中国体外循环杂志2025，Vol.23Issue(4)：302-306,5.DOI:10.13498/j.cnki.chin.j.ecc.2025.04.04
体外膜氧合在威廉姆斯综合征合并先天性心脏病术后的应用
The application of extracorporeal membrane oxygenation in the Williams syndrome postoperatively
摘要
Abstract
Objective THis single-center retrospective study evaluates the clinical outcomes of extracorporeal membrane oxygenation(ECMO)therapy in patients with Williams syndrome(WS)undergoing congenital heart disease correction surgery with cardiopulmonary bypass(CPB).Methods Data of patients under 18 years old with WS who received ECMO support after cardiac surgery from January 2017 to December 2023 at the Shanghai Children's Medical Center were collected,including demographic data,clinical information during CPB and ECMO,complications,and prognosis.Results A total of 8 patients were enrolled in this study.4 cases(50%)failed to wean off CPB and were directly converted to ECMO,1 case(12.5%)received extracorporeal cardiopulmonary resuscitation(ECPR)following cardiac arrest,and the remaining 3 cases(37.5%)required ECMO for postoperative low cardiac output syndrome(LCOS).ECMO support lasted 44-192 hours(mean 99.88±53.81),with mechanical ventilation lasting 66 to 360 hour(mean 257.75±127.67),Intensive care unit stays ranged from 3 to 23 days(mean 15.25±7.91),and total hospitalization ranged from 5 to 55 days(mean 29.5±15.78).Seven patients(87.5%)successfully weaned off ECMO,with an overall survival-to-discharge rate of 87.5%.Conclusion WS patients are prone to LCOS after surgery due to the removal of aortic valve stenosis,which alters coronary circulation.ECMO utilization in WS patients is higher than in general congenital heart disease.ECMO support facilitates adaptation to new coronary perfusion patterns,improving cardiac function with favorable outcomes.Cardiac catheterization can reveal potential anatomical issues and provide targeted treatment.关键词
先天性心脏病/Williams综合征/心脏手术/心肺转流/体外膜氧合Key words
Congenital heart disease/Williams syndrome/Cardiac surgery/Cardiopulmonary bypass/Extracorporeal membrane oxygenation引用本文复制引用
姜磊,王伟,张蔚,郭铮,沈佳,于新迪,黄坚鹄,杨寅愉..体外膜氧合在威廉姆斯综合征合并先天性心脏病术后的应用[J].中国体外循环杂志,2025,23(4):302-306,5.基金项目
上海申康医院发展中心市级医院诊疗技术推广及优化管理项目(SHDC22023228) （SHDC22023228）
