摘要
目的通过单中心研究回顾Williams综合征(WS)合并先天性心脏病患者在心肺转流(CPB)术后接受体外膜氧合(ECMO)治疗的临床结局。方法收集上海儿童医学中心2017年1月至2023年12月期间18岁以下WS患者行心脏病纠治术后应用ECMO的病例资料。包括患者的一般情况、CPB和ECMO期间临床资料、并发症及预后。结果本次研究共纳入8例患者,4例(50%)未成功脱离CPB直接转为ECMO,1例(12.5%)因心脏骤停行体外心肺复苏,其余3例(37.5%)患者因术后低心排血量综合征行ECMO辅助。ECMO辅助时间为44~192(99.88±53.81)h,机械通气时间为66~360(257.75±127.67)h,ICU住院时间为3~23(15.25±7.91)d,总住院时间为5~55(29.5±15.78)d。其中成功撤机为7例,总撤机率87.5%;存活出院为7例,总存活率87.5%。结论WS患者ECMO使用率高于一般先天性心脏病患儿。ECMO支持有助于冠状动脉适应新的灌注模式,同时改善心功能,可获得良好的效果。心导管检查可以揭示潜在的解剖问题,并给予针对性治疗。
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.
作者
姜磊
王伟
张蔚
郭铮
沈佳
于新迪
黄坚鹄
杨寅愉
Jiang Lei;Wang Wei;Zhang Wei;Guo Zheng;Shen Jia;Yu Xindi;Huang Jianhu;Yang Yinyu(Department of Thoracic and Cardiovascular Surgery,Shanghai Children’s Medical Center,Shanghai Jiao Tong University School of Medicine,Shanghai 200127,China)
出处
《中国体外循环杂志》
2025年第4期302-306,共5页
Chinese Journal of Extracorporeal Circulation
基金
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)。
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
Congenital heart disease
Williams syndrome
Cardiac surgery
Cardiopulmonary bypass
Extracorporeal membrane oxygenation
