Selenius Sabina, Ilvesvuo Johanna, Ruotsalainen Hanna, Mattila Ilkka, Pätilä Tommi, Helle Emmi, Ojala Tiina
New Children's Hospital Pediatric Research Center, Helsinki University Hospital, Helsinki, Finland.
Stem Cells and Metabolism Research Program, Faculty of Medicine, University of Helsinki, Helsinki, Finland.
Interdiscip Cardiovasc Thorac Surg. 2023 Aug 3;37(2). doi: 10.1093/icvts/ivad127.
Several studies have reported mortality risk factors associated with hypoplastic left heart syndrome (HLHS). However, these data are ambiguous and mainly focused on the independent effects of these factors. We examined both the independent and the cumulative effects of preoperative risk factors for poor outcome in patients undergoing the Norwood procedure. Moreover, we studied the risk factors associated with prolonged initial hospital stays in these patients.
We performed a retrospective national 18-year observational study of preoperative risk factors for 1 year, as well as total follow-up mortality or need for transplant in patients with HLHS (N = 99) born in Finland between 1 January 2004 and 31 December 2021.
Overall, one-year survival was 85.6%. In a multivariable analysis, having a major extracardiac anomaly and being small for gestational age were significant predictors of one-year mortality or the need for a transplant. Aortic atresia was a predictor of total follow-up mortality. An analysis of the cumulative effect indicated that the presence of 2 risk factors was associated with higher mortality.
HLHS remains the defect with the highest procedural risks for mortality in paediatric cardiac surgery. From a prognostic point of view, recognition of independent preoperative risk factors as well as the cumulative effect of risk factors for mortality is essential.The results of this study were presented orally at the 55th Annual Meeting of the Association for European Paediatric and Congenital Cardiology, Geneva, Switzerland, 28 May 2022.
多项研究报告了与左心发育不全综合征（HLHS）相关的死亡风险因素。然而，这些数据并不明确，且主要关注这些因素的独立作用。我们研究了接受诺伍德手术患者术前不良预后风险因素的独立作用和累积作用。此外，我们还研究了这些患者初始住院时间延长的相关风险因素。
我们对2004年1月1日至2021年12月31日在芬兰出生的HLHS患者（N = 99）进行了一项为期18年的全国性回顾性观察研究，分析其术前风险因素对1年以及总随访期死亡率或移植需求的影响。
总体而言，1年生存率为85.6%。在多变量分析中，存在主要的心外畸形和小于胎龄是1年死亡率或移植需求的显著预测因素。主动脉闭锁是总随访期死亡率的预测因素。累积效应分析表明，存在2个风险因素与更高的死亡率相关。
HLHS仍然是小儿心脏手术中手术死亡率最高的缺陷。从预后的角度来看，识别独立的术前风险因素以及死亡风险因素的累积效应至关重要。本研究结果于2022年5月28日在瑞士日内瓦举行的第55届欧洲小儿和先天性心脏病协会年会上进行了口头汇报。
