Nakamura Akihiro, Ishikawa Yuichi
Fukuoka Children's Hospital, Fukuoka, Japan.
NYS Medical Inc., Shibuya, Tokyo, Japan.
Ann Pediatr Cardiol. 2025 Mar-Apr;18(2):100-104. doi: 10.4103/apc.apc_57_25. Epub 2025 Aug 29.
Despite improvement in the outcome of the Norwood procedure, the prognosis of hypoplastic left heart syndrome (HLHS) remains problematic. The aim of this study was to assess the impact of pre- and perioperative factors and catheterization data on outcomes after the Norwood procedure.
This case-control study included all patients who underwent the Norwood procedure for HLHS at Fukuoka Children's Hospital between January 2000 and October 2011. Subjects ( = 66) were divided into two outcome groups: survivors ( = 41) and nonsurvivors ( = 25). First, we compared the pre- and perioperative data. Second, we compared the catheterization data after the Norwood procedure.
The mean follow-up period was 40 months (range: 1.4-141). Overall, there was a 38% mortality, including early death. The pre- and perioperative data, as well as PaO in room air at catheterization, were not significantly different. However, univariate analysis revealed that the incidence of home oxygen therapy to maintain oxygen saturation >80% was significantly higher in the survivors ( < 0.001). The right ventricular ejection fraction was higher in the survivors (48.7% ± 1.9%, mean ± standard error) than in the nonsurvivors (41.4% ± 2.8%, = 0.041). The severity of tricuspid regurgitation (TR) and the pulmonary blood flow/systemic blood flow ratio were lower in the survivors than in the nonsurvivors. Multivariate logistic regression analysis identified severe TR as the only significant prognostic marker of mortality ( = 0.041).
The severity of TR was associated with the prognosis of HLHS after the Norwood procedure.
尽管诺伍德手术的预后有所改善，但左心发育不全综合征（HLHS）的预后仍然存在问题。本研究的目的是评估术前和围手术期因素以及导管检查数据对诺伍德手术后结局的影响。
本病例对照研究纳入了2000年1月至2011年10月在福冈儿童医院接受诺伍德手术治疗HLHS的所有患者。研究对象（n = 66）分为两个结局组：存活者（n = 41）和非存活者（n = 25）。首先，我们比较了术前和围手术期数据。其次，我们比较了诺伍德手术后的导管检查数据。
平均随访期为40个月（范围：1.4 - 141个月）。总体而言，死亡率为38%，包括早期死亡。术前和围手术期数据以及导管检查时室内空气中的动脉血氧分压无显著差异。然而，单因素分析显示，存活者中为维持氧饱和度>80%而进行家庭氧疗的发生率显著更高（P < 0.001）。存活者的右心室射血分数（48.7% ± 1.9%，平均值±标准误）高于非存活者（41.4% ± 2.8%，P = 0.041）。存活者的三尖瓣反流（TR）严重程度和肺血流量/体循环血流量比值低于非存活者。多因素逻辑回归分析确定严重TR是死亡率的唯一显著预后标志物（P = 0.041）。
TR的严重程度与诺伍德手术后HLHS的预后相关。
