Objective:To review the clinical prognosis and causes of death in children with extracorporeal membrane pulmonary(ECMO) assistance after congenital heart disease(CHD) operation in our center, so as to improve the survival rate of ECMO.Methods:All clinical data of 105 children with VA-ECMO assisted after CHD operation who were admitted to the Department of Cardiothoracic Surgery at Shanghai Children′s Medical Center from January 2017 to December 2019 were collected, and the clinical characteristics and causes of death were retrospectively analyzed.Results:The age of 105 children with ECMO was 110(38, 341)days, the weight was 5.30(3.75, 8.45)kg, and the risk adjustment for congenital heart surgery score was 3(2-3)points.There were no statistically significant differences in gender, age, weight, height, risk adjustment for congenital heart surgery score, the number of ECMO installed in the operating room, the number of ECMO installed after cardiopulmonary resuscitation and ECMO duration between the surviving group ( n=51) and the death group ( n=54)( P>0.05). While there was a significant difference in utilization of continuous renal replacement therapy[7.8% (4/51) vs.38.9% (21/54), P<0.001]. The death mainly occurred within one week after evacuating ECMO(83.3%, 45/54). ECMO installation was most in children aged 1 month to 1 year old(52.4%, 55/105), and the survival rate showed a rise over three years(2017 to 2019), increased from 31.6% (6/19) to 65.0% (13/20). Children with 3 to 5 kg were the most affected (39.0%, 41/105) when ECMO was installed, and the survival rate from 2017 to 2019 increased from 28.6%(4/14) to 75.0%(9/12). The main cause of death was heart failure(48.1%, 26/54), followed by bleeding(18.5%, 10/54)and pulmonary hypertension(13.0%, 7/54). Conclusion:With the progress of surgical technology and cardiopulmonary bypass, and the improvement of postoperative management, the mortality of children with CHD in our hospital after ECMO has decreased year by year during the last three years.However, the mortality of children requiring continuous renal replacement therapy assistance during ECMO is higher.Therefore, it is still necessary to strengthen the maintenance of each organ function during ECMO.

Objective:To explore the reliability of estimated transpulmonary gradient(TPG)by comparing the measured TPG with the estimated TPG in echocardiography.Methods:The cardiothoracic surgery database of Shanghai Children′s Medical Center was reviewed.Children with hemodynamic monitoring and ultrasound findings who underwent total cavopulmonary connection between January 2015 and December 2018 were included.TPG was calculated separately according to the formula.Intraclass correlation efficient was used for consistency test.Results:Finally, 27 patients were selected, including 16 males and 11 females with age(4.0±1.6)years old, weight(15.2±3.3)kg and height(99.1±11.2)cm.There were nine cases (33.3%) of right ventricular double outlet and seven cases (25.9%) of pulmonary atresia.For hemodynamic blood monitoring, TPG was 5-16(10.1±3)mmHg, and its echocardiography parameters were estimated as 5.8-20.3(11±3.3)mmHg.The ICC value was 0.117 which was less than 0.4( P=0.277). Conclusion:TPG estimated by total cavopulmonary connection pipe window during perioperative period is inaccurate and higher than actual value, so invasive hemodynamic monitoring is still recommended during perioperative period.

Objective:To analyze the risk factors of performing diaphragm plication(DPL) after congenital heart disease(CHD) surgery as well as the timing and clinical efficacy.Methods:Data regarding children underwent open heart surgery at Shanghai Children′s Medical Center from January 2017 to December 2019 were reviewed.According to whether DPL was performed after CHD operation or not, the children were divided into DPL group and non-diaphragm plication(NDPL)group.Clinical data including age, surgery, cardiopulmonary bypass(CPB)temperature and time of two groups were compared, meanwhile the risk factors of DPL surgery were analyzed.Based on the median of 8 days between open heart surgery and DPL, children in DPL group were divided into early surgery group(less than 8 days), and delayed operation group(no less than 8 days). The parameters of comparison included ventilator using time, hospital stay time, hospitalization expenditure, postoperative infection to evaluate the timing of DPL and effect.Results:There were 10 309 children after CHD, including 95 in DPL group and 10 214 in NDPL group.In DPL group, there were 52 males(54.7%) and 43 females(45.3%), with age 147(52, 318) d, weight(5.5±4.1) kg, height(56.8±25.6) cm, CPB time(136.8±93.4) min and aortic occlusion time(62.5±50.2) min.Compared with NDPL group, DPL group had younger age, shorter height, lighter weight, higher incidence of preoperative special treatment, higher proportion of reoperation, lower CPB temperature, longer CPB time and longer aortic occlusion time.There were significant differences between two groups( P<0.05). Multivariate Logistic regression analysis showed that younger operative age( OR=0.998, 95% CI 0.998~0.999, P<0.001), staging operation( OR=72.977, 95% CI 39.096~136.211, P<0.001), long CPB time( OR=1.006, 95% CI 1.002~1.011, P=0.008), and pulmonary venoplasty( OR=4.219, 95% CI 2.132~8.350, P<0.001) were independent risk factors for DPL after CHD.Early surgery group had lower postoperative infection rate(43.59% vs. 88.38%, P=0.007), shorter ventilator duration[168.0(99.5, 280.5) h vs.309.9(166.2, 644.5) h, P=0.029], shorter hospital stay duration[27.00(20.75, 35.00)d vs.37.00(28.00, 53.00)d, P<0.001], and lower hospitalization cost[158.36(128.99, 203.11) thousand yuan vs.232.95(174.54, 316.47) thousand yuan, P<0.001] than delayed operation group. Conclusion:Younger age, staging operation, long CPB time, and pulmonary venoplasty are independent risk factors for DPL due to diaphragmatic paralysis after pediatric CHD surgery.Early surgical intervention is beneficial for the recovery of children.

Objective:To examine the effect of surgical treatment in children with pulmonary artery sling and the surgical treatment strategy.Methods:Relevant data of 110 children with pulmonary artery sling admitted to the Department of Cardiac Surgery, Children′s Hospital Affiliated to Shandong University from February 2017 to July 2022 were retrospectively analyzed. There were 55 males and 55 females, aging ( M(IQR)) 9.0 (10.6) months (range: 1 to 96 months). The weight was 7.8 (3.5) kg (range: 2.5 to 25.0 kg). Of the 110 patients, 108 had different degrees of tracheal stenosis and 2 had normal trachea. Left pulmonary artery transplantation and tracheoplasty were performed in 78 patients. Left pulmonary artery transplantation was performed in 30 patients (11 in our hospital and 19 in other hospitals) due to the lack of an early tracheoplasty technique, in which 24 patients needed stage Ⅱ tracheoplasty due to obvious respiratory symptoms and limited activity endurance, and 6 cases did not intervene. Two children with normal trachea only underwent left pulmonary artery transplantation. Results:Among the 78 children who underwent surgery in the same period, 70 cases recovered smoothly after surgery, of whom respiratory symptoms were significantly reduced or disappeared during the 1 to 65 months follow-up, with similar activity endurance to normal children of the same age. Eight cases died, including 4 cases of postoperative multi-drug resistant bacteria infection, died from tracheal anastomotic opening or septic shock, 1 cases with severe congenital heart disease died from postoperative low cardiac output syndrome difficult to correct, 1 case died from blood pressure could not be maintained due to the compressed left pulmonary artery after transplantation, 2 cases of postoperative digestive system diseases (adhesive intestinal obstruction, gastrointestinal bleeding, etc.). The 24 patients in the staging group were followed for 1 to 84 months. All patients needed stage Ⅱ tracheoplasty due to respiratory symptoms and decreased endurance to activity. Eight cases of the non-intervention tracheal group were successfully separated from the ventilator, cured and discharged in a short period of time.Conclusions:Most children with pulmonary artery sling have tracheal stenosis. Children with low degree of tracheal stenosis and inconspicuous respiratory symptoms can only undergo left pulmonary artery transplantation by lateral thoracotomy. For patients combined with severe tracheal stenosis or obvious respiratory symptoms, a simultaneous left pulmonary artery transplantat and tracheoplasty is recommended.

Objective:To examine the treatment strategy of congenital tracheal stenosis associated with non-vascular ring cardiac malformations.Methods:This is a retrospective case series study. Clinic data from 24 children with tracheal stenosis who underwent surgical treatment in the Department of Cardiac Surgery, Children′s Hospital Affiliated to Shandong University from February 2017 to March 2023 were retrospectively collected. There were 16 males and 8 females, aged ( M(IQR)) 6.5 (19.6) months (range: 2.2 to 66.3 months) and weighted 5.95 (4.76) kg (range: 3.2 to 20.0 kg). All patients had obvious respiratory symptoms. Eighteen patients underwent cardiac malformation correction and tracheoplasty at the same time (simultaneous group). Six patients in the staged operation group were treated with cardiac malformation correction in the first stage operation and tracheoplasty in the second stage operation due to missed diagnosis or delayed diagnosis of tracheal stenosis or no condition for tracheoplasty. Slide tracheoplasty was used to correct tracheal stenosis in both groups. The recovery of the children was followed. Wilcoxon sign rank test was used for comparison between the two groups. Results:There was no death during the perioperative period and hospitalization. In the simultaneous group, 1 case with delayed chest closure underwent bedside chest closure after 52 hours, 2 cases were intubated again after operation, and 1 case was implanted with an endotracheal stent. The duration of mechanical ventilation was 40.5 (39.6) hours (range: 19.0 to 438.8 hours). In the staged group, there was 1 case of re-intubation after operation, combined with left vocal cord paralysis and respiratory multidrug-resistant bacterial infection ( Acinetobacter baumanii). One patient underwent 3 times of bronchoscopic balloon dilatation of the right middle bronchus, and heart rate returned to normal range. The duration of mechanical ventilation was 19.0 (21.4) hours (range: 17.1 to 96.7 hours). During follow-up, a patient in the simultaneous group was prone to respiratory infection and had good exercise tolerance, 1 case in the staged group still had sputum stridor in the throat 3 months after the operation, and symptoms improved significantly 6 months after the operation. The other children didn′t have obvious respiratory symptoms. Conclusions:The diagnosis of tracheal stenosis may be delayed or missed when tracheal stenosis is complicated by non-vascular ring cardiac malformations. One-stage correction of tracheal stenosis and cardiac malformation can achieve a good outcome.

Objective:To examine the effect of surgical treatment in children with pulmonary artery sling and the surgical treatment strategy.Methods:Relevant data of 110 children with pulmonary artery sling admitted to the Department of Cardiac Surgery, Children′s Hospital Affiliated to Shandong University from February 2017 to July 2022 were retrospectively analyzed. There were 55 males and 55 females, aging ( M(IQR)) 9.0 (10.6) months (range: 1 to 96 months). The weight was 7.8 (3.5) kg (range: 2.5 to 25.0 kg). Of the 110 patients, 108 had different degrees of tracheal stenosis and 2 had normal trachea. Left pulmonary artery transplantation and tracheoplasty were performed in 78 patients. Left pulmonary artery transplantation was performed in 30 patients (11 in our hospital and 19 in other hospitals) due to the lack of an early tracheoplasty technique, in which 24 patients needed stage Ⅱ tracheoplasty due to obvious respiratory symptoms and limited activity endurance, and 6 cases did not intervene. Two children with normal trachea only underwent left pulmonary artery transplantation. Results:Among the 78 children who underwent surgery in the same period, 70 cases recovered smoothly after surgery, of whom respiratory symptoms were significantly reduced or disappeared during the 1 to 65 months follow-up, with similar activity endurance to normal children of the same age. Eight cases died, including 4 cases of postoperative multi-drug resistant bacteria infection, died from tracheal anastomotic opening or septic shock, 1 cases with severe congenital heart disease died from postoperative low cardiac output syndrome difficult to correct, 1 case died from blood pressure could not be maintained due to the compressed left pulmonary artery after transplantation, 2 cases of postoperative digestive system diseases (adhesive intestinal obstruction, gastrointestinal bleeding, etc.). The 24 patients in the staging group were followed for 1 to 84 months. All patients needed stage Ⅱ tracheoplasty due to respiratory symptoms and decreased endurance to activity. Eight cases of the non-intervention tracheal group were successfully separated from the ventilator, cured and discharged in a short period of time.Conclusions:Most children with pulmonary artery sling have tracheal stenosis. Children with low degree of tracheal stenosis and inconspicuous respiratory symptoms can only undergo left pulmonary artery transplantation by lateral thoracotomy. For patients combined with severe tracheal stenosis or obvious respiratory symptoms, a simultaneous left pulmonary artery transplantat and tracheoplasty is recommended.

Objective:To examine the treatment strategy of congenital tracheal stenosis associated with non-vascular ring cardiac malformations.Methods:This is a retrospective case series study. Clinic data from 24 children with tracheal stenosis who underwent surgical treatment in the Department of Cardiac Surgery, Children′s Hospital Affiliated to Shandong University from February 2017 to March 2023 were retrospectively collected. There were 16 males and 8 females, aged ( M(IQR)) 6.5 (19.6) months (range: 2.2 to 66.3 months) and weighted 5.95 (4.76) kg (range: 3.2 to 20.0 kg). All patients had obvious respiratory symptoms. Eighteen patients underwent cardiac malformation correction and tracheoplasty at the same time (simultaneous group). Six patients in the staged operation group were treated with cardiac malformation correction in the first stage operation and tracheoplasty in the second stage operation due to missed diagnosis or delayed diagnosis of tracheal stenosis or no condition for tracheoplasty. Slide tracheoplasty was used to correct tracheal stenosis in both groups. The recovery of the children was followed. Wilcoxon sign rank test was used for comparison between the two groups. Results:There was no death during the perioperative period and hospitalization. In the simultaneous group, 1 case with delayed chest closure underwent bedside chest closure after 52 hours, 2 cases were intubated again after operation, and 1 case was implanted with an endotracheal stent. The duration of mechanical ventilation was 40.5 (39.6) hours (range: 19.0 to 438.8 hours). In the staged group, there was 1 case of re-intubation after operation, combined with left vocal cord paralysis and respiratory multidrug-resistant bacterial infection ( Acinetobacter baumanii). One patient underwent 3 times of bronchoscopic balloon dilatation of the right middle bronchus, and heart rate returned to normal range. The duration of mechanical ventilation was 19.0 (21.4) hours (range: 17.1 to 96.7 hours). During follow-up, a patient in the simultaneous group was prone to respiratory infection and had good exercise tolerance, 1 case in the staged group still had sputum stridor in the throat 3 months after the operation, and symptoms improved significantly 6 months after the operation. The other children didn′t have obvious respiratory symptoms. Conclusions:The diagnosis of tracheal stenosis may be delayed or missed when tracheal stenosis is complicated by non-vascular ring cardiac malformations. One-stage correction of tracheal stenosis and cardiac malformation can achieve a good outcome.

Objective:To prevent postoperative myocardial oedema or other causes of acute heart failure in congenital cardiac disease,anticipating delay sternal closure may reduce the number of children requiring cardiopulmonary resuscitation after surgery.The aim of this study was to describe the rate of delay sternal closure after surgery for congenital cardiac disease and to analyse the risk factors that may be associated with it.Methods:We retrospectively reviewed all surgeries with extracorporeal circulation in the cardiothoracic surgery intensive care unit of Shanghai Children's Medical Center in the past five years,from September 2014 to December 2018.The study cohort was divided into the delay sternal closure group (n=418) and the control group (routine chest closure,n=12 188) according to whether a delay sternal closure was applicated.Risk factors associated with delay sternal closure were identified by multivariate Logistic regression analysis.Results:A total of 12 606 cases were eligible,of which 418 (3.32%) were in the delayed sternal closure group.The most common cardiac diagnosis in the delayed sternal group was transposition of the great arteries (26.8%,112/418),whereas the most common cardiac diagnosis in the control group was ventricular septal defect (45.9%,5 599/12 188).All-cause mortality in children in the delayed sternal closure group was 3.3% (14/418) compared with 0.4% (46/12 188) in the control group,with a statistically significant difference ( P<0.05).Multivariate Logistic regression analysis showed that the need for delayed sternal closure were associated with age ( OR 0.164,95% CI 0.079-0.338, P<0.001),positive intropic support before surgery ( OR 0.42,95% CI 0.252-0.699, P=0.001),sex（ OR 0.742，95% CI 0.648-1.098， P<0.05），mean body weight（ OR 1.192，95% CI 1.078-1.318， P<0.001），positive intropic support before surgery（ OR 0.370，95% CI 0.252-0.699， P<0.001），complicated surgery ( OR 0.241,95% CI 0.159-0.367, P<0.001) and extracorporeal circulation diversion time ( OR 6.412,95% CI 4.339-9.475, P<0.001). Conclusion:Delayed sternal closure is an important management strategy for congenital cardiac surgery in infants and children.Delayed sternal closure is associated with age，sex,mean body weight at the time of surgery,positive intropic support before surgery，complicated surgery and extracorporeal circulation diversion time.