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在左心室发育不良但形态正常的动脉导管依赖型新生儿中采用双心室修复方法。

Biventricular repair approach in ducto-dependent neonates with hypoplastic but morphologically normal left ventricle.

作者信息

Serraf A, Piot J D, Bonnet N, Lacour-Gayet F, Touchot A, Bruniaux J, Belli E, Galletti L, Planché C

机构信息

Department of Pediatric Cardiac Surgery, Marie-Lannelongue Hospital, Le Plessis-Robinson, France.

出版信息

J Am Coll Cardiol. 1999 Mar;33(3):827-34. doi: 10.1016/s0735-1097(98)00636-6.

Abstract

OBJECTIVES

Increased afterload and multilevel LV obstruction is constant. We assumed that restoration of normal loading conditions by relief of LV obstructions promotes its growth, provided that part of the cardiac output was preoperatively supported by the LV, whatever the echocardiographic indexes.

BACKGROUND

Whether to perform uni- or biventricular repair in ducto dependent neonates with hypoplastic but morphologically normal LV (hypoplastic left heart syndrome classes II & III) remains unanswered. Echocardiographic criteria have been proposed for surgical decision.

METHODS

Twenty ducto dependent neonates presented with this anomaly. All had aortic coarctation associated to multilevel LV obstruction. Preoperative echocardiographic assessment showed: mean EDLW of 12.4 +/- 3.03 ml/m2 and mean Rhodes score of -1.73 +/-0.8. Surgery consisted in relief of LV outflow tract obstruction by coarctation repair in all associated to aortic commissurotomy in one and ASD closure in 2.

RESULTS

There were 3 early and 2 late deaths. Failure of biventricular repair and LV growth was obvious in patients with severe anatomic mitral stenosis. The other demonstrated growth of the left heart. At hospital discharge the EDLVV was 19.4+/-3.12 ml/m2 (p = 0.0001) and the Rhodes score was -0.38+/-1.01 (p = 0.0003). Actuarial survival and freedom from reoperation rates at 5 years were 72.5% and 46%, respectively.

CONCLUSIONS

Biventricular repair can be proposed to ducto dependent neonates with hypoplastic but morphologically normal LV provided that all anatomical causes of LV obstruction can be relieved. Secondary growth of the left heart then occurs; however, the reoperation rate is high.

摘要

目的

后负荷增加和多级左心室梗阻持续存在。我们假设,通过解除左心室梗阻恢复正常负荷条件可促进其生长,前提是部分心输出量术前由左心室提供支持,无论超声心动图指标如何。

背景

对于左心室发育不良但形态正常(左心发育不全综合征II级和III级)的动脉导管依赖型新生儿,是进行单心室还是双心室修复仍未得到解答。已提出超声心动图标准用于手术决策。

方法

20例患有这种异常的动脉导管依赖型新生儿。所有患儿均有主动脉缩窄合并多级左心室梗阻。术前超声心动图评估显示:平均舒张末期左心室壁重量为12.4±3.03 ml/m²,平均罗兹评分为-1.73±0.8。手术包括所有患儿均通过缩窄修复解除左心室流出道梗阻,其中1例联合主动脉瓣交界切开术,2例联合房间隔缺损封堵术。

结果

有3例早期死亡和2例晚期死亡。严重解剖性二尖瓣狭窄患者双心室修复失败且左心室生长不明显。其他患者显示左心生长。出院时舒张末期左心室容积为19.4±3.12 ml/m²(p = 0.0001),罗兹评分为-0.38±1.01(p = 0.0003)。5年时的精算生存率和免于再次手术率分别为72.5%和46%。

结论

对于左心室发育不良但形态正常的动脉导管依赖型新生儿,若能解除左心室梗阻的所有解剖学原因,可考虑进行双心室修复。左心随后会发生继发性生长;然而,再次手术率较高。

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