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[心脏伴有交叉心室,合并左心室射血途径梗阻及二尖瓣异常]

[Heart with criss-cross ventricles associated with an obstruction of the ejection pathway of the left ventricle and a mitral anomaly].

作者信息

Lucet P, Losay J, Piot D, Binet J P

出版信息

Arch Mal Coeur Vaiss. 1981 May;74(5):527-34.

PMID:6794473
Abstract

A case of criss-cross heart with situs inversus associated with subvalvular aortic stenosis and mitral regurgitation in a 9 year old boy is reported. Surgical correction of the valvular abnormalities gave an excellent clinical and haemodynamic result. A review of the literature shows that this abnormality of rotation of the ventricles coexists with a concordant atrioventricular connection in 70 % cases; the ventriculoarterial connection is only discordant in 12 % cases. The right ventricle is always situated above the left ventricle. The most common associated lesions are usually accessible to surgery; ventricular septal defect (98 % cases); stenosis or atresia of the pulmonary artery (70% cases). However, right ventricular hypoplasia is also common (65 % cases). Six patients underwent surgical correction of the associated lesions with 4 successes and 2 deaths. Surgery is possible in these complex cardiac malformations with good results providing a precise preoperative anatomical diagnosis is made.

摘要

本文报道了一名9岁男孩,患有交叉心脏伴内脏反位,并伴有主动脉瓣下狭窄和二尖瓣反流。对瓣膜异常进行手术矫正后,临床和血流动力学结果极佳。文献回顾显示,在70%的病例中,这种心室旋转异常与房室连接一致并存;仅在12%的病例中,心室动脉连接不一致。右心室总是位于左心室上方。最常见的相关病变通常可以通过手术治疗;室间隔缺损(98%的病例);肺动脉狭窄或闭锁(70%的病例)。然而,右心室发育不全也很常见(65%的病例)。6例患者接受了相关病变的手术矫正,4例成功,2例死亡。对于这些复杂的心脏畸形,如果能进行精确的术前解剖诊断,手术是可行的,且效果良好。

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