Objective To summarize and explore the individualized surgical treatment strategy and prognosis of anomalous aortic origin of coronary artery (AAOCA). Methods The clinical data of children with AAOCA admitted to Shanghai Children's Medical Center from March 2018 to August 2021 were retrospectively analyzed. Results A total of 17 children were enrolled, including 13 males and 4 females, with a median age of 88 (44, 138) months and a median weight of 25 (18, 29) kg. All patients received operations. The methods of coronary artery management included coronary artery decapitation in 9 patients, coronary artery transplantation in 5 patients and coronary artery perforation in 3 patients. One patient with severe cardiac insufficiency (left ventricular ejection fraction 15%) received mechanical circulatory assistance after the operation for 12 days. No death occurred in the early postoperative period, the average ICU stay time was 4.3±3.0 d, and the total hospital stay was 14.4±6.1 d. All the children received regular anticoagulation therapy for 3 months after discharge. The median follow-up time was 15 (13, 24) months. All patients received regular anticoagulation therapy for 3 months after discharge. No clinical symptoms such as chest pain and syncope occurred again. The cardiac function grade was significantly improved compared with that before operation. Imaging examination showed that the coronary artery blood flow on the operation side was unobstructed, and no restenosis occurred. Conclusion AAOCA is easy to induce myocardial ischemia and even sudden cardiac death. Once diagnosed, operation should be carried out as soon as possible. According to the anatomic characteristics of coronary artery, the early effect of individualized surgery is satisfactory, and the symptoms of the children are significantly improved and the cardiac function recovers well in the mid-term follow-up.

Objective    To compare and analyze the effect of myocardial protection between HTK and del Nido cardioplegia solutions in neonates with surgeries for transposition of the great arteries. Methods    The clinical data of 208 neonates with complete transposition of the great arteries in our institution from 2014 to 2020 were retrospectively analyzed. According to the cardioplegia solutions utilized in the operations, the patients were divided into two groups: a HTK group and a del Nido group. Propensity score matching was conducted to eliminate the biases. The cardiopulmonary bypass time, aortic cross-clamping time, total amount of cardioplegia solutions, transfusion frequency of cardioplegia, ICU stay time, mechanical support time, inotropic score, hospital stay, left ventricular ejection fraction, N-terminal proBNP and troponin I were compared and analyzed between the two groups after matching. Results    After 1:1 propensity score matching, a total of 54 patients were analyzed with 27 patients in each group. In the HTK group, there were 22 males and 5 females with a median age of 7.0 (2.0, 11.0) d. In the del Nido group, there were 23 males and 4 females with a median age of 8.0 (3.0, 11.0) d. A total of 3 children died after the surgery: 2 (7.4%) patients in the HTK group and 1 (3.7%) patient in the del Nido group. There was no significant difference in hospital mortality between the two groups (P=1.000). The total amount of cardioplegia solutions in the HTK group was significantly higher than that of del Nido group (P<0.001). Transfusion frequency of cardioplegia in del Nido group was significantly higher than that of the HTK group (P=0.043). There was no significant difference in the postoperative ICU time, mechanical support time, length of hospital stay, inotropic score, left ventricular ejection fraction, N-terminal B-type natriuretic peptide precursor or troponin I between the two groups (P>0.05). Conclusion    For neonates with surgeries for complete transposition of the great arteries, HTK cardioplegia solutions can provide effective and safe myocardial protection, which is similar to del Nido cardioplegia solutions.

Objective    To compare and investigate the efficacy and differences of modified B-T shunt, central shunt and right ventricle-pulmonary artery (RV-PA) connection in the treatment of pulmonary atresia with ventricular septal defect (PA/VSD). Methods    A total of 124 children with PA/VSD underwent initial palliative repair in Shanghai Children's Medical Center from September 2014 to August 2019, including 63 males and 61 females, aged 7 days to 15 years. They were divided into in a modified B-T shunt group (55 patients), a central shunt group (22 patients) and a RV-PA connection group (47 patients). The clinical data of these children were retrospectively analyzed. Results    There were 9 early deaths after palliation, with an early mortality rate of 7.3%. The mean follow-up time was 26.5±20.3 months, with 5 patients lost to follow-up, 5 deaths during the follow-up period, and 105 survivors. The 1-year and 5-year survival rates were both 89.7%. The monthly increased Nakata index was 5.2 (–0.2, 12.3) mm2/m2, 9.2 (0.1, 23.6) mm2/m2, 6.3 (1.8, 23.3) mm2/m2 in the modified B-T shunt group, the central shunt group, and the RV-PA connection group, respectively, with no statistical difference among the three groups. The 1-year survival rate was 85.3%, 78.4%, 95.2%, and the 5-year (4-year in the central shunt group) survival rate was 85.3%, 58.8%, 95.2% in the three groups, respectively, with a statistical difference among them (P<0.05). The complete repair rate was 36.5%, 19.0% and 67.4% in the three groups, respectively, with a statistical difference among the three groups (P<0.001). Conclusion    All these three palliative surgical approaches can effectively promote pulmonary vascular development. But compared with systemic-pulmonary shunt, RV-PA connection has a lower perioperative mortality rate and can achieve a higher complete repair rate at a later stage, which is beneficial for long-term prognosis.

A 2-year-old boy was admitted to Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine in Nov 30^th, 2018, due to polydipsia, polyphagia, polyuria accompanied with increased glucose levels for more than 2 weeks. He presented with symmetrical short stature [height 81 cm (－2.2 SD), weight 9.8 kg (－2.1 SD), body mass index 14.94 kg/m² (P₁₀-P₁₅)], and with no special facial or physical features. Laboratory results showed that the glycated hemoglobin A1c was 14%, the fasting C-peptide was 0.3 ng/mL, and the islet autoantibodies were all negative. Oral glucose tolerance test showed significant increases in both fasting and postprandial glucose, but partial islet functions remained (post-load C-peptide increased 1.43 times compared to baseline). A heterozygous variant c.1366C>T (p.R456C) was detected in GATA6 gene, thereby the boy was diagnosed with a specific type of diabetes mellitus. The boy had congenital heart disease and suffered from transient hyperosmolar hyperglycemia after a patent ductus arteriosus surgery at 11 months of age. Insulin replacement therapy was prescribed, but without regular follow-up thereafter. The latest follow-up was about 3.5 years after the diagnosis of diabetes when the child was 5 years and 11 months old, with the fasting blood glucose of 6.0-10.0 mmol/L, and the 2 h postprandial glucose of 17.0-20.0 mmol/L.
Male ; Child ; Humans ; Child, Preschool ; Infant ; Diabetes Mellitus, Type 2/complications* ; Mutation, Missense ; C-Peptide/genetics* ; China ; Insulin/genetics* ; Glucose ; Blood Glucose ; GATA6 Transcription Factor/genetics*

Objective:To study the surgical results of Yasui procedure for interruption of aortic arch and ventricular septal defect(IAA/VSD) associated with aortic stenosis(AS) or aortic atresia(AA).Methods:A retrospective study was performed on all 4 children with IAA/VSD/AS(AA) that underwent a Yasui procedure from Dec 2014 to Dec 2019. Three patients had AS, and 1 patient had AA. The age was from 10 days to 25 months and the weight was from 2.7 kg to 10 kg. The type of IAA was type A in 1 and type B in 3. The diameter and Z value of the aortic annulus were 1.9-4.3 mm and -6.7--3.6. The diameter and Z value of the ascending aorta were 2.6-5.8 mm and -5.4--2.6. The mean Z value of LVEDD and LVEDS was 3.6±2.6 and 3.6±2.9 resparately.Results:All 4 patients survived after the Yasui procedure. Three patients underwent primary repair and 1 patient underwent staged repair. CPB time was 128-283 min and aortic cross-clamping time was 98-171 min. Ventilation time was 93-296 h, and ICU and hospital length of stay was 7-25 days and 18-39 days. Follow-up was complete in all patients from 8 to 92 months, and there was no late death. All the patients were in good biventricular function with NYHA grade Ⅰ in 3 and NYHA grade Ⅱ in 2 patients. No residual left ventricular outflow tract obstruction was detected.Conclusion:The Yasui procedure can be an option for patients with IAA/VSD/AS(AA) and good developed left ventricle and can achieve good early surgical results.

In cardiac surgery such as myocardial infarction, congenital heart disease, and heart transplantation, myocardial ischemia-reperfusion (I/R) damages mitochondria, which in turn causes myocardium energy metabolism disturbance, homeostatic destruction, reactive oxygen species production and DNA damage, and activates myocardium apoptosis and necrosis signals, which eventually lead to myocardial dysfunction or heart failure. Different from the previous I/R treatment targets focusing on a certain pathway or a certain medium, autologous mitochondrial transplantation (AMT) is an emerging method, which transplant viable and respiration-competent mitochondria from healthy organs or tissues to the injured sites which suffer from I/R injury, to replace or repair damaged mitochondria. In recent years, AMT has achieved remarkable results in the prevention and treatment of myocardial I/R. This article reviews the role and mechanism of AMT in I/R myocardial protection.

Objective:To explore and summarize the surgical strategies of biventricular conversion from single-ventricle palliation for the complex congenital heart defects in this study.Methods:From April 2017 to December 2019, a total of 14 cases with complex complex congenital heart defects who had planned to undergo single-ventricle palliation were finally accepted biventricular conversion. According to the development and anatomical characteristics of the ventricles, they were divided into two groups: ventricular well development group(9 cases), all of which showed complex intracardiac defects, and the ventricles well developed, including 4 cases of double outlet right ventricle with remote ventricular septal defect, 2 cases of transposition of great arteries combined with ventricular septal defect and left ventricular outflow tract obstruction, one case of congenitally corrected transposition of the great arteries combined with double outlet right ventricle and interrupted aortic arch, one case of double outlet right ventricle with subpulmonary ventricular septal defect and atrioventricular connection inconsistency, one case of pulmonary atresia with ventricular septal defect. Ventricular unbalanced development group(5 cases), all of which were functional single ventricle, including 3 cases of heterotaxy and 2 cases of unbalanced complete atrioventricular septal defect. The operation time, postoperative complications, death, cardiac function and reoperation of the two groups were compared.Results:There was one early death, with a mortality rate of 7.1%. In the ventricular well development group, the average waiting time for biventricular conversion was(56.4 ± 38.9) months. One case died after the operation. The early complications included 2 case of arrhythmias, 3 cases of pleural effusions and 2 cases of pulmonary infections. In the ventricular unbalanced development group, the average waiting time for biventricular conversion was(20.4 ± 15.8) months, and the average of left ventricular end-diastolic volume was(33.6 ± 2.1) ml/m 2. The early complications included 2 cases of low cardiac outputs, one case of arrhythmia and one case of renal failure. There was no significant difference in operation time, intubation time and ICU time between the two groups. The patients were followed up for 4 months to 3 years, and there was no death. In the ventricular well development group, one case was reoperated due to the obstruction of the anastomosis between the superior vena cava and the right atrial appendage. In the ventricular unbalanced development group, one case was implanted with a permanent pacemaker due to the degree Ⅲ atrioventricular block. The NYHA grade of the whole group was Ⅰ-Ⅱ. Conclusion:Whether the complex congenital heart defects with the ventricles well developed or unbalanced, the comprehensive evaluation before the planned single-ventricle palliation will be helpful to find the appropriate cases for biventricular conversion. According to its anatomical characteristics performed the corresponding biventricular conversion can obtain a satisfied clinical outcomes, although the long-term results still need further follow-up.

Congenital heart disease is the most common congenital defect.With the advancement of surgical techniques, the survival rate of children with congenital heart disease is gradually increasing.However, the neurodevelopmental defects in children with congenital heart disease have gradually increased, which elicits increasing scholars′ attention.Attention deficit hyperactivity disorder(ADHD)is one of the most prominent neurodevelopmental behavior disorders in children.In previous studies, the prevelance of ADHD in cyanotic congenital heart disease was obviously higher than that of the general population, and however, research of congenital heart disease was in its infancy.This paper intends to review the incidence, etiology, pathogenesis, treatment and prognosis of ADHD in congenital heart disease in order to provide references for clinical work.

To evaluate the surgical strategy and follow-up for pulmonary atresia with intact ventricular septum (PA/IVS) in our heart center. Methods    From January 2008 to December 2018, 151 patients with PA/IVS were divided into two groups: a one-stage surgery group (26 patients), including 17 males and 9 females at an average age of 14.7±13.2 months, and a staged surgery group (125 patients) including 72 males and 53 females at an average age of 6.4±6.3 months. The clinical effectiveness of the two groups were analyzed. Results    All patients were followed up for 1-11 years. Eighteen patients died and 19 patients were lost to follow-up. The 1-year, 5-year and 10-year survival rate was 90.2%, 87.0%, and 85.2%, respectively. Two patients died in the one-stage surgery group. Twelve patients died after initial surgery, and 4 patients died after final operation in the staged surgery group. The Z value of tricuspid valve (P=0.013) and severe right ventricular dysplasia (P=0.025) were the risk factors of postoperative death in the patients with PA/IVS. Furthermore, 58 patients completed final operation, and the total number of the final operation (including one-stage radical surgery) accounted for 55.6% (84/151). Five patients accepted the re-operation intervention in the medium-term follow-up. The rest of the patients recovered well. Only 2 patients were classified as grade Ⅲ in cardiac function, and the rest patients were classified as gradeⅠ-Ⅱ. Conclusion    According to the degree of right ventricular hypoplasia, the age at operation and the presence or absence of coronary artery malformation, the individualized surgical strategy could significantly improve the success rate of PA/IVS, and early completion of right ventricular decompression operation is conducive to improve the chance for biventricular repair.

Objective    To compare the outcomes of sutureless technique and conventional technique in the surgical repair for infracardiac total anomalous pulmonary venous connection (TAPVC). Methods    The clinical data of 46 consecutive patients with infracardiac TAPVC undergoing surgical repair in our hospital between June 2014 and April 2019 were retrospectively analyzed. Patients with combined congenital cardiac anomalies such as single ventricle and tetralogy of Fallot were excluded. Patients were divided into a conventional technique group and a sutureless technique group according to the surgical techniques. There were 35 patients in the conventional technique group, including 28 males (80.0%) and 7 females (20.0%) with a median age of 21 (8, 42) d and a median weight of 3.6 (3.0, 4.0) kg. There were 11 patients in the sutureless technique group, including 8 males (72.7%) and 3 females (27.3%) with a median age of 14 (6, 22) d and a median weight of 3.5 (2.9, 3.6) kg. The curative effect of the two groups was compared. Results    There were 5 deaths (10.9%) in the conventional technique group, including 4 in-hospital deaths (8.7%) and 1 late death (2.2%). Overall mortality of the conventional technique group (14.3%, 5/35) was higher than that of the sutureless technique group (0.0%, 0/11), although the difference was not statistically significant (P=0.317). Cox regression analysis showed that sex (P=0.042), age at repair (P=0.028), cardiopulmonary bypass time (P=0.007), aortic cross-clamping time (P=0.018) and duration of ventilation (P=0.042) were risk factors for postoperative mortality. The median follow-up was 18.00 (5.00, 37.75) months. Postoperative pulmonary venous obstruction occurred in 22 patients of the conventional technique group, which was significantly more than that of the sutureless technique group (P=0.000). Conclusion    For infracardiac TAPVC, sutureless technique can reduce the incidence of postoperative pulmonary venous obstruction compared with conventional technique.