目的 探索降钙素原(procalcitonin,PCT)在小儿体外循环(cardiopulmonary bypass,CPB)心脏术后早期的自然变化规律及其影响因素.方法 采用观察性临床研究方法,对2018年6月-2019年12月阜外医院小儿心脏外科中心重症监护病房(pediatric intensive care unit,PICU)收治的年龄3岁以下、先天性心脏病(先心病)手术风险矫正评分(risk adjustment of congenital heart surgery,RACHS)2～5级、CPB下手术、无术前感染或炎性疾病的患儿,连续测定术前至术后第7d的PCT、C-反应蛋白(c-reactive protein,CRP)、白细胞(white blood cell,WBC)计数,同时行痰培养和胸部X线片检查,对体温＞38℃的患儿行血培养,对伤口化脓的患儿行伤口分泌物培养.统计术后7 d内感染及并发症情况,根据术后7 d内有无感染和并发症将入组患儿分为4组:感染+并发症组、单纯感染组、单纯并发症组以及正常组.结果 最终选择住PICU≥4 d的患儿为研究对象,共429例患者入选,其中男268例、女161例,年龄8.0(0.7,26.0)个月.包括单纯感染组145例、单纯并发症组38例、正常组230例以及感染+并发症组16例.小儿CPB心脏术后PCT、CRP、WBC均较术前明显升高,其中CRP、WBC在术后第2 d达到高峰,一直持续到术后第7 d仍高于正常.PCT在术后第1 d达到高峰,一般在术后第5 d降至正常.年龄、体重、RACHS评分、CPB及主动脉阻断时间均与术后早期PCT浓度有一定程度相关性.单纯感染组、单纯并发症组及正常组之间两两比较,年龄、体重、RACHS评分、CPB及主动脉阻断时间等基础资料差异无统计学意义.其中单纯感染组与正常组术后第3～7 d的PCT浓度差异具有统计学意义(P＜0.01),单纯并发症组与正常组术后第1～7 d的PCT浓度差异均有统计学意义(P＜0.01),单纯感染组与单纯并发症组术后第1～5 d的PCT浓度差异有统计学意义(P＜0.05).结论 小儿CPB心脏术后WBC、CRP、PCT均明显升高,从其动态变化趋势看,三者相比,PCT更能预警术后早期感染或并发症的存在.影响小儿CPB心脏术后早期PCT浓度的因素包括年龄、体重、RACHS评分、CPB和主动脉阻断时间、感染以及并发症.

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.

体外膜肺氧合(Extracorporeal Membrane Oxy-genation,ECMO)技术是由氧合器和动脉、静脉回路组成的一个心肺支持系统,能让急性功能衰竭的心肺充分休息,为挽救患儿的生命创造条件.本院开创了云南省该项技术在小儿重症心脏病术后的急救,并获得成功.希望通过这2例的分析总结,为小儿心脏病术后ECMO治疗应用提供一些经验积累,现报告如下.

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 总结主动脉弓离断术后患儿胃肠道并发症的预防护理经验.方法 对11例主动脉弓离断行重建手术患儿给予术前应用前列腺素E的护理,围术期改善全身循环和氧供,监测胃肠道功能和进食管理等.结果2例未出现胃肠道并发症,胃肠道并发症Ⅰ级5例,Ⅱ级2例,Ⅲ级1例,Ⅳ级1例.其中1例Ⅳ级患儿并发多器官功能衰竭而死亡,其余10例存活.存活的10例中1例并存渗漏综合征,经治疗好转;1例术后并发上静脉狭窄行二次手术后治愈.结论通过术前增加下半身的供血、术后改善全身循环和缺氧、严密监测和评估胃肠道功能,合理喂养,可改善胃肠道功能,从而改善小儿主动脉弓离断术后胃肠道并发症患儿的预后.

目的 总结小儿肺血减少性紫绀型先天性心脏病根治术后残余体肺侧枝动脉的治疗策略，以期为类似病例的临床治疗提供参考。方法 回顾性分析云南省阜外心血管病医院小儿外科恢复室(PICU)2018年12月至2020年1月收治的3例肺血减少性紫绀型先天性心脏病根治术后存在残余体肺侧枝动脉的小儿病历资料，采集分析患儿的基本住院资料、临床诊断、治疗经过、术后拔管过程、处理方式、治疗效果、住院时间及预后等信息。结果 3例患儿1女2男，年龄分别为6岁和6月、4月，体重分别为19.5kg、9kg和8.2kg。3例经过介入封堵、呼吸机辅助、体肺侧枝动脉结扎治疗后均成功出院，无特殊并发症。结论 小儿肺血减少性紫绀型先天性心脏病根治术后残余体肺侧枝动脉会延长住院时间、影响康复，应及早识别，可以通过延长呼吸机辅助时间、介入封堵、体肺侧枝动脉结扎三种方式治疗，及时处理预后好。

Development of major aortopulmonary collateral arteries are strongly associated with cyanotic congenital heart disease. However, they have rarely been reported in noncyanotic congenital heart disease. We report a rare case of a newborn originally diagnosed with an atrial septal defect, a ventricular septal defect, and pulmonary arterial hypertension who underwent complete repair. Failure to progress postoperatively lead to the delayed diagnosis of aortopulmonary collateral arteries. Percutaneous embolization and surgical ligation of aortopulmonary collateral arteries resulted in rapid recovery.

目的 结论总结新生儿危重先天性心脏病(Critical congenital heart disease,cCHD)术前术后一体化救治经验.方法 收集2019年3月至2021年3月云南省阜外心血管病医院收治的39例新生儿cCHD临床资料,男24例,女15例.其中早产儿4例,双胎3例;顺产25例,剖宫产14例.出生体重1.5～4.2(2.97±0.59)kg,入院日龄0～28(14.0±8.8)天,入院身高40～57(49.0±4.1)cm,入院体重1.6～4.8(3.0±0.7)kg.39例中30例行手术治疗,其中体外循环下心脏直视手术22例,非体外循环手术8例;矫治手术28例,姑息手术2例;延迟关胸2例.结果 术后呼吸机治疗时间19～2778[48.0(156.5,383.0)]h[因结果呈偏态分布,采用M(P25,P75)表示,下同],ICU停留时间2～152[19.3(23.0,31.7)]d,总住院时间8～159[27.3(33.0,48.5)]d.全组无手术死亡;1例并发坏死性小肠结肠炎,家属拒绝行开腹探查术而放弃治疗,自动出院.随访2～24月,1例于出院1周后因呛奶窒息死亡;另1例因心功能差,术后6个月死亡;其余患儿均恢复良好.结论 cCHD新生儿应迅速转移到设备齐全、技术力量强的小儿心脏中心进行救治.术前维持内环境稳定、平衡体-肺循环阻力等综合治疗,对导管依赖型cCHD应用前列腺素E1泵入,可改善患儿的状态,创造良好的手术机会.及时的手术或介入治疗是救治cCHD患儿的关键,精细的围术期管理是救治成功的重要保障.

目的 分析新生儿复杂先天性心脏病(先心病)手术结果,总结围术期治疗经验.方法 回顾性分析我院2015年1月～2019年12月5年间218例新生儿住院及手术相关的临床资料.结果 全组出院存活210例,死亡8例,手术死亡率为3.7％.术后呼吸机辅助时间为2～389(88 ±85)h,ICU治疗时间为1～35(8 ±6)d,总住院时间为8～42(18 ±9)d.结论 在小儿心脏外科团队各学科间的紧密配合下,对患儿予以积极的术前干预,恰当的手术时机、术式以及个性化的术后治疗策略,新生儿复杂先心病的外科治疗确实能取得良好的临床效果.

1 临床资料 三尖瓣关闭不全是一种常见的心脏瓣膜疾病,以继发性关闭不全多见,合并右心功能不全者再次行外科瓣膜置换术风险大,死亡率高.近年来经导管瓣膜修复和置换技术发展迅速,成为外科高危患者的有效治疗策略.我们报道在单纯超声引导下经导管瓣膜置换成功治疗一例三尖瓣闭锁行右心房、右心室连接术后,三尖瓣置换术后三尖瓣重度关闭不全的病例.

Objective To evaluate the efficacy of pulmonary surfactant(PS)in the treatment of acute respira﹣tory distress syndrome(ARDS)in the infants after congenital heart disease(CHD)operation. Methods The trial Was conducted from January 2012 to June 2017 in FuWai Hospital,and 90 infants Who had ARDS after cardiac surgery Were enrolled. They Were divided into 2 separate groups,PS group(conventional therapy +PS treatment,43 cases)and con﹣trol group(conventional therapy,47 cases). The clinical data,including age,body Weight,risk adjustment for congenital heart surgery 1(RACHS－1)degree,cardiopulmonary bypass( CPB)time,aortic occlusion time,mechanical ventila﹣tion time,intensive care unit(ICU)care time,mortality,complications,partial pressure of oxygen[pa(O2 )],fraction of inspiration O2(FiO2 )and pa(O2 )／FiO2(P／F),Were collected and analyzed. Results The average age,body Weight, RACHS－1 degree,CPB time and aortic occlusion time had no difference betWeen the 2 groups(P＞0. 05). After 24 h of treatment,pa(O2 )in PS group Was(94. 76 ± 13. 25)mmHg(1 mmHg﹦0. 133 kPa),Which Was significantly higher than that in control group[(67. 59 ± 7. 47)mmHg](P﹤0. 01). P／F in PS group Was 170. 37 ± 20. 62,Which Was sig﹣nificantly higher than that in control group(102. 65 ± 15. 29)( P﹤0. 01),While FiO2 in PS group Was(55. 18 ± 6. 89)%,Which Was significantly loWer than that in control group[(68. 59 ± 9. 59)%](P﹤0. 01). The mean me﹣chanical ventilation time[(194. 39 ± 26. 30)h]and ICU care time[(11. 64 ± 3. 26)d]of the experimental group Were shorter than control group[(288. 63 ± 26. 42)h and(16. 65 ± 4. 18)d],and the differences Were statistically significant(P﹤0. 01). Eight deaths occurred,PS group had 1 infant dead,and the control group had 7 deaths,so the mortality in PS group Was 2. 32%,loWer than that in control group(14. 89%),and the difference Was statistically sig﹣nificant(P﹤0. 01). Postoperative complications occurred in 23 patients,of Which PS group had 9 cases postoperative complications and control group had 15 cases,so the incidence of complications in PS group Was 20. 93%,loWer than that in control group(31. 91%),and the difference Was statistically significant(P ﹤0. 01). Conclusions PS has good efficacy in treating ARDS in infants With ARDS after congenital heart disease operation.

目的:回顾性分析肾替代治疗在先天性心脏病术后中的应用.方法:回顾分析2015-01-2017-02于阜外医院进行肾替代治疗的患儿,记录患儿如下资料:人口学数据,诊断,手术情况,术后循环状态,ICU时间,呼吸机辅助时间,肾替代治疗相关情况包括启动时间、肾替代治疗方式、应用时间等,肾功能恢复情况,病情转归.结果:同期小儿中心共手术治疗先天性心脏病8 079例,其中进行肾替代治疗134例(1.66％),单纯腹膜透析100例,连续性肾替代治疗34例,连续性肾替代治疗中有25例曾进行过腹膜透析.腹膜透析启用时间(中位数,四分位间距)为2(0.1,7)d,连续性肾替代治疗启用时间(中位数,四分位间距)为4(0.2,23)d.腹膜透析应用时间(均数±标准差)为(6.15±3.29)d,连续性肾替代治疗应用时间(均数±标准差)为(7.78±5.61)d.全组未发生损害重要器官系统或危及生命的并发症.连续性肾替代治疗组中,发生1例一过性静脉回流障碍,撤除置管后迅速缓解,另1例出现严重消化道出血,需要输注红细胞,停用连续性肾替代治疗后出血停止.连续性肾替代治疗组常见的问题是血管通路引流不畅,影响血流速度.死亡患儿15例,其中腹膜透析患儿10例,连续性肾替代治疗患儿5例;死于肾功能衰竭2例,均为连续性肾替代治疗患儿.结论:先天性心脏病术后肾替代治疗安全、有效,应积极应用.腹膜透析目前仍是治疗的首选,对于腹膜透析无效或效果差、急重症病例或某些特殊病例需要应用连续性肾替代治疗.

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.

目的 评价应用肺表面活性物质(pulmonary surfactant,PS)治疗婴儿先天性心脏病(congenital heart disease,CHD)术后呼吸窘迫综合征(acute respiratory distress syndrome,ARDS)的时效性.方法 应用PS治疗CHD婴儿术后ARDS,符合入选标准患儿43例,年龄(5.3±2.8)个月,体质量(6.4±1.6) kg,记录患儿手术体外循环时间、主动脉阻断时间、机械通气时间、ICU时间、病死率、并发症发生率.记录患儿给药前,给药后2,6,12和24h时间点患儿呼吸功能指标,计算氧合分数(PF)值,氧合指数(OI)和通气指数(Ⅵ).结果患儿体外循环转机时间(151±23) min,主动脉阻断时间(56±12) min.术后ICU治疗时间为(12±3)d;呼吸机辅助时间为(194 ±26)h.全部患儿有1例死亡(病死率为2％),发生术后并发症9例(21％).患儿应用PS后呼吸指标明显好转,PaO2自2h开始逐渐升高至24h,呈时间相关性改善;呼吸机峰压,平均压和给氧浓度FiO2(％)则在给药后24 h内逐渐降低,呈时间相关性改善.PF在24h内相应逐渐增高,Ⅵ和OI在24 h内相应逐渐降低,呈时间相关性改善.结论 CHD婴儿术后ARDS者应用PS治疗,患儿呼吸功能在24h内呈时间相关性改善.

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.

目的 评价肺表面活性物质(pulmonary surfactant,PS)对不同月龄先天性心脏病(先心病)婴儿术后呼吸窘迫综合征(acute respiratory distress syndrome,ARDS)治疗效果.方法 采用PS治疗先心病患儿术后ARDS,其中2012年1月至2017年6月符合入选标准的患者共43例.分为小婴儿组(＜3个月,12例)、婴儿组(3个月至1岁,31例),比较两组患儿用药前后氧合和换气指标的变化情况.结果 小婴儿组体重更低,两组患儿体外循环时间、主动脉阻断时间、机械通气时间、住ICU时间差异无统计学意义,两组患儿在用药前动脉血氧分压(PaO2)、氧吸人浓度(FiO2)、氧合指数(PaO2/FiO2,P/F)及肺泡动脉氧压比(a/A)差异无统计学意义(P＞0.05).用药治疗24 h后,两组患儿与用药前比较,PaO2、P/F及a/A均显著升高;FiO2明显降低,差异有统计学意义(P＜0.05),用药24 h后,小婴儿组PaO2、P/F及a/A显著高于婴儿组,小婴儿组FiO2显著低于对照组.结论 PS治疗对小月龄先心病患儿术后ARDS者有更好的氧合通气改善作用.

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.

目的:ECMO 作为先心病术后心肺支持的重要手段,为受损的心、肺功能的恢复赢得了时间,使一部分以往无法存活的患儿得到了生存的机会.本文旨在探讨小儿多学科团队协作并发症防控模式对降低 ECMO 期间并发症及死亡率的作用.

目的:冠状动脉起源于肺动脉(ALCAPA)是一种少见但危及生命的先天性心脏畸形,部分患儿伴发严重的心脏扩大,心肌功能受损,冠脉移植术后转归存在个体差异.本文分析此类患儿术后早期心脏功能恢复特点,并探讨影响术后早期恢复的相关影响因素.