致心律失常性右室心肌病心力衰竭期的病理特点分析

目的 通过分析致心律失常性右室心肌病(ARVC)心力衰竭期的病理改变,以进一步了解其临床分期与病理表型的关系.方法 从2004-2007 年在阜外心血管病医院接受心脏移植的心力衰竭病例中,收集病理诊断为ARVC的受体心脏8例,测量心脏重量,评价左右心室心腔扩张、心肌细胞肥大、脂肪浸润、纤维化、附壁血栓和伴发心肌炎等指标,注意左心室受累情况,并进行病理分型.结果 8例中的7例为经典型(即右心室改变为主),1例为左优势型(左心室改变为主),未见双室型病例.组织学均为纤维脂肪型,未见单纯脂肪型病例.经典型病例的右心室中、重度扩张,少数有室壁瘤形成,其中6例伴左心室受累,受累左心室轻、中度扩张,心肌广泛间质纤维化,部分病例伴替代性疤痕,而脂肪浸润量小,多局限于心外膜下.左心室心肌细胞肥大普遍.而左优势型的左心室重度扩张,弥漫间质纤维化和局部透壁性脂肪浸润.8例中3例左心室明显肥厚,3例查见双室附壁血栓,1例伴局灶性心肌炎.结论 ARVC心力衰竭期的左心室受累多见而严重,左心室间质纤维化突出,心肌细胞肥大明显,但脂肪替代少见和局限.左、右心室多扩张,可见附壁血栓,应注意与扩张型心肌病等鉴别.     

甲亢性心肌病兔血清cTn-I水平变化及其与心功能的相关性

目的 探讨左心室不同构型的甲状腺功能亢进症(简称甲亢)性心肌病兔血清肌钙蛋白I(cTn-I)水平及其与心功能的相关性。方法 对实验组兔(20只)腹腔注射左甲状腺素 建立甲亢动物模型,对照组兔(10只)每日腹腔注射生理盐水至实验结束。对两组兔行超声心动图检查,同时测定血清中cTn-I水平。实验组依据超声参数分为向心性肥厚(CH)亚组和离心性肥厚(EH)亚组。结果 与对照组比较,CH亚组二尖瓣环平均舒张期峰值速度(Ve)减低(P<0.01),EH亚组二尖瓣环平均收缩期峰值速度(Vs)、Ve明显减低(P均<0.01)。CH亚组和EH亚组血清cTn-I水平明显高于对照组(P均<0.01),EH亚组较CH亚组血清cTn-I水平进一步升高(P<0.01)。cTn-I与Vs及Ve呈负相关(P均<0.01)。结论 血清cTn-I浓度变化结合超声心动图可较敏感地反映甲亢性心肌病心肌损害的有无及其严重程度。     

实时三维超声心动图评价左室心肌致密化不全患者左心室收缩同步性的应用价值

目的 探讨实时三维超声心动图时间-位移参数及17节段时间-容积曲线参数指标在评价左室心肌致密化不全中的应用价值.方法 对10例左室心肌致密化患者(经磁共振证实)、20例正常者(对照组)行实时三维超声心动图检查.结果 左室心肌致密化不全组的17节段时间-容积曲线参数指标:左室16节段、12节段、6节段达最小收缩容积时间的标准差(Tmsv 16-SD,Tmsv 12-SD,Tmsv 6-SD),左室16节段、12节段、6节段达最小收缩容积时间的最大差值(Tmsv 16-Dif,Tmsv 12-Dif,Tmsv 6-Dif)以及左室16节段、12节段、6节段达最小收缩容积时间的标准差的校正值(Tmsv 16-SD%,Tmsv 12-SD%,Tmsv 6-SD%),左室16节段、12节段、6节段达最小收缩容积的时间最大差值的校正值(Tmsv 16-Dif%,Tmsv 12-Dif%,Tmsv 6-Dif%)较正常组显著增高,差异有统计学意义(P均＜0.05),左室心肌致密化不全组的时间-位移参数中位移平均值、最小值低于正常对照组,差异有统计学意义(P＜0.05).结论 实时三维超声心动图时间-位移参数显像及17节段时间-容积曲线参数指标能快速准确评价左室心肌致密化不全患者心肌节段功能及收缩同步性.

Abstract：

Objective To investigate the value of real-time three-dimensional echocardiography(RT-3DE) timing-excursion parametric index and 17 segment time-volume curves index in patients with leftventricular noncompaction(LVNC). Methods Ten patients with LVNC (proven by MRI) ,twenty subjects with normal LV function were examined by Philips iE33 with X3-1 probe. Results Parameter index (including Tmsv 16-SD,Tmsv 12-SD,Tmsv 6-SD,Tmsv 16-Dif,Tmsv 12-Dif,Tmsv 6-Dif,Tmsv 16-SD%,Tmsv 12-SD%, Tmsv 6-SD%, Tmsv 16-Dif%, Tmsv 12-Dif%, Tmsv 6-Dif%) of 17 segment time to minimal systolic volume was significantly higher in patients with LVNC than that in subjects with normal LV function( all P＜0.05). Average and minimum value of excursion was significantly lower in patients with LVNC than that in subjects with normal LV function ( P＜0.05). Conclusions RT-3DE with timeexcursion parameters and 17 segment of time- volume curve parameters can rapidly and accurately evaluate left ventricular systolic synchrony in patients with LVNC.     

Extracellular matrix proteins and matrix metalloproteinases differ between various right and left ventricular sites in end-stage cardiomyopathies

This study was undertaken to investigate whether there might be differences in the distribution of extracellular matrix (ECM) proteins and matrix metalloproteinases (MMPs), depending on their specific sites within the heart. We investigated 33 explanted human hearts, 15 with dilated cardiomyopathy (DCM) and 18 with ischemic cardiomyopathy (ICM). Transmural samples from the right ventricle, the interventricular septum and the left ventricle, either from near the apex or from near the base were taken from every heart. Frozen sections were processed for connective tissue staining and immunohistochemistry for collagens type I, III, IV, laminin and fibronectin, as well as MMP-1, -2 and -9. Volume densities of laminin in ICM as well as of fibronectin and collagen types I and IV in DCM showed significant differences between right and left ventricular sites. The volume densities of matrix proteins usually did not reveal significant differences among the three left ventricular sites tested in both DCM and ICM. MMPs partly showed differences between the right and the left ventricular myocardium. These results suggest that the distributions of ECM proteins and MMPs differ between the two ventricles in both end-stage DCM and ICM. This gives rise to the hypothesis that a specific pattern of ECM degradation exists in the right and left ventricular myocardium.     

Morphometric comparison of mitochondria and myofibrils of cardiomyocytes between hypertrophic and dilated cardiomyopathies

Summary We performed an ultrastructural, morphometric comparison of mitochondria and myofibrils of cardiomyocytes using endomyocardial biopsy specimens in hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM). Biopsies came from the right ventricular side of the interventricular septum in nine patients with HCM, nine with DCM, and nine controls with arrhythmia and/or ST depression. Morphometric analysis was carried out using electron microscopic photographs and an image analyser. Mitochondria were significantly greater in number and smaller in size in HCM than in the control group. In DCM, the size of mitochondria was also significantly smaller than in the control group, although their number was similar to that of the control group. No statistically significant difference was found regarding the size of mitochondria between HCM and DCM. The percentages of both mitochondrial and myofibrillar areas in cytoplasm were smaller in the DCM than the HCM and control groups, though no difference was seen between the latter two. The ratio of mitochondrial area to myofibrillar area was almost the same in each group. These results suggest increased mitochondrial function to match hypertrophic cardiomyocytes in HCM, and decreased mitochondrial function and cardiomyocytic contractility in DCM.     

儿童心肌病的磁共振特征

目的:分析儿童心肌病磁共振的临床影像学特点.方法:回顾性搜集我院64例临床诊断儿童心肌病患者,其中心内膜弹力纤维增生症(EFE)26例,肥厚型心肌病(HCM)14例,扩张型心肌病(DCM) 16例,限制型心肌病(RCM)4例,致心律失常性右室心肌病(ARVC)2例,心肌致密化不全(NVM)2例.采用1.5T磁共振扫描,不能配合的患儿用0.5 mL/kg水合氯醛镇静.所有患儿行黑血快速自旋回波(TSE)、亮血平衡稳态自由进动梯度回波(balanced SSFP)电影,26例行快速自旋回波心肌延迟增强,28例行相位敏感反转恢复(PSIR)心肌延迟增强扫描.结果:26例EFE均有左室心腔扩大,9例伴心肌变薄;16例心肌收缩运动幅度减低;左房增大7例;左室肌小梁增多增厚13例;瓣膜返流18例;心包积液7例,胸腔积液4例.14例HCM共有66个左室节段心肌增厚,室间隔的增厚14例,共累及45个节段,左室游离壁增厚12例,共累及21个节段.1例右室心尖部受累.左室内乳头肌异常4例.左室肌小梁增多增厚6例.16例DCM均有心腔扩大,左室心肌变薄,左房左室同时显著增大12例,8例为全心增大,仅左室增大2例.13例患者心室壁收缩运动幅度均明显减低.左室游离壁肌小梁增粗增厚11例.瓣膜返流13例.心包积液9例,胸腔积液7例.4例RCM,均为双心室受累,舒张运动受限,心室腔大小正常,双侧心房明显扩张,6例瓣膜返流.下腔静脉扩张4例.心包积液3例.2例ARVC,MRI均可见右室流出道扩张,局部室壁变薄,“手风琴征”阳性.2例NVM,左室心内膜及肌小梁明显增厚,交叉呈网,相邻心肌变薄,NC/C约3.1～7.5.6例延迟强化,HCM 1例,EFE 2例,DCM 3例.结论:儿童心肌病的临床表现多样化,多伴有心功能异常,磁共振影像学表现有一定的特点,两者密切结合可以作出诊断.     

儿童心肌致密化不全的临床与超声心动图特点

目的探讨儿童心肌致密化不全(NVM)的临床及超声心动图特点。 方法回顾性分析2013年8月至2017年8月,于四川大学华西第二医院经超声心动图诊断为NVM的74例患儿病例资料,总结儿童NVM临床特点与超声心动图特点。 结果①本组74例儿童NVM的主要门诊/入院诊断为：先天性心脏病占32.4%(24/74),NVM占28.4%(21/74),肺炎占21.6%(16/74)。主要临床表现为：心前区收缩期杂音(31.1%,23/74),心力衰竭(25.7%,19/74),心影增大(17.6%,13/74)及心律失常(4.1%,3/74)等。②本组74例儿童NVM的超声心动图特点为：91.9%(68/74)为左室型NVM(LVNC);左心室受累为95.9%(71/74),以心尖合并心尖部下壁及侧壁受累、心尖受累为主。超声心动图测量致密心肌厚度为(2.28±0.86) mm,非致密心肌厚度为(6.94±1.75) mm,非致密心肌厚度与致密心肌厚度比值为(3.50±1.76)。本组大部分病例心腔增大,并且以左房、室为主;左心室收缩功能减低为33.8%(25/74)。孤立性NVM为60.8%(45/74),非孤立性NVM主要合并先天性心脏病(25.7%,19/74)及扩张性心肌病(8.1%,6/74)。74例患儿均无心内血栓形成。 结论儿童NVM的临床表现无特异性,临床诊断较困难。超声心动图能观察心肌形态、评价心肌运动及诊断心内合并畸形,是确诊儿童NVM的首选影像学检查方法。     

国内外儿童心肌病研究现状及前沿分析

背景　儿童心肌病是一种预后差、病死率及致残率较高的疾病。历年来,国内外研究者对其进行了积极的探索与研究,发表了大量相关文献。目的　分析国内外儿童心肌病研究的现状及趋势,为相关研究提供参考。方法　英文文献以Web of Science核心合集数据库（SCI-Expanded、SSCI、ESCI）、PubMed数据库为数据来源,中文文献以中国知网、万方数据知识服务平台为数据来源,检索儿童心肌病相关文献,检索时间为建库至2018-04-12。采用Notepad++软件补充缺失信息,中、英文文献分别采用EndNote X7软件、CiteSpace 5.2.R1软件去重处理。采用Bibexcel软件统计刊载文献的期刊。采用CiteSpace 5.2.R1软件分析文献的发表年代、国家/地区、研究机构、期刊、研究热点及前沿。结果　共纳入9 816篇儿童心肌病领域的英文文献,数量整体呈上升趋势,2014-2017年呈下降趋势;共纳入585篇中文文献,2014-2017年呈下降趋势;英文文献中有362篇为我国发表,文献数量呈上升趋势,2015-2017年增加幅度较大。1997-2017年共160个国家/地区发表文献,美国的文献数量最多,中国位居第9名。英文文献数量前5名的研究机构主要位于美国,文献数量最多的为Harvard University（美国）,研究机构合作网络较为密集,与其他机构合作较多;中文文献及我国发表的英文文献的数量最多的研究机构为上海交通大学,研究机构合作网络稀疏,与其他机构合作较少。9 816篇英文文献共发表在1 769种期刊,585篇中文文献共发表在321种期刊。英文文献的研究热点为体外膜氧合、心血管疾病、基因型-表型相关性、有价值的预后工具和临床结局;国内中英文文献的聚类标签即研究热点为蒽环霉素诱导的心脏毒性、ATPase缺陷型线粒体内膜蛋白ATAD3A、肌球蛋白结合蛋白C、功能突变、TAZ突变、儿童白血病、基因产物表达、慢性病和家族性房间隔缺损。儿童心肌病的研究前沿为科学管理、临床结局、心血管磁共振、心室辅助装置、遗传学与相应指南。结论　儿童心肌病研究成果显著,我国该领域的研究逐渐面向国际,但研究水平仍待提升,未形成稳定的大规模研究方向,应注重机构间合作。     

蛛网膜下腔出血后的早期心脏异常

约50%～70%的患者在蛛网膜下腔出血后会继发心脏异常,而心脏异常又会加重脑组织缺血缺氧,可能还会促进迟发性缺血缺氧性脑病的发生.很多研究人员借助心电图、心脏彩超和血清心肌酶检测来观察这种变化的临床特征、持续时间及其与预后的关系.文章就蛛网膜下腔出血早期心脏异常的原因以及心电图、心脏彩超和心肌酶谱改变的临床特征方面的研究进展做一综述.