Hirata Yasutaka, Miyata Hiroaki, Hirahara Norimichi, Murakami Arata, Kado Hideaki, Sakamoto Kisaburo, Sano Shunji, Takamoto Shinichi
Department of Cardiac Surgery, The University of Tokyo, Bunkyo, Japan.
Department of Health Policy and Management, School of Medicine, Keio University, Shinjuku, Japan.
Pediatr Cardiol. 2018 Jan;39(1):111-119. doi: 10.1007/s00246-017-1735-1. Epub 2017 Sep 21.
Bilateral pulmonary artery banding (PAB) has emerged to be an attractive option as an initial procedure for the treatment of hypoplastic left heart syndrome (HLHS), and some centers report excellent survival. However, its usage is variable among institutions and its true efficacy is unknown due to reporting biases. We aimed to describe the results of bilateral PAB use, preoperative risk factors, and long-term outcomes compared with primary Norwood procedure, using a national database. Infants who underwent bilateral PAB or Norwood procedure as an initial palliation for HLHS between January 2008 and December 2012 listed in the Japan Congenital Cardiovascular Surgery Database (JCCVSD) were included. The total number of patients diagnosed with HLHS was 334. Bilateral PABs were performed for 256 patients and primary Norwood procedures for 78 patients, as an initial procedure. Actuarial 5-year survival was 59.0%. The primary Norwood procedure group had better 5-year survival [75.5%; 95% confidence interval (CI) 63.2-84.1] than the bilateral PAB group (75.5 vs. 54.0%, log-rank p < 0.001). However, the bilateral PAB group had more significant risk factors. When the risk-adjusted outcomes were evaluated, there was no significant difference between the primary Norwood group and the bilateral PAB group (76.4 vs. 78.1%, log-rank p = 0.87) in higher volume institutions. The primary Norwood group had better 5-year survival than the bilateral PAB group, but preoperative risk was higher in the bilateral PAB group. Because outcomes are comparable when performed at higher HLHS volume institutions, proper patient selection is important in achieving good long-term result.
双侧肺动脉环缩术(PAB)已成为治疗左心发育不全综合征(HLHS)初始手术的一个有吸引力的选择,一些中心报告其生存率良好。然而,各机构对该手术的使用情况不一,且由于报告偏差,其真正疗效尚不清楚。我们旨在利用一个全国性数据库,描述双侧PAB的使用结果、术前危险因素以及与一期诺伍德手术相比的长期预后。纳入了2008年1月至2012年12月期间在日本先天性心血管外科数据库(JCCVSD)中登记的、接受双侧PAB或诺伍德手术作为HLHS初始姑息治疗的婴儿。诊断为HLHS的患者总数为334例。作为初始手术,256例患者接受了双侧PAB,78例患者接受了一期诺伍德手术。5年精算生存率为59.0%。一期诺伍德手术组的5年生存率[75.5%;95%置信区间(CI)63.2 - 84.1]优于双侧PAB组(75.5%对54.0%,对数秩检验p < 0.001)。然而,双侧PAB组有更多显著的危险因素。在评估风险调整后的结果时,在手术量较大的机构中,一期诺伍德组和双侧PAB组之间无显著差异(76.4%对78.1%,对数秩检验p = 0.87)。一期诺伍德组的5年生存率优于双侧PAB组,但双侧PAB组的术前风险更高。由于在HLHS手术量较大机构进行手术时结果具有可比性,因此正确选择患者对于取得良好的长期效果很重要。