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[一例威廉姆斯综合征合并主动脉瓣上狭窄的成功手术病例:手术过程的探讨]

[A successful surgical case of supravalvular aortic stenosis with Williams syndrome: the investigations of the procedures].

作者信息

Ichihara T, Watanabe T, Yasuura K, Tanaka M, Abe T, Tsuji A, Nagashima M

机构信息

Department of Thoracic Surgery, Nagoya University School of Medicine, Japan.

出版信息

Kyobu Geka. 1994 Oct;47(11):929-33.

PMID:7967265
Abstract

We experienced a successful surgical case in which a 3-year-old boy with a characteristic elfin face, heart murmur, and mental retardation, underwent extended patch aortoplasty using equine pericardium for congenital supravalvular aortic stenosis. The aortography performed before operation, and demonstrated diffuse stenosis just above the aortic valve, which was a typical hour-glass type. The preoperative peak systolic pressure gradient between the left ventricle and ascending aorta was 120 mmHg, and was improved postoperatively. In this procedure only one cusp was incised, resulting in no deformity of the aortic valve and no obstruction of coronary arteries. In conclusion this method was excellent for the diffuse type of supravalvular aortic stenosis.

摘要

我们经历了一例成功的外科手术病例,一名3岁男孩,面容如小精灵般独特,伴有心脏杂音和智力发育迟缓,因先天性主动脉瓣上狭窄接受了使用马心包的扩大补片主动脉成形术。术前进行了主动脉造影,显示主动脉瓣上方弥漫性狭窄,为典型的沙漏型。术前左心室与升主动脉之间的收缩期峰值压力梯度为120 mmHg,术后有所改善。在此手术中仅切开一个瓣叶,未导致主动脉瓣畸形及冠状动脉阻塞。总之,该方法对于弥漫型主动脉瓣上狭窄效果极佳。

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Kyobu Geka. 1994 Oct;47(11):929-33.
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