肥厚型梗阻性心肌病

肥厚型梗阻性心肌病化学消融术

—基础与临床

PercutaneousTransluminalSeptalMyocardialAblationofHypertrophic

ObstructiveCardiomyopathy第1页肥厚型心肌病(hypertrophiccardiomyopathy，HCM)是一种以心肌进行性肥厚、心室腔进行性缩小为特性病理特点：左心室血液充盈受阻，舒张期顺应性下降分型：梗阻型和非梗阻型两型人群发病率0.02%-0.2%概述第2页病死率1.4%（小朋友可高至6%)，其中猝死0.7%，心衰0.5%，中风0.2%Koga等报道日本肥厚型心肌病患者预后，5年随访年病死率为2.3%～2.9%，其中心尖肥厚型心肌病病死率仅为0.3%，约1/10患者逐渐发生左心室扩张和心力衰竭，最后类似扩张型心肌病被称为扩张期肥厚型心肌病概述第3页遗传性原因：是主要病因，大约50%～55%肥厚型心肌病患者有家族史，属于常染色体显性遗传病，肥厚型心肌病遗传学说已被公认钙调整紊乱概述—病因：不明第4页症状与体征心电图特性超声心动图特性：非对称性室间隔增厚>12mm，室间隔/左室后壁>1.3；室间隔厚度≥18mm并有二尖瓣收缩期前移，可辨别梗阻性与非梗阻性左心室造影：左心室流出道压力差及左室形态特性能够确立诊断核磁共振（MRI)：室壁增厚和流出道狭窄诊断

第5页第6页MechanicalimpedanceatthesubaorticlevelOwingtomitralvalvesystolicanteriormotion(SAM)andmid-systoliccontactwithventricularseptumMitralregurgitationduetoincompleteleafletcoaptationObstructiveMechanism第7页Leftventricular

outflowtractobstructionispresentatrestinapproximately

25%ofHCMpatientsInaddition,50%of

patientswithoutobstructionatrestcangeneratesignificant

intraventriculargradientswithexerciseObstructiveMechanism第8页

LVOTPGLAAOLVPosteriorwallSeptumSAMLVOTRV第9页SAMSign第10页LVOTPG第11页Howtoreducethehypertrophy?SeptalmyectomyPTSMA第12页药品外科切除经皮室间隔化学消融术（PTSMA)双强起搏器HCOM治疗第13页Intracoronaryethyl

alcohol

orphenolinjectionablatesaconitine-inducedventriculartachycardiaindogsChemicalablationbysubendocardial

injection

of

ethanol

viacatheter--preliminaryresultsinthe

pig

heart.Conclusion:

Intracoronary

ethanol

ablationisapromisingtechniqueforthetreatmentofarrhythmias.Significantarrhythmiasandadecreaseinleftventricularejectionfractionareassociatedwiththistechnique.AlcoholSeptalAblation(SAS),InoueHetal,JAmCollCardiol.1987Dec;10(6):1342-9WeismüllerPetal.EurHeartJ.1991Nov;12(11):1234-9第14页Intracoronary

ethanol

ablationin

swine:characterizationofmyocardialinjuryintargetandremotevascularbedsConclusion:Intracoronary

ethanol

ablationLesionsaregenerallyproducedwithinthedistributionofthetargeted

coronary

bed,butarealsofrequentlyassociatedwithrefluxtoasecondvasculardistribution.AlcoholSeptalAblation(SAS)HainesDE,etal

JCardiovascElectrophysiol.

1994Jan;5(1):41-9.理论基础第15页In1994,Sigwartwasthefirsttoreportasuccessfulnonsurgicalmyocardialreductionafterocclusionoftheseptalbranchusing96%alcohol.---Non-surgicalmyocardialreductionforhypertrophicobstructivecardiomyopathy------SASPTSMAaimsdirectlytoreducethehypertrophiedinterventricularseptumwithassociatedexpansionoftheLVOTandreductionofthesubaorticgradient.PercutaneousTransluminalSeptalMyocardialAblation(PTSMA)SigwartU.Lancet.1995;346:211e4第16页Myocardial

contrast

echocardiography:areproducibletechniqueof

myocardialopacifi-cationforidentifyingregional

perfusion

deficitsTargetingpercutaneoustransluminalseptal

ablation

forHOCMbyintraproceduralECHOmonitoring.AlcoholSeptalAblation(SAS)TeiCetal.Circulation.1983Mar;67(3):585-93.FaberL,etal.JAmSocEchocardiogr.2023Dec;13(12):1074-9.第17页The“1stSeptalUnit”(consistof:the1stseptalcoronarybranchanditsdependent(asymmetric)septalhypertrophy,atthelevelofmitral-septalcontact)inHOCMAlcoholSeptalAblation(SAS)Poloetal.TexHeartInstJ2023;34:336-46第18页AlcoholSeptalAblation(SAS)1stSeptalUnit1stSeptalUnit第19页SymptomaticHCMpatientswithaNYHAclassofatleastⅢdespiteofoptimaltherapy.Patientswithsubstantialside-effectsofmedicationhighoutflowtractgradients(≥50mmHgatrestor≥100mmHgunderstress)canbeverified.PTSMAIndications第20页Clinicalsymptoms:amaurosis,syncope,angina,externaldyspnea,etalEchoMRICAGIsotopemyocardialimagingHolterProvocativetestEvaluationbeforeSAS第21页Wallandseptalthickness:Base,middleandapexsegmentLVOTobstruction:SAM

signandpressuregradeECHO第22页LVOTPG(PG=219mmHg)第23页WallandseptalthicknessLVOTobstructionMRI第24页ProvocativeTest—LatentobstructionDifferentiateobstructivetype（RestingandLatenttype)ConfirmingindicationsforSASorSurgerytherapy

中华心血管病杂志

2023;36:412-414.

第25页CoronaryArteryAngiogram:LesionsorSeptalBranches

1septalbranch3septalbranches第26页NetStructureofSeptalBranch第27页ObstructivepositionandmeasuringpressureLVAngiogram流出道最窄处距积极脉瓣约25mm，压差80mmHG第28页LVOTPG80mmHGLVPAOP第29页术前常规安装临时起搏器（经右颈内静脉)。MPA1导管经右桡动脉置于左心室内，测量左室腔内压力曲线。6Fr导引导管（EBU3.5）经右股动脉置于左冠状动脉，连续监测LVOTPG。PTSMA术第30页BMW导丝至消融第1间隔支（S1），沿导引钢丝将合适OTW球囊（2.0×9mm）送至靶间隔支近段,加压扩张球囊（6-12atm）经中心腔注入造影剂或声学造影剂确定间隔支供血区域是否在肥厚梗阻部位，评定有没有交通支开放。超声心动图评定----注射酒精前最后评定，最关键PTSMA术第31页PTSMA操作技术关键是确定靶间隔支。间隔支大小及分布变异很大，20%患者第1间隔支供应右心室游离壁；40%患者瓣下室间隔不是完全由第1间隔支供应，5%患者不能确定靶间隔区域。室间隔由多种细小间隔支供应操作难度较大。术中靶间隔支确定第32页经OTW球囊中心腔注入造影剂或声学造影剂超声心动图：确定间隔支供血区域是否在肥厚梗阻部位；心肌声学造影（MCE）：更清楚，并能判断有没有交通支造影评定有没有交通支开放术中靶间隔支确定第33页MCE提升PTSMA安全性使用第三代微泡造影剂在介入术中进行超声心肌声学造影（MCE）微泡造影剂可使拟消融血管供血范围愈加明确，帮助确定靶血管，避免误消融。第34页MCE提升PTSMA安全性第35页经OTW球囊中心腔迟缓注入xml无水酒精，总量不超出3ml保持压力泵压力注射酒精前透视注意球囊位置和充盈情况酒精慢，0.1~0.3ml/次，每次间隔1min注意积极脉压力变化，压差下降时应当升高或不变，一旦下降要查找原因注意心率、节律—AVB监测有没有交通支开放—最危险PTSMA术—注入无水酒精（最关键）第36页LADS1AfterS1ablationOTWOTWBeforeablation第37页Baseline1weekFollow-up1yearFollow-up

磁共振随诊

AmJCardio2023;106:1487-1491.(IF3.9)

第38页

AmJCardio2023;106:1487-1491.(IF3.9)

院所青年基金

MRI可精确测量PTSMA消融位置和范围大小消融后左心室重量显著下降无水酒精用量与消融范围存在有关关系MRI评价PTSMA效果第39页同位素心肌灌注显像示PTSMA术后

患者室间隔灌注有不一样程度减低

中华核医学杂志2023;30:176-179.第40页国内外各组术者PTSMA急性期疗效术者病例数成功率%致死率%装起搏器%乔树宾203910.990.49Gietzen50？43Faber91972.211Kuhn172？2.3？Seggiwiss260901.25.8Schweinfurt659201.5第41页

疗效和随访—PTSMA

n=171PTSMA术前术后急性期（1周）术后远期（1年）左心室流出道压力阶差（mmHg）97.6±38.252.4±35.8**

47.3±38.6**

室间隔厚度(mm)

22.7±5.420.7±4.6△

16.8±4.4**

左心房内径(mm)

43.8±7.342.4±7.5△

32.8±15.6**

左心室射血分数(%)

72.8±8.173.2±9.5△

73.3±9.3△

术后晕厥消失病例/原有晕厥病例//82/86胸闷、胸痛改善%//130/171*p<0.05，**p<0.01，△p=NS第42页In-hospitaldeathCompleteheartblockventricularfibrillationAcutemitralregurgitationRightbundlebranchblockperforationPTSMAComplications第43页Procedure-relatedmortalityisaround1%to2%atexperiencedcentersSeptalMyocardialinfarctionencompassingupto10%oftheoverallLVmassVentriculararrhythmiasoccurin5%ofpatientsduringhospitalizationPersistentcompleteAVblock,with10%to20%,requiringaPPMComplications（研究资料）第44页ComplicationnRate(%)transitoryheartblock123