肥厚型梗阻性心肌病培训教学课件

1、肥厚型梗阻性心肌病化学消融术基础与临床Percutaneous Transluminal Septal Myocardial Ablation of Hypertrophic Obstructive Cardiomyopathy肥厚型心肌病(hypertrophic cardiomyopathy，HCM)是一种以心肌进行性肥厚、心室腔进行性缩小为特征病理特点：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%概述病死率1.4%（儿童可高至6%)，其中猝死0.7%，心衰0.5%，中风0.2%Koga等报道日本的肥厚型心肌病患者预后，5年随访的年病死率为

2、2.3%2.9%，其中心尖肥厚型心肌病病死率仅为0.3%，约1/10的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 心肌病概述遗传性因素： 是主要病因，大约50%55%的肥厚型心肌病患者有家族史，属于常染色体显性遗传病，肥厚型心肌病的遗传学说已被公认钙调节紊乱概述病因：不明症状与体征心电图特征超声心动图特征：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，可区分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征可以确立诊断核磁共振（MRI)：室壁增厚和流出道狭窄诊断Mechanical impedance at

3、the subaortic levelOwing to mitral valve systolic anterior motion (SAM) and mid-systolic contact with ventricular septumMitral regurgitation due to incomplete leaflet coaptationObstructive MechanismLeft ventricular outflow tract obstruction is present at rest in approximately25% of HCM patients In a

4、ddition, 50% of patients without obstruction at rest can generate significantintraventricular gradients with exerciseObstructive Mechanism LVOTPGLAAOLVPosterior wallSeptumSAMLVOTRVSAM SignLVOTPGHow to reduce the hypertrophy?Septal myectomyPTSMA药物外科切除经皮室间隔化学消融术（PTSMA)双强起搏器HCOM治疗Intracoronary ethylalc

5、oholor phenol injection ablates aconitine-induced ventricular tachycardia in dogsChemical ablation by subendocardialinjection ofethanolvia catheter-preliminary results in thepigheart.Conclusion: Intracoronaryethanolablation is a promising technique for the treatment of arrhythmias. Significant arrhy

6、thmias and a decrease in left ventricular ejection fraction are associated with this technique.Alcohol Septal Ablation (SAS), Inoue H et al,J Am Coll Cardiol. 1987 Dec;10(6):1342-9Weismller P et al. Eur Heart J.1991 Nov;12(11):1234-9Intracoronaryethanolablation inswine: characterization of myocardia

7、l injury in target and remote vascular bedsConclusion: Intracoronaryethanolablation Lesions are generally produced within the distribution of the targetedcoronarybed, but are also frequently associated with reflux to a second vascular distribution.Alcohol Septal Ablation (SAS)Haines DE ,et al J Card

8、iovasc Electrophysiol.1994 Jan;5(1):41-9.理论基础In1994, Sigwart was the first to report a successful nonsurgical myocardial reduction after occlusion of the septal branch using 96% alcohol.-Non-surgical myocardial reduction for hypertrophic obstructive cardiomyopathy- SASPTSMA aims directly to reduce t

9、he hypertrophied interventricular septum with associated expansion of the LVOT and reduction of the subaortic gradient.Percutaneous Transluminal Septal Myocardial Ablation (PTSMA)Sigwart U.Lancet. 1995;346:211e4Myocardialcontrastechocardiography: a reproducible technique ofmyocardial opacifi-cation

10、for identifying regionalperfusiondeficitsTargeting percutaneous transluminal septal ablationfor HOCM by intraprocedural ECHO monitoring.Alcohol Septal Ablation (SAS)Tei Cet al. Circulation.1983 Mar;67(3):585-93.Faber L,et al. J Am Soc Echocardiogr.2000 Dec;13(12):1074-9.The “1st Septal Unit”(consist

11、 of : the 1st septal coronary branch and its dependent (asymmetric) septal hypertrophy, at the level of mitral-septal contact) in HOCMAlcohol Septal Ablation (SAS)Polo et al. Tex Heart Inst J 2007;34:336-46Alcohol Septal Ablation (SAS)1st Septal Unit1st Septal UnitSymptomatic HCM patients with a NYH

12、A class of at least despite of optimal therapy.Patients with substantial side-effects of medication high outflow tract gradients (50mmHg at rest or100mmHg under stress) can be verified.PTSMA IndicationsClinical symptoms: amaurosis, syncope, angina, external dyspnea, et alEchoMRICAGIsotope myocardial

13、 imagingHolterProvocative testEvaluation before SASWall and septal thickness: Base, middle and apex segmentLVOT obstruction: SAM sign and pressure gradeECHOLVOTPG (PG=219mmHg)Wall and septal thicknessLVOT obstructionMRIProvocative TestLatent obstructionDifferentiate obstructive type（Resting and Late

14、nt type)Confirming indications for SAS or Surgery therapy 中华心血管病杂志 2008;36:412-414.Coronary Artery Angiogram: Lesions or Septal Branches 1 septal branch3 septal branches Net Structure of Septal BranchObstructive position and measuring pressureLV Angiogram流出道最窄处距主动脉瓣约25mm，压差80mmHGLVOTPG80mmHGLVPAOP术前

15、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BMW导丝至消融第1间隔支（S1），沿导引钢丝将合适的OTW球囊（2.09mm）送至靶间隔支的近段,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，评估有无交通支开放。超声心动图评估-注射酒精前最后评估，最关键PTSMA术PTSMA操作技术的关键是确定靶间隔支。间隔支的大小及分布变异很大，20%的患者第1间隔支供应右心室的游离壁；40%的患者瓣下室间隔不是完全由

16、第1间隔支供应，5%的患者不能确定靶间隔区域。室间隔由多个细小间隔支供应的操作难度较大。术中靶间隔支的确定经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）：更清晰，并能判断有无交通支造影评估有无交通支开放术中靶间隔支的确定MCE提高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的供血范围更加明确，协助确定靶血管，避免误消融。MCE提高PTSMA安全性经OTW球囊中心腔缓慢注入xml 无水酒精，总量不超过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，0.10.3

17、ml/次，每次间隔1min注意主动脉压力变化，压差下降时应该升高或不变，一旦下降要查找原因注意心率、节律AVB监测有无交通支开放最危险PTSMA术注入无水酒精（最关键）LADS1After S1 ablationOTWOTWBefore ablationBaseline 1 week Follow-up 1 year Follow-up 磁共振随诊 Am J Cardio 2010;106:1487-1491. (IF 3.9) Am J Cardio 2010;106:1487-1491. (IF 3.9)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明显下降无水酒

18、精用量与消融范围存在相关关系 MRI评价PTSMA效果同位素心肌灌注显像示PTSMA术后患者室间隔灌注有不同程度的减低 中华核医学杂志 2010;30:176 -179.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203910.990.49Gietzen 50？43Faber 91972.211Kuhn 172？2.3？Seggiwiss 260901.25.8Schweinfurt 659201.5疗效和随访PTSMAn=171 PTSMA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）97.638.2 52.435.8* 47.3

19、38.6* 室间隔厚度(mm) 22.75.4 20.74.6 16.84.4* 左心房内径(mm) 43.87.3 42.4 7.5 32.815.6* 左心室射血分数(%) 72.88.1 73.29.5 73.39.3 术后晕厥消失病例/原有晕厥病例 / / 82/86 胸闷、胸痛改善% / / 130 /171* p0.05，* p0.01，p=NSIn-hospital deathComplete heart blockventricular fibrillationAcute mitral regurgitationRight bundle branch blockperforat

20、ionPTSMA ComplicationsProcedure-related mortality is around 1% to 2% at experienced centers SeptalMyocardial infarction encompassing up to 10% of the overall LV massVentricular arrhythmias occur in 5% of patients during hospitalizationPersistent completeAV block, with 10% to 20% , requiring a PPMComplica