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.

Near-infrared spectroscopy (NIRS) can be employed to monitor regional oxygen saturation (rSO 2), to reflect the oxygen metabolism and local effective blood perfusion of the measured site in a real-time and noninvasive manner.NIRS has been extensively used in monitoring blood perfusion of brain, kidney, gastrointestinal tract and organ function evaluation in different conditions.During the perioperative period of congenital heart disease, hemodynamic fluctuation may easily cause organ hypoperfusion, which would lead to the development of ischemia, hypoxia and internal environment disorder, thus resulting in harmful changes in tissue oxygenation status.However, there is no change in the arterial oxygen saturation (SaO 2) and mixed venous saturation (SvO 2). NIRS can be employed to sensitively monitor the changes of tissue rSO 2, so as to prevent further injury caused by tissue ischemia and hypoxia.This paper briefly describes the basic principle, limitations and new progress of NIRS in the perioperative application of CHD, in order to provide new ideas and directions for its clinical application in the perioperative period of CHD, early evaluate the patient′s condition and guide clinical work.

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.

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.

Objective To investigate the influencing factors for energy balance and the relationship be-tween energy balance and clinical outcome in PICU mechanical ventilation children. Methods Children with mechanical ventilation who were hospitalized in PICU for more than 3 days from June 2015 to May 2016 were collected, resting energy expenditure was measured by resting energy metabolic detector, and the nutrition bal-ance was calculated, the influencing factors on energy balance was analyzed by regression analysis. Results A total of 104 mechanical ventilation children were included with the average energy consumption of ( 265. 4 ± 63. 2) kJ/kg in the first 3 days and the average energy supply of (219. 8±82. 9) kJ/kg, and failed to reach the target value ( P<0. 05) . As the time of hospitalization was prolonged, the supply of energy increased gradu-ally, the SAPS Ⅱ ( r=-0. 609, P=0. 000) , mechanical ventilation time ( r=-0. 456, P=0. 000) , ICU stay time ( r=-0. 646, P=0. 000) , the number of organ failure ( r=-0. 568, P=0. 000) , infection complications ( r=-0. 859, P=0. 000) were negatively correlated to energy supply balance; regression analysis showed that complications of nosocomial infection ( P = 0. 000 ) , number of organ failure ( P = 0. 000 ) , mechanical ventilation time ( P=0. 000) , ICU retention time ( P=0. 001) were predictors of energy supply balance. Con-clusion Insufficient supply of energy for the first three days of mechanical ventilation is high in the critically ill children. Factors affecting energy supply balance include patient's organ failure, infection complications, me-chanical ventilation time, and ICU retention time, which suggest that the strengthening of the nutritional man-agement of the critically ill children with mechanical ventilation will benefit to the clinical outcome.

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.

Objective To explore the feasibility of percutaneous puncture for ventricular septal defect(VSD).Methods From March 2015,we did percutaneous punctured transcatheter device closure of VSD for 22 patients.The patients included 15 males and 7 females with age from 2 years 6 months to 11 years 7 months, weight from 13.5 kg to 44.0 kg.Among 22 pa-tients,6 were diagnosed with residual shunt after VSD repair, with size of residual shunt from 4.8 mm to 7.0 mm.Residual shunts are perimembranous, conoventricular and intracristal.The other 16 patients were diagnosed with isolated VSD,with de-fect size from 3.5 to 5.1 mm.For all patients,we punctured directly through the right ventricular surface into the right ventri-cle in the 3th or 4th intercostal space of the left sternal border.Next, we succeeded to extract the guide wire after inserting a conveyor tube for 21 patients.Subsequently,7-10 mm VSD closure device were delivered via the conveyor tube.Only one patient was changed to perform occlusion of VSD through chest small incision due to improper selection of punctured position. Results A total of 21 closure devices were placed for 21 patients.18 of them are equilateral device,and 3 are decentered de-vice,with size from 5 mm to 10 mm.There were small amount of pericardial effusion in 4 patients using TEE examination,and no future treat was performed after observation.For one-year follow-up, all patients have recovered very well.Conclusion Percutaneous punctured transcatheter device closure fits for treatment for children with residual shunt after VSD repair and isola-ted VSD.It has a good recent result.

Objective To explore the feasibility of hybrid procedure of percutaneous puncture for residual shunt after ventricular septal defect(VSD) repair.Methods From March 2015,we did percutaneous punctured transcatheter device closure of residual shunt after VSD repair for four patients.The patients included 2 males and 2 females with age from 4 years 9 months to 11 years 7 months and weight from 18 kg to44 kg.Four patients are diagnosed for4 years after VSD repair,3 years after tetralogy of Fallot (TOF),3 years after coarctation of aorta(CoA) combined with VSD,and 1 year after VSD repair,respectively.The withsize of residual shunt from 4.8 mm to 6.8 mm.Residual shunt are perimembranous,conoventricular and intracristal.Punctured directly through the right ventricular surface into the right ventricle in the 4-th or 5th intercostal space of the left sternal border.Next,extracted the guide wire after inserting a conveyor tube.Subsequently,7-10 mm VSD closure device were delivered via the conveyor tube.Results All the four operations were succeeded.A total of four closure devices were placed.Two of them are equilateral devices,and two are decentered devices,with size from 7mm to 9mm.Echocardiographyexamination revealed no significant pericardial effusion.Post-operative ultrasonic cardiogram showed the VSD closure device on site,with no pericardia1 effusion and no obvious morphological abnormalities of the valve.For one-year follow-up,the cardiac functionsof all patients were well.Conclusion Percutaneous punctured transcatheter device closure is a new,efficient and reliable method of treatment for children withresidual shunt after VSD repair.This method also can extend and offer a new avenue for valvular prosthesis and Perimembranous VSD.