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心肌病,肥大性

心肌病,肥大性的相关文献在1999年到2017年内共计118篇,主要集中在内科学、临床医学、基础医学 等领域,其中期刊论文118篇、专利文献440058篇;相关期刊43种,包括中国病理生理杂志、磁共振成像、中国临床医学影像杂志等; 心肌病,肥大性的相关文献由473位作者贡献,包括刘延玲、姜腾勇、李靖等。

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心肌病,肥大性

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  • 刘延玲
  • 姜腾勇
  • 李靖
  • 吕强
  • 吕秀章
  • 吴学思
  • 周蕾
  • 王浩
  • 胡大一
  • 许迪
  • 期刊论文
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    • 滕飞; 刘婷; 戴旭
    • 摘要: Objective: The feature tracking technique of cardiac magnetic resonance (CMR-FT) was used to analyze the cardiac function and myocardial segmental strain in patients with hypertrophic cardiomyopathy (HCM), and to explore the feasibility of CMR-FT in the detection of early deformation abnormalities in patients with hypertrophic cardiomyopathy. Materials and Methods: 17 hypertrophic cardiomyopathy patients and 14 healthy subjects as control cases were enrolled in our study. CMR was performed on 3.0 T scanner with the sequence of cine steady-state free precession. Cardiac functional segmental strain (38 segments of HCM and 38 segments of healthy subjects) was measured using CVI software. Results:Left ventricular function parameters (left ventricular end-diastolic volume, left ventricular end-systolic volume and left ventricular ejection fraction) had no difference between HCM patients and healthy subjects (all P>0.05). Segmental strain were significantly lower in patients with HCM compared to healthy subjects (all P0.05),肥厚的心肌节段的圆周应变、长轴应变及峰值收缩期圆周应变、峰值收缩期长轴应变均低于正常的心肌节段[(-5.26±2.70)%vs(-11.68±2.06)%,(-7.92±5.07)%vs(-13.93±3.89)%,(-10.44±5.46)%vs(-18.43±2.99)%,(-12.29±8.17)%vs(-20.26±2.93)%,P均<0.05].结论 对于心功能正常的肥厚性心肌病患者,CMR-FT技术能够早期检测出肥厚患者肥厚心肌节段应变的变化,提示心肌应变量的改变能够比左心室功能参数更早地发现心肌收缩功能异常.
    • 唐海林; 黎鹏; 胡功排; 李俐; 方辉; 傅国胜; 赵博文
    • 摘要: Objective To assess left atrial (LA) function by two dimensional speckle tracking echocardiography (2DSTE) in patients with hypertrophic obstructive cardiomyopathy (HOCM) before and one year after percutaneous transluminal septal myocardial ablation (PTSMA).Methods The study included 31 patients with HOCM who underwent PTSMA performed between November 2012 and December 2014 in Sir Run Run Shaw Hospital.Mean age was 51 ± 13 years,and 13 patients were men,18 patients were women.New York Heart Association class was 2.6± 0.7 at baseline.If the target region was correctly marked by the injection of the echo contrast agent,ethanol was slowly (0.5 ml/min) injected to a maximum of 3 ml.Ten minutes after the injection of ethanol,the balloon was deflated.A successful procedure was defined as a ≥ 50% reduction in the LV outflow tract peak systolic gradient compared to baseline level.Each patient with HOCM had a complete two-dimensional transthoracic echocardiography assessment before and one year after the PTSMA.The measurements included basal septal thickness,left ventricular outflow tract (LVOT) gradient,mitral regurgitation (MR) grade,left atrial dimensions,left ventricular (LV) ejection fraction and tissue Doppler parameters of lateral mitral annular e'and septal mitral annular e'.The LA wall was tracked on a frame-by-frame basis using 2D-STE,and LA volume waveforms were generated.The maximum LA volume (LAVmax),minimal LA volume (LAVmin),and the LA volume before atrial contraction (LAVpre-a) were measured.The LA reservoir function was calculated as left atrial expansion index (LAEI) and left atrial diastolic emptying index (LADEI).The LA conduit function was calculated as the passive emptying percentage of total emptying (PE) and the passive emptying index (PEI).The LA booster function was calculated as the active emptying percentage of total emptying (AE) and the active emptying index (AEI).The comparisons between patient New York Heart Association class,echocardiography and LA function parameter before and after PTSMA were conducted by paired t test.Results Compared with pre-operative values,LVOTG,MR grade,LA end-diastolic size,E/lateral e'ratio,septal mitral annular e'decreased in patients one year after the PTSMA [(35±26) mmHg vs (74135) mmHg,0.9310.35 vs 2.27±0.73,(40±6) mm vs (45±6) mm,12.6±3.8 vs 14.6±4.6,(4.3± 1.1) cm/s vs (5.0± 1.3) cm/s],whereas New York Heart Association class,Lateral mitral annular e',E/septal e'ratio [1.4±0.5 vs 2.6±0.7,(6.9±2.3) cm/s vs (5.8± 1.7) cm/s,20.2±7.1 vs 17.0±5.2] increased (t=6.55,4.78,2.60,2.84,2.59,-3.32,-2.67,-2.93,all P < 0.05).Compared with pre-operative values,LAVImax,LAVImin,LAVIpre-a,AE,AEI(%) decreased in patients one year after the PTSMA [(32.7±7.1) ml/m2 vs (38.9±9.3) ml/m2,(16.3±4.4) ml/m2 vs (20.7±5.5) ml/m2,(23.1±5.7) ml/m2 vs (30.5±7.0) ml/m2,(41.5±8.7)% vs (53.8± 10.4)%,(29.4±3.9)% vs (32.1±4.6)%],(t=3.65,3.11,3.38,3.92,2.17,all P < 0.05);whereas the LAEI(%),LADEI(%),PE(%),PEI(%) [(100.1 ± 19.3)% vs (87.9± 14.7)%,(50.2±9.8)% vs (46.7±9.1) %,(58.5± 11.8) % vs (46.1 ±9.1) %,(26.9±5.2)% vs (21.6±4.8)%] increased (t=-2.82,-2.33,-3.89,-2.74,all P < 0.05).Conclusions The study showed the improvement in LA reservoir and conduit function,while the reduction in LA booster pump function in patients one year after PTSMA.%目的 应用二维斑点追踪技术对肥厚型梗阻性心肌病(HOCM)患者经皮经冠状动脉室间隔化学消融术(PTSMA)术前及术后1年左心房功能的评估.方法 选取2012年11月至2014年12月在浙江大学医学院附属邵逸夫医院行PTSMA手术HOCM患者31例,其中,男性13例,女性18例,年龄22~71岁,平均年龄(51±13)岁.心功能NYHA分级为(2.6±0.7)级.如果靶区域超声造影剂显示正常,通过球囊中心腔向导管腔内缓慢推注无水乙醇增大剂量3.0 ml,保持球囊充盈10 min.以LVOTG下降≥50%作为手术成功的标准.于室间隔化学消融术前及术后1年接受超声心动图检查.测量包括左心室流出道压差(LVOTG),二尖瓣关闭不全(MR)的程度,左心房前后径,左心室射血分数(LVEF)和组织多普勒(TDI)测定二尖瓣环舒张早期峰值速度e'(取室间隔和侧壁).采用二维斑点追踪技术自动追踪左心房壁,并获取左心房容积曲线,获得左心房最大容积(LAVmax)、左心房最小容积(LAVmin)及左心房收缩前容积(LAVpre-a).计算出左心房储备功能包括左心房充盈扩张分数(LAEI)及左心房舒张期排空分数(LADEI)、左心房管道功能包括左心房被动排空比(PE)及被动排空指数(PEI)、左心房泵功能包括左心房主动排空比(AE)及主动排空指数(AEI).PTSMA术前及术后患者心功能NYHA分级、超声心动图测量结果、左心房容积参数之间的比较采用配对f检验.结果 PTSMA术后1年与术前比较,LVOTG、MR的程度、左心房前后径、E/侧壁e'比值、二尖瓣环室间隔e'均下降,差异均有统计学意义[(35±26) mmHg vs (74±35) mmHg(1 mmHg=0.133 kPa)、0.93±0.35 vs 2.27±0.73、(40±6) mm vs (45±6) mm、12.6±3.8 vs14.6±4.6、(4.3±1.1) cm/s vs(5.0±1.3) cm/s,t=6.55、4.78、2.60、2.84、2.59,P均<0.05];心功能NYHA分级、二尖瓣环侧壁e'、E/室间隔e'比值增加,差异均有统计学意义[1.4±0.5 vs 2.6±0.7、(6.9±2.3) cm/s vs (5.8±1.7) cm/s、20.2±7.1 vs 17.0±5.2,t=-3.32、-2.67、-2.93,P均<0.05].消融术后1年与术前比较,LAVImax,LAVImin、LAVIpre-a、AE、AEI(%)下降[(32.7±7.1) ml/m2vs (38.9±9.3) ml/m2、(16.3±4.4) ml/m2 VS (20.7±5.5) ml/m2、(23.1±5.7) ml/m2vs (30.5±7.0) ml/m2、(41.5±8.7)%vs (53.8±10.4)%、(29.4±3.9)%vs (32.1±4.6)%],差异均有统计学意义(t=3.65、3.11、3.38、3.92、2.17,P均<0.05);LAEI(%)、LADEI(%)、PE(%)、PEI(%) 增加[(100.1±19.3)%vs(87.9±14.7)%、(50.2±9.8)% vs (46.7±9.1)%、(58.5±11.8)% vs (46.1±9.1)%、(26.9±5.2)%vs (21.6±4.8)%],差异均有统计学意义(t=-2.82、-2.33、-3.89、-2.74,P均<0.05).结论 HOCM患者室间隔消融术后1年左心房的储备及管道功能得到改善,而左心房泵功能及主动收缩耗能下降.
    • 乔铅; 郝应禄; 李燕萍
    • 摘要: 肥厚型心肌病(hypertrophic cardiomyopathy,HCM)是常见的常染色体显性遗传性心脏病,其病因复杂,临床表现多样,临床特征是左心室非对称性的肥厚,且这种肥厚不能用其他心脏疾病或全身疾病来解释.随着HCM诊治手段的不断发展、更新,对HCM认识也越来越深入,其治疗方法亦在不断发展.目前治疗上仍主要以防治并发症(尤其是心源性猝死)、减轻患者症状、延缓疾病进展为主,包括一般治疗、药物治疗和非药物治疗.通过阅读相关文献,就HCM的治疗方法作一综述.
    • 李阳; 梁蕊; 林泽喜; 魏文峰; 刘子杰
    • 摘要: [目的]探讨炎症因子的表达与心肌肥厚性改变的相关性.[方法]将40只体重在180~200 g的雄性SD大鼠随机分为两组,实验组(25只)制作压力负荷性心肌肥厚大鼠模型,对照组肾动脉不作处理.手术56 d后用ELISA方法测量比较两组心肌组织肿瘤坏死因子(TNF-α)与白细胞介素-6(IL-6)的含量,并比较两组左室心肌重量.[结果]两组大鼠TNF-α和IL-6水平比较差异有显著性(P<0.05);且实验组大鼠的左室壁明显肥厚,重量明显增加(P<0.05).[结论]实验组大鼠心肌组织TNF-α、IL-6表达水平与左室心肌重量成正相关,并参与了左室肥厚的形成过程.%[Objective]To explore the correlation between the expression of inflammatory factors and the change about myocardial hypertrophy. [Methods]Forty healthy male Sprague-Dawley rats weighed 180~200 g were randomly divided into two groups. Experiment group ( n =25) was used for establishing rat model of pressure-overload hypertrophy, while control group did not receive the operation of renal artery. Inflammatory factors such as tumor necrosis factor-alpha(TNF-α) and interleukin-6(IL-6) in myocardium were measured and compared between two groups by using ELISA 56 days after the operation. [Results]There was significant difference in TNF-α and IL-6 between two groups( P <0. 05). In experiment group, left ventricular wall of rats had obvious hypertrophy and the weight significantly increased( P <0. 05). [Conclusion]The expression of TNF-a and IL-6 in myocardium is positively correlated with left ventricular mass of rats in experiment group, which participates in the formation of left ventricular hypertrophy.
    • 刘杨
    • 摘要: 目的:观察短期应用阿托伐他汀对扩张型心肌病(DCM)心力衰竭患者心功能及C反应蛋白(CRP)、血浆脑钠肽(BNP)的影响.方法:选择DCM心力衰竭患者40例,随机分为治疗组20例,对照组20例.对照组常规应用纠正心力衰竭药物治疗,治疗组在常规治疗基础上加用阿托伐他汀40 mg,每晚1次,疗程12周.2组入选后次日及治疗12周后测定左心室射血分数(LVEF)、血浆CRP、BNP水平.结果:2组LVEF较治疗前显著提高(P<0.01),治疗组与对照组差异有统计学意义(P<0.01).2组CRP和BNP水平均较治疗前显著降低(P<0.01),治疗组与对照组差异均有统计学意义(P<0.01).结论:短期应用阿托伐他汀可降低DCM心力衰竭患者CRP水平,改善心功能,有效抑制BNP水平.其改善心功能作用机制可能与他汀类调脂以外的抗炎作用有部分关系,短期应用他汀类治疗DCM心力衰竭可能是有益的.%Objective:To observe the effect of atorvastatin on cardiac function, C-reaction protein(CRP) and serum level of brain natriuretic peptide(BNP) in patients of heart failure(HF) with dilated cardiomyopathy(DCM). Methods :Forty patients with DCM and HF were randomly divided into treatment group and control group. The control group was given conventional drug therapy. The treatment group was given atorvastatin(40 mg/d, 12 weeks) in addition to routine treatment. The left ventricular ejection fraction (LVEF), CRP and BNP were measured before and after treatment. Results: After 12 weeks, the LVEF was dramatically higher in all patients ( P < 0.01 ) ,and the LVEF in treatment group was significantly higher than that in the conventional therapy group( P < 0.01 ). The serum level of CRP and BNP was dramatically lower in all patients( P < 0.01 ). Also, the serum level of CRP and BNP was significantly lower in the treatment group when compared with that of control group(P < 0.01 ). Conclusions :Short-term use of atorvastatin can decrease serum levels of CRP and BNP in Patients of HF with DCM, and improve cardiac function. The mechanism of action may be related to anti-inflammatory effeet. It may be useful to short-term use of statin therapy.
    • 贾鹏; 郭万华; 杜明华; 高玲
    • 摘要: 目的 探讨门控心肌灌注显像评价肥厚型心肌病患者心肌缺血情况的临床应用价值.方法 选取核素心肌灌注显像均为阳性的69例临床确诊的肥厚型心肌病住院患者,分为冠状动脉造影阳性(管腔狭窄≥50%)和阴性(管腔狭窄<50%)2组,对比其心肌缺血的门控心肌灌注显像特点,并进行两样本t检验.结果 冠状动脉造影阳性组19例,其中9例表现为可逆性心肌缺血,10例表现为不可逆性心肌缺血;8例射血分数(EF)升高,为(69.1±2.8)%,11例下降,为(42.8±2.1)%.冠状动脉造影阴性组50例,其中37例表现为可逆性心肌缺血,13例表现为不可逆性心肌缺血;38例EF值升高,为(70.8±4.0)%,12例下降,为(48.9±2.7)%.2组缺血范围[(29.7±17.8)%与(24.1±16.0)%]、缺血严重程度和EF值组间差异均有统计学意义(t=9.28,16.51和2.65,P<0.001,<0.001和<0.01).结论 门控心肌灌注显像发现肥厚型心肌病合并冠心病患者心肌缺血情况要严重于无合并冠心病患者;冠心病对于改变肥厚型心肌病的病理生理过程、自然病程和预后可能起重要作用.%Objective To evaluate the value of gated ~(99)Tc~m-methoxyisobutylisonitrile (MIBI) myocardial perfusion imaging in detection of myocardial ischemia in hypertrophic cardiomyopathy.Methods Sixty-nine patients with clinically proven hypertrophic cardiomyopathy were divided into 2 groups using coronary angiogram as "gold standard":positive group(n=19,narrowing≥50%) and negative group (n=50,narrowing<50%).Gated ~(99)Tc~m-MIBI myocardial perfusion imaging was performed and positive in all 69 patients (41 males,28 females,aged 35-75 years).Comparative analysis between the two groups was carried out using t-test.Results In the positive group,reversible and irreversible perfusion defects were detected in 9 and 10 patients,respectively.Left ventricular ejection fraction (LVEF) increased to (69.1±2.8)% in 8 patients and decreased to(42.8±2.1)% in 11 patients.In the negative group,reversible and irreversible perfusion defects were found in 37 and 13 patients,respectively.LVEF increased to(70.8±4.0)% in 38 patients and decreased to(48.9±2.7)% in 12 patients.The values of ischemic area,severity and extent of perfusion defect,and LVEF were significantly different between the two groups(t=9.28,16.51,2.65;P<0.001,<0.001,<0.01,respectively).Conclusions Gated ~(99)Tc~m-MIBI myocardial perfusion imaging is valuable in assessing patients with hypertrophic cardiomyopathy.Detection for the presence or absence of coexisting coronary artery disease and myocardial ischemia has an important prognostic indication and management indication for these patients.
    • 袁建松; 乔树宾; 田月琴; 韩萍萍; 张万春; 杨伟宪; 高润霖; 陈纪林; 杨跃进
    • 摘要: Objective To evaluate the use of gated SPECT in patients with hypertrophic obstructive cardiomyopathy (HOCM) and the effects of percutaneous transluminal septal myocardial ablation (PTSMA) on myocardial perfusion.Methods 99 Tcm-methoxyisobutylisonitrile (MIBI) and 18F-fluorodeoxyglucose (FDG) images were performed in 31 HOCM patients before PFSMA and in 15 patients 3-7 d after PTSMA.The images in different left ventricular segments were analysed by using scores.Results In 99Tcm-MIBI images, uptake decreased at the septal regions in 12 HOCM patients (80.0%, 12/15) after PTSMA, 18F-FDG images also showed decreased uptake at the septal regions in 5 HOCM patients (33.3%, 5/15) after PTSMA.Conclusion 99Tcm-MIBI images might be an important method to evaluate PTSMA results, and 18 F-FDG images showed important value as reference.%目的 了解肥厚梗阻型心肌病(HOCM)患者行经皮室间隔心肌化学消融术(PTSMA)前后心肌灌注和代谢的变化.方法 对31例HOCM患者在PTSMA前进行了99Tcm-甲氧基异丁基异腈(MIBI)灌注显像和18F-脱氧葡萄糖(FDG)代谢显像,其中15例进行术后急性期(3~7d)99Tcm-MIBI灌注显像和18F-FDG代谢显像复查,以半定量记分法评价各室壁节段灌注和代谢变化情况.结果 99Tcm-MIBI灌注显像:90.3%(28/31)的HOCM患者PTSMA术前室间隔放射性浓聚,PTSMA术后80.0%(12/15)的HOCM患者室间隔灌注有不同程度的减低;18F-FDG代谢显像在PTSMA术前54.8%(17/31)的患者表现为代谢异常,术后33.3%(5/15)的患者代谢异常降低,以间隔后段最为明显.结论 99Tcm-MIBI SPECT灌注显像可作为评价PTSMA术后效果的重要手段.18F-FDG代谢显像有重要的参考价值.
    • 许旭东; 宋晓伟; 荆清; 秦永文
    • 摘要: 目的:分析大鼠心肌肥厚过程中microRNAs(miRNAs)的表达变化.方法:通过肾上腹主动脉缩窄(abdominal aortic constriction,AAC)的方法建立大鼠心肌肥厚模型.实时定量PCR检测大鼠肥厚心肌中miRNAs的表达变化.结果:大鼠AAC后1周,心重/体重比以及心肌肥厚标志分子心房钠尿肽、β-重链肌球蛋白的编码基因Nppa、myh7表达明显升高,表明心肌肥厚模型建立成功;对肥厚心肌的miRNAs表达检测表明,miR-199a表达显著升高,miR-1、miR-133、miR-181a及miR-499表达显著降低.AAC 后4周,miR-21、miR-24和miR-214表达显著升高,miR-181a表达显著降低, miR-23a、miR-133、miR-145、miR-199a和miR-499表达无明显改变.结论:心肌肥厚后miRNAs表达发生改变,可能在心肌肥厚病理过程中发挥着重要的调节作用.
    • 孙牧川; 李亚明; 李娜; 刘浩; 尹雅芙; 李德顺; 梁晓光; 王勃
    • 摘要: 目的 探讨肥厚型心肌病(HCM)患者心肌99Tcm-甲氧基异丁基异腈(MIBI)早、晚期清除率的变化以及早、晚期清除率与左室壁心肌肥厚程度的关系.方法 对临床确诊为HCM的15例患者行99Tcm-MIBI静态平面及门控SPECT显像.比较HCM患者99Tcm-MIBI早期(注药后90 min)及晚期(注药后4 h)清除率与健康对照组(健康志愿者12名)间的差异(采用SPSS 13.0软件,行t检验),并对早、晚期清除率与左室壁心肌肥厚程度行Pearson直线相关性分析.结果 HCM患者组早期及晚期心肌99Tcm-MIBI清除率分别为(27.77±2.60)%及(42.66±3.30)%,健康对照组分别为(18.90±3.70)%及(31.27±4.04)%,2组比较差异均有统计学意义(t值分别为-7.320,-8.069,P均<0.01).HCM患者组左室壁肥厚心肌最大厚度为(26.53±6.57)mm,健康对照组为(15.92±1.29)mm,2组比较差异亦有统计学意义(t值为-6.110,P<0.01).HCM早期及晚期清除率与左室壁肥厚心肌最大厚度间有较好的相关性(r值分别为0.611及0.873,P<0.05和<0.01).结论 HCM患者心肌99Tcm-MIBI早期及晚期清除率明显高于健康对照组,且早、晚期清除率与左室壁心肌肥厚程度均有一定的相关性.%Objective The aims of this study were two. One was to investigate the changes in early and late washout rates of 99Tcm-methoxyisobutylisonitrile (MIBI) in patients with hypertrophic cardiomyopathy (HCM) and the other was to analyze the relationships between early and late washout rates and the hypertrophic wall thickness of left ventricle. Methods Fifteen patients who were clinically diagnosed with HCM underwent 99Tcm-MIBI static planar and gated SPECT imaging. Early (90 min after the intravenous injection) and late (4 hafter the intravenous injection) washout rates of 99gTcm-MIBI between HCM and normal control groups (n=12)were compared. Linear correlation (Pearson) was assessed between early and late washout rates and the hyper-trophic wall thickness of left ventricle treasured by gated SPECT. Results Statistically higher early and late washout rates of 999Tcm-MIBI were observed in HCM than in normal control groups [(27.77±2.60)% vs (18.90±3.70)%, t=-73.20, P<0.01 and (42.66±3.30) vs (31.27±4.04)%, t=-8.069, P<0.01]. Moreover, statistically higher maximum wall thickness over left ventricle was observed in HCM than in normal control groups [(26.53±6.57)mm vs (15.92±1.29) mm, t=-6.110, P<0.01]. Further-more, significantly positive correlation between early and late washout rates and the maximal wall thickness of left ventricle obtained by gated SPECT was demonstrated (r=0.611, P<0.05 and r=0.873, P<0.01). Conclusions Both early and late washout rates of 99Tcm-MIBI and maximum wall thickness over left ventricle were significantly higher in patients with HCM than in control groups. The higher the maximum wall thickness over left ventricle, the higher early and late washout rates in 99Tcm-MIBI studies was noted.
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