Division of Paediatric Cardiology, Labatt Family Heart Centre, Hospital for Sick Children, Toronto, Ontario, Canada.
Department of Anesthesiology and Critical Care Medicine, Children's Hospital, Los Angeles, CA, USA.
Cardiol Young. 2021 Jan;31(1):105-113. doi: 10.1017/S1047951120003376. Epub 2020 Oct 26.
Treatment of hypoplastic left heart syndrome varies across institutions. This study examined the impact of introducing a standardised programme.
This retrospective cohort study evaluated the effects of a comprehensive strategy on 1-year transplant-free survival with preserved ventricular and atrioventricular valve (AVV) function following a Norwood operation. This strategy included standardised operative and perioperative management and dedicated interstage monitoring. The post-implementation cohort (C2) was compared to historic controls (C1). Outcomes were assessed using logistic regression and Kaplan-Meier analysis.
The study included 105 patients, 76 in C1 and 29 in C2. Groups had similar baseline characteristics, including percentage with preserved ventricular (96% C1 versus 100% C2, p = 0.28) and AVV function (97% C1 versus 93% C2, p = 0.31). Perioperatively, C2 had higher indexed oxygen delivery (348 ± 67 ml/minute/m2 C1 versus 402 ± 102ml/minute/m2 C2, p = 0.015) and lower renal injury (47% C1 versus 3% C2, p = 0.004). The primary outcome was similar in both groups (49% C1 and 52% C2, p = 0.78), with comparable rates of death and transplantation (36% C1 versus 38% C2, p = 0.89) and ventricular (2% C1 versus 0% C2, p = 0.53) and AVV dysfunction (11% C1 versus 11% C2, p = 0.96) at 1-year. When accounting for cohort and 100-day freedom from hospitalisation, female gender (OR 3.7, p = 0.01) increased and ventricular dysfunction (OR 0.21, p = 0.02) and CPR (OR 0.11, p = 0.002) or ECMO use (OR 0.15, p = 001) decreased the likelihood of 1-year transplant-free survival.
Standardised perioperative management was not associated with improved 1-year transplant-free survival. Post-operative ventricular or AVV dysfunction was the strongest predictor of 1-year mortality.
左心发育不全综合征的治疗方法因机构而异。本研究旨在探讨引入标准化方案的影响。
本回顾性队列研究评估了一项综合策略对行 Norwood 手术后 1 年无移植存活率的影响，该策略包括标准化手术和围手术期管理以及专门的中间期监测。实施后队列（C2）与历史对照组（C1）进行比较。使用逻辑回归和 Kaplan-Meier 分析评估结果。
本研究纳入 105 例患者，其中 C1 组 76 例，C2 组 29 例。两组基线特征相似，包括心室（96% C1 与 100% C2，p=0.28）和房室瓣（97% C1 与 93% C2，p=0.31）功能保存率。围手术期，C2 组的氧供指数更高（348±67ml/minute/m2 C1 与 402±102ml/minute/m2 C2，p=0.015），肾损伤发生率更低（47% C1 与 3% C2，p=0.004）。两组主要结局相似（49% C1 与 52% C2，p=0.78），死亡率和移植率相当（36% C1 与 38% C2，p=0.89），心室（2% C1 与 0% C2，p=0.53）和房室瓣功能障碍（11% C1 与 11% C2，p=0.96）在 1 年时也相似。当考虑到队列和 100 天无住院天数时，女性（OR 3.7，p=0.01）、心室功能障碍（OR 0.21，p=0.02）和心肺复苏（OR 0.11，p=0.002）或体外膜肺氧合（ECMO）使用（OR 0.15，p=0.001）降低了 1 年无移植存活率。
标准化围手术期管理并未提高 1 年无移植存活率。术后心室或房室瓣功能障碍是 1 年死亡率的最强预测因素。
