Electro‐vectorcardiographic demonstration of bifascicular block associated with ventricular preexcitation

Down syndrome occurs more frequently in the offsprings of older pregnant women and may be associated with atrioventricular septal defect. This refers to a broad spectrum of malformations characterized by a deficiency of the atrioventricular septum and abnormalities of the atrioventricular valves caused by an abnormal fusion of the superior and inferior endocardial cushions with the midportion of the atrial septum and the muscular portion of the ventricular septum.     

急性心肌梗死后左心室游离壁破裂患者临床特点分析

目的探讨急性心肌梗死(acute myocardial infarction,AMI)后发生左心室游离壁破裂(free wall rupture,FWR)患者的临床特点及危险因素。方法入选2010年12月至2018年12月南京医科大学附属南京医院明确诊断为AMI的患者4221例,其中发生FWR的患者81例(FWR组),按照1:5匹配原则,随机选取未发生心脏破裂(且未发生室间隔穿孔)的患者405例作为非FWR组,比较两组患者临床基线资料及预后。结果(1)AMI患者中出现FWR的风险为1.9%。(2)与非FWR组相比,FWR组患者年龄偏大,前壁心肌梗死较多,心功能更差,接受手术治疗(包括经皮支架植入和冠状动脉旁路移植术)的患者比例偏低,且所有患者均出现院内死亡。(3)91.4%的患者FWR发生在AMI起病1周之内,其中24 h内发生FWR 37例(45.7%)。(4)COX回归分析发现,年龄(HR=1.055,95%CI:1.032~1.078,P<0.001)、急性前壁心肌梗死(HR=1.907,95%CI:1.211~3.002,P=0.005)和手术治疗(HR=0.126,95%CI:0.072~0.220,P<0.001)是AMI患者出现FWR的独立预测因子。结论AMI患者发生FWR的风险约为1.9%,而且通常发生在心肌梗死1周内,高龄和急性前壁心肌梗死患者容易发生FWR,而手术治疗能明显降低FWR风险。     

非阻塞性冠状动脉粥样硬化患者血管内皮功能与左室舒张功能的关系

目的 探讨非阻塞性冠状动脉粥样硬化患者血管内皮功能与左室舒张功能的关系。方法 选取2013.6～2015.6宣武医院心脏内科收治的因胸痛疑诊冠心病、经冠脉造影检查证实为非阻塞性冠状动脉粥样硬化症（冠状动脉狭窄＜50%）患者119例，其中男性55例，女性64例，年龄31～85岁，平均年龄60岁。专业培训人员采用标准化问卷调查记录患者临床资料，行超声心动图和肱动脉血流介导内皮依赖性血管舒张功能（FMD）检测，分为FMD正常组（FMD＞10%）和减低组（FMD≤10%）。结果 两组人群基本临床资料无显著性差异（P值均＞0.05）。左室结构指标室间隔厚度、左室后壁厚度、左室舒张末内径、左室质量指数两组间比较无显著性差异（P值均＞0.05）。左室收缩功能指标每搏输出量、心输出量、左室短轴缩短率及射血分数组间比较也无显著性差异（P值均＞0.05）。FMD降低组较FMD正常组E/A比值显著减低（P＜0.001）、E/e’比值显著升高（P=0.006），控制年龄等影响因素，偏相关分析显示FMD与E/A比值存在显著正相关（r=0.261，P=0.005），FMD与E/e’呈显著负相关（r=-0.203，P=0.033）。结论 在非阻塞性冠状动脉粥样硬化人群中，内皮功能与左室舒张功能密切相关，内皮功能不全患者左室舒张功能明显减退。     

益气养阴解毒方联合环磷腺苷葡胺注射液治疗病毒性心肌炎35例

目的:探讨益气养阴解毒方联合环磷腺苷葡胺注射液对病毒性心肌炎患者心功能及外周血清肿瘤坏死因子-α(tumor necrosis factor-α,TNF-α)、转化生长因子-β1(transforming growth factor-β1,TGF-β1)和干扰素-γ(interferon-γ,IFN-γ)水平的影响。方法:将70例病毒性心肌炎患者按照随机数字法分为对照组和观察组,每组各35例。两组患者均给予常规治疗,对照组在常规治疗的基础上给予环磷腺苷葡胺注射液静脉滴注,观察组在对照组的基础上联合益气养阴解毒方治疗。比较两组患者的临床疗效及治疗前后左室短轴缩短分数(left ventricular shortening fraction,LVFS)、左心室射血分数(left ventricular ejection fraction,LVEF)、心脏指数(cardiac index,CI)、TNF-α、TGF-β1和IFN-γ水平。结果:观察组有效率为88.57%,对照组有效率为68.57%,两组患者有效率比较,差异有统计学意义(P<0.05)。两组患者治疗后LVEF、LVFS高于本组治疗前,且观察组高于对照组,差异具有统计学意义(P<0.05)。两组患者治疗后TGF-β1、TNF-α水平低于治疗前比较,IFN-γ高于治疗前,且观察组TGF-β1、TNF-α低于对照组,IFN-γ高于对照组,差异均有统计学意义(P<0.05)。两组患者不良反应发生率比较,差异无统计学意义(P>0.05)。结论:益气养阴解毒方联合环磷腺苷葡胺注射液治疗病毒性心肌炎疗效更显著,可减轻机体的炎症反应,恢复患者心功能。     

Clinical and molecular spectrum of Wiedemann-Steiner syndrome,an emerging member of the chromatinopathy family

Wiedemann-Steiner syndrome (OMIM #605130) is a rare congenital malformation syndrome characterized by hypertrichosis cubiti associated with short stature; consistent facial features, including long eyelashes, thick or arched eyebrows with a lateral flare, wide nasal bridge, and downslanting and vertically narrow palpebral fissures; mild to moderate intellectual disability; behavioral difficulties; and hypertrichosis on the back. It is caused by heterozygous pathogenic variants in KMT2A. This gene has an established role in histone methylation, which explains the overlap of Wiedemann-Steiner syndrome with other chromatinopathies, a heterogeneous group of syndromic conditions that share a common trigger: The disruption of one of the genes involved in chromatin modification, leading to dysfunction of the epigenetic machinery.