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Alterations in cardiac connexin expression in cardiomyopathies. 心肌病患者心脏连接蛋白表达的改变。
Pub Date : 2006-01-01 DOI: 10.1159/000092572
Nicholas J Severs, Emmanuel Dupont, Neil Thomas, Riyaz Kaba, Stephen Rothery, Revti Jain, Katherine Sharpey, Christopher H Fry

Gap junctions, assembled from connexins, form the cell-to-cell pathways for propagation of the precisely orchestrated patterns of current flow that govern the synchronized rhythm of the healthy heart. As in most tissues and organs, multiple connexin types are co-expressed in the heart; the connexins Cx43, Cx40 and Cx45 are found in distinctive combinations and relative quantities in different, functionally specialized subsets of cardiomyocytes. Alterations in connexin expression and gap junction organization, now a well-documented feature of human cardiomyopathies, potentially contribute to the pro-arrhythmic substrate. In the diseased ventricle, the most consistently reported quantitative alteration involves heterogeneous reduction in Cx43 expression and disruption of the normal ordered pattern of Cx43 gap junction distribution. Additional studies suggest that upregulation of Cx40 and Cx45 may also feature in the failing ventricle, the former restricted to ischemic cardiomyopathy and localized to the subendocardial region. By correlating data from studies on the human patient with those from animal and cell models, alterations in connexin expression and gap junction organization have emerged as important factors to be considered in understanding the pro-arrhythmic substrate found in human cardiomyopathies.

由连接蛋白组装而成的间隙连接,形成了细胞间的传导途径,用于精确编排的电流模式的传播,从而控制健康心脏的同步节律。与大多数组织和器官一样,多种连接蛋白类型在心脏中共同表达;连接蛋白Cx43、Cx40和Cx45在不同功能特化的心肌细胞亚群中以不同的组合和相对数量存在。连接蛋白表达和间隙连接组织的改变,现在是人类心肌病的一个充分证明的特征,可能有助于促进心律失常的底物。在病变心室中,最一致报道的定量改变包括Cx43表达的异质性减少和Cx43间隙连接正常有序分布模式的破坏。其他研究表明,Cx40和Cx45的上调也可能出现在心室衰竭中,前者仅限于缺血性心肌病,并局限于心内膜下区域。通过将人类患者的研究数据与动物和细胞模型的研究数据相关联,连接蛋白表达和间隙连接组织的改变已经成为理解人类心肌病中发现的促心律失常底物的重要因素。
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引用次数: 69
Endocardial cushion defects: embryology,anatomy and pathophysiology. 心内膜垫缺陷:胚胎学、解剖学和病理生理学。
Pub Date : 2004-01-01 DOI: 10.1159/000079791
Rubin S Cooper
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引用次数: 2
Strategies for management of postcardiotomy cardiogenic shock following valvular heart surgery. 心脏瓣膜手术后心源性休克的处理策略。
Pub Date : 2004-01-01 DOI: 10.1159/000079794
Deon W Vigilance, Mehmet C Oz
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引用次数: 5
The Howard Gilman Foundation Lecture. Where have we come from and where are we going? Valve management past, present and future. 霍华德·吉尔曼基金会讲座。我们从哪里来,又往哪里去?阀门管理的过去、现在和未来。
Pub Date : 2004-01-01 DOI: 10.1159/000079778
Joseph K Perloff

Leonardo da Vinci's anatomical drawings of quadricuspid, tricuspid, and bicuspid aortic valves underscored the hydraulic superiority of a three leaflet valve with cuspal equality. William Harvey demonstrated that venous valves were designed for unidirectional flow and to prevent reflux from the heart, observations that served as the basis of his immortal de Mortu Cordis. Joseph Rouanet of Paris proposed that heart sounds originated from the closing movements of cardiac valves. The Cardiodynamics of Mitral Insufficiency by Wiggers and Feil was followed three decades later by Paul Wood's An Appreciation of Mitral Stenosis. The Bland/Sweet operation indirectly addressed mitral stenosis by means of a venous shunt. Sir Henry Souttar's early digital repair of mitral stenosis was later reintroduced independently by Harken and Bailey; Doyen, Sellers, and Brock employed surgical valvotomy for pulmonary stenosis, and Bailey employed surgical valvotomy for aortic stenosis. Management of abnormal cardiac valves includes repair (reconstruction), replacement with mechanical or biologic prostheses, and interventional catheterization. The first mechanical valve was inserted extracardiac by Hufnagel into the descending thoracic aorta of patients with severe aortic regurgitation. The Starr caged ball mechanical prosthesis was designed for intracardiac replacement of an abnormal cardiac valve. The peripheral flow ball valve was followed by hydraulically superior and less thrombogenic central flow monoleaflet or bileaflet mechanical valves, and by homograft and heterograft bioprosthetic valves. Improved methods of preparing exogenous bioprostheses and innovative techniques of aortic valve reconstruction are evolving. Cardiac catheterization as a therapeutic intervention is routinely applied to stenotic mitral, aortic and pulmonary valves, and transcatheter replacement of an abnormal pulmonary valve is now a reality.

列奥纳多·达·芬奇的四尖瓣、三尖瓣和二尖瓣主动脉瓣的解剖图强调了三瓣瓣尖相等的水力优势。威廉·哈维证明,静脉瓣膜是为单向流动而设计的,可以防止心脏回流,这些观察结果是他不朽的《德·莫图·考迪斯》的基础。巴黎的约瑟夫·鲁阿内(Joseph Rouanet)提出,心音起源于心脏瓣膜的闭合运动。Wiggers和Feil的《二尖瓣功能不全的心脏动力学》三十年后,Paul Wood的《二尖瓣狭窄的欣赏》紧随其后。Bland/Sweet手术通过静脉分流间接治疗二尖瓣狭窄。亨利·索塔爵士早期二尖瓣狭窄的数字修复术后来被哈肯和贝利独立地重新引入;Doyen、Sellers和Brock对肺动脉狭窄采用手术瓣膜切开术,Bailey对主动脉狭窄采用手术瓣膜切开术。异常心脏瓣膜的处理包括修复(重建)、机械或生物假体置换以及介入导管置入术。第一个机械瓣膜由Hufnagel插入严重主动脉反流患者的心外胸降主动脉。Starr固定球机械假体是设计用于心脏内异常瓣膜置换术的。外周血流球阀之后是液压性能优越且血栓发生率低的中央血流单小管或双小管机械阀,以及同种移植物和异种移植物生物假体阀。制备外源性生物假体的改进方法和主动脉瓣重建的创新技术正在不断发展。心导管置入术作为一种治疗干预常规应用于狭窄的二尖瓣、主动脉瓣和肺动脉瓣,经导管置换术治疗异常的肺动脉瓣现已成为现实。
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引用次数: 6
Perspectives on diseases of the thoracic aorta. 胸主动脉疾病的观点。
Pub Date : 2004-01-01 DOI: 10.1159/000079786
John A Elefteriades
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引用次数: 7
Valve repair versus replacement when aortic regurgitation is caused by aortic root aneurysms: relative advantages and disadvantages and the impact of decision on surgical indications. 主动脉根部动脉瘤引起主动脉返流时瓣膜修复与置换术:相对优势和劣势以及决定对手术指征的影响。
Pub Date : 2004-01-01 DOI: 10.1159/000079783
Leonard N Girardi
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引用次数: 0
Assessment of myocardial damage in regurgitant valvular disease. 反流性瓣膜病的心肌损害评价。
Pub Date : 2004-01-01 DOI: 10.1159/000079784
Jagat Narula
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引用次数: 0
Ventricular arrhythmias in mitral regurgitation: frequency, clinical and prognostic importance, management before and after mitral valve surgery. 二尖瓣返流引起的室性心律失常:频率,临床和预后重要性,二尖瓣手术前后的处理。
Pub Date : 2004-01-01 DOI: 10.1159/000079789
Clare Hochreiter, Jeffrey S Borer, Andrew Yin, Phyllis G Supino, Edmund M Herrold, Karl Krieger, O Wayne Isom
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引用次数: 5
Valve surgery in the asymptomatic patient with aortic regurgitation: current indications and the effect of change rates in objective measures. 无症状主动脉瓣返流患者的瓣膜手术:当前适应症和客观指标变化率的影响。
Pub Date : 2004-01-01 DOI: 10.1159/000079782
Jeffrey S Borer, Phyllis G Supino, Clare Hochreiter, Edmund M Herrold, Andrew Yin, Karl Krieger, O Wayne Isom
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引用次数: 2
Surgery for atrioventricular septal defects. 房室间隔缺损的外科治疗。
Pub Date : 2004-01-01 DOI: 10.1159/000079792
Jan M Quaegebeur, Rubin S Cooper
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引用次数: 2
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