Liu Wei, Wen Chen, Shentu Jin, Ma Ruixiang, Zhang Hao, Zhu Zhongqun, Shi Guocheng, Chen Huiwen
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
Interdiscip Cardiovasc Thorac Surg. 2023 Mar 2;36(3). doi: 10.1093/icvts/ivad013.
This study sought to review our single-institutional surgical experience in paediatric Ebstein anomaly (EA).
We retrospectively reviewed the paediatric patients with EA undergoing operation between 2004 and 2020. The time-to-event analysis was studied using Kaplan-Meier estimates. Cox regression model was used to identify risk factors for recurrent moderate-severe or greater tricuspid regurgitation (TR).
A total of 188 patients at a median age of 3.0 [interquartile range (IQR), 1.6-5.6] years were included, among whom 108 (57.4%) underwent cone reconstruction (CR). Bidirectional cavopulmonary shunt was required in 53 patients (28.2%). There were no in-hospital deaths. The median follow-up time was 5.6 (IQR, 2.9-8.9) years. Twenty-three (12.2%) developed recurrent moderate-severe or greater TR, among whom 9 required reoperation and 1 had late death. There was a lower incidence of recurrent TR (P = 0.006) and reoperation for TR (P = 0.037) in the CR group compared with the non-CR group. There was no difference in the incidence of recurrent TR (P = 0.61), reoperation (P = 0.9) and death (P = 0.48) among patients aged <1, 1-4 and 4-18 years.
Acceptable outcomes can be anticipated in paediatric EA undergoing CR in terms of freedom from TR of > moderate degree at a mid-term follow-up.
本研究旨在回顾我们在小儿埃布斯坦畸形（EA）方面的单机构手术经验。
我们回顾性分析了2004年至2020年间接受手术的小儿EA患者。采用Kaplan-Meier估计进行事件发生时间分析。使用Cox回归模型确定中度至重度或更严重三尖瓣反流（TR）复发的危险因素。
共纳入188例患者，中位年龄为3.0岁[四分位间距（IQR），1.6 - 5.6岁]，其中108例（57.4%）接受了圆锥重建（CR）。53例患者（28.2%）需要进行双向腔肺分流术。无院内死亡。中位随访时间为5.6年（IQR，2.9 - 8.9年）。23例（12.2%）出现中度至重度或更严重的TR复发，其中9例需要再次手术，1例晚期死亡。与非CR组相比，CR组TR复发率（P = 0.006）和因TR再次手术率（P = 0.037）较低。年龄＜1岁、1 - 4岁和4 - 18岁患者的TR复发率（P = 0.61）、再次手术率（P = 0.9）和死亡率（P = 0.48）无差异。
在中期随访中，接受CR治疗的小儿EA患者在避免中重度以上TR方面可获得可接受的结果。
