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1、肥厚性心肌病的器械治疗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1.HCM的基本特征2.HCM的ICD 治疗进展3.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
2、基本特征（2）猝死的主要危险因素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）1.HCM的基本特征2.HCM的ICD 治疗进展3.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
3、。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。2008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
4、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,coronaryarterydis
5、ease;CHD,*Regardedaspossible(butnotdefinitive)evidenceforhypertrophiccardiomyopathyatautopsywithmildlyincreasedLVwallthickness(1519mm)andheartweight(44776g).Includesmostcommonly,Kawasakidisease,sicklecelltraitandsarcoid.MaronBJ,Circulation 2009;119: 10851092HCM心律失常的发生具有不可预测性Timeintervalbetweenimplan
6、tationofimplantablecardioverter-defibrillator(ICD)andfirstappropriateintervention.Variabletimedelayafterimplantationisevident,withsomedevicedischargesoccurringrelativelyearlyandothers510yearslater(bluebars).HourlydistributionofappropriateICDinterventionsoverthe24-hdayfor126ventriculartachycardia/ven
7、tricularfibrillationeventsin63patientswithHCM.心脏猝死的危险分层PyramidprofilecurrentlyusedtoidentifypatientsathighestriskforSCDwhoarepotentialcandidatesforanimplantablecardioverter-defibrillator(ICD).BP,bloodpressure;LV,leftventricular;LVH,leftventricularhypertrophy;NSVT,nonsustainedventriculartachycardia;V
8、T,ventriculartachycardia.*Followingalcoholseptalablation,sustainedVThasbeenreportedinasignificantminorityofpatients(10%)overtheshortterm.DirectrelationbetweenmagnitudeofLVhypertrophy(maximummaxwallthicknessbyechocardiography)andSCDrisk.Mildhypertrophygenerallyconveyslowerriskandextremehypertrophy(wa
9、llthickness30mm)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-cardiovascularmagneticresonanceshows
10、delayedenhancement(ie,scar)involvingthethinaneurysmrim(arrowheads)andcontiguousmyocardium(largearrow);smallapicalthrombusisevident(smallarrow).(C)Largetransmuralventricularseptalscar(arrow)resultingfromalcoholablation(arrow)(reproducedwithpermission).(D)“End-stage”heartshowingextensiveandtransmurals
11、eptalscarring,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-oldmanwithhypertro
12、phiccardiomyopathy(HCM)andseptalscarringwhosurvivedanepisodeofventricularfibrillation(VF)becauseofICDintervention(A).Contrast-enhancedCMRimageshowingtransmuralDEwithhighsignalintensityoccupyingsubstantialproportionofseptum(arrows).(B)Withoutcontrast,showingmoderateasymmetrichypertrophyoftheventricul
13、arseptum(VS;21mm).(C)IntracardiacelectrogramshowingVFinterruptedbydefibrillationshock(arrow).MaronBJ,Am J Cardiol 2008;肌节组成及突变示意图Schematicrepresentationofthecomponentsofahalfsarcomere.Componentsinwhichcardiomyopathy-associatedmutationsarefoundareunderlined.MHC突变突变Arg403-Gln猝死患者的猝死患者的心肌细胞形态特征心肌细胞形态特征
14、A, Gross heart specimen from a 13-year-old male competitive athlete showing isproportionate thickening of the ventricular septum (VS) with respect to the left ventricular (LV) free wall (RV indicates right ventricular wall); B, marked disarray of cardiac muscle cells in the isproportionately thicken
15、ed VS with adjacent hypertrophied cells arranged in a chaotic pattern at oblique and perpendicular angles, forming the typical disorganized architecture of HCM; C, LV myocardium showing several abnormal intramural coronary arteries with markedly thickened walls and narrowed lumen, dispersed within r
16、eplacement fibrosis Maron, B. J. JAMA 2002;287:1308-1320Copyrightrestrictionsmayapply.糖原储积性疾病表现为HCM和WPWLAMP2型心肌病特征 (A)Froma14-year-oldboywithsuddencardiacdeathandaseptalthicknessof65mm(heartweight1,425g).(B)Clustersofmyocyteswithvacuolatedsarcoplasm(stainedred)embeddedinanareaofscar(stainedblue;Mass
17、ontrichrome).(C)Disorganizedarrangementofmyocytesmosttypicalofsarcomerichypertrophiccardiomyopathy.(D)Intracardiacelectrogram.Theimplantablecardioverter-defibrillatorelicited5defibrillationshocksthatfailedtointerruptventricularfibrillation(280beats/min).心脏性猝死的家族史心脏性猝死的家族史 (Top)Intracardiacelectrogra
18、mobtainedat1:20amwhileasleep5yearsafterplacementofanimplantablecardioverter-defibrillator(ICD).Froma35-year-oldmanwithhypertrophiccardiomyopathywhoreceivedprophylacticICDbecauseoffamilyhistoryofSCDandmarkedventricularseptalthickness(31mm).(A)Ventriculartachycardia(VT)beginsabruptly,at200beats/min.(B
19、)DefibrillatorsensesVTandcharges.(C)VTdeterioratesintoventricularfibrillation(VF)(D)defibrillatorissues20-Jshock(arrow),restoringsinusrhythmimmediately.Virtuallyidenticalsequenceoccurred9yearslaterduringsleep;patientisnow52yearsoldandasymptomatic.(Reproducedwithpermission.)(Bottom)Flow-chartofICD-re
20、latedoutcomein506highriskHCMpatientsfromaninternational,multicenterICDregistryHCM主要危险因素与预后 (Top)Appropriateimplantablecardioverter-defibrillator(ICD)interventionrates(per100person-years)arenotsignificantlydifferentwithrespectto1,2or3riskfactors.(Bottom)Cumulativeratesforfirstappropriatedeviceinterve
21、ntioninpatientswith1,2or3riskfactors.MaronBJ,Circulation 2009;119: 108510921.HCM的基本特征2.HCM的ICD 治疗进展3.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
22、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（2008年AHA/HRS)HCM梗阻型的血流动力梗阻型的血流动力学异常学异常LV流出道二尖瓣前叶增厚的间隔舒张期收缩期1101例例HCM随访随访6.36.2年。结论：休息下年。结论：休息下LVOT压差压差30mmHg是是HOCM死亡、心衰的独立危险因素。死亡、心衰的独立危险因素。 Martin S NEnglJMed2003;348:295-303DDD起搏器植入后起搏器植入后舒张期收缩期起搏导线
23、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起搏器植入后起搏器植入后v多中心多中心, ,随机随机, ,双盲双盲,3,3个月交叉试验个月交叉试验药物治疗无效的药物治疗无效的8383例患者，平均年龄例患者，平均年龄5353岁岁, ,随访随访1 1年。比较主动起搏（年。比较主动起搏（DDDDDD，最适最适AVAV间期）间期）对对非主动起搏（非主动起搏（AAIAAI起搏，起搏，3030次次/ /分）的疗效。分）的疗效。84%84%患者生活质量和症状改善患者生活质量和症状改善. .PIC 研究研究药物治疗无效的药物治疗无效的4848例患者例患者,LVOT,LVOT压差压差 50mmHg50m
24、mHgv随机双盲随机双盲3 3个月交叉试验个月交叉试验在双盲试验阶段，患者生活质量和症状改在双盲试验阶段，患者生活质量和症状改善善,DDD,DDD起搏和起搏和AAIAAI起搏组无差别。起搏组无差别。v6 6个月个月非盲试验阶段非盲试验阶段患者生活质量和症状在患者生活质量和症状在DDDDDD组明显改善。组明显改善。 安慰剂效应？ M-PATHY 研究研究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（25ms
25、ec125msec)：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最佳AVAV间期的间期的程控程控最佳AVD调整原则：v有效的AVD短于自身PR间期v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AVD（ms）50100150压差(mmHg)615262Lancet,1992,339:1318-1323DDD起搏治疗不能改善起搏治疗不能改善LVOT梗阻的原因梗阻的原因起搏器参数程控不当 心室激动提前不适当心室激动提前不适当（AV延迟值过长）延迟值过长） 干扰左房排空（干扰左房排空（ AV延迟值过
26、短）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心室电极位于室间隔的近端或高位 异位乳头肌阻塞异位乳头肌阻塞LOVT 原发的二尖瓣反流原发的二尖瓣反流 左室腔中部梗阻左室腔中部梗阻 心房或心房或/和心室的快速心律失常和心室的快速心律失常 左室舒张功能障碍左室舒张功能障碍 药物治疗不当药物治疗不当 DDD起搏对起搏对HOCM的远期疗效仍争论，的远期疗效仍争论，没有证据表明没有证据表明DDD起搏可改善起搏可改善HOCM患患者的预后。者的预后。严格选择病例严格选择病例-HOCM-HOCM伴窦性心动过缓伴窦性心动过缓/ /不能耐受不能耐受BBBB者者程控最佳的程控最佳的AVDAVD药物控
27、制房颤药物控制房颤争论还将继续？争论还将继续？Simultaneous measurement of aortic and left ventricular pressures.Haruki S et al. Eur J Heart Fail 2010;12:94-97PublishedonbehalfoftheEuropeanSocietyofCardiology.Allrightsreserved.TheAuthor2009.Forpermissionspleaseemail:journals.permissionsoxfordjournals.org.Simultaneous measu
28、rement of aortic and left ventricular pressures. (A) The baseline pressure gradient was 127 mmHg. (B) DDD pacing at a rate of 80/min and an atrioventricular interval of 150 ms reduced the gradient to 27 mmHg. (C) After 5 min of disopyramide infusion (1.0 mg/kg) without DDD pacing, the gradient was 28 mmHg. (D) The combination of DDD pacing with an atrioventricular interval of 150 ms and disopyramide reduced the gradient to 4 mmH
