房间隔缺损合并先天性纤维蛋白原缺乏的体外循环围术期管理:1例报告并文献复习  

Perioperative management of atrial septal defect with congenital fibrinogen deficiency by cardiopulmonary bypass:A case report and literature review

作　　者：李杨[1] 李洪利[1] 齐德林 冯磊 李超 丁瑞田 刘燕晖[1] 尚学斌[1] Li Yang;Li Hong-Li;Qi De-Lin;Feng Lei;Li Chao;Ding Rui-Tian;Liu Yan-Hui;Shang Xue-Bin(Department of Cardiac Surgery,Xuanwu Hospital,Capital Medical University,Beijing 100053,China)

机构地区：[1]首都医科大学宣武医院心脏外科,北京100053

出　　处：《解放军医学杂志》2021年第3期258-262,共5页Medical Journal of Chinese People's Liberation Army

基　　金：首都医科大学宣武医院院级课题(XWJL-2019024)。

摘　　要：目的报道1例房间隔缺损(ASD)合并先天性纤维蛋白原缺乏(CFD)的诊治过程并文献复习。方法回顾性分析首都医科大学宣武医院住院治疗的1例在体外循环(CPB)下行不停跳心内直视缺损修补术的继发孔ASD合并CFD患者的临床资料,检索PubMed数据库,综合文献结果,探讨合并纤维蛋白原(FIB)缺乏的患者行CPB心脏手术的围术期管理经验。结果本例患者女,30岁,因体检发现ASD 8个月入院。入院时心脏超声检查示:房间隔中部回声中断约34 mm,彩色多普勒超声探及收缩期左向右分流,三尖瓣反流压差66 mmHg,估测肺动脉收缩压76 mmHg,射血分数(EF)64%。凝血功能:凝血酶原时间>120 s,凝血酶时间>240 s,活化部分凝血活酶时间>180 s,FIB <0.6 g/L。术前连续静脉输注人FIB 4 g/d,维持血浆FIB>1.5 g。3 d后在CPB下行不停跳ASD修补术。术中输注人FIB 2 g,鱼精蛋白中和后再给予人FIB 2 g。手术时间3.5 h,CPB时间45 min。术后每天补充FIB 4 g,连续5 d。第3天拔除胸腔引流管,术后第7天出院,未发生出血及血栓栓塞并发症。3个月后门诊随访,患者手术切口愈合佳,复查心脏超声提示房间隔未见残余分流,EF为67%。检索PubMed数据库并手动筛选后共获得英文文献8篇,共875例体外循环心脏手术后低FIB血症患者,男604例(69.0%),女271例(31.0%);其中6篇为个案报告,包括婴幼儿1例(出生后5 d,体重2.5 kg),成人5例,均为男性。FIB缺乏的患者行CPB心脏手术,围术期需要补充FIB、冷沉淀或新鲜冰冻血浆,以预防术后严重的出血并发症。结论合并FIB缺乏的患者行CPB心脏手术,需根据血浆中FIB定量和凝血相关指标,做出个体化的围术期管理方案。Objective To report a case of atrial septal defect(ASD)with congenital fibrinogen deficiency(CFD),and review the literature.Methods A case of secondary ASD with CFD treatment in Xuanwu Hospital of Capital Medical University was reported,and the defect was repaired under CPB on beating heart.The management experience of cardiac surgery under CPB in patients with CFD was summarized by means of PubMed database and literature analysis.Results This patient was female,30 years old.She was admitted to the hospital with the complaint that ASD was found for 8 months after physical examination.On admission,echocardiography showed that the atrial septum was interrupted by about 34 mm.Color Doppler flow imaging(CDFI)detection indicated systolic left to right shunt,the pressure difference of tricuspid regurgitation was 66 mmHg,systolic pulmonary artery pressure(SPAP)was 76 mmHg,ejection fraction(EF)was 64%.Coagulation function:Prothrombin time was>120 s,thrombin time>240 s,activated partial thrombin time>180 s,and fibrinogen<0.6 g/L.Preoperative continuous intravenous infusion of fibrinogen 4 g per day was to maintain the plasma fibrinogen>1.5 g.Three days later ASD repair was performed under CPB on beating heart.Infusion of fibrinogen 2 g was performed by venous injection during operation,and another fibrinogen 2 g was given after heparin was neutralized by protamine.Operation time was 3.5 hours,and CPB time was 45min.Fibrinogen 4 g per day was supplemented postoperatively for 5 days.At the 3rd day chest drainage tube was removed.The patient was discharged at the 7th day.There were no hemorrhage and thrombosis complications.After 3 months,outpatient follow-up showed the surgical incision healed well,echocardiography showed no residual shunt in the atrial septum,and EF value was 67%.A total of 8 English literatures were obtained after searching PubMed database and manually screening,including 875 patients with hypofibrinogenemia after CPB cardiac surgery.Among them,604 were males(69.0%)and 271 were females(31.0%).There were 6

关 键 词：心脏外科 纤维蛋白原缺乏 体外循环 围术期管理 

分 类 号：R856.5[医药卫生—航空、航天与航海医学]