiblepatientsaredefinedbyallofthefollowing:a.Clinical:Severedyspneaorchestpain(usuallyNYHAfunctionalclassesIIIorIV)oroccasionallyotherexertionalsymptoms(suchassyncopeornearsyncope)thatinterferewitheverydayactivityorqualityoflifedespiteoptimalmedicaltherapy.b.Hemodynamic:DynamicLVOTgradientatrestorwithphysiologicprovocation50mmHgassociatedwithseptalhypertrophyandSAMofthemitralvalve.c.Anatomic:Targetedanteriorseptalthicknesssufficienttoperformtheproceduresafelyandeffectivelyinthejudgmentoftheindividualoperator.IIIaIIbIIIInvasiveTherapiesConsultationwithcentersexperiencedinperformingbothsurgicalseptalmyectomyandalcoholseptalablationisreasonablewhendiscussingtreatmentoptionsforeligiblepatientswithHCMwithseveredrug-refractorysymptomsandLVOTobstruction.Surgicalseptalmyectomy,whenperformedinexperiencedcenters,canbebeneficialandisthefirstconsiderationforthemajorityofeligiblepatientswithHCMwithseveredrug-refractorysymptomsandLVOTobstruction.Surgicalseptalmyectomy,whenperformedatexperiencedcenters,canbebeneficialinsymptomaticchildrenwithHCMandsevererestingobstruction(>50mmHg)forwhomstandardmedicaltherapyhasfailed.IIIaIIbIIIIIIaIIbIIIBIIIaIIbIIISurgicalSeptalMyectomyNishimuraRAetal.NEJM.2004.350(13):1320.SurgicalmyectomyActuarialsurvivalwas99%,98%,and95%at1,5,and10years,respectively(whenconsideringHCM-relatedmortality).SCDorappropriateICDdischargeaftermyectomyis0.9%.Nonetheless,surgicalmyectomydoesnoteliminatetheneedtoassesseachpatient’sriskforSCDandtoconsiderplacementofanICDinthosewithasignificantriskburdenEffectofSeptalMtectomyComplicationsafterMyectomycompleteheartblockisapproximately2%withmyectomy(higherinpatientswithpreexistingrightbundle-branchblock),butinmyectomypatientswhohavehadpreviousalcoholseptalablation,riskismuchhigher(50%to85%)Iatrogenicventricularseptaldefectoccursin1%ofpatients.aorticvalveormitralvalveinjuryis1%InvasiveTherapiesWhensurgeryiscontraindicatedortheriskisconsideredunacceptablebecauseofseriouscomorbiditiesoradvancedage,alcoholseptalablation,whenperformedinexperiencedcenters,canbebeneficialineligibleadultpatientswithHCMwithLVOTobstructionandseveredrug-refractorysymptoms(usuallyNYHAfunctionalclassesIIIorIV).Alcoholseptalablation,whenperformedinexperiencedcenters,maybeconsideredasanalternativetosurgicalmyectomyforeligibleadultpatientswithHCMwithseveredrug-refractorysymptomsandLVOTobstructionwhen,afterabalancedandthoroughdiscussion,thepatientexpressesapreferenceforseptalablation.TheeffectivenessofalcoholseptalablationisuncertaininpatientswithHCMwithmarked(i.e.,>30mm)septalhypertrophy,andthereforetheprocedureisgenerallydiscouragedinsuchpatients.IIIaIIbIIIBIIIaIIbIIIBIIIaIIbIIIAlcoholSeptalAblationBraunwald.AtlasofHeartDiseases:Cardiomyopathies,Myocarditis,andPericardialDisease.1998.1995-AlcoholSeptalAblationBeforeAfterLimitationsThelikelihoodofimplantationofapermanentpacemakeris4-to5-foldhigherafterseptalablationthanafterseptalmyectomyClinicalandhemodynamicbenefitmaybedelayedforupto3monthsafterseptalablation.Furthermore,patientswithmassiveseptalthicknessapproachingorexceeding30mmmayexperiencelittleornobenefitfromseptalablation.ComplicationsTemporarycompleteatrioventricularblockoccursduringtheprocedure50%Persistentcompleteheartblockpromptingimplantationofapermanentpacemakeroccursin10%to20%ofpatientsbasedontheavailabledataApproximately5%ofpatientshavesustainedventriculartachyarrhythmiasduringhospitalization.Thein-hospitalmortalityrateisupto2%.ComplicationsoccurrenceofsustainedventriculararrhythmiasandSCDfollowingseptalablationinabout3%to10%ofpatientsbothwithorwithoutriskfactorsforSCD.InvasiveTherapiesSeptalreductiontherapyshouldnotbedoneforadultpatientswithHCMwhoareasymptomaticwithnormalexercisetoleranceorwhosesymptomsarecontrolledorminimizedonoptimalmedicaltherapy.SeptalreductiontherapyshouldnotbedoneunlessperformedaspartofaprogramdedicatedtothelongitudinalandmultidisciplinarycareofpatientswithHCM.IIIaIIbIIIIIIaIIbIIIHarmHarmInvasiveTherapiesMitralvalvereplacementforreliefofLVOTobstructionshouldnotbeperformedinpatientswithHCMinwhomseptalreductiontherapyisanoption.AlcoholseptalablationshouldnotbedoneinpatientswithHCMwithconcomitantdiseasethatindependentlywarrantssurgicalcorrection(e.g.,CABGforCAD,mitralvalverepairforrupturedchordae)inwhomsurgicalmyectomycanbeperformedaspartoftheoperation.AlcoholseptalablationshouldnotbedoneinpatientswithHCMwhoare<21yearsofageandisdiscouragedinadults<40yearsofageifmyectomyisaviableoption.IIIaIIbIIIIIIaIIbIIIIIIaIIbIIIHarmHarmHarmPacingInpatientswithHCMwhohavehadadual-chamberdeviceimplantedfornon-HCMindications,itisreasonabletoconsideratrialofdual-chamberatrial-ventricularpacing(fromtherightventricularapex)forthereliefofsymptomsattributabletoLVOTobstruction.PermanentpacingmaybeconsideredinmedicallyrefractorysymptomaticpatientswithobstructiveHCMwhoaresuboptimalcandidatesforseptalreductiontherapy.IIIaIIbIIIBIIIaIIbIIIBDualchamberpacemakerTheoverallreductioninoutflowtractgradientwasmodest(25%to40%)withsubstantialvariationamongindividualpatients.Aconsistentimprovementinsymptomswithadecreaseingradientandobjectiveimprovementinexercisedurationisseenin50%ofpatients.Theoverallsuccessrateintermsofsymptomreliefandgradientreductionissignificantlylowerthanthatseeninpatientswhoundergoseptalmyectomy.TherapeuticStrategiesNishimuraetal.NEJM.2004.350(13):1323.PacingPermanentpacemakerimplantationforthepurposeofreducinggradientshouldnotbeperformedinpatientswithHCMwhoareasymptomaticorwhosesymptomsaremedicallycontrolled.Permanentpacemakerimplantationshouldnotbeperformedasafirst-linetherapytorelievesymptomsinmedicallyrefractorysymptomaticpatientswithHCMandLVOTobstructionwhoarecandidatesforseptalreduction.IIIaIIbIIIIIIaIIbIIIBNoBenefitNoBenefitICDCurrentSCDriskstratificationdoesnotidentifyallpatientsatriskforventriculararrhythmiasandSCDSCDRiskStratificationAllpatientswithHCMshouldundergocomprehensiveSCDriskstratificationatinitialevaluationtodeterminethepresenceofthefollowing:a.Apersonalhistoryforventricularfibrillation,sustainedVT,orSCDevents,includingappropriateICDtherapyforventriculartachyarrhythmias.?b.AfamilyhistoryforSCDevents,includingappropriateICDtherapyforventriculartachyarrhythmias.?c.Unexplainedsyncope.d.DocumentedNSVTdefinedas≥3beatsat≥120bpmonambulatory(Holter)ECG.e.MaximalLVwallthickness≥30mm.?AppropriateICDdischargeisdefinedasICDtherapytriggeredbyVTorventricularfibrillation,documentedbystoredintracardiacelectrogramorcycle-lengthdata,inconjunctionwiththepatient’ssymptomsimmediatelybeforeandafterdevicedischarge.IIIaIIbIIIBICDSCDRiskStratificationItisreasonabletoassessbloodpressureresponseduringexerciseaspartofSCDriskstratificationinpatientswithHCM.SCDriskstratificationisreasonableonaperiodicbasis(every12to24months)forpatientswithHCMwhohavenotundergoneICDimplantationbutwouldotherwisebeeligibleintheeventthatriskfactorsareidentified(12to24months).IIIaIIbIIIBIIIaIIbIIISCDRiskStratificationTheusefulnessofthefollowingpotentialSCDriskmodifiersisunclearbutmightbeconsideredinselectedpatientswithHCMforwhomriskremainsborderlineafterdocumentationofconventionalriskfactors:CMRimagingwithLGEb.Doubleandcompoundmutations(i.e.,>1)c.MarkedLVOTobstructionIIIaIIbIIIIIIaIIbIIIIIIaIIbIIIBSCDRiskStratificationInvasiveelectrophysiologictestingasroutineSCDriskstratificationforpatientswithHCMshouldnotbeperformed.IIIaIIbIIIHarmSelectionofPatientsforICDsThedecisiontoplaceanICDinpatientswithHCMshouldincludeapplicationofindividualclinicaljudgment,aswellasathoroughdiscussionofthestrengthofevidence,benefits,andriskstoallowtheinformedpatient’sactiveparticipationindecisionmaking(Figure4).ICDplacementisrecommendedforpatientswithHCMwithpriordocumentedcardiacarrest,ventricularfibrillation,orhemodynamicallysignificantVT.IIIaIIbIIIIIIaIIbIIIBSelectionofPatientsforICDsItisreasonabletorecommendanICDforpatientswithHCMwith:SuddendeathpresumablycausedbyHCMin≥first-degreerelatives.AmaximumLVwallthickness≥30mm.Oneormorerecent,unexplainedsyncopalepisodesAnICDcanbeusefulinselectpatientswithNSVT(particularlythose<30yearsofage)inthepresenceofotherSCDriskfactorsormodifiers?.AnICDcanbeusefulinselectpatientswithHCMwithanabnormalbloodpressureresponsewithexerciseinthepresenceofotherSCDriskfactorsormodifiers?.?SCDriskmodifiesarediscussedinSectionofthefulltextguideline.IIIaIIbIIIIIIaIIbIIIIIIaIIbIIISelectionofPatientsforICDsItisreasonabletorecommendanICDforhigh-riskchildrenwithHCM,basedonunexplainedsyncope,massiveLVhypertrophy,orfamilyhistoryofSCD,