维持性血液透析患者心脏结构及功能与骨形态发生蛋白2的关系

目的:分析维持性血液透析(MHD)患者心脏结构及功能与骨形态发生蛋白2(BMP-2)的关系。方法:纳入2016年1月-2017年6月在武汉大学中南医院血液透析中心行MHD治疗的患者,运用酶联免疫吸附技术检测血清BMP-2,记录一般资料、实验室检查及心脏多普勒彩超结果。观察心脏瓣膜钙化组和非钙化组、左心室肥厚组和非肥厚组患者各指标,分析心脏结构及功能改变的危险因素及与BMP-2的关系。结果:共纳入109例MHD患者,其中32.11%患者发生心脏瓣膜钙化,57.8%发生左心室肥厚,98.63%出现舒张功能不全,BMP-2中位数32.64(27.42,58.52)pg/mL。心脏瓣膜钙化组BMP-2水平、血磷、钙磷乘积、铁蛋白、年龄、透析龄均高于非钙化组(P<0.05)。左心室肥厚组血红蛋白和左心室射血分数低于非肥厚组(P<0.05),铁蛋白和透析龄高于非肥厚组(P<0.05),而BMP-2在两组间无差异。Logistic回归分析显示,高BMP-2是心脏瓣膜钙化的危险因素。Spearman相关分析示,二尖瓣血流舒张早期最大流速/二尖瓣心房收缩期最大血流比(E/A)与收缩压呈正相关,与血红蛋白呈负相关。受试者工作特征(ROC)曲线分析显示,血清BMP-2对于预测MHD患者心脏瓣膜钙化的曲线下面积为0.758,95%CI为0.651～0.865,P<0.001,最佳截取值为42.04 pg/mL,灵敏度为0.600,特异度为0.824。结论:MHD患者心脏瓣膜钙化与血清BMP-2密切相关,高水平的BMP-2是心脏瓣膜钙化的危险因素,且BMP-2对心脏瓣膜钙化具有一定诊断价值。但MHD患者左心室肥厚及舒张功能不全与BMP-2无关。     

体外循环心脏直视手术后急性消化道大出血

急性消化道大出血是体外循环心脏直视手术后较少见的并发症。唐斌等报告1,060例体外循环心脏手术中,术后并发急性消化道大出血者5例(0.47％)。我院自1980年8月至1988年6月共施行体外循环心脏直视手术206例,术后发生此并发症者二例,占0.97％。现报告如下。例1 女,12岁,诊断为先心房间隔缺损,既往无溃疡病史。于1982年2月15日在低温体外循环下行房间隔缺损修补术。术后出     

心房间隔高位缺损伴室间隔缺如一例

心房间隔高位缺损伴室间隔缺如一例姜文霞汤如勇附属甘泉医院病理科（上海，２０００６５）关键词心脏缺损，先天性；尸体解剖分类号Ｒ３６１．１新生儿尸解发现先天性心脏严重畸形—心房间隔高位缺损伴室间隔缺如较罕见，现报告如下。１临床资料患儿男性，出生后１１２ｈ...     

心脏复杂畸形外科手术治疗11例总结

本文11例心脏复杂畸形中均为二种以上的心内畸形,其中紫绀型先天性心脏病8例,非紫绀型先天性心脏病3例。病种如下:法乐氏三联症3例(1例并有动脉导管未闭),法乐氏四联症、五联症各2例,双腔右心室(均合并室间隔缺损)2例,右冠状动脉——右房瘘并房间隔缺损1例,心房间隔缺损(Ⅱ孔中央型)并二尖瓣前瓣裂1例。本文着重讨论了心脏复杂畸形病人术前诊断方法;手术中对各种畸形矫治的方法。并讨论了心内直视手术中对心肌保护的重要性及其心肌保护的综合方法的优点及其效果。     

SciMed膜式氧合器在我院临床应用

香港亚洲心肺机公司技术人员,代表美国SciMed生命系统有限公司,前来我国进行技术交流及商品推销,于10月12日到达南京,与南京医学院附属医院、南京部队总医院的胸外科医师及有关人员进行了座谈。介绍了该公司的膜式氧合器(膜肺)性能及临床应用问题,并先后在二个医院作了动物实验和临床应用。用SciMed膜式氧合器体外循环装置,为4例先天性心脏病患者施行了根治手术,包括原发孔型心房间隔缺损、继发孔型心房间隔缺损各1例,心室间隔缺损2例。心肺转流时间23～45分钟,术中术后血气分析及血生化指标     

SCN5A基因突变与心律失常的研究进展

近年来分子生物学及分子电生理的迅速发展,开创了心律失常机制研究新纪元。心律失常与离子通道基因表达异常明确相关,多个离子通道基因的突变可引起各种心律失常。目前,已知绝大多数的原发性心电异常都是由编码各主要离子通道亚单位的基因突变引起的,因此,这类病可称为“离子通道病”。Nav1.5通道是人类主要的心脏钠离子通道类型,负责动作电位的起始和传播,由SCN5A基因编码。自从在长QT综合征(long QT syndromeL,QTS)家系中发现心脏钠离子通道α亚基的编码基因SCN5A第一个突变以来,目前已经发现数百个突变与一系列遗传性心律失常有关,如长QT综合征3型(LQT3)、Brugada综合征、进展性心脏传导阻滞(PCCD)、扩张型心肌病(DCM)、婴儿猝死综合征(SIDS)等。近年来发现SCN5A基因突变与病态窦房结综合征(SSS),房性心律失常(心房颤动,心房静止),室性心律失常和起搏夺获不良密切相关。本文将详细阐述近年来SCN5A基因突变在SSS、房性心律失常(心房颤动,心房静止)、起搏夺获不良、室性心律失常和长QT综合征3型的研究进展,SCN5A功能获得性和功能丧失致病突变潜在的机制以及目前存在的问题和挑战。      

胸腔镜下房间隔缺损修补术的护理配合

房间隔缺损(Atrial septal defect,ASD)为心脏胚胎期心房间隔发育不全,出现房间隔缺损.早期可无症状,随年龄增加可出现劳累后心悸、气急、乏力、咳嗽及咯血等;晚期可出现心力衰竭.对于ASD患者的手术治疗常规是在直视体外循环下行缺损修补术.我院2003年成功地实施了3例电视胸腔镜下(Video-assisted Thoracoscopic surgery,VATS)房间隔缺损修补术,效果满意,术后患者恢复良好.现将手术护理方面配合要点总结如下.     

BMP-2在骨骼生长发育中的作用及研究进展

骨形态发生蛋白(BMPs)是一类多功能的生长因子，属于转化生长因子-β (TGF-β)超家族，其在心脏、神经、软骨的形成以及成骨等发育过程中都是必不可少的。而骨形态发生蛋白2 (BMP-2)作为第一个被发现的BMP，近年来已被广泛研究，其在骨骼发育、成年后骨形成与改建过程中发挥着关键性作用。本文就BMP-2的结构与功能、其激活和调节的信号通路、临床应用和并发症等方面做一综述，旨在为基础研究和临床治疗提供帮助。     

间隔缺损术后Ⅲ度房室传导阻滞发生的临床探讨

目的探讨间隔缺损术后发生Ⅲ度房室传导阻滞(CAVB)的原因、防治措施与转归。方法对178例间隔缺损患儿术后进行回顾性分析,其中房缺58例,室缺120例,经体外循环下心脏直视术后11例发生CAVB,采用起搏器加激素为主的治疗方案。对178例中11例间隔缺损术后发生CAVB的缺损部位、手术方式、发生时间、持续时间和转归进行分析。结果178例间隔缺损术后发生CAVB 11例,经治疗7例患儿恢复正常心律出院,1例患儿持续房室传导阻滞(AVB),2例为间歇Ⅲ度AVB,1例患儿猝死。发现术后房室传导阻滞发生与缺损部位、手术方式以及阻断主动脉时间、心肌局部组织水肿等有关。结论间隔缺损的部位及手术路径是导致心脏直视术后发生AVB的主要影响因素,故熟悉传导束与缺损部位的解剖关系,改良手术路径是防止术后发生AVB的关键。多数AVB能恢复正常心律,少数发展为持续AVB,持续时间在1个月以上者预后差,应考虑安装永久起搏器。     

腹部三维超声模拟内镜模式评价先天性心脏间隔缺损的价值

目的探讨应用腹部三维超声表面成像摸拟内镜模式对评价先天性心脏间隔缺损的价值。方法对30例心脏间隔缺损病例进行二维彩色多谱勒检查,心脏缺损部位应用腹部三维超声内镜模式进行三维表面成像,比较二维图像与三维图像的差别。结果 30例患者中,25例房间隔缺损(房缺)、3例室间隔缺损(室缺)三维重建成功,腹部三维超声对房缺、室缺可获满意图像;另2例三维重建失败。三维成像较二维图像更易观察缺损全貌及缺损的游离缘。房缺与室缺的二维超声测值分别与其三维超声测值呈直线正相关(r分别为0.806、0.703,P<0.05),房缺与室缺的三维超声测值亦与术中所用的封堵器直径呈直线正相关(r分别为0.798、0.755,P<0.05)。结论三维超声与二维超声测值及封堵器直径有良好的相关性,腹部三维超声内镜模式表面成像可对先天性心脏间隔缺损程度进行较好的评价。     

Connexin43基因缺陷新生小鼠心脏组织病理学研究

目的观察不同程度Connefin43基因缺陷对新生小鼠心脏发育的影响。方法采用Cx43基因敲除(Cx43 KO)和CMV43^CT两种Cx43基因缺陷的小鼠模型,聚合酶链反应鉴定基因型。然后将新生小鼠心脏分离固定,HE染色观察心脏的形态结构。普通C57BL6／SJ小鼠作为对照。结果所有11只纯合型Cx43 KO小鼠生后1d内均死亡,心脏表现为严重右室流出道梗阻。在20只纯合型CMV43^CT新生小鼠中,有12只生后2d内死亡,其心脏不仅表现有右室流出道梗阻,还出现了房间隔缺损、室间隔缺损等其他心脏畸形;而另外8只未见心脏异常。所有杂合型Cx43 KO和CMV43^CT基因缺陷的小鼠生后均存活,心脏病理学检测未见异常。结论Cx43基因缺陷与心脏发育异常有密切的关系,但不同类型和不同程度的Cx43基因功能缺失对心脏发育的影响也不尽相同。     

房间隔缺损三种治疗方法的比较研究

目的总结和比较房间隔缺损三种现代治疗方法：经导管介入封堵术、非体外循环下微创房间隔缺损封堵术和体外循环下心脏直视修补术。方法2007年9月-2008年7月我院采用内科经导管介入封堵、非体外循环下微创封堵和体外循环下心脏直视手术治疗房间隔缺损共103例,术前和术后随访时进行检查对比。结果内科经导管介入封堵术共37例,35例封堵成功,2例封堵失败。非体外循环下微创封堵术15例,15例均封堵成功。体外循环下心脏直视修补术51例,51例均获成功。结论封堵术是近年来发展起来的治疗方法,导管介入封堵术适用于缺损典型的、边缘完整的患者。非体外循环微创封堵术适用于缺损大的、边缘缺失和肺动脉高压体外循环风险高的患者,体外循环下心脏直视修补术适用于房间隔缺损有合并畸形的患者。     

25-year mortality after surgical repair of congenital heart defect in childhood. A population-based cohort study.

OBJECTIVE--To determine long-term survival and the cause of death after repair of one of eight congenital heart defects in childhood. DESIGN--Cohort study. SETTING--General community. PARTICIPANTS--All Oregon residents with one of eight congenital heart defects, which was repaired surgically between 1958 and 1989 when the patient was aged 18 years or younger, including (1) tetralogy of Fallot; (2) isolated ventricular septal defect; (3) isolated atrial septal defect; (4) coarctation of the aorta; (5) aortic valvular stenosis; (6) pulmonary valvular stenosis; (7) transposition of the great arteries; and (8) patent ductus arteriosus. Follow-up of this cohort of 2701 individuals was obtained from 94%. MAIN OUTCOME MEASURE--Mortality from cardiac and noncardiac causes. RESULTS--Age at surgery and operative mortality have decreased significantly over the last 30 years. Late cardiac mortality at 25 years after surgery was 5% for tetralogy of Fallot and isolated ventricular septal defect, 10% for coarctation of the aorta, 17% for aortic stenosis, 5% for pulmonic stenosis, and less than 1% for patent ductus arteriosus; there were no late cardiac deaths after atrial septal defect repair. For transposition, late cardiac mortality was 15% at 15 years after the Mustard operation and was 2% at 10 years after the Senning operation. CONCLUSION--Surgical repair of most congenital heart defects is associated with lingering cardiac mortality, particularly for aortic stenosis, coarctation, and transposition.     

Atrial septal defect of the persistent ostium primum type with hypoplastic right ventricle in a Welsh pony foal

Valvular competency of the foramen ovale (patent foramen ovale) is regarded as a common finding in the neonatal foal and usually occurs in isolation. True atrial septal defects appear to be uncommon and are usually associated with other congenital cardiac lesions. The present report describes a case of atrial septal defect type 1 (persistent ostium primum) complicated by hypoplastic right ventricle, and tricuspid dysplasia, in a Welsh Mountain pony foal, and discusses the embryogenesis of the abnormality. A critical review of the literature suggests that atrial septal defects may occur more frequently than they are reported, and that on occasion they may be described erroneously as patent foramen ovale. The clinical significance of uncomplicated discontinuity of the atrial septum is slight, depending upon the size and location of the defect. Complicated atrial septal defects vary in clinical significance according to the nature of the associated defects.     

Presentation of atrial septal defect in infancy.

Four patients with atrial septal defects and congestive heart failure in the first year of life had none of the classic auscultatory findings of atrial septal defect. Three of four had murmurs suggestive of innocent pulmonary-artery branch stenosis.     

单中心、机器人辅助下房间隔缺损修补术(英文)

目的总结单中心、由同一术者完成的机器人辅助下房间隔缺损修补术的临床经验。方法 2007年1月至2011年9月,115例继发孔型房间隔缺损患者接受了机器人辅助下房缺修补术。患者中位年龄35岁,中位缺损直径为28 mm。术中采用外周体外循环技术,右侧胸壁开3个直径8 mm的小孔及1个15 mm的工作孔,术者于机器人系统操控台前、三维手术视野下遥控机械臂完成房缺修补或同期三尖瓣成形。术中食道超声评估手术效果,出院前及术后1个月常规心脏超声复查。结果本组病例中无手术死亡或术中术式转化,其中54例房缺修补在心脏停跳下完成,61例于心脏不停跳下完成。术中检查及术后超声复查未见房缺残余分流。平均手术时间为(257.4±79.5)min,平均体外循环时间(81.6±32.7)min。心脏不停跳组的手术时间和体外循环较心脏停跳组明显缩短;两组患者的呼吸机辅助时间、ICU时间、术后引流量和住院时间无明显差别。结论利用机器人系统可在心脏停跳或不停跳下安全完成继发孔房间隔缺损修补,手术效果良好。     

应用胎儿心脏超声对先天性心脏病检测的价值评价

目的评价应用胎儿心脏超声对先天性心脏病检测的临床价值和意义。方法选取于2009年1月～2013年1月来我院行产前检查的孕妇986例,应用超声共筛查出胎儿先天性心脏病10例。结果经超声诊断单心室1例,法乐四联症2例,房间隔缺损3例,肺动脉瓣狭窄伴右室发育不良1例,左心室发育不良1例,室间隔缺损2例。结论胎儿超声心动图可以提高先天性心脏病的检出率,对降低婴儿出生缺陷起着不可替代的作用,可作为筛选妊娠中、晚期胎儿心脏病的首选检查方法之一。     

Clinical Application of Three－dimensional Transesophageal Echocardiography in the Diagnosis of Atrial Septal Defect

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Assessment of atrial septum morphology by live three-dimensional echocardiography

Summary To evaluate the morphology of atrial septum by the live three-dimensional echocardiography (L3DE) and its value of clinical application, L3DE was performed in 62 subjects to observe the morphological characteristics and dynamic change of the overall anatomic structure of atrial septum. The study examined 49 patients with atrial septal defect (ASD), including 3 patients with atrial septal aneursym, and 10 healthy subjects. ASD in the 35 patients was surgically confirmed. The maximal diameters of ASD were measured and the percentages of area change were calculated. The parameters derived from L3DE were compared with intraoperative measurements. The results showed that L3DE could directly and clearly display the morphological features of overall anatomic structure of normal atrial septum, repaired and artificially-occluded atrial septum, atrial septal aneurysm. The defect area in ASD patients changed significantly during cardiac cycle, which reached a maximum at end-systole and a minimum at end-diastole, with a mean change percentage of 46.6%, ranging from 14.8% to 73.4%. The sizes obtained from L3DE bore an excellent correlation with intraoperative findings (r=0.90). It is concluded that L3DE can clearly display the overall morphological features and dynamic change of atrial septum and measure the size of ASD area accurately, which is important in the decision to choose therapeutic protocols.