Anomalous aortic origin of the coronary artery(AAOCA) refers to the abnormal initiation, route, or distribution of coronary arteries, which is generally believed to be caused by abnormal or incomplete development of embryonic coronary arteries, and is a rare congenital cardiovascular malformation. It can exist independently without other congenital heart disease. With the development of medical science and people's understanding of AAOCA, more and more AAOCA has been detected, and its clinical significance has attracted more and more attention. Based on abnormal coronary artery opening, malignant or potential contorts can cause long-term blood flow dynamic change, appear abnormal blood vessel hardening of the arteries, at the same time due to walk in the pulmonary artery and the ascending aorta between two vessels, vulnerable to the extrusion of large blood vessels, which caused a temporary blood flow in coronary artery interruption, can cause acute angina pectoris, myocardial infarction, arrhythmia, exercise-induced cardiac syncope. This review focuses on the anatomy, diagnosis and treatment of coronary artery abnormalities arising from aorta.

Objective:To determine the risk factors of reoperation and mortality after complete atrioventricular septal defect repair, and to evaluate the medium and long-term prognosis.Methods:From March 2008 to March 2022, a total of 266 children were selected from the Department of Thoracic and Cardiovascular Surgery, Nanjing Children's Hospital, who underwent the complete atrioventricular septal defect repair. Exclusion of children with conotrucal anomaly such as tetralogy of Fallot, transposition of the great arteries, and right ventricular double outlet. Demographic characteristics, surgical data, postoperative follow-up and associated risk factors were analyzed.Results:All the children were repaired with modified single-piece method for the first time, and 26 children were reoperated because of severe left atrioventricular valve regurgitation, left ventricular outflow tract obstruction and atrioventricular block. The 1-year, 3-year and 5-year overall survival rate and freedom from reoperation rate of all children were (98.1±0.8)%, (97.3±1.0)%, (96.2±1.2)%, (96.6±1.1)%, (93.9±1.5)% and (92.2±1.7)%, respectively. A total of 11 (42.3%) early reoperations and 15 (57.7%) late reoperations were performed, of which 1-year, 3-year and 5-year survival rates were (92.3±5.2)%, (82.1±8.3)% and (76.6±9.4)% respectively. Multifactorial analysis showed that age <3 months and left atrioventricular regurgitation >grade 2 at 24 hours postoperatively were independent risk factors for reoperation, whereas age <3 months and experience of reoperation were independent risk factors for death of children.Conclusion:Complete atrial septal defects have excellent surgical outcomes, but some children still require reoperation, and age <3 months and postoperative left atrioventricular valve regurgitation(LAVVR)>2 grades remain important predictors of their surgical prognosis.

Congenital heart disease (CHD) is the most common birth defect, and in recent years, its neurodevelopmental disorder (NDD) has been gradually emphasized, among which neurodevelopmental disorder in complex congenital heart disease (CCHD) is more common, and the research on related risk factors is gradually deepening. The risk factors of CCHD neurodevelopmental disorder are complex and diversified, with synergistic and cumulative effects, which can be classified into preoperative, surgical-related and postoperative according to the time period, and involve various aspects such as genetics, hemodynamic disorders, extracorporeal circulation, nursing care, and family and social factors. In this paper, we try to review the risk factors of neurodevelopmental disorders in CCHD by combining the latest domestic and international studies.

Objective:To evaluate the feasibility of measuring pulmonary venous blood flow spectrum by transesophageal ultrasound in assessing intraoperative left atrial pressure (LAP) in pediatric patients with complex congenital heart disease.Methods:Twenty-five pediatric patients with complex congenital heart disease of either sex, aged < 3 yr, of American Society of Anesthesiologists physical status Ⅲ or Ⅳ, with New York Heart Association class Ⅲ or Ⅳ, undergoing surgery with general anesthesia and requiring LAP monitoring during surgery, were selected.Transesophageal echocardiography was used to record the left pulmonary venous blood flow spectrum during surgery.S wave velocity (PV S), D wave velocity (PV D), AR wave velocity (PV AR), and deceleration time of pulmonary venous diastolic flow (DT D) were measured at 15 min after termination of CPB.The ratio of S wave peak velocity to D wave peak velocity (S/D ratio) was calculated.Measurement was carried out for 3 consecutive cardiac cycles, and the average value was calculated.The LAP was simultaneously measured through the left atrial piezometer.Pearson correlation analysis was performed between PV S, PV D, PV AR, DT D, S/D ratio and LAP. Results:PV S, PV D, PV AR and S/D ratio had no correlation with LAP ( r=-0.06, 0.21, 0.19, -0.38, respectively, P>0.05), while DT D was negatively correlated with LAP ( r=-0.84, P<0.05). Conclusions:DT D measured by transesophageal ultrasound can be used to evaluate intraoperative LAP in pediatric patients with complex congenital heart disease.

Objective:To investigate the common types, surgical treatment and effects of tracheal stenosis in children.Methods:A total of 23 children with tracheal stenosis in our hospital from December 2017 to August 2020 were retrospectively reviewed, including 14 males and 9 females. The mean age at operation was(8.9±5.8)months(range: 2-3 months) and the mean weight was(6.4±2.3)kg(range: 4.2-10.5 kg). The common types of tracheal stenosis were complete tracheal ring in 9 children, tracheomalacia in 10 and subglottic membranous annular hyperplasia in 4. The type of congenital heart diseases included 10 patients of pulmonary artery sling, 1 of tetralogy of Fallot, 5 of ventricular septal defect, 1 of pulmonary atresia, and 1 of right aortic arch with aberrant left subclavian artery. Slide tracheoplasty was performed in 9 patients, external splint in 8, endotracheal stent in 2 and tracheal dilation in 4. All children were followed up after 1, 3, 6, and 12 months of operation with CT and bronchoscopy.Results:There was 1 death in all 23 patients and the mortality was 4.3%, which died of granulation tissue hyperplasia after slide tracheoplasty. Reoperation was performed in 1 patient with endotracheal stent. All patients were followed for 1 to 24 months. Clinical symptoms of tracheal stenosis disappeared and the results of CT and bronchoscopy were satisfied.Conclusion:Slide tracheoplasty is the effective surgical method for complete trachea ring. 3D printing bioresorbable external splint is a promising method for the treatment of tracheomalacia.

BACKGROUND: Little is known about the effects of environmental cobalt exposure on insulin resistance (IR) in the general adult population. We investigated the association between cobalt concentration and IR. METHODS: A total of 1281 subjects aged more than 20 years with complete blood cobalt data were identified from the National Health and Nutrition Examination Survey (NHANES) 2015-2016 cycle. Blood cobalt levels were analyzed for their association with IR among all populations and subgroups by sex. Regression coefficients and 95% confidence intervals (CIs) of blood cobalt concentrations in association with fasting glucose, insulin and homeostatic model assessment of insulin resistance (HOMA-IR) were estimated using multivariate linear regression after adjusting for age, sex, ethnicity, alcohol consumption, body mass index, education level, and household income. A multivariate generalized linear regression analysis was further carried out to explore the association between cobalt exposure and IR. RESULTS: A negative association between blood cobalt concentration (coefficient = - 0.125, 95% CI - 0.234, - 0.015; P = 0.026) and HOMA-IR in female adults in the age- and sex-adjusted model was observed. However, no associations with HOMA-IR, fasting glucose, or insulin were found in the overall population. In the generalized linear models, participants with the lowest cobalt levels had a 2.74% (95% CI 0.04%, 5.50%) increase in HOMA-IR (P for trend = 0.031) compared with subjects with the highest cobalt levels. Restricted cubic spline regression suggested that a non-linear relationship may exist between blood cobalt and HOMA-IR. CONCLUSIONS These results provide epidemiological evidence that low levels of blood cobalt are negatively associated with HOMA-IR in female adults.
Adult ; Aged ; Aged, 80 and over ; Cobalt/blood* ; Cross-Sectional Studies ; Environmental Pollutants/blood* ; Female ; Homeostasis ; Humans ; Insulin/blood* ; Insulin Resistance ; Male ; Middle Aged ; Nutrition Surveys ; Sex Factors ; United States ; Young Adult

Objective:To analyze the early and middle term clinical effects of mitral valve repair in children with mitral insufficiency.Methods:From January 2012 to January 2019, a total of 202 cases of children with mitral insufficiency treated by mitral valve repair were selected from the department of cardiothoracic surgery of Nanjing Children's Hospital, patients with atrioventricular septal defect, single ventricle and ischemic mitral regurgitation were excluded. Echocardiography was used to compare the preoperative and postoperative left ventricular function and degree of regurgitation in children to evaluate the early and middle term efficacy of mitral valvuloplasty.Results:There were 5 cases of early death(5/202, 2.5%) and 3 cases of late death(3/202, 1.5%). The mean follow-up time was(19.49±17.48) months(1-68 months). Postoperative echocardiography showed that the left heart function and mitral regurgitation were significantly improved.Conclusion:Mitral valvuloplasty can significantly correct mitral insufficiency in children, and it has satisfactory mid-term efficacy and good clinical value.

To investigate the anatomical classification, diagnosis, indications, surgical methods, surgical techniques, intraoperative management, postoperative management and surgical efficacy of mitral valve malformation. Using the internationally used Delphi procedure, PubMed, Medline, The Cochrane Library, Wanfang and other databases were searched to review domestic and foreign literatures on congenital mitral valve deformity from January 1940 to February 2020, and select the ones of higher quality as evidence. In addition, with regard to the multiple disputes in the diagnosis and treatment of congenital mitral valve, experts from pediatric cardiac surgery and related disciplines have been convened for discussion for many times, and finally the following consensus has been formed to guide the clinical treatment and provide theoretical and technical guidance for the surgical treatment of congenital mitral valve malformation in China.

OBJECTIVETo compare the efficacy of thoracoscopic surgery versus thoracotomy in repairing esophageal atresia type Ⅲ with tracheoesophageal fistula (EA/TEF) in neonates.

METHODSA 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.

RESULTSThe 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, <0.05], but the postoperative ventilation time was shorter[(55±22) h vs. (65±19) h, <0.05] and the first oral feeding was earlier in thoracoscopic surgery group[(3.2±1.1) d vs (3.9±1.3) d, <0.05]. No statistical difference was observed in the ratio of red blood cell transfusion, length of hospital stay and drainage tube indwelling time between two groups (all >0.05). The incidence of lung complications in thoracotomy group was higher than that in thoracoscopic surgery group (20.0% vs. 9.1%, <0.01), while there were no significant differences in the incidence of other postoperative complications between two groups. There was no death in thoracoscopic surgery group, while 2 patients died in thoracotomy group.

CONCLUSIONSThoracoscopic repair is a preferred surgical procedure for EA/TEF in neonates.

Congenital esophageal atresia is one of the serious birth defects. Identifying the etiology and mechanism of esophageal development can provide clues for the effective prevention and treatment of congenital esophageal atresia. Recent studies have shown that a variety of transcription factors and signaling pathways (including Wnt signaling pathway, bone morphogenetic protein signaling pathway, SHH signaling pathway, vascular endothelial growth factor signaling pathway) are involved in esophageal proliferation, differentiation and other processes, promoting the normal development of esophagus. Understanding the regulatory mechanisms during the normal development of esophagus can give reference for the prevention and treatment of congenital esophageal atresia. This article reviews the research progress on related transcription factors and signaling pathways in esophageal development.