Department of Paediatric Cardiology, Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Gothenburg, Sweden.
Department of Pediatrics, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Sweden.
Cardiol Young. 2020 Mar;30(3):353-360. doi: 10.1017/S1047951119003263. Epub 2020 Jan 10.
Norwood surgery has been available in Sweden since 1993. In this national cohort study, we analysed transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia.
Patients were identified from the complete national cohort of live-born with hypoplastic left heart syndrome/aortic atresia 1993-2010. Analysis of survival after surgery was performed using Cox proportional hazards models for the total cohort and for birth period and gender separately. Thirty-day mortality and inter-stage mortality were analysed. Patients were followed until September 2016.
The 1993-2010 cohort consisted of 208 live-born infants. Norwood surgery was performed in 121/208 (58%). The overall transplantation-free survival was 61/121 (50%). The survival was higher in the late period (10-year survival 63%) than in the early period (10-year survival 40%) (p = 0.010) and lower for female (10-year survival 34%) than for male patients (10-year survival 59%) (p = 0.002). Inter-stage mortality between stages I and II decreased from 23 to 8% (p = 0.008). For male patients, low birthweight in relation to gestational age was a factor associated with poor outcome.
The survival after Norwood surgery for hypoplastic left heart syndrome/aortic atresia improved by era of surgery, mainly explained by improved survival between stages I and II. Female gender was a significant risk factor for death or transplantation. For male patients, there was an increased risk of death when birthweight was lower than expected in relation to gestational age.
1993 年以来，瑞典已经开展了诺伍德手术。在这项全国性队列研究中，我们分析了主动脉瓣闭锁的左心发育不全综合征患者行诺伍德手术后无移植生存情况。
我们从 1993 年至 2010 年出生的所有左心发育不全综合征/主动脉瓣闭锁患儿的完整全国队列中确定了患者。使用 Cox 比例风险模型对总队列以及按出生时期和性别分别进行了手术后生存情况分析。分析了 30 天死亡率和各阶段死亡率。随访至 2016 年 9 月。
1993 年至 2010 年队列包括 208 例活产婴儿。208 例患儿中 121 例行诺伍德手术（58%）。无移植总体生存率为 61/121（50%）。晚期（10 年生存率 63%）生存率高于早期（10 年生存率 40%）（p = 0.010），女性（10 年生存率 34%）低于男性（10 年生存率 59%）（p = 0.002）。I 期至 II 期之间的各阶段死亡率从 23%降至 8%（p = 0.008）。对于男性患儿，出生体重与胎龄的比值低是预后不良的一个因素。
随着手术时代的进步，左心发育不全综合征/主动脉瓣闭锁行诺伍德手术后的生存率得到了提高，这主要是由于 I 期至 II 期之间的生存率得到了改善。女性性别是死亡或移植的显著危险因素。对于男性患儿，出生体重与胎龄的比值低会增加死亡风险。
