肥厚型梗阻性心肌病培訓(xùn)教學(xué)課件

1、肥厚型梗阻性心肌病化學(xué)消融術(shù)基礎(chǔ)與臨床Percutaneous Transluminal Septal Myocardial Ablation of Hypertrophic Obstructive Cardiomyopathy肥厚型心肌病(hypertrophic cardiomyopathy，HCM)是一種以心肌進(jìn)行性肥厚、心室腔進(jìn)行性縮小為特征病理特點：左心室血液充盈受阻，舒張期順應(yīng)性下降分型：梗阻型和非梗阻型兩型人群發(fā)病率0.02%-0.2%概述病死率1.4%（兒童可高至6%)，其中猝死0.7%，心衰0.5%，中風(fēng)0.2%Koga等報道日本的肥厚型心肌病患者預(yù)后，5年隨訪的年病死率為

2、2.3%2.9%，其中心尖肥厚型心肌病病死率僅為0.3%，約1/10的患者逐漸發(fā)生左心室擴(kuò)張和心力衰竭，最后類似擴(kuò)張型心肌病被稱為擴(kuò)張期肥厚型 心肌病概述遺傳性因素： 是主要病因，大約50%55%的肥厚型心肌病患者有家族史，屬于常染色體顯性遺傳病，肥厚型心肌病的遺傳學(xué)說已被公認(rèn)鈣調(diào)節(jié)紊亂概述病因：不明癥狀與體征心電圖特征超聲心動圖特征：非對稱性室間隔增厚12mm，室間隔/左室后壁1.3；室間隔厚度18mm并有二尖瓣收縮期前移，可區(qū)分梗阻性與非梗阻性左心室造影：左心室流出道壓力差及左室形態(tài)特征可以確立診斷核磁共振（MRI)：室壁增厚和流出道狹窄診斷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藥物外科切除經(jīng)皮室間隔化學(xué)消融術(shù)（PTSMA)雙強(qiáng)起搏器HCOM治療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.理論基礎(chǔ)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 中華心血管病雜志 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流出道最窄處距主動脈瓣約25mm，壓差80mmHGLVOTPG80mmHGLVPAOP術(shù)前

15、常規(guī)安裝臨時起搏器（經(jīng)右頸內(nèi)靜脈)。MPA1導(dǎo)管經(jīng)右橈動脈置于左心室內(nèi)，測量左室腔內(nèi)壓力曲線。6Fr 導(dǎo)引導(dǎo)管（EBU3.5）經(jīng)右股動脈置于左冠狀動脈，連續(xù)監(jiān)測LVOTPG。PTSMA術(shù)BMW導(dǎo)絲至消融第1間隔支（S1），沿導(dǎo)引鋼絲將合適的OTW球囊（2.09mm）送至靶間隔支的近段,加壓擴(kuò)張球囊（6-12atm）經(jīng)中心腔注入造影劑或聲學(xué)造影劑確定間隔支供血區(qū)域是否在肥厚梗阻部位，評估有無交通支開放。超聲心動圖評估-注射酒精前最后評估，最關(guān)鍵PTSMA術(shù)PTSMA操作技術(shù)的關(guān)鍵是確定靶間隔支。間隔支的大小及分布變異很大，20%的患者第1間隔支供應(yīng)右心室的游離壁；40%的患者瓣下室間隔不是完全由

16、第1間隔支供應(yīng)，5%的患者不能確定靶間隔區(qū)域。室間隔由多個細(xì)小間隔支供應(yīng)的操作難度較大。術(shù)中靶間隔支的確定經(jīng)OTW球囊中心腔注入造影劑或聲學(xué)造影劑超聲心動圖：確定間隔支供血區(qū)域是否在肥厚梗阻部位；心肌聲學(xué)造影（MCE）：更清晰，并能判斷有無交通支造影評估有無交通支開放術(shù)中靶間隔支的確定MCE提高PTSMA安全性使用第三代微泡造影劑在介入術(shù)中進(jìn)行超聲心肌聲學(xué)造影（MCE）微泡造影劑可使擬消融血管的供血范圍更加明確，協(xié)助確定靶血管，避免誤消融。MCE提高PTSMA安全性經(jīng)OTW球囊中心腔緩慢注入xml 無水酒精，總量不超過3ml保持壓力泵壓力注射酒精前透視注意球囊位置和充盈情況酒精慢，0.10.3

17、ml/次，每次間隔1min注意主動脈壓力變化，壓差下降時應(yīng)該升高或不變，一旦下降要查找原因注意心率、節(jié)律AVB監(jiān)測有無交通支開放最危險PTSMA術(shù)注入無水酒精（最關(guān)鍵）LADS1After S1 ablationOTWOTWBefore ablationBaseline 1 week Follow-up 1 year Follow-up 磁共振隨診 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可精確測量PTSMA消融位置和范圍大小消融后左心室重量明顯下降無水酒

18、精用量與消融范圍存在相關(guān)關(guān)系 MRI評價PTSMA效果同位素心肌灌注顯像示PTSMA術(shù)后患者室間隔灌注有不同程度的減低 中華核醫(yī)學(xué)雜志 2010;30:176 -179.國內(nèi)外各組術(shù)者PTSMA急性期療效術(shù)者 病例數(shù)成功率%致死率%裝起搏器%喬樹賓203910.990.49Gietzen 50？43Faber 91972.211Kuhn 172？2.3？Seggiwiss 260901.25.8Schweinfurt 659201.5療效和隨訪PTSMAn=171 PTSMA術(shù)前 術(shù)后急性期（1周） 術(shù)后遠(yuǎn)期（1年）左心室流出道壓力階差（mmHg）97.638.2 52.435.8* 47.3

19、38.6* 室間隔厚度(mm) 22.75.4 20.74.6 16.84.4* 左心房內(nèi)徑(mm) 43.87.3 42.4 7.5 32.815.6* 左心室射血分?jǐn)?shù)(%) 72.88.1 73.29.5 73.39.3 術(shù)后暈厥消失病例/原有暈厥病例 / / 82/86 胸悶、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