Lin Hsin-Chia, Wu Mei-Hwan, Wang Jou-Kou, Lin Ming-Tai, Chen Chun-An, Lu Chun-Wei, Chen Yih-Sharng, Huang Shu-Chien, Chiu Shuenn-Nan
Department of Pediatrics, National Taiwan University Children's Hospital, Taipei, Taiwan; Department of Pediatrics, National Taiwan University Hospital Yunlin Branch, Yunlin, Taiwan.
Department of Pediatrics, National Taiwan University Children's Hospital, Taipei, Taiwan.
J Formos Med Assoc. 2022 Jan;121(1 Pt 1):89-97. doi: 10.1016/j.jfma.2021.01.014. Epub 2021 Feb 3.
BACKGROUND/PURPOSE: Fontan operation is the standard surgical procedure for achieving long-term survival in single-ventricular complex congenital heart diseases (SV-CHD). We aim to identify the perioperative outcomes and impact of heterotaxy syndrome (HS) after Fontan operation in a tertiary pediatric cardiology center.
Medical records were reviewed for all patients who received Fontan operation and who were born between 1997 and 2017 in our institution. Preoperative, operative, and postoperative risk factors for perioperative mortality and morbidity were analyzed.
Totally, 154 patients were enrolled (103 SV-CHD and 51 HS), and the male to female ratio was 92:62. The mean age of Fontan operation was 5.1 years, and extracardiac conduit comprised the majority (90.9%) of Fontan operation. Overall perioperative event-free survival to discharge was 91.6% (84.3% in HS and 95.1% in other SV-CHD, P = 0.032). For secondary outcomes, length of intensive care stay and duration of pleural effusion drainage were not significantly different between patients with HS and other SV-CHD, but postoperative arrhythmia was more common in HS group (31.4% vs. 12.6%, P = 0.005). In multivariable regression analysis, preoperative risk factors including operation year before 2007 and high PAP and postoperative factors of elevated postoperative CVP were associated with worse outcomes. HS was not a predictor of worse outcome after adjusting for preoperative PAP and operation era.
Surgical outcome has improved much in current era. Perioperative outcome is poorer in patients with HS than other SV-CHD, but HS is not a predictor of perioperative mortality after adjusting for hemodynamic factors.
背景/目的：Fontan手术是单心室复杂先天性心脏病（SV-CHD）实现长期生存的标准外科手术。我们旨在确定在一家三级儿科心脏病中心进行Fontan手术后的围手术期结果及心脏异构综合征（HS）的影响。
回顾了1997年至2017年在我们机构出生且接受Fontan手术的所有患者的病历。分析围手术期死亡率和发病率的术前、术中及术后危险因素。
共纳入154例患者（103例SV-CHD和51例HS），男女比例为92:62。Fontan手术的平均年龄为5.1岁，心外管道Fontan手术占大多数（90.9%）。总体围手术期无事件生存至出院率为91.6%（HS组为84.3%，其他SV-CHD组为95.1%，P = 0.032）。对于次要结局，HS组和其他SV-CHD组患者在重症监护病房住院时间和胸腔积液引流持续时间方面无显著差异，但HS组术后心律失常更常见（31.4%对12.6%，P = 0.005）。在多变量回归分析中，术前危险因素包括2007年前的手术年份和高肺动脉压（PAP）以及术后中心静脉压（CVP）升高的术后因素与较差结局相关。在调整术前PAP和手术时代后，HS不是较差结局的预测因素。
当前时代手术结果有了很大改善。HS患者的围手术期结局比其他SV-CHD患者差，但在调整血流动力学因素后，HS不是围手术期死亡率的预测因素。
