部分室间隔切除治疗肥厚型梗阻性心肌病

采用部分肥厚室间隔切除的方法治疗肥厚型梗阻性心肌病10例,1例同期行二尖瓣置换术。手术均获成功,无死亡者。认为部分肥厚室间隔切除治疗肥厚型梗阻性心肌病能有效解除左室流出道梗阻,效果满意。     

二尖瓣附瓣四例并文献复习

目的 探讨二尖瓣附瓣患者的超声心动图及临床表现.方法 4例成人患者经多普勒超声心动图检查确诊二尖瓣附瓣.结果 4例患者均有心脏杂音,但2例无明显症状.超声心动图显示2例患者为单纯二尖瓣附瓣伴左室流出道梗阻;1例患者合并梗阻性肥厚型心肌病,在服用负变力性药物后左室流出道内的收缩期压力阶差降至正常;另1例患者合并多种先天性心脏畸形,但无左室流出道梗阻征象.结论 二尖瓣附瓣可能并不极为罕见,超声心动图能检出本病,并与其他左室流出道狭窄-梗阻性异常进行鉴别.     

二尖瓣置换术后瓣周漏合并获得性室间隔缺损介入封堵1例

正1临床资料患者男,71岁。因"周围性水肿8个月余"入华中科技大学同济医学院附属协和医院。患者8个月前因肥厚型梗阻性心肌病及冠心病、重度二尖瓣关闭不全在外院实施冠状动脉旁路移植术+左心室流出道疏通+二尖瓣置换(机械瓣)术+起搏器植入术,术后经胸超声心动图检查发现室间隔穿孔,穿孔部位位于室间隔肌部中段,考虑与术中左心室流出道疏通过度有关,当时未行特殊处理。术后患者     

经皮室间隔化学消融术对梗阻性肥厚型心肌病二尖瓣反流的影响

目的 评价经皮室间隔化学？肖融术对梗阻性肥厚型心肌病二尖瓣反流的影响。方法选择2007年3月至2012年2月在广东省人民医院诊断为梗阻性肥厚型心肌病的患者41例为研究对象,年龄（51．1±2．2）岁,其中男20例,女21例。利用Sigward法行经皮室间隔化学消融术,浓度99％的无水酒精注入冠状动脉间隔支内,记录术前、术后左心室流出道压力阶差的变化,消融术前及术后第一天通过超声心动图测量左心室流出道压差及二尖瓣反流面积变化。结果导管法术前、术后左心室流出道压力阶差分别为（101．9±33．9）mmHg（1mmHg=0．133kPa）和（54．3±34．7）mmHg,两者比较差异有统计学意义（P〈0．01）。术前、术后二尖瓣反流面积分别为（7．2±2．1）cm。和（4．0±3．0）m2,两者比较差异有统计学意义（P〈0．01）。线性柏关回归分析显示,术前、术后二尖瓣反流面积的变化与左心室流出道压力阶差变化相关（r=0．589,P〈0．01）。结论经皮室间隔化学消融术可以降低梗阻性肥厚型心肌病患者左心室流出道压力阶差,使二尖瓣收缩期前向运动减轻,二尖瓣反流减少：二尖瓣反流的减少可以作为评价经皮冠状动脉室间隔化学消融术疗效的指标之一。     

肥厚型心肌病左室流出道梗阻(54)

肥厚型心肌病临床并非少见,严重者可发生左室流出道梗阻。左室流出道是指左室与主动脉之间狭长的解剖学结构,上方为室间隔,下方为二尖瓣前叶。有一定比例的肥厚型心肌病患者存在左室流出道梗阻,这些患者存在主动脉瓣下室间隔局部的明显肥厚,并在收缩期向     

二尖瓣成形术与二尖瓣置换术治疗退行性二尖瓣关闭不全的远期疗效比较研究

背景退行性变是西方国家二尖瓣关闭不全常见的病因,与二尖瓣置换术(MVR)相比,二尖瓣成形术(MVP)不仅无需长期抗凝,避免了抗凝相关并发症,而且能够有助于左心功能的维护。目前MVR与MVP治疗退行性变的远期疗效尚不明确。目的比较MVP与MVR治疗退行性二尖瓣关闭不全的远期疗效。方法选取2003—2008年在俄罗斯巴库列夫心血管外科研究中心接受瓣膜手术的退行性二尖瓣关闭不全患者274例为研究对象。根据手术方式的不同,将患者分为MVP组(129例)和MVR组(145例)。收集患者一般资料,统计患者5年、10年累积生存率、无脑卒中发生率及再次瓣膜手术情况。分别根据患者随访终点时死亡情况、脑卒中发生情况,将患者分为死亡组(42例)和非死亡组(232例)、脑卒中组(30例)和非脑卒中组(244例)。采用单因素分析和多因素Cox回归分析探讨退行性二尖瓣关闭不全患者死亡、发生脑卒中的影响因素。结果MVP组患者体外循环时间、主动脉阻断时间、术后住院时间长于MVR组(P<0.05)。死亡组患者体外循环时间、呼吸机使用时间长于非死亡组,MVP率低于非死亡组(P<0.05)。脑卒中组患者入院时血肌酐、入院时左心室射血分数(LVEF)高于非脑卒中组,MVP率低于非脑卒中组(P<0.05)。多因素Cox回归分析结果显示,MVP是退行性二尖瓣关闭不全患者死亡[HR=0.053,95%CI(0.005,0.585)]、发生脑卒中[HR=0.095,95%CI(0.013,0.698)]的保护因素(P<0.05)。MVP组患者5年累积生存率低于MVR组,10年累积生存率高于MVR组(P<0.05)。MVP组患者5年、10年累积无脑卒中发生率低于MVR组(P<0.05)。MVP组和MVR组患者再次瓣膜手术率比较,差异无统计学意义(P>0.05)。结论MVP是退行性二尖瓣关闭不全患者死亡、发生脑卒中的保护因素,对于退行性二尖瓣关闭不全患者,行MVP者的远期预后优于行MVR者,临床应积极推广MVP。     

肥厚型梗阻性心肌病经皮经腔间隔心肌消融术与室间隔心肌切除术疗效比较

目的应用超声心动图比较肥厚型梗阻性心肌病患者经皮经腔间隔心肌消融术与室间隔心肌切除术的疗效。方法分别于经皮经腔间隔心肌消融术及室间隔心肌切除术前、后测量肥厚型梗阻性心肌病患者左室流出道压差,比较术前及术后压差。结果肥厚型梗阻性心肌病患者在进行经皮经腔间隔心肌消融术与室间隔心肌切除术后,左室流出道压差均较术前明显减低。经皮经腔间隔心肌消融术肥厚型心肌病患者术前左室流出道压差(99±19)mm Hg(1 mm Hg=0.133 kPa),术后降至(36±20) mm Hg(P<0.05)。进行室间隔心肌切除术肥厚型心肌病患者术前左室流出道压差(117±32) mm Hg,术后降至(28±17) mm Hg(P<0.05)。经皮经腔间隔心肌消融术与室间隔心肌切除术患者术后左室流出道压差差异无统计学意义[(36±20)mm Hg比(28±17)mm Hg]。结论经皮经腔间隔心肌消融术可以明显减低肥厚型梗阻性心肌病患者左室流出道压差,且与室间隔心肌切除术疗效相似。     

Liwen术式治疗轻度室间隔肥厚伴左室流出道严重梗阻并随访2年一例

对优化药物治疗后效果不佳的梗阻性肥厚型心肌病,采用课题组的创新术式经皮超声引导下室间隔心肌内射频消融术(即Liwen术式),成功地治疗了 1例室间隔轻度肥厚但伴有严重左室流出道狭窄的肥厚型心肌病患者.患者术后恢复良好,无左心室流出道梗阻或心律失常.     

梗阻性肥厚型心肌病治疗方法的现状与进展

梗阻性肥厚型心肌病是一种以室间隔非对称性肥厚、二尖瓣前叶收缩期前向运动征、左室流出道梗阻和不同程度的二尖瓣反流为主要特征的常染色体显性遗传性疾病。目前该病可通过药物、外科手术、经皮室间隔化学消融术等方式治疗,从而改善患者的临床症状,减少并发症及预防心源性猝死。现就梗阻性肥厚型心肌病治疗方法的现状与进展做一综述。     

扩大室间隔切除治疗肥厚型梗阻性心肌病的临床研究

目的总结扩大室间隔切除治疗肥厚型梗阻性心肌病（HOCM）的临床经验。方法2009年2月至2011年12月我院外科治疗肥厚型梗阻性心肌病42例,其中男性23例,女性19例,年龄13～66（38±6）岁,体重45±85（62．4±14．7）kg。手术在全身麻醉低温体外循环下完成,按常规经主动脉切13行室间隔心肌切除术（改良Morrow技术）,同期完成二尖瓣机械瓣膜置换术2例、冠状动脉旁路移植术5例、主动脉瓣置换术3例。围术期及术后随访心脏超声心动图（UCG）或食管超声心动图（TEE）评价左心房（LA）、左心室（LV）、左室流出道流速及压差（LVOT）、左室射血分数（LVEF）、二尖瓣结构和功能。结果全组患者手术均获得成功,无停机困难,体外循环（84．7±13．8）min,主动脉阻断（60．3±18．4）min。术后所有患者症状均明显改善或消失。超声心动图显示室间隔厚度较术前明显减小,左心室流出道内径较术前明显增宽,左心室流出道压差下降。SAM患者术后超声心动图示SAM消失。二次转机行二尖瓣置换术2例。住院时间11～23d,平均12d。34例获得6～26（14±3）个月随访,随访中进行超声心动图检查。术后10d．6个月及1年的超声心动图均提示LVOTG和室间隔厚度（IVST）较术前显著下降（P〈0．05）;除术前外,其他各时间点的LVOTG和IVST间差异无统计学意义。术后1年心功能（NYHA分级）：I级30例、Ⅱ级3例、Ⅲ级1例。结论扩大室间隔心肌切除术可避免行二尖瓣置换术治疗肥厚型梗阻性心肌病,左心室舒张功能改善,有利于左心室的重塑。患者症状及心功能改善,治疗结果满意。     

Mitral regurgitation in hypertrophic obstructive cardiomyopathy: relationship to obstruction and relief with myectomy

OBJECTIVES

This study examined: 1) the impact of myectomy on postoperative mitral regurgitation (MR) and 2) the association between the severity of MR and the left ventricular outflow tract (LVOT) gradient.

BACKGROUND

For patients with hypertrophic obstructive cardiomyopathy (HOCM) and MR, controversy exists as to whether myectomy alone is sufficient in eliminating MR. Furthermore, the relationship between the degree of MR and the LVOT peak gradient has not been well defined.

METHODS

We performed pre- and postoperative transthoracic as well as intraoperative transesophageal studies in 104 consecutive patients with HOCM undergoing septal myectomy. Left ventricular outflow tract gradient and the nature of MR were assessed.

RESULTS

In the 93 patients without independent mitral valve disease, a relationship was observed between MR severity and the LVOT gradient. Left ventricular outflow tract gradient (mean ± standard deviation) for trivial, mild, moderate and severe MR were: 23.2 ± 19.1, 43.8 ± 25.4, 70.1 ± 21.0 and 104 ± 21.0 mm Hg (p < 0.001). Early postoperative, MR was absent or trivial in 80%, mild in 19% and moderate in 1%. None of these patients required additional mitral valve surgery. For patients with independent mitral valve disease (n = 11), five required mitral valve surgery as well as myectomy. The remainder had significant reductions in the degree of MR with myectomy alone.

CONCLUSIONS

For patients with HOCM and MR not due to independent mitral valve disease, myectomy significantly reduced the degree of MR, without requirement for additional mitral valve surgery. In these patients the severity of MR was directly related to the magnitude of the LVOT gradient.     

肥厚型梗阻性心肌病的外科治疗及围术期治疗策略

目的 总结肥厚型梗阻性心肌病（Hypertrophicobstructivecardiomyopathy,HOCM）的外科治疗效果,探讨围术期治疗策略。方法 回顾性分析2012年6月至2013年10月我院由单一术扦实施外科手术治疗的HOCM忠并75例,刃性47例（47／75,63％）,女性28例（28／75,37%）．年龄10—66（42．92±15．07）岁,术前左室流出道峰值压差（LVOTGP）为50—270（86．98±42．69）mmHg（1mmHg=0．133kPa）、令=部患行均接受室间隔心肌切除术（改良扩大Morrow术）．同期行冠状动脉旁路移植术6例,室壁确切除术1例,二尖瓣置换术4．二尖瓣成形术9,主动脉瓣置换术2,三尖瓣成形术3,先心病2例．围术期常规行心脏超声心动图、心电图及胸部X线片检查,评价超声心动图检查指标、二尖瓣的结构和功能改变。结果 全组无围术期或远期死亡。全组体外循环时间66—258（J33．00±39．83）分钟,升主动脉阻断时间45—157（84．71±25．85）分钟,机械通气时间8-396（2447±44．78）小时,术后住ICU时间1-27（299±3．23）天,术后住院时间6—35（10．20±5．31）天,术后胸腔积液12例,二次插管1例,气管切外1例,床旁血液滤过治疗1例,主动脉内球囊反搏1例,无气胸、无二次开胸探查及二次手术.术后片心房内径（37.31±4．34mm vs43．50±5．89mm,P=O．000）,左室流出道峰值压差（12．31±7.00mmHg vs 86．98±42．69mmHg,P=0．000）,室间隔厚度（15．41±5．00mmvs22．34±6．20mm．P=O．000）与术前比较均减小或降低。二尖瓣关闭好或仅有轻度反流,二尖瓣前向运动（SAM征）基本消失。术后发生的主要心律失常包括完全性左束支传导阻滞、室内传导阻滞、完全性房室传导阻滞和心房颤动等。远期随访患者症状消失或仅有轻度症状,生活质量明显改善,心动能NYHA分级级别较术前降低I—II级,无远期死亡、并发症或再次手术。结论外科室间隔心肌切除术治疗肥厚型梗阻性心肌病具有良好的手术效果,能够安全有效地解除左室流出道的梗阻,消除二尖瓣SAM征,改善临床症状。术后并发症主要为心律失常表现为传导束传导异常和心房颤动．具有较好的近远期生存率。     

隐匿性肥厚梗阻性心肌病的外科治疗

目的明确隐匿性肥厚梗阻性心肌病的手术适应症,评价改良扩大室间隔肥厚心肌切除术(改良Morrow手术)治疗隐匿性肥厚梗阻性心肌病的手术效果。方法回顾性分析2009年10月至2011年8月阜外医院心外科手术治疗的74例肥厚梗阻性心肌病病人资料,其中5例符合隐匿性肥厚梗阻性心肌病标准,其中男4例,女1例,平均年龄33(22～43)岁,静息和运动激发试验后左室流出道压差(LVOTGP)平均为29.4(13～49)mmHg和105.2(91-124)mmHg,二尖瓣为少量(4例)或少中量(1例)返流。手术为全麻低温体外循环下经主动脉切口行改良扩大Morrow手术。分析比较患者手术前后超声心动图,通过门诊、电话随诊。结果本组围手术期存活率100%,手术效果好,无严重并发症,1例同期行二尖瓣置换术。主动脉阻断时间平均70.4分钟(35-157分钟),平均体外循环时间102.6分钟(49-225分钟)。患者术后LVOT压差平均为3.75mmHg(0--8mmHg),与术前相比有显著差异(p0.01)。术后二尖瓣为无返流4例,少量返流1例。平均随访11(4-20)个月,全部患者症状消失或减轻,心功能I～II级,无死亡或再次手术。结论运动激发试验用于隐匿性肥厚梗阻性心肌病患者的筛选有利于避免漏诊,改良Morrow手术治疗符合手术指征的隐匿性肥厚梗阻性心肌病具有良好的手术效果,早中期疗效满意。     

Left ventricular outflow tract obstruction due to anomalous insertion of papillary muscle.

A 56-year-old man who complained of quadrantic hemianopsia was admitted to determine its etiology. Cerebral angiography revealed no organic stenosis. Echocardiography showed clear direct continuity between a hypertrophied anterolateral papillary muscle and the anterior mitral leaflet, and the left ventricular (LV) outflow tract (LVOT) was narrowed by the presence of an accessory papillary muscle. The LVOT obstruction caused an intra-LV pressure overload that resulted in LV concentric hypertrophy. Arrhythmia, such as paroxysmal atrial fibrillation (PAF), was thought to have caused a cerebral embolism. Mitral valve replacement (MVR), septal myectomy, and myectomy of the abnormal papillary muscle were performed, and complete release of the LVOT obstruction was accomplished. Anomalous insertion of papillary muscle is a rare cause of LVOT obstruction. Echocardiography was useful in identifying the papillary muscle malformation, and surgery was completely curative.     

肥厚型梗阻性心肌病患者二尖瓣叶长度及对合形态异常的超声心动图随访研究

目的:应用超声心动图评估肥厚型梗阻性心肌病(HOCM)患者二尖瓣叶长度及对合形态的特点,以及单纯行扩大室间隔切除术后二尖瓣叶对合形态的改变。方法:入组2012年至2017年间中国医学科学院阜外医院由同一个有经验术者行扩大室间隔切除术(术中未特殊处理二尖瓣叶)的HOCM患者52例为HOCM组,同时纳入性别和年龄相匹配的健康受试者23例为正常对照组。比较两组二尖瓣参数以及左心房、室指标的差异,且随访观察HOCM组患者在扩大室间隔切除术前、术后二尖瓣参数和左心房、室指标的变化以及是否出现新发的、有血流动力学意义的二尖瓣反流。平均随访(7.10±7.57)个月。结果:与正常对照组比较,HOCM组患者二尖瓣瓣环前后径(Ann D)、二尖瓣前叶长度(ALL)、后叶长度(PLL)、前乳头肌根部直径(APM)和反乳头肌根部直径(PPM)均增大(P均<0.01),前叶在瓣环上的投影长度(Pro L)及其占瓣环总长度的比例(Pro R)均减小(P均<0.01),左心房前后径(LAD)增大,室间隔基底段厚度(IVS)明显增加,左心室舒张末期内径(LVEDD)减小(P<0.01),瓣叶对合高度(Ten H)无差异(P>0.01),瓣叶对合点到前室间隔的最近距离(C-sept)及乳头肌连线平面与前室间隔的距离(S-P)均明显减小(P<0.01)。与术前比较,HOCM组患者在扩大室间隔切除术后,二尖瓣反流明显减少(P<0.01),二尖瓣反流均在轻度以下,未见新发的有血流动力学意义的反流;Ann D、Pro L、Pro R、Ten H与术前比较均无明显差异(P均>0.01),C-sept及S-P较术前均明显增大(P均<0.01);LAD、IVS、左心室后壁厚度(LVPWD)、左心室射血分数(LVEF)较术前均明显减小,LVEDD较术前增大(P均<0.01)。结论:HOCM患者经过充分的室间隔切除,二尖瓣反流均能得到明显改善。虽然二尖瓣前后叶均延长,但术后中期随访结果未见新发的、有血流动力学意义的二尖瓣反流。HOCM患者二尖瓣对合形态异常、对合点前移,手术解除梗阻后,异常的对合形态未见恢复。     

Outcome of patients with hypertrophic obstructive cardiomyopathy after percutaneous transluminal septal myocardial ablation and septal myectomy surgery

OBJECTIVES: This study was conducted to evaluate follow-up results in patients with hypertrophic obstructive cardiomyopathy (HOCM) who underwent either percutaneous transluminal septal myocardial ablation (PTSMA) or septal myectomy. BACKGROUND: Controversy exists with regard to these two forms of treatment for patients with HOCM. METHODS: Of 51 patients with HOCM treated, 25 were treated by PTSMA and 26 patients via myectomy. Two-dimensional echocardiograms were performed before both procedures, immediately afterwards and at a three-month follow-up. The New York Heart Association (NYHA) functional class was obtained before the procedures and at follow-up. RESULTS: Interventricular septal thickness was significantly reduced at follow-up in both groups (2.3 +/- 0.4 cm vs. 1.9 +/- 0.4 cm for septal ablation and 2.4 +/- 0.6 cm vs. 1.7 +/- 0.2 cm for myectomy, both p < 0.001). Estimated by continuous-wave Doppler, the resting pressure gradient (PG) across the left ventricular outflow tract (LVOT) significantly decreased immediately after the procedures in both groups (64 +/- 39 mm Hg vs. 28 +/- 29 mm Hg for PTSMA, 62 +/- 43 mm Hg vs. 7 +/- 7 mm Hg for myectomy, both p < 0.0001). At three-month follow-up, the resting PG remained lower in the PTSMA and myectomy groups (24 +/- 19 mm Hg and 11 +/- 6 mm Hg, respectively, vs. those before procedures, both p < 0.0001). The NYHA functional class was also significantly improved in both groups (3.5 +/- 0.5 vs. 1.9 +/- 0.7 for PTSMA, 3.3 +/- 0.5 vs. 1.5 +/- 0.7 for myectomy, both p < 0.0001). CONCLUSIONS: Both myectomy and PTSMA reduce LVOT obstruction and significantly improve NYHA functional class in patients with HOCM. However, there are benefits and drawbacks for each therapeutic method that must be counterbalanced when deciding on treatment for LVOT obstruction.     

Successful alcohol septal ablation for late recurrence of left ventricular outflow tract obstruction after surgical myectomy in hypertrophic obstructive cardiomyopathy

An 18-year-old male patient, known with familial hypertrophic obstructive cardiomyopathy underwent a septal myectomy 10 years ago for significant left ventricular outflow tract (LVOT) obstruction. During follow-up a progressive increase in LVOT gradient was noted in association with severe mitral valve regurgitation. The patient underwent percutaneous alcohol septal ablation to induce regression of left ventricular hypertrophy. Coronary angiography, with intracoronary contrast and guided by echocardiographic imaging, was applied for localisation of the appropriate septal branch. The vessel was subsequently injected with 1.5 cc ethanol. No procedure-related complications were reported. The LVOT gradient decreased from 90 mmHg to 48 and 45 mmHg at rest 6 weeks and 6 months, respectively, after the procedure. Mitral valve regurgitation was significantly reduced. This case nicely illustrates the feasibility of percutaneous alcohol septal ablation for recurrent LVOT obstruction 10 years after myectomy.     

Mitral regurgitation disappearance after cibenzoline treatment in a patient with hypertrophic obstructive cardiomyopathy

Hypertrophic obstructive cardiomyopathy (HOCM), which shows left ventricular outflow pressure gradient (LVPG), is often complicated with mitral regurgitation (MR). We examined a 62-year-old Japanese female with HOCM and MR. Ultrasound echocardiography showed severe MR, asymmetrical septal hypertrophy, systolic anterior movement of the mitral valve anterior leaflet, and left ventricular outflow stenosis. Her LVPG, measured using continuous wave Doppler recording, was 118 mmHg. During heart catheterization, the aortic pressure and left ventricular pressure were simultaneously measured. An intravenous injection of 70 mg cibenzoline decreased the LVPG from 110 mmHg to 16 mmHg. Left ventriculography was performed immediately after the injection and did not show MR. This clearly demonstrates that cibenzoline decreases LVPG in patients with HOCM and also improves the MR that arises from LVPG.