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[双侧肺动脉环扎术对左心发育不全综合征(HLHS)及其变异型手术及长期预后的影响]

[Impacts of bilateral pulmonary artery banding on the surgical and long-term outcomes in hypoplastic left heart syndrome( HLHS) and its variants].

作者信息

Sakamoto Takahiko, Harada Yorikazu

机构信息

Department of Cardiovascular Surgery, Nagano Children's Hospital, Azumino, Japan.

出版信息

Kyobu Geka. 2014 Apr;67(4):278-85.

Abstract

Although Norwood-type operation is a fundamental procedure for hypoplastic left heart syndrome and its variants, the surgical risk is still high even in the high volume center. Bilateral pulmonary artery (PA) banding( BPAB) is an alternative procedure to avoid the risk in the neonatal period. We clarified the impacts of BPAB on the surgical and long-term outcomes in HLHS and its variants. Consecutive 85 patients( classical HLHS:52, variant:33) who underwent surgical intervention between July, 1993 and December, 2012 were evaluated. Fetal diagnosis was done in 37 patients (44%). Forty-two patients underwent primary Norwood operation[ Gp N, Blalock-Taussig( BT):29, right-ventricle( RV)-PA conduit:13] and the remaining 43 patients underwent BPAB followed by Norwood operation. Among them, 4 patients did not reach Norwood [9%, atrioventricular valve regurgitation (AVVR):1, infection:1, preoperative shock:2). The survived 39 patients were divided into 2 groups;Gp BN (BPAB → Norwood+BT/RV-PA conduit, n=22), Gp BG [BPAB → Norwood+bidirectional Glenn procedure(BDG), n=17]. BPAB for HLHS and its variants is useful in terms of lower mortality. The 2nd stage palliation is so important that modified Norwood procedure with BT shunt or RV-PA conduit contribute to the lower morbidity including neurological outcome. Norwood+BDG strategy might be beneficial for the high risk patients such as with poor cardiac function or low body weight.

摘要

尽管诺伍德式手术是治疗左心发育不全综合征及其变体的基本术式,但即便在手术量大的中心,手术风险依然很高。双侧肺动脉环扎术(BPAB)是一种可避免新生儿期风险的替代术式。我们阐明了BPAB对左心发育不全综合征及其变体的手术及长期预后的影响。对1993年7月至2012年12月间接受手术干预的85例连续患者(典型左心发育不全综合征:52例,变体:33例)进行了评估。37例患者(44%)进行了胎儿诊断。42例患者接受了一期诺伍德手术[Gp N,布莱洛克-陶西格(BT)分流术:29例,右心室(RV)-肺动脉导管:13例],其余43例患者接受了BPAB,随后进行诺伍德手术。其中,4例患者未进行诺伍德手术[9%,房室瓣反流(AVVR):1例,感染:1例,术前休克:2例]。存活的39例患者分为2组;Gp BN(BPAB→诺伍德+BT/RV-肺动脉导管,n=22),Gp BG[BPAB→诺伍德+双向格林手术(BDG),n=17]。BPAB用于左心发育不全综合征及其变体在降低死亡率方面是有用的。二期姑息治疗非常重要,采用BT分流术或RV-肺动脉导管的改良诺伍德手术有助于降低包括神经学预后在内的发病率。诺伍德+BDG策略可能对心功能差或体重低等高风险患者有益。

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