O'Connor Mario, Adler Joel T, Venardos Neil M, Ponce-Rivera Monica S, Fraser Charles D, Mery Carlos M, Well Andrew
Department of Surgery and Perioperative Care, Dell Medical School at the University of Texas, Austin, Austin, TX, USA.
Department of Pediatrics, Dell Medical School at the University of Texas, Austin, Austin, TX, USA.
Pediatr Transplant. 2025 Aug;29(5):e70110. doi: 10.1111/petr.70110.
Congenital heart disease (CHD) frequently coexists with noncardiac malformations. Among which, biliary atresia (BA) occurs in approximately 5%-15% of patients Despite this, outcomes of liver transplantation (LT) in patients with CHD and BA remain unknown.
A retrospective review of the Pediatric Health Information System (PHIS) database from January 1, 2004 to October 30, 2023. All patients who underwent LT and had a diagnosis of BA were included. Multiorgan transplants were excluded. International Classification of Diseases 9th and 10th editions were utilized to identify patients with a diagnosis consistent with CHD.
A total of 1677 patients were identified with 983 (59%) female, 811 (5%) white non-Hispanic, and a median age at transplant of 11.6 (interquartile range [IQR]: 7.7-26.6) months. A CHD diagnosis was present in 83 (5%). Overall, the majority of transplants were performed in the CHD population in the modern era (2016-2023) (42/83.51%). CHD had a longer median preoperative length of stay (LOS) compared to non-CHD (1 [1.0-15.0] vs. 1 [0-6.0], p = 0.031). No increased risk of in-hospital mortality was evident (OR: 1.61, 95% CI: 0.37-6.95, p = 0.519). On multivariable analysis, CHD was associated with a 29% (95% CI: 11.04-50.34) increase in LOS (p < 0.001), 25% (95% CI: 6.98-46.47, p = 0.005) increase in postoperative LOS, and was not associated with increased risk for 30-day readmission (OR: 1.14; CI: 0.53-2.45, p = 0.736). Over a median follow-up of 3.5 years (IQR: 0.86-7.78) years, no difference in retransplantation rate was evident.
LT in patients with CHD and BA is safe. Although patients with CHD display heightened utilization of in-hospital resources, no discernible variance in long-term outcomes was observed. However, additional research is imperative to comprehensively elucidate the influence of CHD on management decisions and outcomes throughout the liver transplant process.
先天性心脏病（CHD）常与非心脏畸形并存。其中，约5%-15%的患者会发生胆道闭锁（BA）。尽管如此，CHD合并BA患者的肝移植（LT）结局仍不明确。
对2004年1月1日至2023年10月30日的儿科健康信息系统（PHIS）数据库进行回顾性分析。纳入所有接受LT且诊断为BA的患者。排除多器官移植患者。采用国际疾病分类第9版和第10版来识别诊断为CHD的患者。
共识别出1677例患者，其中983例（59%）为女性，811例（5%）为非西班牙裔白人，移植时的中位年龄为11.6个月（四分位间距[IQR]：7.7-26.6）。83例（5%）患者诊断为CHD。总体而言，大多数移植手术是在现代（2016-2023年）CHD患者群体中进行的（42/83，占51%）。与非CHD患者相比，CHD患者术前中位住院时间（LOS）更长（1[1.0-15.0]天 vs. 1[0-6.0]天，p = 0.031）。住院死亡率无明显增加风险（OR：1.61，95%CI：0.37-6.95，p = 0.519）。多变量分析显示，CHD与LOS增加29%（95%CI：11.04-50.34，p < 0.001）、术后LOS增加25%（95%CI：6.98-46.47，p = 0.005）相关，且与30天再入院风险增加无关（OR：1.14；CI：0.53-2.45，p = 0.736）。中位随访3.5年（IQR：0.86-7.78年），再移植率无明显差异。
CHD合并BA患者的LT是安全的。尽管CHD患者对医院资源的利用增加，但长期结局未观察到明显差异。然而，需要进一步研究以全面阐明CHD对肝移植全过程管理决策和结局的影响。
