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[流量可调式双侧肺动脉环扎术(FABPAB)用于左心发育不全综合征(HLHS)或HLHS变异型的有效性研究]

[A study of the usefulness of flow-adjustable bilateral pulmonary artery banding (FABPAB) for hypoplastic left heart syndrome( HLHS) or HLHS variant].

作者信息

Miyamoto Takashi

机构信息

Department of Cardiovascular surgery, Gunma Children's Medical Center, Shibukawa, Japan.

出版信息

Kyobu Geka. 2014 Apr;67(4):262-5.

PMID:24917154
Abstract

BACKGROUNDS

Bilateral pulmonary artery banding( BPAB), a relatively minimally invasive procedure, has begun to be undertaken as the initial operation for the treatment of hypoplastic left heart syndrome (HLHS) or HLHS variant. In the opinion of the present authors, it is practicable to avoid stepwise operations with cardiopulmonary bypass (CPB) in newborn patients by conducting an initial operation consisting of flow-adjustable bilateral pulmonary artery banding (FABPAB) combined with transcatheter pulmonary artery balloon dilatation( PABD), followed in a subsequent treatment course by transcatheter PABD, which would allow for more minimally invasive adjustment of the pulmonary artery bloodflow. This study was conducted as a retrospective evaluation of the usefulness of this therapeutic strategy.

METHODS

Fourteen patients who underwent BPAB as the initial operation for HLHS/HLHS variant between April 2008 and October 2013 were included in this study. Stepwise surgical repair including BPAB was performed via a median sternotomy as the initial operation, without the use of CPB. And 7 patients underwent transcatheter PABD successfully.

RESULTS

In this series, 7 patients developed a significant pulmonary artery index (PAI) after PABD (pre PABD:148.3±63.9 vs. post PABD:232.5±73.2, p<0.05). No need of the pulmonary artery reconstruction at the 2nd operation. One patient failed to survive the operation, in which difficulty in adjustment of atrioventricular valvular regurgitation was encountered after the Norwood procedure. There were 3 patients who underwent the Norwood combined BDG at the mean age of 4.6 months and the mean body weight of 5.5 kg. Fontan procedure was accomplished in 3 of the 6 patients of the survivor, while 3 patients were on standby for the Fontan procedure.

CONCLUSION

We performed BPAB as the initial operation combined with PABD in the treatment of HLHS/HLHS variant, which enabled avoidance of the initial operation with cardiopulmonary bypass at the neonatal stage and developed a significant pulmonary artery index after PABD.

摘要

背景

双侧肺动脉环缩术(BPAB)是一种相对微创的手术,已开始作为治疗左心发育不全综合征(HLHS)或HLHS变异型的初始手术。在本文作者看来,对于新生儿患者,通过进行由流量可调双侧肺动脉环缩术(FABPAB)联合经导管肺动脉球囊扩张术(PABD)组成的初始手术,随后在后续治疗过程中进行经导管PABD,从而更微创地调整肺动脉血流,避免在体外循环(CPB)下进行分步手术是可行的。本研究旨在对该治疗策略的有效性进行回顾性评估。

方法

本研究纳入了2008年4月至2013年10月期间接受BPAB作为HLHS/HLHS变异型初始手术的14例患者。包括BPAB在内的分步手术修复作为初始手术通过正中胸骨切开术进行,不使用CPB。7例患者成功接受了经导管PABD。

结果

在该系列中,7例患者在PABD后肺动脉指数(PAI)显著升高(PABD前:148.3±63.9 vs. PABD后:232.5±73.2,p<0.05)。无需在第二次手术时进行肺动脉重建。1例患者手术未能存活,在诺伍德手术后遇到房室瓣反流调整困难。3例患者在平均年龄4.6个月、平均体重5.5 kg时接受了诺伍德联合双向格林手术(BDG)。6例存活患者中有3例完成了Fontan手术,3例患者等待进行Fontan手术。

结论

我们在HLHS/HLHS变异型的治疗中采用BPAB作为初始手术并联合PABD,这能够避免新生儿期在体外循环下进行初始手术,并在PABD后使肺动脉指数显著升高。

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