An infant with Costelio syndrome complicated with fatal hypertrophic obstructive cardiomyopathy

We report a 3-month-old girl with Costelio syndrome complicating fatal hypertrophic obstructive cardio-myopathy. She had typical findings of this syndrome, slight dyspnea and persistent wheezing. Doppler echocardiography revealed asymmetric septal hypertrophy and systolic anterior movement of the anterior mitral leaflet. There was grade 1 mitral regurgitation. Although once her heart failure had been controlled medically, she died suddenly following deterioration of her heart condition. Costelio syndrome can complicate fatal hypertrophic obstructive cardiomyopathy.     

氨基胍对糖尿病大鼠心脏功能及心肌超微结构的影响

目的 :探讨非酶糖化抑制剂 氨基胍 (amin oguanidine ,AG)对链尿佐菌素 (streptozotocin ,STZ)糖尿病大鼠心肌的保护作用 ,为糖尿病心肌病的防治提供参考。方法 :建立STZ糖尿病大鼠模型 ,随机分为对照组、糖尿病组和氨基胍治疗组 ,AG剂量为15 0mg·kg-1·d-1。 12周时测定大鼠血清果糖胺含量、心脏重量指数、左室内压最大上升和下降率 (±dp dtmax)值 ,电镜观察心肌超微结构 ,测量心肌毛细血管基底膜厚度。结果 :糖尿病组大鼠血清果糖胺含量、心脏重量指数明显增高 ,±dp dtmax降低 ;超微结构显示心肌肌原纤维排列紊乱 ,线粒体肿胀变性 ,间质胶原增生 ,微血管内皮细胞肿胀、基底膜明显增厚。氨基胍治疗组血清果糖胺含量降低、心脏重量指数明显下降、±dp dtmax 值上升 ,微血管基底膜增厚减轻 ,间质胶原减少 ,心肌细胞超微结构异常减轻。结论 :糖尿病时存在心脏功能异常、心肌肥厚和超微结构的改变 ,早期应用AG在一定程度上可阻抑糖尿病心肌病变的发展。     

儿童先天代谢异常伴发的肥厚型心肌病

儿童先天代谢异常是导致肥厚型心肌病（HCM）的重要病因, 掌握每种疾病的特征性表现有助于正确的诊断及鉴别诊断。多数先天代谢异常伴发HCM属于常染色体隐性遗传,少数为常染色体显性遗传X连锁遗传方式,部分线粒体病呈母系遗传。常规心电图、超声心动图等心脏检查即可以为部分疾病的基础病因找到诊断线索。随着酶替代疗法等新的治疗方法的不断进步,加强心脏评估,恰当地针对原发病治疗,多学科协同合作将为越来越多的患者生存提供可能。     

O-GlcNAc修饰在糖尿病心肌病中的作用及机制研究进展

<正>糖尿病是一种慢性代谢性紊乱疾病。据统计,我国2021年糖尿病患者数量已高达1.41亿[1]。糖尿病引发的心血管意外已成为患者死亡的主要原因,且死亡率明显高于非糖尿病患者[2]。糖尿病心肌病(diabetic cardiomyopathy, DCM)是一种因长期高血糖引起的心脏结构和功能异常的疾病,与多种信号通路有关。     

黄芪精口服液对围生期心肌病患者心功能不全及辅助T细胞调节作用的研究

[摘要]目的探讨中药黄芪精口服液对围生期心肌病患者心功能不全及辅助T细胞的调节作用。方法36例围生期心肌病住院患者随机分为常规治疗对照组与加用黄芪精口服液治疗组,治疗8周后应用超声心动图观察患者心功能,检测心功能不全实验室相关指标N末端脑钠肽前体（NT—proBNP）及免疫相关指标IFN-γ、IL-2、IL-4、IL-10并进行疗效的比较。结果治疗8周后2组心率、左室收缩末内径和左室舒张末内径均较治疗前显著减小（P均〈0．05）,左室射血分数、左室短轴缩短率、每搏量和心排血量较治疗前显著提高（P均〈0．05）,以上各项指标治疗组均较对照组改善明显（P均〈0．05）;NT-proBNP在治疗8周后2组皆有显著下降,治疗组下降更显著（P〈0．05）;血清IFN-γ和IL-2明显降低,IL-4和IL-10水平明显升高。结论黄芪精口服液配合常规治疗围生期心肌病患者可以显著改善心功能,提高治疗效果,并使辅助T细胞的平衡从Th1向Th2移动。     

糖尿病性心肌病的发病机理探析

目的：探讨糖尿病性心肌病（DC）的发病机制。方法：查阅研读近年的大量文献，将所获信息整理分类，得出结论。结果：糖尿病性心肌病（DC）是以持续高血糖、RAS的作用、Ca^2＋转运异常等因素共同作用，使存心肌细胞代谢障碍，导致心脏结构和功能障碍，心肌细胞凋亡，心肌间质纤雏化，引起左心室舒张收缩功能不全，心室肥厚重构，功能渐丧而致心力衰竭。结论：从以上各方面探讨了DC的发病机制，希望能为DC早期防治提供理论依据。     

翁维良老师治疗酒精性心肌病经验

翁维良教授从事中西医内科临床工作50余年,擅用活血化瘀治疗心血管病及各种慢性疑难疾病。结合多年临床经验,翁老认为酒精性心肌病病机多属以心气(阳)亏虚为主,湿热、瘀血、痰饮、毒热内蕴为标的虚实夹杂证。治疗时,在完全戒酒的基础上,配合中药益气温阳扶助正气,同时辨证应用健脾、祛湿、活血、祛痰、解毒等祛除实邪治法,疗效肯定。文章旨在介绍翁维良教授辨治酒精性心肌病的临证经验。     

Electrocardiographic and Echocardiographic Features That Distinguish Anomalous Origin of the Left Coronary Artery from Pulmonary Artery from Idiopathic Dilated Cardiomyopathy

Many authors have reported noninvasive means of diagnosing anomalous left coronary artery from pulmonary artery (ALCAPA) and differentiating ALCAPA from idiopathic dilated cardiomyopathy (DCM). Systematic evaluation using these noninvasive diagnostic modalities is not available. To distinguish between ALCAPA and DCM using a systematic approach, we examined 23 patients with ALCAPA (age 1 month to 23 years, median 7 months) and 23 patients with DCM (age 5 days to 16 years, median 6.6 months). Standard 12-lead electrocardiograms (ECG) and 2-dimensional (2-D) and color Doppler echocardiograms were performed. A logistic regression model was applied using ALCAPA diagnosis as the dependent variable and ECG and echocardiographic findings as independent variables. A scoring system was created to establish the ALCAPA diagnosis based on results from the logistic regression. On the logistic regression, the ECG feature of QT pattern in aVL (Q wave ≥ 3 mm deep with an inverted T wave) and echocardiographic features of right coronary artery diameter to aortic annulus ratio ≥ 0.14, increased papillary muscle echogenicity, and Doppler color flow of LCA from aorta or pulmonary artery were the most significant differentiating features between the ALCAPA and DCM groups. A scoring system was developed using the previous five variables and assigning a score of 1 to each variable (−1 to Doppler color flow of LCA from aorta). The scoring system had sensitivity of 100% and specificity of 91% for ALCAPA diagnosis. Compared with previous reported diagnostic features in differentiating ALCAPA and DCM, the scoring system had a much higher specificity and positive predictive value. In conclusion, we selected the most useful ECG and echocardiographic features to differentiate between ALCAPA and DCM and created a scoring system to aid clinical diagnosis. This scoring system may be useful in evaluating children with acute congestive heart failure.     

不同透析方式对尿毒症患者心脏结构的影响

目的:比较血液透析(hemodialysis,HD)与腹膜透析(peritoneal dialysis,PD)治疗对尿毒症患者心脏结构的影响。方法:选择我院住院诊断为尿毒症的患者42例,随机分为血液透析组和腹膜透析组各21例,分别于透析治疗后1个月及透析后6个月行心脏彩色多普勒检查,测量常规指标:左心房内径(LA)、左室舒张末径(LVEDd)、左室后壁舒张末厚度(LVPWd)、室间隔舒张末厚度(IVSd)。结果:血液透析及腹膜透析治疗1个月后较透析前LA、LVEDd、IVSd及LVPWd有明显减小(P<0.05),6个月后LA、LVEDd、IVSd及LVPWd较1个月时亦有所减小(P<0.05);透析治疗1个月及6个月后腹膜透析组对心脏结构改善均明显优于血液透析组(P<0.05)。结论:腹膜透析及血液透析均能改善尿毒症患者的心脏结构,而腹膜透析效果更优于血液透析,随着透析时间延长,心脏结构改善更明显。