肥厚性心肌病的器械治疗.ppt

肥厚性心肌病的器械治疗,阜外心血管病医院滕思勇,内容提要,HCM的基本特征HCM的ICD治疗进展HCM的DDD治疗进展,肥厚性心肌病（HCM）是一种复杂的、相对常见的遗传性心脏疾病。经过40年的严密调查和注册研究，发现HCM是所有年龄段的患者致残和死亡的重要原因。由于临床表现、自然史和预后的显著差异，对于心血管专家来说，HCM的治疗依然存在许多争议。,基本特征（1）,HCM年死亡率约为1.4%，其中猝死0.7%，心衰0.5%，中风0.2%。猝死可为HCM的首发表现。猝死也可发生在疾病平稳期虽然大部分猝死发生于青少年，但猝死并不局限于青少年，猝死会持续在所有年龄组中发生,基本特征（2）,猝死的主要危险因素:持续室速家族性猝死史恶性突变类型（如：-MHC基因Arg403-Gln的家系）晕厥史反复发作的非持续性室速左室肥厚（室壁30mm）,基本特征（3）,HCM的基本特征HCM的ICD治疗进展HCM的DDD治疗进展,内容提要,心脏性猝死是肥厚性心肌病患者死亡的常见原因。大约有10的肥厚性心肌病患者被认为有心脏性猝死的危险性。肥厚性心肌病猝死高危患者：(1)猝死幸存者；(2)自发持续性心动过速；(3)猝死家族史；(4)不明原因晕厥史；(5)运动后血压反应异常，收缩压不升高反而降低者；(6)左室壁或室间隔厚度30mm；流出道压力阶差50mmHg。50以上的肥厚性心肌病高危患者10年内将发生心脏性猝死。肥厚性心肌病是35岁以下运动员心脏性猝死的最主要原因。,心脏性猝死的一级和二级预防的多个前瞻性多中心随机临床试验的结果（AVID、CASH、MADIT、MADIT-、MUSTT、SCD-HeFT、COMPANION）已经充分证明ICD是肯定的效果最佳和唯一可靠的预防心脏性猝死的选择，能够有效降低心脏性猝死高危患者的病死率。,2019年ACC/AHA/ESC室性心律失常治疗和心脏性猝死预防指南把SCD一级和二级预防的建议合并，对SCD的一级预防提到更加显著位置，HCM患者出现以下情况为植入ICD类适应症：1）自发持续性VT、无论血液动力学是否稳定。2）有晕厥史、电生理检查明确诱发有血液动力学不稳定的持续性VT或VF。肥厚型心肌病患者有一项以上主要SCD危险因素，包括心脏骤停史、自发持续性VT、自发非持续性VT、SCD家族史、不明原因晕厥史、左室厚度30mm、运动时血压反应异常，建议植入ICD。-,心脏性猝死（SCD）的发病年龄SinglemostfrequentcauseofSCDinyouncompetitiveathletesintheU.S.ARVC,arrhythmogenicrightventricularcardiomyopathy;AS,aorticvalvestenosis;CAD,coronaryarterydisease;CHD,*Regardedaspossible(butnotdefinitive)evidenceforhypertrophiccardiomyopathyatautopsywithmildlyincreasedLVwallthickness(1519mm)andheartweight(44776g).Includesmostcommonly,Kawasakidisease,sicklecelltraitandsarcoid.,MaronBJ,Circulation2009;119:10851092,HCM心律失常的发生具有不可预测性Timeintervalbetweenimplantationofimplantablecardioverter-defibrillator(ICD)andfirstappropriateintervention.Variabletimedelayafterimplantationisevident,withsomedevicedischargesoccurringrelativelyearlyandothers510yearslater(bluebars).HourlydistributionofappropriateICDinterventionsoverthe24-hdayfor126ventriculartachycardia/ventricularfibrillationeventsin63patientswithHCM.,心脏猝死的危险分层PyramidprofilecurrentlyusedtoidentifypatientsathighestriskforSCDwhoarepotentialcandidatesforanimplantablecardioverter-defibrillator(ICD).BP,bloodpressure;LV,leftventricular;LVH,leftventricularhypertrophy;NSVT,nonsustainedventriculartachycardia;VT,ventriculartachycardia.*Followingalcoholseptalablation,sustainedVThasbeenreportedinasignificantminorityofpatients(10%)overtheshortterm.DirectrelationbetweenmagnitudeofLVhypertrophy(maximummaxwallthicknessbyechocardiography)andSCDrisk.Mildhypertrophygenerallyconveyslowerriskandextremehypertrophy(wallthickness30mm)isassociatedwiththehighestrisk.,HCM合并持续性室性心动过速的影像和病理特征(A)Massivehypertrophywithventricularseptal(VS)thicknessof55mm.(B)Akineticthin-walledLVapicalaneurysmwithmidcavitymuscularapposition.D,distal(cavity);LA,leftatrium;P,proximal(cavity);(B1)Contrast-cardiovascularmagneticresonanceshowsdelayedenhancement(ie,scar)involvingthethinaneurysmrim(arrowheads)andcontiguousmyocardium(largearrow);smallapicalthrombusisevident(smallarrow).(C)Largetransmuralventricularseptalscar(arrow)resultingfromalcoholablation(arrow)(reproducedwithpermission).(D)“End-stage”heartshowingextensiveandtransmuralseptalscarring,extendingintotheanteriorwall(arrowheads).,HCM心脏核磁显像延迟提示致心律失常基质的存在Ventriculartachyarrhythmiasonambulatory(Holter)ECG,includingnonsustainedVT(NSVT),aresignificantlymorefrequentinthepresenceofDE.PVC,prematureventricularcontraction;SVT,supraventriculartachycardia.A21-year-oldmanwithhypertrophiccardiomyopathy(HCM)andseptalscarringwhosurvivedanepisodeofventricularfibrillation(VF)becauseofICDintervention(A).Contrast-enhancedCMRimageshowingtransmuralDEwithhighsignalintensityoccupyingsubstantialproportionofseptum(arrows).(B)Withoutcontrast,showingmoderateasymmetrichypertrophyoftheventricularseptum(VS;21mm).(C)IntracardiacelectrogramshowingVFinterruptedbydefibrillationshock(arrow).,MaronBJ,AmJCardiol2019;,肌节组成及突变示意图Schematicrepresentationofthecomponentsofahalfsarcomere.Componentsinwhichcardiomyopathy-associatedmutationsarefoundareunderlined.,MHC突变Arg403-Gln猝死患者的心肌细胞形态特征A,Grossheartspecimenfroma13-year-oldmalecompetitiveathleteshowingisproportionatethickeningoftheventricularseptum(VS)withrespecttotheleftventricular(LV)freewall(RVindicatesrightventricularwall);B,markeddisarrayofcardiacmusclecellsintheisproportionatelythickenedVSwithadjacenthypertrophiedcellsarrangedinachaoticpatternatobliqueandperpendicularangles,formingthetypicaldisorganizedarchitectureofHCM;C,LVmyocardiumshowingseveralabnormalintramuralcoronaryarterieswithmarkedlythickenedwallsandnarrowedlumen,dispersedwithinreplacementfibrosis,Maron,B.J.JAMA2019;287:1308-1320,Copyrightrestrictionsmayapply.,糖原储积性疾病表现为HCM和WPW,LAMP2型心肌病特征(A)Froma14-year-oldboywithsuddencardiacdeathandaseptalthicknessof65mm(heartweight1,425g).(B)Clustersofmyocyteswithvacuolatedsarcoplasm(stainedred)embeddedinanareaofscar(stainedblue;Massontrichrome).(C)Disorganizedarrangementofmyocytesmosttypicalofsarcomerichypertrophiccardiomyopathy.(D)Intracardiacelectrogram.Theimplantablecardioverter-defibrillatorelicited5defibrillationshocksthatfailedtointerruptventricularfibrillation(280beats/min).,心脏性猝死的家族史(Top)Intracardiacelectrogramobtainedat1:20amwhileasleep5yearsafterplacementofanimplantablecardioverter-defibrillator(ICD).Froma35-year-oldmanwithhypertrophiccardiomyopathywhoreceivedprophylacticICDbecauseoffamilyhistoryofSCDandmarkedventricularseptalthickness(31mm).(A)Ventriculartachycardia(VT)beginsabruptly,at200beats/min.(B)DefibrillatorsensesVTandcharges.(C)VTdeterioratesintoventricularfibrillation(VF)(D)defibrillatorissues20-Jshock(arrow),restoringsinusrhythmimmediately.Virtuallyidenticalsequenceoccurred9yearslaterduringsleep;patientisnow52yearsoldandasymptomatic.(Reproducedwithpermission.)(Bottom)Flow-chartofICD-relatedoutcomein506highriskHCMpatientsfromaninternational,multicenterICDregistry,HCM主要危险因素与预后(Top)Appropriateimplantablecardioverter-defibrillator(ICD)interventionrates(per100person-years)arenotsignificantlydifferentwithrespectto1,2or3riskfactors.(Bottom)Cumulativeratesforfirstappropriatedeviceinterventioninpatientswith1,2or3riskfactors.,MaronBJ,Circulation2009;119:10851092,HCM的基本特征HCM的ICD治疗进展HCM的DDD治疗进展,内容提要,肥厚型心肌病伴窦房结功能异常或房室传导阻滞需植入永久性起搏器者。（伴或不伴左室流出道梗阻）药物治疗无效、静息或应激时流出道梗阻的肥厚型心肌病患者，不推荐植入永久性起搏器，为IIb类（证据级别A级）推荐。（强调个体化治疗）有SCD风险（主要SCD风险：心脏骤停史，自发持续性VT，自发非持续性VT，SCD家族史，晕厥，左室厚度30mm，运动时血压反应异常；可能的SCD风险：房颤、心肌缺血、左室流出道梗阻、突变高危、强竞技性体力活动时）应植入DDD-ICD。无症状或有症状但药物可控制、没有左室流出道梗阻证据的肥厚型心肌病患者禁止植入永久性起搏器。,HCM起搏器治疗指南（2019年AHA/HRS),HCM梗阻型的血流动力学异常,LV流出道,二尖瓣前叶,增厚的间隔,舒张期,收缩期,1101例HCM随访6.36.2年。结论：休息下LVOT压差30mmHg是HOCM死亡、心衰的独立危险因素。,MartinSNEnglJMed2019;348:295-303,DDD起搏器植入后,舒张期,收缩期,起搏导线,经导管测流出道压差,起搏前,起搏治疗后,LV收缩压,动脉压,DDD起搏器植入后,多中心,随机,双盲,3个月交叉试验药物治疗无效的83例患者，平均年龄53岁,随访1年。比较主动起搏（DDD，最适AV间期）对非主动起搏（AAI起搏，30次/分）的疗效。84%患者生活质量和症状改善.,PIC研究,药物治疗无效的48例患者,LVOT压差50mmHg随机双盲3个月交叉试验在双盲试验阶段，患者生活质量和症状改善,DDD起搏和AAI起搏组无差别。6个月非盲试验阶段患者生活质量和症状在DDD组明显改善。安慰剂效应？,M-PATHY研究,DDD起搏治疗梗阻型HCM的适应症,LVOT压差静息30mmHg激发后50mmHg室间隔基部而非心尖部、心室中部的心肌肥厚无慢性或频繁发作的阵发性房颤,DDD起搏治疗梗阻性HCM的技术参数,心室起搏位点：起搏电极必须置于真正的右室尖AV间期（25msec125msec)：必须短于窦性心律的PR间期AV间期程控期间监测左室和主动脉压力，必要时行激发试验尽量保证窦性心律,最佳AV间期的程控,最佳AVD调整原则：有效的AVD短于自身PR间期保持适当房室同步，维持左室的前负荷Jeanrenaud观察13例不同AVD的血流动力学变化,Lancet,1992,339:1318-1323,DDD起搏治疗不能改善LVOT梗阻的原因,起搏器参数程控不当心室激动提前不适当（AV延迟值过长）干扰左房排空（AV延迟值过短）其他相关的异常心室电极位于室间隔的近端或高位异位乳头肌阻塞LOV