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[关于一名7岁儿童病例的劳布里-佩齐综合征的治疗]

[Treatment of Laubry-Pezzi syndrome apropos of a case in a 7-year-old child].

作者信息

Fouchard J, Bechet A M, Cabrol C, Degeorges M

出版信息

Arch Mal Coeur Vaiss. 1975 Jan;68(1):105-12.

PMID:804870
Abstract

Report of one case of a Laubry and Pezzi syndrome. In spite of the already severe aortic incompetence at the age of 4 years, operation was postponed. Three years later, at the first signs of bad tolerance of the aortic incompetence the operation was undertaken including: -- closure of the ventricular septal defect by a patch, and correction of aortic incompetence by a metallic-ball Starr's prosthesis n 9. After an observation period of 18 months, the result of operation is satisfactory, in spite of signs of haemolysis. Comparison with the data from the literature led to the following conclusions: 1) in a patient with ventricular septal defect the demonstration of a diastolic murmur indicates closure of the septal defect. 2) When the aortic incompetence is a severe one, it should be corrected urgently, whatever the age of the child, if badly tolerated. In the very young child, if it is well tolerated, the operation might be postponed in view of the difficulties of plastic operations and the hazards of prostheses in such patients. 3) A moderate aortic incompetence constitutes the most difficult indication; a conservative operation seems to be preferable to a valve replacement by a prosthesis, a homograft or a heterograft. The results obtained by some techniques of valvular plasty after observation periods amounting to 12 years are considerable.

摘要

劳布里和佩齐综合征1例报告。尽管该患者在4岁时就已出现严重的主动脉瓣关闭不全,但手术被推迟。三年后,在出现主动脉瓣关闭不全耐受性差的最初迹象时,进行了手术,包括:——用补片闭合室间隔缺损,并用9号金属球斯塔尔假体矫正主动脉瓣关闭不全。经过18个月的观察期,尽管有溶血迹象,但手术结果令人满意。与文献数据比较得出以下结论:1)在室间隔缺损患者中,舒张期杂音的出现表明室间隔缺损已闭合。2)当主动脉瓣关闭不全严重时,如果耐受性差,无论患儿年龄多大,都应紧急矫正。在非常年幼的儿童中,如果耐受性良好,鉴于整形手术的困难以及此类患者使用假体的风险,手术可能会推迟。3)中度主动脉瓣关闭不全是最困难的适应症;保守手术似乎比用假体、同种异体移植物或异种异体移植物置换瓣膜更可取。经过长达12年的观察期后,一些瓣膜成形术技术取得的结果相当可观。

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