Division of Pediatric Cardiac Surgery, Stanford University School of Medicine, Lucile Packard Children's Hospital Stanford, Stanford, CA, USA.
Division of Pediatric Cardiology, Stanford University School of Medicine, Lucile Packard Children's Hospital Stanford, Stanford, CA, USA.
Eur J Cardiothorac Surg. 2022 Dec 2;63(1). doi: 10.1093/ejcts/ezac553.
Children with Alagille syndrome often have complex forms of congenital heart defects with the majority having peripheral pulmonary artery stenosis (PPAS) and pulmonary valve atresia (PA) or pulmonary valve stenosis. Children with Alagille syndrome also have variable amounts of liver dysfunction. The purpose of this study was to evaluate the impact of liver dysfunction on outcomes in children with Alagille syndrome undergoing congenital heart surgery.
This was a retrospective review of 69 patients with Alagille syndrome who underwent congenital heart surgery at our institution. The underlying diagnoses included PPAS (n = 29), tetralogy of Fallot with PPAS (n = 14), tetralogy with PA (n = 3), PA with ventricular septal defect and major aortopulmonary collateral arteries (n = 21) and one each with D-transposition and supravalvar aortic stenosis.
The median age at surgery was 16 months (range 0-228 months). Procedures performed included PPAS repair (n = 43), tetralogy with PA repair (n = 3), unifocalization procedures (n = 21) and other (n = 2). Forty-two (61%) patients had mild or no liver dysfunction, while 26 (38%) had moderate or severe liver dysfunction. The median cardiopulmonary bypass time was 345 min (341 with liver dysfunction, 345 without liver dysfunction). There were a total of 8 operative (12%) deaths and 3 late (4%) deaths. Six operative and 2 late deaths occurred in patients with liver dysfunction (combined 30.7%) versus 2 operative and 1 late death (combined 7.1%) for patients without liver dysfunction (P < 0.05).
These results suggest that liver dysfunction has a profound impact on survival in children with Alagille syndrome undergoing congenital heart surgery.
患有 Alagille 综合征的儿童常伴有复杂的先天性心脏病，其中多数存在外周肺动脉狭窄（PPAS）和肺动脉瓣闭锁（PA）或肺动脉瓣狭窄。Alagille 综合征患儿也存在不同程度的肝功能障碍。本研究旨在评估肝功能障碍对接受先天性心脏手术的 Alagille 综合征患儿结局的影响。
这是对在我院接受先天性心脏手术的 69 例 Alagille 综合征患儿的回顾性研究。基础诊断包括 PPAS（n=29）、PPAS 伴法洛四联症（n=14）、PA 伴室间隔缺损和主要体肺侧支动脉（n=3）、PA 伴室间隔缺损和大主肺动脉侧支动脉（n=21）和一例法洛四联症伴主动脉瓣上狭窄。
手术时的中位年龄为 16 个月（范围 0-228 个月）。手术包括 PPAS 修复术（n=43）、PA 伴法洛四联症修复术（n=3）、单腔化手术（n=21）和其他手术（n=2）。42（61%）例患儿有轻度或无肝功能障碍，26（38%）例患儿有中度或重度肝功能障碍。体外循环时间中位数为 345 分钟（肝功能障碍者为 341 分钟，无肝功能障碍者为 345 分钟）。共有 8 例（12%）手术死亡和 3 例（4%）晚期死亡。肝功能障碍组有 6 例手术死亡和 2 例晚期死亡（合计 30.7%），无肝功能障碍组有 2 例手术死亡和 1 例晚期死亡（合计 7.1%）（P<0.05）。
这些结果表明，肝功能障碍对接受先天性心脏手术的 Alagille 综合征患儿的生存率有深远影响。
