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OBJECTIVES Vascular rings are rare anomalies of congenital heart disease that cause respiratory and gastrointestinal symptoms. This study assessed the long-term outcomes of patients with vascular ring division. METHODS A multi-institution retrospective review of 371 patients with vascular rings undergoing surgical division at 3 paediatric cardiac institutions between November 2007 and October 2019 was performed. RESULTS The complete vascular rings consisted of a double aortic arch (24.5%), right aortic arch with left ligamentum arteriosum (36.7%) and left aortic arch, with right ligamentum arteriosum (0.5%). The incomplete vascular rings consisted of a pulmonary artery sling (22.9%), left aortic arch with aberrant right subclavian artery (15.1%) and innominate artery compression syndrome (0.3%). Respiratory symptoms included stridor (71.4%), wheezing (49.1%), coughing (31.5%), gastrointestinal symptoms included choking (12.4%), dysphagia (3.2%) and emesis (1.9%). Only one patient died after discharge, yielding a late mortality rate of 0.3% (1/360). The 10-year overall survival rate was 96.8%. Postoperative complications were reported in 51 patients, 15 of whom required reoperation. The 10-year freedom from reoperation rate was 95.9%. Follow-up was completed in 95.4% (354/371) of patients, with a mean follow-up time of 4.3 ± 2.9 years (range from 1 to 13 years). Twenty patients (5.6%) experienced residual symptoms during long-term follow-up. CONCLUSIONS The outcomes of vascular ring division are excellent. A Kommerell diverticulum >1.5 times the aberrant left subclavian artery origin is an operative indication for primary resection. Tracheomalacia is a risk factor for reoperation and residual symptoms, and preoperative fibrobronchoscopy is important for evaluation.

Vitamin E is the most abundant lipid-soluble antioxidants present in plasma; however, the relationship between serum vitamin E and change in body mass index (BMI)-for-age Z scores in adolescents has not been well described. This study is a cross-sectional study. Data were analyzed from 4014 adolescents who participated in the National Health and Nutrition Examination Survey. The nutritional status was calculated by BMI Z scores and was classified into normal weight, overweight, and obese. Multivariable-adjusted logistic regression was used to examine the association between serum vitamin E levels with overweight/obesity. Besides, the interaction effects between potential confounders and vitamin E on obesity were further evaluated. After adjusting potential confounders, serum vitamin E levels were negatively associated with overweight/obesity in girls but not in boys. Per standard deviation increment in vitamin E concentrations was associated with a 92% decreased risk of obesity in females. Besides, lower quartiles of serum vitamin E were associated with a higher risk of overweight/obesity in girls. Moreover, the inverse association between serum vitamin E levels and obesity was also found in most subgroups through subgroup analysis. Our study supports the negative association between serum vitamin E levels and overweight/obesity in adolescents. A higher serum vitamin E level may be associated with a reduced probability of obesity in girls, but not in boys.

Background The coronavirus disease 2019 (COVID-19) has caused a global pandemic, resulting in considerable mortality. The risk factors, clinical treatments, especially comprehensive risk models for COVID-19 death are urgently warranted. Methods In this retrospective study, 281 non-survivors and 712 survivors with propensity score matching by age, sex, and comorbidities were enrolled from January 13, 2020 to March 31, 2020. Results Higher SOFA, qSOFA, APACHE II and SIRS scores, hypoxia, elevated inflammatory cytokines, multi-organ dysfunction, decreased immune cell subsets, and complications were significantly associated with the higher COVID-19 death risk. In addition to traditional predictors for death risk, including APACHE II (AUC = 0.83), SIRS (AUC = 0.75), SOFA (AUC = 0.70) and qSOFA scores (AUC = 0.61), another four prediction models that included immune cells subsets (AUC = 0.90), multiple organ damage biomarkers (AUC = 0.89), complications (AUC = 0.88) and inflammatory-related indexes (AUC = 0.75) were established. Additionally, the predictive accuracy of combining these risk factors (AUC = 0.950) was also significantly higher than that of each risk group alone, which was significant for early clinical management for COVID-19. Conclusions The potential risk factors could help to predict the clinical prognosis of COVID-19 patients at an early stage. The combined model might be more suitable for the death risk evaluation of COVID-19.

Background:The COVID-19 pandemic has been considered a great threat to global public health. We aimed to clarify the risk factors associated with the development of acute respiratory distress syndrome (ARDS) and progression from ARDS to death and construct a risk prediction model.Methods:In this single-centered, retrospective, and observational study, 796 COVID-19 patients developed ARDS and 735 COVID-19 patients without ARDS were matched by propensity score at an approximate ratio of 1:1 based on age, sex and comorbidities. Demographic data, symptoms, radiological findings, laboratory examinations, and clinical outcomes were compared between those with or without ARDS. Univariable and multivariable logistic regression models were applied to explore the risk factors for development of ARDS and progression from ARDS to death and establish a comprehensive risk model. Results:Higher SOFA, qSOFA, APACHE II and SIRS scores, elevated inflammatory cytokines, dysregulated multi-organ damage biomarkers, decreased immune cell subsets were associated with higher proportion of death (34.17% vs 1.22%; PPPPP+ T cells (OR=0.983, 95%CI=0.976-0.990; P-CD19+ B cells (OR=0.992, 95%CI=0.988-0.997; P=0.003) as novel risk factors. Most importantly, the predictive accuracy of the combined model integrating four score systems and these risk factors demonstrated highest among all models for the development of ARDS (AUC= 0.904) and the progression from ARDS to death (AUC= 0.959).Conclusion:COVID-19 patients with ARDS were more likely to develop into death. The potential risk factors and the comprehensive prediction model could be helpful to identify patients that are at risk of developing ARDS with poor prognosis at an early stage, which might help physicians to formulate a timely therapeutic strategy.

Background The coronavirus disease 2019 (COVID-19) has caused global pandemic, resulting in considerable mortality. The risk factors, clinical treatments and especially comprehensive risk models for COVID-19 death are urgently warranted. Methods In this retrospective study, 281 non-survivors and 712 survivors with propensity score matching by age, sex and comorbidities were enrolled from January 13, 2020 to March 31, 2020. Results Higher SOFA, qSOFA, APACHE II and SIRS scores, hypoxia, elevated inflammatory cytokines, multi-organ dysfunction, decreased immune cells subsets and complications were significantly associated with the higher COVID-19 death risk. In addition to traditional predictors for death risk, including APACHE II (AUC = 0.83), SIRS (AUC = 0.75), SOFA (AUC = 0.70) and qSOFA scores (AUC = 0.61), another four prediction models that included immune cells subsets (AUC = 0.90), multiple organ damage biomarkers (AUC = 0.89), complications (AUC = 0.88) and inflammatory-related indexes (AUC = 0.75) were established. Additionally, the predictive accuracy of combining these risk factors (AUC = 0.950) was also significantly higher than that of each risk group alone, outperforming previous risk models, which was significant for early clinical management for COVID-19. Conclusions The potential risk factors could help to predict the clinical prognosis of COVID-19 patients at an early stage. The combined model might be more suitable for the death risk evaluation of COVID-19.

Background: The COVID-19 pandemic has been considered as the great threat to global public health. We aimed to clarify the risk factors associated with the development of acute respiratory distress syndrome (ARDS) and progression from ARDS to death and construct a risk prediction model.Methods: In this single-centered, retrospective, and observational study, 796 COVID-19 patients developed ARDS and 735 COVID-19 patients without ARDS were matched by propensity score at an approximate ratio of 1:1 based on age, sex and comorbidities. Demographic data, symptoms, radiological findings, laboratory examinations, and clinical outcomes were compared between with or without ARDS. Univariable and multivariable logistic regression models were applied to explore the risk factors for development of ARDS and progression from ARDS to death and establish a comprehensive risk model. Results: Higher SOFA, qSOFA, APACHE II and SIRS scores, elevated inflammatory cytokines, dysregulated multi-organ damage biomarkers, decreased immune cell subsets were associated with higher proportion of death (34.17% vs 1.22%; PPPPP+ T cells (OR=0.983, 95%CI=0.976-0.990; P-CD19+ B cells (OR=0.992, 95%CI=0.988-0.997; P=0.003) as novel risk factors. Most importantly, the predictive accuracy of the combined model integrating four score systems and these risk factors demonstrated highest among all models for the development of ARDS (AUC= 0.904) and the progression from ARDS to death (AUC= 0.959).Conclusion: COVID-19 patients with ARDS were more likely to develop into death. The potential risk factors and the comprehensive prediction model could be helpful to identify patients developed ARDS with poor prognosis at an early stage, which might help physicians to formulate a timely therapeutic strategy.

Background: Inflammatory myofibroblastic tumor (IMFT) is a rare neoplasm mainly affecting children and young adults. We conducted a retrospective study to evaluate the clinical features and treatment alternatives of childhood inflammatory myofibroblastic tumors.Methods: A total of 19 patients who were pathologically diagnosed with IMT between December 2008 and October 2018 were included. Collected data were demographic information, main complaints, tumor characteristics, treatment, pathological results, immunohistochemical analysis, and prognosis.Results: The male/female ratio was 13:6. The mean age at disease onset was 44.9 ± 33.9 months (range 4 to 111 months). The mean tumor size was 6.5 ± 4.0 cm (range 1.2 to 17.0 cm). The most common site was the abdomen (13/19). The most commonly used detection tool was CT. Eleven patients (57.9%) had aggressive tumor growth, including eight receiving extensive resection and three receiving palliative resection due to high local invasiveness and postoperative chemotherapy. Eight cases whose tumors were completely enveloped received complete resection. Immunohistochemistry was performed for 17 patients and ALK positivity was found in 11 patients. Despite three children lost to follow-up, sixteen patients were followed up for 6 to 132 months (average 63.9 months, median 66 months). Of which, twelve children survived with no evidence of IMT, and four cases (21%) showed local recurrences (two of them died). No distant metastasis was detected.Conclusions: IMT is rare in children with various locations, mostly appearing in the abdomen. Whether the tumor could be completely removed, the location and the invasiveness of surrounding tissues might be highly prognosis-related.

Background Results on the association between trans-β-carotene and obesity are less clear and little is known about how their relationship may be affected by plasma glucose levels.The present study aimed to evaluate the relationships between trans-β-carotene and obesity and to investigate whether plasma glucose levels had a modifying effect on these relationships. Methods Children aged 6-18 years were selected from the National Health and Nutrition Examination Survey(NHANES) (2001–2006) (n =8030). The serum trans-β-carotene levels were divided into tertiles, and their associations with obesity were evaluated using multivariable-adjusted linear regression models adjusted for potential confounding factors. The interaction effects between trans-β-carotene levels and plasma glucose levels on obesity were further evaluated. Results In the fully adjusted model, using serum trans-β-carotene as natural log-transformed continuous variable, the negative association between trans-β-carotene level and obesity were confirmed. In addition, plasma glucose levels significantly modified the inverse association between trans-β-carotene and obesity (p value for interaction: 0.09). A stronger association of trans-β-carotene levels with obesity was found in higher plasma glucose levels (more than100 mg/dl) than in lower plasma glucose levels. Further, a non-linear relationship was detected between trans-β-carotene and obesity in participants with higher plasma glucose levels, with an inflection point of 2.7 (trans-β-carotene =14.88 ug/dl). The effect sizes and confidence intervals for the left and right sides of the inflection point were 0.10 (0.00 to 0.2) and 6.7 (0.1 to 348.2), respectively. Conclusion Our findings indicate that the association between trans-β-carotene concentration and obesity is stronger in individuals with higher plasma glucose population than in those with lower plasma glucose levels.

BACKGROUND: Video-assisted thoracic surgery (VATS) has been increasingly used in pediatric patients. We evaluated the outcomes of VATS and thoracotomy for pediatric mediastinal tumors. METHODS: A total of 137 patients who underwent surgery for mediastinal tumors at our department from March 2012 to September 2018 were recruited in this retrospective study. Forty-three patients were treated by VATS and ninety-four underwent thoracotomy (two patients who underwent open surgery were excluded from the study because they were lost to follow-up). Data including demographic information, tumor characteristics, operative time, conversion of surgery, blood transfusion, postoperative hospital stay, mortality and recurrence were collected and compared between the two groups. RESULTS: No significant difference was found in gender, age, weight and tumor distribution between the two groups. The number of patients with malignant tumors who underwent thoracotomy was significantly higher than those who received VATS (78.0% vs. 22.0%, P=0.04). Patients received thoracotomy had significantly larger mean tumor diameter than those who underwent VATS (7.6±3.8 vs. 4.4±1.7 cm, P

// Jun Li 1, * , Shiwei Yang 1, * , Zhening Pu 2, * , Juncheng Dai 2 , Tao Jiang 2 , Fangzhi Du 2 , Zhu Jiang 2 , Yue Cheng 2 , Genyin Dai 1 , Jun Wang 1 , Jirong Qi 3 , Liming Cao 1 , Xueying Cheng 1 , Cong Ren 1 , Xinli Li 4 , Yuming Qin 1 1 Department of Cardiology, Children's Hospital of Nanjing Medical University, Nanjing 210008, China 2 Department of Epidemiology and Biostatistics, Jiangsu Key Lab of Cancer Biomarkers, Prevention and Treatment, Collaborative Innovation Center for Cancer Medicine, School of Public Health, Nanjing Medical University, Nanjing 211166, China 3 Department of Cardiothoracic Surgery, Children's Hospital of Nanjing Medical University, Nanjing 210008, China 4 Department of Cardiology, The First Affiliated Hospital, Nanjing Medical University, Nanjing 210029, China * These authors contributed equally to this work Correspondence to: Shiwei Yang, email: jrdoctoryang@163.com Yuming Qin, email: doctor025ym@163.com Xinli Li, email: xinli3267@yeah.net Keywords: total anomalous pulmonary venous return (TAPVR), genetics, whole-exome sequencing (WES), rare genetic variant, congenital disease Received: August 01, 2016 Accepted: February 06, 2017 Published: February 17, 2017 ABSTRACT As a rare type of Congenital Heart Defects (CHD), the genetic mechanism of Total Anomalous Pulmonary Venous Return (TAPVR) remains unknown, although previous studies have revealed potential disease-driving regions/genes. Blood samples collected from the 6 sporadic TAPVR cases and 81 non-TAPVR controls were subjected to whole exome sequencing. All detected variations were confirmed by direct Sanger sequencing. Here, we identified 2 non-synonymous missense mutations: c.C652T, p.R218W in activin A receptor type II-like 1 (ACVRL1), c.C717G, p.D239E in sarcoglycan delta (SGCD). Our results offered the landscape of mutations for TAPVR in Chinese population firstly and are valuable in the mutation-based pre- and post-natal screening and genetic diagnosis for TAPVR.

Background Primary cardiac tumors in children are extremely rare. This study aimed to analyze clinical treatment and follow-up of pediatric primary cardiac tumors. Patients and methods We performed a retrospective analysis by searching the medical records of 75 patients diagnosed with pediatric primary cardiac tumors from June 2005 to August 2017 in our institution. We followed operative patients every half year in the first postoperative year and then at least every year. If the patients had no serious symptoms or hemodynamic changes, they received nonoperative management and were followed regularly every year. Results Nineteen patients underwent surgery at our department for serious symptoms and critical hemodynamic changes. Four patients had postoperative complications. Two died of low cardiac output syndrome and arrhythmia after surgery. One patient with myxomas had tumor recurrence and one had been found of another rhabdomyoma after surgery. The other 14 patients recovered well. Fifty-six patients had nonoperative management. Four were lost in follow-up. Two patients with malignant tumors died of unknown causes after discharge. The remaining patients had no severe symptoms or tumor growth during follow-up. Conclusions Clinical treatment of pediatric primary cardiac tumors should be performed individually. Most pediatric primary cardiac tumors are benign, and spontaneous regression is possible, especially for rhabdomyomas. The principle purpose of surgical treatment is to restore normal hemodynamics and protect important structures and cardiac tissue.

To compare the efficacy of thoracoscopic surgery versus thoracotomy in repairing esophageal atresia type Ⅲ with tracheoesophageal fistula (EA/TEF) in neonates.A retrospective analysis was conducted in 97 neonates who underwent EA/TEF repair between January 2012 and December 2017 in the Affiliated Children's Hospital of Nanjing Medical University, including 75 patients receiving thoracotomy and 22 patients receiving thoracoscopic surgery. The perioperative data and the incidence of early postoperative complications were compared between two groups.The operations were completed in all patients. One child (4.5%) in thoracoscopic surgery group was converted to thoracotomy with modified Livaditis procedure due to the long distance of two blind ends (4 cm) and thinner distal end. The operation time was longer in thoracoscopic surgery group[(143±48) min vs. (120±40) min,Thoracoscopic repair is a preferred surgical procedure for EA/TEF in neonates.

The risk-benefit profile of transpyloric vs gastric feeding in mechanically ventilated (MV) patients has not been definitively established.To evaluate the risks and benefits of transpyloric feeding compared with gastric feeding in mechanically ventilated patients.We systematically searched MEDLINE, Google Scholar, EMBASE, and the Cochrane Central Register of Controlled Trials databases for eligible articles through June 21, 2013. Randomized controlled trials (RCTs) that reported a comparison between gastric and transpyloric feeding in MV patients were selected. Two reviewers independently extracted data on populations, methods, outcomes, and risk of bias. Ventilator-associated pneumonia (VAP) was considered the primary outcome.A total of 8 RCTs, including 835 MV patients, were identified and analyzed. Our pooled findings indicated that there was a significant reduction in VAP through transpyloric feeding compared with gastric feeding (relative risk [RR], 0.67; 95% confidence interval [CI], 0.53 to 0.85; P = .001) but not in mortality (RR, 1.08; 95% CI, 0.86 to 1.36; P = .49), length of mechanical ventilation (mean difference [MD], -0.16; 95% CI, -0.75 to 0.43; P = .59), length of stay in the intensive care unit (MD, -0.91; 95% CI, -2.75 to 0.94; P = .34), incidence of diarrhea (RR, 0.9; 95% CI, 0.66 to 1.23; P = .50), and incidence of vomiting (RR, 0.82; 95% CI, 0.25 to 2.72; P = .75).Transpyloric feeding in MV adults was associated with significantly less incidence of VAP compared with gastric feeding. No differences were observed in other outcomes, suggesting that the difference observed in the incidence of VAP may be spurious and needs confirmation.

Perioperative nutritional support has become a hot topic in the clinical management of congenital heart disease (CHD). Postoperative enteral nutrition (EN) offers many benefits, such as protection of the intestinal mucosa, reduced risk of infection, and low clinical costs. Interruptions in EN frequently influence nutritional support and clinical outcomes. We, therefore, aimed to determine the causes of interruptions in postoperative EN in CHD patients and discuss clinical counter measures.We analyzed the data of 360 CHD patients to determine the causes of interruptions in postoperative EN and develop possible clinical strategies to prevent such interruptions.Of the 360 patients (aged from 1 month to 6 years), 198 patients had at least one EN interruption. The total number of interruptions was 498 (average, 2.52 interruptions/ patient). Non-gastrointestinal factors (airway management, fluid overload, invasive procedure, increased intracranial pressure, feeding tube block, and clinical deterioration) accounted for 67.8% (338/498) of all interruptions and gastrointestinal factors (vomiting, gastrointestinal bleeding, diarrhea, constipation, and large gastric residual volume) accounted for 32.2% (160/498). The total number of interruptions and the number of interruptions due to gastrointestinal factors were significantly higher in younger patients (aged from 1-12 months) than in older patients (aged from 1-6 years).Non-gastrointestinal factors were the main causes of interruptions in postoperative EN in CHD patients. Younger patients had a greater number of interruptions as a whole, and more interruptions caused by gastrointestinal factors. Gastrointestinal factors can be reduced by tube feeding and use of gastrointestinal motility drugs.

Ventricular septal defects (VSDs) are estimated to account for 20 to 30% of all congenital heart defects (CHDs). Although a residual shunt is the most common complication of VSD surgery, a second operation that applies the surgical repair method is very difficult because it can increase the possibility of uncontrolled bleeding and the severity of tissue adhesion. Here, we present the first case of percutaneous punctured transcatheter device closure of a residual shunt after VSD repair as a novel method to further develop for the treatment of children with congenital heart disease.

Object The purpose of this study was to determine the effects of diazoxide on apoptosis and the relative mechanisms in a model of brain injury induced by cerebral ischemia/reperfusion (I/R) during deep hypothermia. Methods Three-week-old Sprague–Dawley male rats were randomly and equitably divided into sham-operated group, placebo-treated group and diazoxide-treated group respectively. Specific examination of the regional cerebral blood flow (rCBF) was measured in the three groups continuously during the operation by laser Doppler flowmetry. Terminal deoxynucleotidyl transferase mediated dUTP nick end labeling (TUNEL) was showed DNA fragmentation. The mRNA expressions of cytochrome c and full-length caspase-3 were determined by RT-PCR, while the protein expressions of cytochrome c and cleaved caspase-3 were determined by immunohistochemistry at 1 h, 6 h, 24 h, 72 h and 7 days after I/R, respectively. Cytosolic release of cytochrome c at 24 h after I/R was also confirmed by Western blot. Results rCBF was significantly decreased in both of placebo-treated and diazoxide-treated group just after ischemia in the time interval 0–5 min, and had no obvious changes in all the time intervals during the operation. Diazoxide preconditioning significantly decreased the percentage of TUNEL-positive staining cells. The mRNA expressions of cytochrome c and full-length caspase-3 in diazoxide-treated group were significantly decreased. In addition, diazoxide provided a significant reduction in the protein expressions of cytochrome c and cleaved caspase-3. Conclusion These results suggested that the neuroprotective effects of diazoxide against cerebral I/R injury during deep hypothermia correlated with the reduction of DNA fragmentation, prevention of mitochondrial cytochrome c release and inhibition of caspase-3 activation.

Objective: To summarize the technique and clinical experience of the hybrid procedure with cardiopulmonary bypass in children with muscular ventricular septal defect (mVSD). Methods: From January 2006 to June 2010, 45 cases of mVSD underwent hybrid procedures with cardiopulmonary bypass (CPB) under the guidance of transesophageal echocardiography. mVSDs were closed with devices under direct vision in the 45 cases. Fourteen patients had another lesion that required surgical repair. Large membranous VSDs were closed with a pericardial patch after the initiation of CPB in 38 cases. Results: Out of the 45 cases, 42 had only one occluder and three had two occluders. The size of the device for mVSD closure ranged from 3 to 8 mm. All cases recovered smoothly after treatment without residual shunting, aortic or mitral valve regurgitation, or restriction of surrounding structures. All the children survived the operation with no late deaths during the follow-up. Conclusion: The hybrid procedure is safe and effective for the closure of congenital heart defects in children. # 2011 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.