Department of Cardiothoracic Surgery, Norton Children's Hospital, University of Louisville, Louisville, KY, USA.
Department of Pediatrics, Norton Children's Hospital, University of Louisville, Louisville, KY, USA.
Eur J Cardiothorac Surg. 2024 Jun 3;65(6). doi: 10.1093/ejcts/ezae204.
End-stage congenital heart disease (CHD) in children with heterotaxy syndrome might necessitate a heart transplant (HTx). An HTx in heterotaxy patients can be associated with several technical (e.g. redo, systemic/pulmonary-venous/situs anomalies, pulmonary artery reconstruction) and extra-cardiac (e.g. ciliary dyskinesia, infections, gastrointestinal) challenges. Our goal was to determine if heterotaxy syndrome is associated with increased early or late transplant risks.
The United Network for Organ Sharing transplant database was merged with the Paediatric Health Information System administrative database to identify children with heterotaxy who received an HTx. Characteristics and outcomes were compared between children with heterotaxy and contemporaneous non-heterotaxy congenital and non-congenital cardiomyopathy control groups.
After we merged the databases, we divided our cohort of 1122 patients into 3 groups: the heterotaxy (n = 143), group the non-heterotaxy congenital (n = 428) group and the cardiomyopathy (n = 551) group. There were differences in the characteristics between the 3 groups, with the heterotaxy group being comparable to the non-heterotaxy congenital group. The waiting list duration was longer for the heterotaxy than for the non-heterotaxy congenital and cardiomyopathy groups (91 vs 63 vs 56 days, P < 0.001). Early post-transplant complications were similar for all groups except for operative mortality, which was 1% for the cardiomyopathy and 4% for the heterotaxy and non-heterotaxy congenital groups (P < 0.001). The post-transplant hospital stay was shorter for the cardiomyopathy (57 days) compared to the non-heterotaxy congenital (99 days) and heterotaxy (89 days) groups (P < 0.001). Whereas rejection prior to discharge was comparable between the heterotaxy and the CHD groups, it was higher at 1 year for the heterotaxy (22%) than for the non-heterotaxy congenital (19%) and cardiomyopathy (13%) groups (P < 0.001). Survival at 5 years was superior for the cardiomyopathy (87%) compared to the heterotaxy (69%) and non-heterotaxy congenital groups (78%) (P < 0.001). For the heterotaxy group, no risk factors affecting survival were identified on multivariable analysis.
Regardless of the complexity, an HTx in selected children with heterotaxy is associated with good mid-term outcomes. Despite early results that are comparable to those of other patients with CHD, the increasing rejection rate at 1 year and the relatively accelerated attrition at mid-term warrant further follow-up. Due to database limitations in defining morphologic and surgical details, further work is warranted to delineate anatomical and surgical variables that could affect survival.
患有异构综合征的儿童终末期先天性心脏病（CHD）可能需要进行心脏移植（HTx）。异构患者的 HTx 可能与多种技术（例如，再次手术、系统性/肺静脉/位置异常、肺动脉重建）和心脏外（例如，纤毛运动障碍、感染、胃肠道）挑战相关。我们的目标是确定异构综合征是否与早期或晚期移植风险增加有关。
将美国器官共享联合网络的移植数据库与儿科健康信息系统的行政数据库合并，以确定接受 HTx 的异构患者。将异构患者与同期非异构先天性和非先天性心肌病对照组的特征和结果进行比较。
在合并数据库后，我们将 1122 名患者的队列分为 3 组：异构组（n=143）、非异构先天性组（n=428）和心肌病组（n=551）。3 组之间存在特征差异，异构组与非异构先天性组相当。与非异构先天性组和心肌病组相比，异构组的等待名单时间更长（91 天 vs 63 天 vs 56 天，P<0.001）。除手术死亡率外，所有组的早期移植后并发症相似，心肌病组为 1%，异构组和非异构先天性组为 4%（P<0.001）。与非异构先天性组（99 天）和异构组（89 天）相比，心肌病组（57 天）的移植后住院时间更短（P<0.001）。尽管在出院前的排斥反应在异构组和 CHD 组之间相似，但在 1 年时，异构组（22%）的排斥反应高于非异构先天性组（19%）和心肌病组（13%）（P<0.001）。5 年生存率方面，心肌病组（87%）优于异构组（69%）和非异构先天性组（78%）（P<0.001）。在多变量分析中，未发现影响生存的危险因素。
无论复杂程度如何，对于选择的异构儿童进行 HTx 与良好的中期结果相关。尽管早期结果与其他 CHD 患者相当，但 1 年时排斥反应率的增加和中期相对加速衰减需要进一步随访。由于数据库在定义形态学和手术细节方面的限制，需要进一步的工作来描绘可能影响生存的解剖学和手术变量。
