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[房室不一致但心室-动脉无不一致。附3例报告]

[Atrioventricular discordance without ventriculo-arterial discordance. Apropos of 3 cases].

作者信息

Rey C, Laurent J M, Ducloux G, Dupuis C

出版信息

Arch Mal Coeur Vaiss. 1983 May;76(5):504-12.

PMID:6411022
Abstract

Three cases of atrioventricular without ventriculo-arterial discordance are reported. The first case was a young woman with situs solitus, atrioventricular discordance and ventriculo-arterial concordance, large atrial and small ventricular septal defects. The functional tolerance was relatively good. The second case was an infant girl with dextrocardia by dextroversion and abdominal situs solitus. The child had atrioventricular discordance with ventriculo-arterial concordance and an ostium primum atrial septal defect. Cyanosis was mild and, in addition, complete heart block was diagnosed on the fourth day of life. The functional tolerance was good in this case too and growth was normal. The third case was a young girl with levocardia, atrial situs inversus, and abdominal situs inversus. A double outlet right ventricle was diagnosed at catheterisation with atrioventricular discordance, ventricular septal defect, and severe valvular and infundibular pulmonary stenosis. The first palliative procedure was performed at eight months: a left Blalock-Taussig anastomosis. As cyanosis has recurred a second procedure is being considered, the child now being 5 years old. Different forms of atrioventricular discordance without ventriculo-arterial discordance have been described: atrioventricular discordance with ventriculo-arterial concordance; atrioventricular discordance with double outlet right ventricle; atrioventricular discordance with double outlet left ventricle; atrioventricular discordance with a single vessel issuing from the right or left ventricle. With reference to their personal cases and those described in the literature, the authors describe the anatomy, physiopathology and the clinical consequences of the different forms encountered in their cases. The different surgical techniques for each anatomical form of this condition are discussed.

摘要

报告了3例房室不协调但心室-动脉无不一致的病例。第一例是一名年轻女性,心脏位置正常,房室不协调但心室-动脉协调,有较大的房间隔缺损和较小的室间隔缺损。功能耐受性相对良好。第二例是一名女婴,因右旋心和腹部脏器位置正常而出现右位心。患儿有房室不协调但心室-动脉协调,并有原发孔型房间隔缺损。青紫较轻,此外,在出生后第4天诊断出完全性心脏传导阻滞。该病例的功能耐受性也良好,生长正常。第三例是一名年轻女孩,左位心,心房反位,腹部脏器反位。心导管检查诊断为右心室双出口,伴有房室不协调、室间隔缺损以及严重的瓣膜和漏斗部肺动脉狭窄。在8个月时进行了第一次姑息手术:左布莱洛克-陶西格吻合术。由于青紫复发,现正考虑进行第二次手术,患儿现已5岁。已经描述了房室不协调但心室-动脉无不一致的不同形式:房室不协调但心室-动脉协调;房室不协调伴右心室双出口;房室不协调伴左心室双出口;房室不协调伴单一血管发自右心室或左心室。作者结合他们的个人病例以及文献中描述的病例,阐述了他们所遇到的不同形式的解剖结构、病理生理学及临床后果。并讨论了针对这种情况每种解剖形式的不同手术技术。

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