Knirsch Walter, De Silvestro Alexandra, von Rhein Michael
Pediatric Cardiology, Pediatric Heart Center, Department of Surgery, University Children's Hospital Zurich, Switzerland.
Children's Research Center, University Children's Hospital Zurich, Switzerland.
Front Pediatr. 2023 Jan 16;10:1099283. doi: 10.3389/fped.2022.1099283. eCollection 2022.
Patients with hypoplastic left heart syndrome (HLHS) undergoing staged palliation until Fontan procedure are at risk for impaired neurodevelopmental (ND) outcome. The Hybrid procedure with bilateral pulmonary artery banding, ductal stenting, and balloon atrioseptostomy may offer a less invasive stage I procedure compared to the Norwood stage I procedure avoiding early neonatal cardiopulmonary bypass (CPB) surgery. Despite altered fetal cerebral hemodynamics, the type of stage I procedure may be a covariate influencing ND outcome and functional outcome may also be altered due to postponing neonatal CPB surgery. Within this review, we analyzed ND outcome as well as functional outcome after Hybrid procedure as stage I procedure.
The review analyzed original publications (OPs) published before March 15, 2022, identified by Cochrane, EMBASE, OVID, Scopus, and Web of science. An OP was included if short-to-long-term neurodevelopment outcome, brain development, somatic, and cardiac outcome in patients for HLHS and variants treated by Hybrid procedure were analyzed. In addition to database searches, we reviewed all references of the analyzed OP to obtain a comprehensive list of available studies. The author, year of publication, demographic characteristics of study population, study design (prospective or retrospective), study assessment, and main findings were summarized.
Twenty-one OPs were included with data of patients with ND outcome and functional cardiac outcome. Overall, there is an impaired mid-term ND outcome in patients with Hybrid procedure as stage I for HLHS. Only slight differences between stage I procedures (Hybrid vs. Norwood) in two comparing studies have been determined affecting right ventricular remodeling, short- and mid-term ND outcome, reduced brain growth until two years of age, sufficient quality of life, and altered hemodynamics influencing brain volumes and cerebral perfusion pattern.
Despite some minor differences regarding the mid-term follow-up in patients with HLHS comparing Hybrid vs. Norwood procedure, its impact on ND outcome seems rather low. This may be explained by the large number of covariates as well as the small study populations and the different selection criteria for patients undergoing Hybrid or Norwood procedure as stage I.
接受分期姑息治疗直至Fontan手术的左心发育不全综合征（HLHS）患者存在神经发育（ND）结局受损的风险。与Norwood I期手术相比，双侧肺动脉束带、导管支架置入和球囊房间隔造口术的杂交手术可能提供一种侵入性较小的I期手术，避免早期新生儿体外循环（CPB）手术。尽管胎儿脑血流动力学发生了改变，但I期手术的类型可能是影响ND结局的一个协变量，并且由于推迟新生儿CPB手术，功能结局也可能发生改变。在本综述中，我们分析了作为I期手术的杂交手术后的ND结局以及功能结局。
本综述分析了Cochrane、EMBASE、OVID、Scopus和Web of science识别的2022年3月15日前发表的原始出版物（OPs）。如果分析了接受杂交手术治疗的HLHS及其变体患者的短期至长期神经发育结局、脑发育、躯体和心脏结局，则纳入一篇OP。除了数据库检索外，我们还查阅了所分析OP的所有参考文献，以获得可用研究的综合列表。总结了作者、发表年份、研究人群的人口统计学特征、研究设计（前瞻性或回顾性）、研究评估和主要发现。
纳入了21篇OP，包含ND结局和心脏功能结局患者的数据。总体而言，对于HLHS患者，作为I期手术的杂交手术中期ND结局受损。在两项比较研究中，I期手术（杂交手术与Norwood手术）之间仅确定了细微差异，这些差异影响右心室重塑、短期和中期ND结局、两岁前脑生长减缓、足够的生活质量以及影响脑容量和脑灌注模式的血流动力学改变。
尽管在HLHS患者中比较杂交手术与Norwood手术的中期随访存在一些细微差异，但其对ND结局的影响似乎相当小。这可能是由于大量的协变量、研究人群规模小以及作为I期手术接受杂交手术或Norwood手术的患者的不同选择标准所致。
