Background:Hypoplastic left heart syndrome (HLHS) is a severe and complex congenital heart defect characterized by the underdevelopment of the left-sided cardiac structures. Management of HLHS typically involves multiple staged surgical interventions, often requiring repeated use of cardiopulmonary bypass (CPB). Optimizing CPB strategies is crucial for improving outcomes in these patients. Case Description:We present the case of a pediatric patient with HLHS who underwent eight surgical procedures, six of which involved the use of CPB. The case highlights the evolution of CPB management strategies across multiple operations, including transitions between peripheral and central cannulation techniques. Detailed clinical history, imaging findings, operative details, and postoperative outcomes were analyzed. Particular attention was paid to the challenges encountered during CPB weaning and the corresponding intraoperative interventions. Conclusions:Our experience suggests that individualized CPB management, meticulous preoperative planning, and dynamic intraoperative adjustments are essential for optimizing surgical outcomes in complex HLHS cases.

Background Prolonged intensive care unit (ICU) stays consume medical resources and increase medical costs. This study identified risk factors associated with prolonged postoperative intensive care unit (ICU) stay in children with total anomalous pulmonary venous connection (TAPVC). Methods The medical records of 85 patients who underwent surgical repair of TAPVC were retrospectively analyzed. The patients were divided into prolonged-stay and standard-stay groups. The prolonged stay group included all patients who exceeded the 75th percentile of the ICU stay duration, and the standard stay group included all remaining patients. The effects of patient variables on ICU stay duration were investigated using univariate and logistic regression analyses. Results Patient median age was 41 (18–103) days, and median weight was 3.80 (3.30–5.35) kg.Postoperative duration of ICU stay was 11–68 days in the prolonged stay group (n = 23) and 2–10 days in the standard stay group (n = 62). Lower preoperative pulse oximetry saturation (SpO 2 ), higher intraoperative plasma lactate levels, and prolonged postoperative mechanical ventilation were independent risk factors for prolonged ICU stay. Preoperative SpO 2 < 88.5%, highest plasma lactate value > 4.15 mmol/L, and postoperative mechanical ventilation duration was longer than 53.5 h, were associated with increased risk of prolonged ICU stay. Young age, low body weight, subcardiac type, need for vasoactive drug support, emergency surgery, long anesthesia time, low SpO 2 after anesthesia induction, long cardiopulmonary bypass (CPB) and aortic clamp times, high lactate level, low temperature, large volume of ultrafiltration during CPB, large amounts of chest drainage, large red blood cells (RBCs) and plasma transfusion, and postoperative cardiac dysfunction may be associated with prolonged ICU stay. Conclusions Lower preoperative SpO 2 , higher intraoperative plasma lactate levels, and prolonged postoperative mechanical ventilation were independent risk factors for prolonged ICU stay in children with TAPVC. When SpO 2 was lower than 88.5%, the highest plasma lactate value was more than 4.15 mmol/L, and the postoperative mechanical ventilator duration was longer than 53.5 h, the risk of prolonged ICU stay increased. Improved clinical management, including early diagnosis and timely surgical intervention to reduce hypoxia time and protect intraoperative cardiac function, may reduce ICU stay time.

Background Pediatric acute fulminant myocarditis (AFM) is a very dangerous disease that may lead to acute heart failure or even sudden death. Previous reports have identified some prognostic factors in adult AFM; however, there is no such research on children with AFM on venoarterial extracorporeal membrane oxygenation (VA-ECMO). This study aimed to find relevant prognostic factors for predicting adverse clinical outcomes. Methods A retrospective analysis was performed in an affiliated university children’s hospital with consecutive patients receiving VA-ECMO for AFM from July 2010 to November 2020. These children were classified into a survivor group (n=33) and a non-survivor group (n=8). Patient demographics, clinical events, laboratory findings, and electrocardiographic and echocardiographic parameters were analyzed. Results Peak serum creatinine (SCr) and peak creatine kinase isoenzyme MB during ECMO had joint predictive value for in-hospital mortality (p=0.011, AUC=0.962). Based on multivariable logistic regression analysis, peak SCr level during ECMO support was an independent predictor of in-hospital mortality (OR=1.035, 95% CI 1.006 to 1.064, p=0.017, AUC=0.936, with optimal cut-off value of 78 μmol/L). Conclusion Tissue hypoperfusion and consequent end-organ damage ultimately hampered the outcomes. The need for left atrial decompression indicated a sicker patient on ECMO and introduced additional risk for complications. Earlier and more cautious deployment would likely be associated with decreased risk of complications and mortality.

Background Congenital heart disease(CHD)is one of the main supportive diseases of extracorporeal membrane oxygena-tion in children.The management of extracorporeal membrane oxygenation(ECMO)for pediatric CHD faces more severe challenges due to the complex anatomical structure of the heart,special pathophysiology,perioperative complications and various concomitant malformations.The survival rate of ECMO for CHD was significantly lower than other classifica-tions of diseases according to the Extracorporeal Life Support Organization database.This expert consensus aims to improve the survival rate and reduce the morbidity of this patient population by standardizing the clinical strategy.Methods The editing group of this consensus gathered 11 well-known experts in pediatric cardiac surgery and ECMO field in China to develop clinical recommendations formulated on the basis of existing evidences and expert opinions.Results The primary concern of ECMO management in the perioperative period of CHD are patient selection,cannulation strategy,pump flow/ventilator parameters/vasoactive drug dosage setting,anticoagulation management,residual lesion screening,fluid and wound management and weaning or transition strategy.Prevention and treatment of complications of bleeding,thromboembolism and brain injury are emphatically discussed here.Special conditions of ECMO management related to the cardiovascular anatomy,haemodynamics and the surgical procedures of common complex CHD should be considered.Conclusions The consensus could provide a reference for patient selection,management and risk identification of periop-erative ECMO in children with CHD.

体外膜肺氧合（extracorporeal membrane oxygenation, ECMO）技术已成为急性心功能衰竭患儿的临床首选治疗手段之一 [1]，其适用的儿科病种包括暴发性心肌炎 [2]和复杂先天性心脏病术后体外循环脱机困难等引起的严重心功能衰竭等 [3]。常用的插管方式包括颈部插管和正中插管 [4]。静脉-动脉体外膜氧合（veno-arterial ECMO, VA-ECMO）能有效恢复患儿全身循环灌注，但对左心室（left ventricle, LV）的潜在影响不容忽视 [5]。主动脉插管持续泵血增加主动脉根部压力和左室后负荷，进一步加重左室缺血和室性心律失常 [6]。左心室血栓可导致脑梗等致死性并发症 [7]。左心减压能快速有效降低左心负荷，为心脏功能恢复争取更多的时间 [8]。近年来，ECMO支持下，左心减压对于严重左室功能不全的患儿的重要性已得到广泛认可 [9]。截止2020年9月，浙江大学医学院附属儿童医院心脏中心共完成94例患儿V-A ECMO心脏支持，儿童80例（85.1%），新生儿14例（14.9%），撤机率59.6%。2010—2019年V-A ECMO支持下左心减压共完成5例，4例死亡、1例存活，现将相关临床资料和经验分析汇总如下。

女性患儿,1岁5个月,体重10 kg,因"咳嗽5d,喘息3 d,发现心率快1 d"于2019-01-16急诊收治入ICU.入院查体:体温38.1℃,心率174次/min,呼吸36次/min,血压 94/63 mmHg(1 mmHg=0.133 kPa),意识清,心音低,心律不齐.心电图提示阵发性室性心动过速(图1).

Objective:To investigate the preschool period neurodevelopmental status and analyze the perioperative data which associated with delayed neurodevelopment in patients underwent antergrade cerebral perfusion(ACP).Methods:To access the preschool period neurodevelopmental status in patients underwent ACP using Griffiths mental development scale-Chinese(GDS-C). Patients were classified as normal development group(ND) and low development group(LD) depending on the outcomes of assessment. Perioperative data including age, weight, CPB time, aortic cross-clamp time, mean arterial pressure, ACP time and flow were analyzed retrospectively.Results:62 children who met the inclusion criteria, of which 19 were accessed by GDS-C scale. Fourteen cases were lagged in general quotient(GQ) compared with normal children. The outcomes of assessment in six subscales of GDS-C scale indicated that 13 cases were delayed in language(C) and practical reasoning(F). Eight cases were delayed in locomotor(A) and personal-social(B). Eleven cases were delayed in eye-hand coordination(D). Ten cases were delayed in performance(E). The children in LD group had significant longer CPB time and aortic cross-clamp time than those in ND group. There were no differences between two groups in other perioperative data.Conclusion:The incidence of preschool period neurodevelopmental delay after ACP in infants is relatively high. In detailed analysis, their language and practical reasoning ability are lagged significantly. In addition, the longer time of CPB and aortic cross-clamp are associated with the neurodevelopmental delay.

Venoarterial extracorporeal membrane oxygenation (VA ECMO) has been considered as the first-line treatment for acute fulminant myocarditis (AFM) when traditional treat-ment is ineffective.Peripheral vascular VA ECMO can partially reduce right ventricular preload,but it can increase left ventricular(LV) afterload.1 The increased afterload may cause difficulty in LV blood ejection in patients with severely impaired LV function.In addi-tion,it may result in secondary LV dilatation,pulmonary edema,intraventricular throm-bosis,and even increased LV diastolic pressure,leading to myocardial ischemia and irreversible cardiac function,affecting the prognosis of the disease.2 Timely LV decompression can help to improve the prognosis.3 Here,we report a successful case of applying a surgical minimal invasive left atrial decompression method,and we discuss the appropriate timing and method of LV decompression during ECMO supporting in pediatric AFM.

目的 介绍体外膜氧合(ECMO)中血栓形成的判断和处理临床经验,提高对小婴儿ECMO出凝血管理的认识水平,减少血栓栓塞并发症.方法 回顾分析1例小婴儿先天性主动脉弓缩窄术后体外循环脱机困难行静脉-动脉(V-A)EC?MO中并发主动脉插管内血栓及肢体血栓栓塞,结合相关病例报告文献分析该并发症的诊断、原因及处理.结果 患儿成功撤离ECMO出院,右上肢和双下肢的血栓消退,但左手1～5指坏死截指,ECMO中并发少量硬膜下出血.2011年1月至2019年10月期间英文文献病例报告儿童ECMO中血栓形成5例,中文未见文献报道.结论 动脉插管内血栓形成是V-A ECMO的严重并发症,及时更换ECMO管路和插管或许能减少体循环栓塞症状,安全有效的溶栓策略还有待于临床经验积累.

Background Extracorporeal membrane oxygenation (ECMO) support on D-transposition of the great arteries (D-TGA) carries formidable challenges. Methods A retrospective study was performed on pediatric patients with D-TGA supported by ECMO from July 2007 to December 2019. This study summarized the clinical experience of ECMO support in pediatric patients with D-TGA preoperative, intraoperative, and postoperative. Results Overall, 16 children with D-TGA received ECMO support during this period. Two (2 of 16) were supported before cardiac surgery, 3 (3 of 16) were supported postoperatively in the intensive care unit, and 11 (11 of 16) failed to wean off cardiopulmonary bypass. Two cases of preoperative ECMO support for patients with D-TGA with an intact ventricular septum and restrictive atrial septum due to severe hypoxemia died. In this study, D-TGA with coronary artery malformation and other complicated deformities died (8 of 14), whereas uncomplicated D-TGA without coronary artery malformation all survived (6 of 14). The wean-off rate of ECMO patients supported in D-TGA was 62.5% (10 of 16), while the 30-day survival rate was 44% (7 of 16). Conclusion Although a promising ECMO weaning rate was obtained, 30-day survival of this population was frustrating, mainly attributed to the original anatomy of coronary arteries and the concomitant deformities.

目的 总结及探讨右侧颈动脉、颈内静脉切开插管技术在小儿体外膜肺氧合(extracorpo-real membrane oxygenation,ECMO)中的应用价值.方法 回顾性收集2009年11月至2019年2月在浙江大学医学院附属儿童医院经右侧颈部插管ECMO治疗的重症患儿作为研究对象,分析其疾病类型、年龄、体重、ECMO持续时间、并发症及转归等情况.结果 本研究共纳入70例行右侧颈部插管ECMO治疗的重症患儿,其中男童39例,女童31例,均在充分镇静、镇痛、肌松及呼吸机支持下行右侧颈动脉和颈内静脉切开插管.3例插管失败,其余均插管成功,其中3例因静脉狭窄扩大切口远端插管,1例新生儿静脉撕裂扩大切口远端插管,1例迷走神经损伤导致声音嘶哑,2例插管顶端位置不佳予以调整插管,1例出现插管头端血栓予以更换插管,18例在心肺复苏(cardiopulmonary resuscitation,CPR)下插管.ECMO转机均采用V-A模式,插管手术时间(41.1±32.3)min,ECMO转机时间(167.5±133.5)h,中位运行时间120 h.51例成功撤机,撤机时行近心端动静脉结扎.结论 右侧颈动脉、颈内静脉切开插管在小儿ECMO治疗中是安全有效的.

甲型H1N1流感病毒具有很强的嗜肺性,55%重症感染患者可迅速进展为急性呼吸窘迫综合征(acute respiratory distress syndrome ,ARDS),病死率为40%～46%,此类患者对经典机械通气治疗效果欠佳.体外膜氧合(extracorporeal membrane oxygenation ,ECMO)支持现已逐渐成为严重呼吸衰竭的最终治疗手段.然而目前国内在小儿领域,ECMO辅助治疗重症甲型流感相关报道少见.本文总结报道了3例甲型H1N1流感并发ARDS患儿经ECMO治疗的诊疗过程.

目的 总结先天性心脏病体外膜氧合(ECMO)支持患儿的死亡原因,以进一步提高生存率.方法 回顾性分析2010年1月至2018年12月实施ECMO支持的先天性心脏病患儿33例.结果 33例行ECMO支持的先天性心脏病患儿中,死亡率为54.5％(18/33).单纯左心功能不全的完全性大动脉转位(TGA)生存率80％(4/5),辅助效果佳;TGA合并冠脉畸形和其他复杂畸形的死亡率100％(7/7);1例左心发育不良存活;术后心源性休克死亡率29％(2/7);复杂先天性心脏病患儿术前严重低氧急诊手术术后转ECMO的死亡率33.3％(1/3),而TGA术前严重低氧血症先采用ECMO支持2例均于术前死亡;2例ECMO期间再次手术干预的患者均存活出院.结论 ECMO为先天性心脏病并发难治性心衰的患儿提供了生存机会,心脏功能的可逆程度、心脏畸形的类型以及ECMO时机的把握是先天性心脏病患儿ECMO支持是否成功的主要原因.

目的 观察婴幼儿围心脏手术期循环中铁调素水平动态变化情况,初步探索其与临床预后的相关性.方法 选取2017年1月在心肺转流(CPB)下行心脏手术、年龄<3岁的先天性心脏病患儿18例.于手术前,CPB前,CPB后1 h、6 h、12 h和24 h,采用ELISA法检测血浆中铁调素的质量浓度.在手术后第一天和第二天分别检测患儿肝肾功能并记录血肌酐值.记录患儿一般临床资料、住心脏重症监护室(CICU)时间和手术后住院时间.对血浆铁调素水平与临床相关参数的关系进行分析.以P<0.05为差异具有统计学意义.结果 血浆中铁调素水平在CPB后1 h即较手术前明显升高(P<0.001),且在CPB后24 h仍明显高于术前水平(P<0.001).CPB术后1 h血浆中铁调素的质量浓度与患儿术后肾功能障碍明显正相关,表现为与手术后血肌酐水平正相关(r=0.76,P<0.001);还与患儿住CICU时间(r=0.69,P=0.001)和手术后住院时间(r=0.7,P=0.001)正相关.结论 婴幼儿CPB术后循环中升高的铁调素与术后血肌酐水平、住院时间有关,可能作为影响婴幼儿CPB下心脏手术预后的早期预警指标.

目的 研究儿童体外循环管路残余机血和术中自体血洗涤回输及优化心肺转流(CPB)管路两项血液保护措施对临床结果的影响.方法 选择2010年至2012年期间在本院实施CPB手术的单纯室间隔缺损(VSD)或房间隔缺损(ASD)患儿54例,体重15～28 kg,随机分为对照组(CON组,29例)和自体血回输组(CS组,25例),另选择2015年体重10～18 kg的24例患儿,实施自体血回输加优化体外循环管路组(CS+OC组,24例).收集三组患儿的年龄、体重、性别、术前和术后血红蛋白(Hb)、肌酐值;术中CPB时间、主动脉阻断时间、最低红细胞比容(HCT)、最低温度、停机时HCT;围术期输血量、术后乳酸、白细胞和C反应蛋白、术后呼吸道相关的主要临床事件、呼吸机使用时间、ICU时间、住院时间及24 h胸腔引流量,并进行比较.结果 CS组和CS+OC组围术期未输红细胞(RBC),CON组围术期输RBC 1.5(1.0～2.0)U;CS组术后第三天Hb显著低于CON组;CS+OC组年龄、体重、预充量和呼吸机时间显著低于CS组;其余数据比较无差异.结论 儿童CPB术后残余机血和术中自体血洗涤回输,能使体重20 kg左右简单先天性心脏病患儿做到围术期不输异体血,自体血回输联合管路优化可以使10 kg以上单纯VSD或ASD患儿实现无血手术,对术后早期临床结果没有负面影响,减少异体输血的潜在风险.

Background To summarize the experience of management of persistent pulmonary hypertension of the newborn (PPHN) with extracorporeal membrane oxygenation (ECMO) support. Methods We presented three neonates with PPHN supported by ECMO in our center. Medical records and patient management notes were retrospectively reviewed. Results For two neonates with congenital diaphragmatic hernia (CDH), diaphragm repair surgery was done under ECMO support. One patient was weaned from ECMO after 73 hours, and recovered well at the last follow-up after 1 year. The other patient was weaned from ECMO after 167 hours, and he died from septic shock 21 days after decannulation. For the neonate with idiopathic PPHN, ECMO was withdrawn successfully. Conclusions ECMO is an effective rescue means for refractory PPHN. Appropriate intervention timing, accurate coagulation, and volume management are important.

We report a neonatal case of the use of alteplase for the lysis of a large aortic arch thrombus formed during extracorporeal membrane oxygenation support. Alteplase (0.1-0.15 mg/kg/hour) was infused for thrombolysis, and meanwhile, unfractionated heparin was administrated at 5-10 U/kg/hour for the anticoagulation purpose. Alteplase was successfully administered to this neonate after the repair of congenital diaphragmatic hernia on extracorporeal membrane oxygenation, and the patient survived without apparent catastrophic long-term complications. It is reasonable to consider alteplase therapy during extracorporeal membrane oxygenation support in this setting.

Retrospective analysis was performed at an affiliated university children’s hospital with consecutive patients receiving a venoarterial extracorporeal membrane oxygenation (VA-ECMO) for refractory cardiogenic shock from July 2007 to May 2018. Fifty-six patients underwent VA-ECMO for refractory cardiogenic shock with the median age of 39.0 (1.5, 103.5) months were included. Median ECMO duration was 87 h, and the median length of hospital stay was 22 days. Successful ECMO weaning rate was 68%. Thirty-day mortality in this cohort was 39% (22/56), among which the mortality of fulminant myocarditis and postcardiotomy cardiogenic shock (PCS) were 23% (6/26) and 52% (12/23), respectively. Multivariate Cox proportional hazard regression analysis identified prolonged prothrombin time (PT) > 6 s and elevated lactate level 24 h after ECMO initiation were associated with 30-day mortality.

目的 总结先天性膈疝围术期体外膜氧合(ECMO)治疗的临床经验.方法 回顾分析6例先天性膈疝(CDH)围术期在本院接受ECMO治疗患儿的临床资料(男3例,女3例,年龄12～74 h,体重2.71～3.88 kg),总结ECMO适应证和时机选择、ECMO下患者转运、手术时机、出血和血栓形成的处理等临床经验.结果 本组1例主动脉弓缩窄合并CHD患者因体外循环术后脱机困难转为ECMO,5例患者因围术期低氧血症酸中毒常规处理无效建立ECMO,其中1例患儿经本院ECMO团队会诊后在当地医院建立ECMO,运行平稳后转运至本院;4例术前建立ECMO,内环境改善后在ECMO辅助下行膈肌修补术,术后继续ECMO辅助;1例膈肌修补术后出现肺高压低氧血症,经颈部置管建立静脉-动脉(V-A)ECMO.ECMO辅助时间73～170 h.6例患儿中4例出院,1例ECMO撤离后21 d死于感染性休克,1例ECMO中并发坏死性小肠结肠炎和DIC死亡.主要并发症是出血、血栓形成、毛细血管渗漏.结论 ECMO是治疗常规处理无效的CDH围术期低氧血症的有效手段.转运方案、ECMO时机和手术时机选择应根据患儿的疾病状态制定个性化的治疗方案,精准管理包括抗凝、液体管理等的各个环节,有助于减少并发症,提高存活率.