目的 探讨静脉泵入不同剂量曲前列尼尔对高危全腔静脉-肺动脉连接(total cavo-pulmonary connection,TCPC)术后患儿的血流动力学参数及术后恢复指标的影响.方法 2018-2021年,阜外医院小儿外科中心TCPC术后189例患儿中,接受静脉泵入曲前列尼尔治疗的高危患儿26例,其中男12例、女14例,年龄4(3,6)岁,体重(17.6±6.2)kg.根据实际接受的曲前列尼尔维持剂量,将患儿分为高剂量组[维持量＞10 ng/(kg·min),15例]和低剂量组[维持量≤10 ng/(kg·min),11例].比较两组患儿静脉泵入曲前列尼尔前与达到维持量后24h内的血流动力学参数变化,并比较两组患儿术后恢复指标是否存在差异.结果 高剂量组患儿内脏异位综合征比例高于低剂量组(66.7％vs.18.2％,P=0.021).观察期间两组患儿肺动脉平均压均有不同程度的降低,低剂量组由(11.9±3.6)mm Hg 降至(11.0±3.3)mm Hg(P=0.013),高剂量组由(12.9±4.7)mm Hg 降至(10.2±3.4)mm Hg(P=0.001),高剂量组的降幅大于低剂量组(P=0.010).两组患儿的术后恢复指标差异无统计学意义(P＞0.05).副作用方面,高剂量组患儿在用药6～12 h之间,需短暂上调血管活性药物剂量维持血压稳定.结论 静脉泵入曲前列尼尔治疗高危TCPC术后患儿,高剂量曲前列尼尔降低肺动脉压力的疗效更优,但要注意高剂量使用时可能需要上调血管活性药物维持血压稳定.

目的 评价活化部分凝血活酶时间(aPTT)联合活化凝血时间(ACT)诊断接受静脉-动脉体外膜肺氧合(VA-ECMO)支持的患儿所出现的出血并发症临床事件的价值.方法 收集2013年1月～2020年10月在中国医学科学院阜外医院接受心脏外科术后接受ECMO支持的患儿(年龄6月龄～6岁)的临床数据.该入选人群于ECMO运行期间,通常每隔(1～3)h同时测量一次aPTT和ACT.根据体外生命支持组织(ELSO)抗凝指南定义ECMO支持期间患儿的出血事件.采用Pearson相关评估ACT、aPTT之间的相关性.并采用线性趋势卡方方法分析ACT与aPTT的联合指标是否与ECMO支持期间患儿的出血事件存在线性变化的趋势.利用Logistic回归联合aPTT与ACT进行受试者工作特征(ROC)曲线诊断,从而评价该联合指标在诊断心脏术后ECMO期间患儿所出现的出血并发症的敏感度与特异度,并且通过计算其曲线下面积(AUC),进而评价其准确性.结果 共纳入58例患儿,根据出血事件定义分为非出血组(n=19)和出血组(n=39).单因素分析显示,与非出血组患儿相比,出血组患儿在安装ECMO当日的红细胞计数(P＜0.05)、血红蛋白浓度(P＜0.01)及红细胞比容(P＜0.05)均较低;出血组患儿的新鲜冰冻血浆(P＜0.05)及纤维蛋白原(P＜0.05)的输注量较多,开胸探查止血的构成比较高(P＜0.01);ACT与aPTT之间存在中度程度相关(r=0.558,P＜0.01);随着ACT与aPTT值的增高,该研究人群于ECMO期间出现出血事件存在线性变化的趋势(P=0.01);采用aPTT值联合ACT值筛查出血事件,其AUC=0.757.结论 aPTT联合ACT是筛查心脏术后ECMO期间患儿所出现的出血并发症的有效方法,且该方法具有良好准确性.

目的 通过对高危血栓风险先心病儿童在术后阿司匹林初次使用前血小板聚集率的测定,探讨此类人群血小板聚集率的基础状态及主要影响因素,为药物效果正确评价提供前期基础.方法 纳入对象为2020年4月～2020年9月在中国医学科学院北京协和医学院国家心血管病中心阜外医院小儿外科中心住院手术的所有先心病儿童,具有高危血栓风险并且签订知情同意书,在其手术后阿司匹林初次使用前进行血小板聚集率(PAG-AA)测定,了解此类人群的血小板基础聚集率状态.并通过单因素及多因素分析,探讨主要影响因素.结果 最终满足入选条件进行基础PAG-AA检测患者共196例(男,116),年龄13个月(12天～6岁),体质量(10.2±4.9)kg.基础状态的 PAG-AA 中位值为 20.7％(1.3％～86.5％),其中 67.3％PAG-AA 均处于低下状态(＜55％),47.9％PAG-AA处于严重低下状态(≤20％).体外循环手术患者术后基础PAG-AA显著低于非体外循环患者(31±27 vs.58±28,P＜0.05);体外循环术后3天内检测者显著低于3天后检测者(26±26 vs.49±26,P＜0.01);紫绀病人显著高于非紫绀病人(39±27 vs.26±26,P＜0.05).多因素回归分析显示体外循环与否以及术后检测时间是影响先心病儿童基础PAG-AA检测值的主要影响因素,紫绀并无显著影响.结论 高危血栓风险先心病儿童在术后阿司匹林初次使用前血小板聚集率的基础值波动范围较大,但大多数均位于正常值以下,严重低下者占比近50％.其主要影响因素为是否行体外循环以及术后检测时点.体外循环术后3天内PAG-AA显著减低,是否紫绀对基础状态测定值无显著影响.评价阿司匹林的药物效果应该以体外循环影响基本结束后的PAG-AA结果为参考基准,建议以手术3天后为宜.

Background Aspirin following unfractionated heparin is the most common anticoagulation strategy for pediatric patients who experienced cardiac surgery at high risk of thrombosis. The platelet aggregation test is the golden method to evaluate the aspirin effect on platelet function. However, the platelet aggregation basal status before postoperative aspirin initiation and the related clinical influencing factors hasn't been investigated systemically in this population. Methods In a prospective cohort of 247 children, arachidonic acid-induced platelet aggregation (PAG-AA) was measured by means of light transmission aggregometry (LTA) before the first dose of aspirin after cardiac surgical procedure and the perioperative variables were also collected. Distribution of this population's PAG-AA basal status was described. Univariate and multivariate logistic regression analysis were performed to identify the main influencing factors of PAG-AA. Results The median time of aspirin administration was 2 (1–27) days after surgery and the corresponding median value of basal PAG-AA was 20.70% (1.28–86.49%), with 67.6% population under 55% and 47.8% population under 20%. Patients undergoing cardiopulmonary bypass (CPB) had a significantly lower basal PAG-AA than those without (30.63 ± 27.35 vs. 57.91 ± 27.58, p = 0.013). While patients whose test done within 3 days after CPB had a significantly lower PAG-AA than those out of 3 days (25.61 ± 25.59 vs. 48.59 ± 26.45, p = 0.001). Univariate analysis implied that the influencing factors of the basal PAG-AA including CPB use, test time point, cyanosis, and platelet count. Multivariate regression analysis indicated that only CPB use, test time point, and platelet count were the main independent influencing factors for the basal PAG-AA. Conclusion The majority of children have impaired basal platelet aggregometry responses before postoperative aspirin initiation. The main influencing factors are CPB use, test time point, and platelet count. To establish the platelet aggregometry baseline prior to commencement of aspirin therapy, testing should be performed 3 days later following the procedure when effect of CPB is basically over.

目的 确定接受心脏手术后的患者再次转入重症监护室(intensive care unit,ICU)的独立预测因素.方法 从重症监护医学信息数据库-Ⅲ(Medical Information Mark for Intensive Care Ⅲ,MIMIC-Ⅲ)中提取研究所需的患者数据;根据"再次转入ICU"的定义将其分为对照组与病例组.主要结果采用最小绝对收缩与选择算子(The least absolute shrinkage and selection operator,LASSO)回归、单因素以及多因素Logistic回归分析确定术后再次转入ICU的独立预测因素,并在此基础上构建列线图模型.结果 最终共纳入4 420例接受心脏手术后的患者进行回顾性分析,其分为对照组(n= 4 183,占94.6％)与病例组(n= 237,占5.4％).多因素logistic回归分析显示术前并发心律失常(OR= 0.723,95％CI:0.546-0.958,P＜0.05),外周血管疾病(OR = 0.658,95％CI:0.465-0.930,P＜0.05),肾功能不全(OR = 0.649,95％CI:0.428-0.983,P＜0.05),电解质紊乱(OR = 0.549,95％CI:0.380-0.792,P＜0.01),充血性心力衰竭(OR = 0.476,95％CI:0.358-0.633,P＜0.01),药物滥用(OR= 0.453,95％CI:0.206-0.992,P＜0.05),酗酒(OR = 0.402,95％CI:0.206-0.786,P＜0.01),失血性贫血(OR = 0.260,95％CI:0.085-0.796,P＜0.05),由急诊手术入院(OR = 2.906,95％CI:2.118-3.986,P＜0.01),术后并发重症脓毒症(OR = 0.304,95％CI:0.095-0.974,P＜0.05)为心脏术后患者再次转入ICU的独立预测因素.并且,该模型具有良好的区分能力.结论 本研究建立的预测心脏术后再次转入ICU风险的列线图模型可能有助于ICU医生识别高风险患者.然而,在模型推荐用于临床实践之前,该模型需要进一步的外部验证.

目的 明确儿童法洛四联症(tetralogy of Fallot,TOF)根治术后出血的危险因素并构建具有预测术后出血风险性能的列线图.方法 回顾性分析2018年11月至2019年6月在我院行TOF根治手术儿童的临床数据.术后出血定义为术后24 h内的胸腔积液量≥16 mL/kg,相当于本研究人群的第75个百分位数.主要结果采用最小绝对收缩与选择算子(the least absolute shrinkage and selection operator,LASSO)回归、单因素以及多因素logistic回归分析确定术后出血的独立预测因素,在此基础上构建列线图,并分析其一致性、区分度.结果 入选儿童105例.出血组术后24h胸腔积液量明显高于非出血组(P＜0.0001).多因素logistic回归分析显示,患儿低体重[odds ratio(OR)=0.538,95％confidence interval(CI)0.369～0.787,P=0.001]、术前高血红蛋白浓度(OR=1.036,95％CI 1.008～1.066,P=0.013)、术中主动脉阻断时间长(OR=1.022,95％CI 1.000～1.044,P=0.048)是术后出血风险独立预测因素.在内部验证中,列线图C-指数为0.835(95％CI 0.745～0.926),并且其校准曲线质量高.结论 本研究建立的列线图在评估TOF根治术后出血风险方面显示出良好的一致性与区分度.

目的 比较先天性心脏病患儿手术的活化部分凝血活酶时间(activated partial thromboplastin time,aPTT)监测肝素的两种抗凝状况.方法 入选(2018年6月～2019年12月)在阜外医院接受体外循环下双向格林手术全腔静脉与肺动脉连接术的(6月～6岁)患儿50例.所有普通肝素剂量调整都基于aPTT指导的肝素监测方案进行.根据能否在48 h内达到治疗性抗凝并且之后始终维持aPTT于治疗范围内,将入选患儿分为达标组(n=27)与未达标组(n=23).采用单因素分析比较两组间的围术期临床资料.结果 与未达标组比较,达标组的患儿在启用肝素24 h与48 h时的aPTT均较长(P＜0.01);且在肝素启用48 h达标组患儿的抗凝血酶Ⅲ水平较高(P＜0.01),术后24 h与48 h内的胸管引流量较少(P＜ 0.05);48 h内的新鲜冰冻血浆总输注量较少(P＜0.05).机械通气时间与ICU住院时间较短(P＜0.01).但两组患儿间的肝素维持剂量差异无统计学意义.结论 达到基于aPTT指导的肝素监测方案标准可以改善患儿在腔静脉-肺动脉连接术后的出血情况;缩短机械通气时间;减少ICU住院时间.

目的 总结完全性大动脉转位(TGA)一期大动脉调转(ASO)术后早期临床特点及围术期处理策略,评价年龄结构及畸形复杂程度对术后恢复进程的影响,并分析导致术后ICU延迟恢复的相关风险因素.方法 回顾性分析2015-2017年我院连续231例行一期ASO手术的TGA患儿临床资料,其中男165例、女66例,年龄3d至10岁.按照术后ICU滞留时间从短到长排序,将排在第75％位点的ICU滞留时间定义为分组的界值.将患儿分为正常恢复组(177例)与延迟恢复组(54例),比较两组患儿的围术期临床资料,分析延迟恢复的危险因素.结果 231例患儿中,超龄患儿占比约一半(49.6％).延迟恢复组平均ICU滞留时间(23.9±15.6)d,而正常恢复组平均ICU滞留时间仅为(4.9±2.3)d.合并弓部病变、延迟关胸、术后肺部感染是ASO术后延迟恢复的独立影响因素,超龄对总体恢复并未产生显著影响.结论 严格把握手术适应证及良好的术后管理,绝大多数TGA患儿ASO术后仍可获得满意的早期效果,但面临并发症增加的问题,导致延迟恢复.合并弓部病变、延迟关胸、术后肺部感染是导致ASO术后延迟恢复的独立影响因素.

目的 探讨吸入一氧化氮(NO)对肺血减少型紫绀型先天性心脏病(CHD)根治术后患儿血流动力学的效应.方法 2014～2018年,阜外医院小儿外科中心实施肺血减少型紫绀型CHD根治手术1764例,纳入停机后测定右心室收缩压与主动脉收缩压之比≥75％的患儿共61例,其中男41例、女20例,年龄20.5 (9.0,39.0)个月,体重(12.5±7.8)kg.将患者分为常规治疗组(33例)和联合治疗组(常规治疗联合NO吸入治疗,28例).观察NO吸入后24 h内的血流动力学参数变化,并对比两组患儿主要临床指标的变化.结果 两组患儿的人口学特征及手术相关指标差异均无统计学意义(P＞0.05).联合治疗组患儿在吸入NO后24 h内总体的血流动力学效应表现为:血管活性药物评分(ⅥS)降低(21.6±6.6vs.17.3±7.2,P=0.020)的同时血压升高[收缩压(73.7±9.7) mm Hg vs.(90.8±9.1)mm Hg,P＜0.001],中心静脉压降低[(10.0±3.1)mm Hg vs.(7.9±2.1)mm Hg,P=0.020],尿量增加[(2.8±1.7)mL/(kg.h) vs.(4.9±2.2)mL/(kg.h),P＜0.001],血乳酸降低[(2.2±1.7)mmol/L vs.(1.2±0.5)mmol/L,P＜0.001].常规治疗组术后24 h VIS差异无统计学意义(22.1±7.9vs.20.0±8.5,P=0.232).联合治疗组患儿对肾脏替代治疗的需求少于常规治疗组,特别是在术式中等复杂[心脏手术的危险等级评分(RACHS-1)≤3级]的病例中更明显(9.5％vs.40.7％,P=0.016).结论 对合并肺血管阻力增高的肺血减少型紫绀型CHD根治手术后的围术期治疗,联合NO吸入治疗可有效改善血流动力学状态.相比于常规治疗,联合NO吸入降低VIS的效果更明显,并可减少对肾脏替代治疗的需求,有利于患儿的术后恢复.

目的:总结应用可吸收聚左旋乳酸(PLLA)微骨板外固定术治疗先天性心脏病(先心病)合并支气管软化患儿的经验.方法:回顾性分析2017年12月至2019年1月,在我中心接受可吸收PLLA微骨板外固定术治疗先心病合并支气管软化的患儿8例,其中男性2例,女性6例.平均年龄(8.4±4.1)个月,平均体重(6.5±1.2)kg.观察术后机械通气时间、重症监护病房(ICU)时间、呼吸系统并发症等评估治疗效果.并于术后1、3、6、12个月复查超声心动图和胸部CT.结果:8例患儿中7例成功接受同期心脏畸形矫治和支气管可吸收PLLA微骨板外固定术,1例成功接受单纯支气管可吸收PLLA微骨板外固定手术.全组无围术期死亡,术后呼吸机辅助时间(24.4±23.7)小时,ICU时间(3.8±3.8)天.平均随访时间(6.5±6.2)个月,超声心动图显示所有患者心脏畸形矫治满意,CT显示支气管通畅.结论:采用可吸收PLLA微骨板外固定术治疗先心病合并支气管软化简便易行,治疗效果明显,可显著缩短机械通气时间及ICU时间,降低费用.近期效果满意.

目的 探讨婴儿期法洛四联症(tetralogy of Fallot,TOF)根治术后患儿快速康复(fast track,FT)的可行性及相关风险因素.方法 回顾性分析2017年1月至2017年12月我院年龄小于1岁TOF根治术病例156例.FT的定义为患者从手术室回PICU后24小时内撤离呼吸机.按照此定义,将患者分成FT组与非FT组.结果 总体达到FT治疗比例为71.8％.FT组机械通气时间、ICU住院时间比非FT组明显降低(P＜0.05).二元logestic回归分析发现右室压/左室压(RVP/LVP)大于60％、高LAP是FT治疗的独立危险因素.结论 FT对于大多数婴儿期TOF根治术后的患儿是可行的.RVP/LVP比值、LAP是影响FT治疗的风险因素.

Objective: To explore the clinical value of extravascular lung water monitoring for rapid recovery in pediatric patients after complete repair of tetralogy of Fallot (TOF). Methods: A total of 43 pediatric patients received complete repair of TOF were studied. The pulse contour cardiac index (PCCI), global end diastolic volume index (GEDI), stroke volume variation (SVV), systemic vascular resistance index (SVRI), global ejection fraction (GEF), maximum of pressure increase in aorta (dPmax), extravascular lung water index (EVWI) and pulmonary vascular permeability index (PVPI) were recorded by pulse-indicated continuous cardiac output (PICCO) monitoring at immediately enter pediatric ICU (PICU) and 6h, 12h, 18h, 24h post-operation. Meanwhile, the heart rate, blood pressure, central venous pressure (CVP), left atrium pressure (LAP) and balance of liquid were monitored; mechanical ventilation time, PICU stay time, re-intubation,re-occlusion of major aortopulmonary collateral arteries (MAPCAs) and other complications were recorded. Based on post-operative mechanical ventilation time, the patients were divided into 2 groups: Rapid recovery (R) group, patients with mechanical ventilation≤24h, n=29 and Delayed recovery (D) group, patients with mechanical ventilation>24h, n=14. Results: Compared with group D, group R had the shorter mechanical ventilation time (14.2±8.0) h vs (86.3±44.5) h and PICU stay time (2.5±1.7) days vs (5.3±3.6) days, both P<0.05; decreased PVPI at immediately enter PICU and 6h, 12h, 18h, 24h post-operation as (4.9±1.3 vs 6.4±1.5),(5.1±1.8 vs 6.5±1.3),(4.8±2.0 vs 6.5±1.6),(4.4±1.1vs 6.9±1.8), (4.4±2.5 vs 6.5±2.2) respectively, all P<0.05; Lower ELWI at 12h and 18h post-operation as(20.9±6.1) ml/kg vs (26.8±5.7) ml/kg and(19.1±5.5) ml/kg vs (26.7±5.5)ml/kg, both P<0.05. Group R had no patient received re-occlusion of MAPCAs after operation, while Group D had 3. No death, no catheter-related complication occurred in either group. Conclusion: MAPCAs may increase extravascular lung water, pulmonary vascular permeability and cause lung perfusion, therefore affect the early recovery of complete repair of pediatric TOF. PICCO monitoring may conduct bedside quantitative observation of lung perfusion, combining with ELWI and PVPI, clinicians may identify and manage MAPCAs as necessity for rapid recovery in relevant patients.

Although the use of vasopressin has become commonplace in pediatric patients with vasodilatory shock after cardiac surgery, its efficacy and hemodynamic effects have not been systematically documented. Furthermore, previous studies were mainly limited patients with left heart anomalies. To date, the use of vasopressin in patients with right heart anomalies has not yet been reported. To clarify the hemodynamic effects of vasopressin on pediatric patients with vasodilatory shock after cardiopulmonary bypass, 70 consecutive patients, most of whom with right heart anomalies, were retrospectively analyzed in Fuwai Hospital from October 2013 to September 2015. Vasopressin was administered continuously at a dose of 0.0002 to 0.002 u/kg/min. Hemodynamics, urine output, and catecholamine vasopressor doses were compared before and after vasopressin initiation. Results showed that besides the significant increase in blood pressure at 2 h after vasopressin administration, the systemic vascular resistance index also prominently elevated from 894.3 +/- 190.8 dyn/s to 1138.2 +/- 161.4 dyn/s per cm(5) per m(2), while the heart rate, right atrial pressure, pulmonary artery pressure had a trend of decline. Subsequently, the fluid requirement, the catecholamine vasopressor requirement both decreased and urine output increased. Lactate concentration showed a later remarkable decline at 12 h since vasopressin administration. All the 70 patients survived to hospital discharge. In conclusion, low dose of vasopressin administration was associated with great and timely hemodynamic improvement for pediatric patients with vasodilatory shock after cardiac surgery without any significant adverse effects.

Objective To recognize the risk factors of unplanned re-interventions within 30 days after pediatric cardiac surgery and evaluate the outcome of re-interventions. Methods We retrospectively analyzed the clinical data of 202 children in Fuwai Hospital between January 1, 2015 and August 31, 2017. There were 115 males and 87 females at average age of 32.4 months with range of 3 days to 14 years. Results There were 202 children who underwent unplanned re-intervention during 30 days post-operation, including 54 re-adjustments of pulmonary blood flow, 34 re-corrections for residual cardiac abnormalities, 28 cardiopulmonary resuscitations, 38 for coagulation problems, 19 pericardial drainages, 11 palliative re-operations to deliver heart load and 6 diaphragmatic folds and 12 others. The mortality rate among children who underwent unplanned re-inventions after cardiac surgery was 10.9％ (22/202). It was much higher than those free from re-interventions (0.7％). Time of mechanical ventilation was 284.3 (11-2 339) h, and mean ICU stay was 17.7 (1-154) d, significantly longer than those free from re-interventions at the same period. Conclusion Unplanned reinterventions after pediatric cardiac surgery is associated with higher mortality rate and longer recovery time. Early identifying risk factors and re-intervention can reduce the complications and improve the prognosis.

Objective To explore the risk factors for prolonged mechanical ventilation in children with trans-position of great arteries and intact ventricular septum who underwent arterial switch operation. Methods This study was a retrospective,single center study. One hundred and twenty patients with transposition of great arteries and intact ventricular septum who underwent primary arterial switch operation between January 2014 and December 2016 at Fuwai Hospital were eligible for this study. The data of patients from pediatric intensive care unit database and electronic medical records were collected. The data related to postoperative respiratory assist time were collected,including demo-graphic data,preoperative diagnosis,intraoperative data,and postoperative recovery data. The patients were divided into 2 groups according to ventilation time which were prolonged mechanical ventilation group(ventilation time > 72 hours) and non - prolonged mechanical ventilation group(ventilation time ≤72 hours). The data of 2 groups were analyzed by using single factor analysis,and the P≤0. 2 factors were processed into Logistic regression analysis. Results Ninety -six patients were enrolled including 22 patients in prolonged ventilation group and 74 patients in non - prolonged me-chanical ventilation group. No statistical significance was found in 2 groups in gender,age,weight,preoperative lactate, hemoglobin,use of prostaglandin E1,mechanical ventilation,cardiopulmonary time,aortic clamping time,the ratio of left ventricular pressure to right ventricular pressure,immediate postoperative plasma lactate,and vasoactive inotropic score. The weight and postoperative left atrial pressure were significantly different between 2 groups with P < 0. 2. Weight were (3. 5 ± 0. 9)kg in prolonged mechanical ventilation group and (3. 9 ± 1. 0)kg in non - prolonged mechanical ventila-tion group (P = 0. 117). Left atrial pressures were (7. 9 ± 1. 9)mmHg(1 mmHg = 0. 133 kPa)in prolonged mechani-cal ventilation group and (6. 7 ± 2. 0)mmHg in non - prolonged mechanical ventilation group(P = 0. 015). The weight and left atrial pressure were processed into Logistic regression analysis and the results revealed that high left atrial pres-sure was the risk factor for ventilation prolongation(OR = 1. 048,P = 0. 020). Respiratory assist time in prolonged and non - prolonged ventilation group was 112(80,194)h and 26(17,46)h,respectively;ICU time in prolonged and non - prolonged ventilation group was 10(1,14)d and 4(3,6)d,respectively;and all the differences were significant (all P = 0. 000). The number of death in each group was 1 with no significant difference(P = 0. 420). Conclusions High left atrial pressure is the risk factor for prolonged mechanical ventilation in children with transposition of great ar-teries and intact ventricular septum following primary arterial switch operation.

Objective To analyze the incidence rate of postoperative coagulopathy for pediatric patients with congenital heart disease (CHD) by thromboelastograph(TEG).Methods 661 pediatric patients after cardiac surgery with CPB were divided into two groups by the type of CHD and oxygen saturation,cyanotic group(n=198)and acyanotic group(n=463) and put in subgroups based on age.All of them received TEG measurement postoperatively.TEG results were then recorded and compared between two groups.Results There were 306 patients(46.3％)postoperatively with the abnormal TEG results mainly because of platelet dysfunction (43.7％),other including low fibrinogen(13％) and low clotting factors(4.2％).For cyanotic group,it's significant higher compared to acyanotic group,63.4％ and 39.7％,respectively.The rate of low clotting factors and fibrinogen were both high in cyanotic group than those acynotic group,P＜0.01,while the platelet dysfunction rate was similar between two groups,P＞0.05.The subgroup analyses found that patients younger than 1 year old,the incidence of postoperative coagulopathy with abnormal TEG results was higher than other patients.However,low fibrinogen and platelet dysfunction are more often in cyanotic patients under 1 to 3 years old.Conclusion According to TEG monitoring,pediatric patients after cardiac surgery are more likely to suffer from coagulopathy,mainly because of platelet dysfunction.Younger children and cyanosis may also be some of the high risk factors contributing to postoperative coagulopathy.Older cyanotic chlidren is also the high risk population.

Objective: To explore the relationship between pre-operative severe cyanosis (SC) and the early post-operative recovery in pediatric patients with corrective operation of Tetralogy of Fallot (TOF) Methods: A retrospective cohort study was conducted in 271 pediatric TOF patients who received corrective operation in our hospital from 2010-03-01 to 2013-03-01. Based on pre-operative hemoglobin≥180g/L, the patients were divided into 2 groups: SC group,n=48 and Non-SC group,n=223. Univariate analysis was performed to compare the differences between 2 groups; binary Logistic regression analysis was carried out to identify significant changes which was defined byP<0.01 in univariate analysis. Results: Univariate analysis indicated that compared with Non-SC group, the patients in SC group had the higher incidence of post-operative complications,P<0.001, longer time to reach negative fluid balance,P=0.006 and prolonged mechanical ventilation time,P=0.001; while the post-operative kidney injury was similar between 2 groups,P=0.036. Binary Logistic analysis presented that the patients in SC group needed prolonged mechanical ventilation time (OR=3.432, 95% CI 1.014-5.978,P=0.015), longer time to reach negative fluid balance (OR=4.823, 95% CI 2.586-8.941,P=0.002), the higher incidence of post-operative complications (OR=14.322, 95% CI 7.114-26.251,P<0.001).&nbsp;Conclusion: Pediatric TOF patients with pre-operative SC had the higher incidence of early post-operative complications, prolonged mechanical ventilation time and longer time to reach negative fluid balance those resulting delayed post-operative recovery.

目的：目前对小儿心脏术后短期的高血糖是否需要干预，以及最佳的目标血糖范围尚未形成一致的结论。本研究假设婴幼儿心脏外科术后早期适度的血糖控制有利于改善早期预后。

目的 通过分析体外膜肺氧合(ECMO)技术在小儿复杂先天性心脏病术后应用前后的临床数据及治疗情况,为进一步提高ECMO辅助病人抢救的成功率提供依据.方法 回顾分析2012年1月至2015年6月我中心31例接受ECMO辅助患儿的临床资料.患儿应用ECMO的指征为:术中难以脱离体外循环机和术后出现严重低心排或心跳骤停,经常规治疗仍无效者.结果 患儿年龄53天～8.0岁,体重4.3 ～ 28.3kg.ECMO辅助时间70～355(146.48±66.82)小时,31例患儿中脱离ECMO辅助22例(71.0％),最终存活15例(48.4％),死亡16例(51.6％).死亡组患儿应用ECMO辅助前的血管活性药物评分平均为64.69,明显高于存活组的48.03.死亡原因有心功能持续不恢复9例、脑死亡3例、感染2例、肾功能衰竭2例.结论 ECMO适应症的选择和期间的精细管理、降低相关并发症是影响ECMO辅助疗效的影响因素.

Objective To investigate the methods of early identification and early intervention for newborn with life-threatening congenital heart disease.Methods Between January 2010 and December 2010,223 neonates with serious congenital cardiac malformations were hospitalized in PICU of Fuwai Hospi-tal.Results The most type of cardiac lesions was complete transposition of the great arteries,accounting for 59%(131 cases),and the second was total anomalous pulmonary venous connection,17%(39 cases).For the primary clinical symptoms,the most common were any cyanosis,dyspnea and cardiac murmur,accounting for 91 %(204 cases),56%(125 cases)and 53%(1 18 cases),respectively.Fifty-nine cases developed into critical conditions such as severe hypoxia,metabolic acidosis and heart failure and were sent to PICU for emergency rescue.Early intervention included maintaining ductus arteriosus open,correcting internal environ-ment disturbances,treatment of heart failure,and surgical treatment as soon as possible.Four cases died before operation and 10 cases were abandoned to continue care,which all died within 12 days after discharge.In 209 cases who received operation,9 cases died.The total operation mortality was 4.3%.Within 3 to 63 month following-up,the late death was in 2 cases,2 cases received two-stage corrective operation,and three for reop-eration.The others all were in normal cardiac function and growth.Conclusion Most of neonatal life-threat-ening congenital cardiac malformations were ductus dependent such as transposition of the great arteries and total anomalous pulmonary venous connection,which the baby needs immediate diagnosis and management for survival.Early recognition,appropriate preoperative management and operation as soon as possible are the key to rescue.