The complement component 4 binding protein alpha(C4BPA)gene is the alpha chain of complement binding protein 4.As a plasma protein involved in the complement and coagulation systems,it can influence immune responses and lipid metabolism.In order to study the polymor-phism of C4BPA gene and its correlation with milk quality traits in Chinese Holstein cows,genom-ic DNA was extracted from blood samples of 92 Chinese Holstein cows,and the target fragment of C4BPA gene was amplified by PCR,and the association analysis was performed by using direct se-quencing to obtain the SNP loci and milk quality traits.The results showed that among the four SNPs found at the third intron of the C4BPA gene,I3-11 G＞A was highly significantly correlated with milk protein and urea nitrogen(P＜0.05),I3-291 T＞G was significantly correlated with lac-tose(P＜0.05),I3-374 C＞T was highly significantly correlated with lactose and urea nitrogen(P＜0.05),and I3-375 T＞G was highly significantly correlated with lactose(P＜0.05),milk pro-tein and urea nitrogen.The chi-square test values for each point indicated that the population was in genetic equilibrium.Individuals of haplotype combination H1 H1 had the highest lactose content,and haplotype combination H1H2 can be used as the best haplotype combination in the molecular selection work of dairy cows.

Objective:To analyze and explore the selection of secondary intervention strategies for residual issues following congenital aortic valve disease surgery in children, as well as to evaluate their prognostic efficacy.Methods:A retrospective analysis was conducted on the clinical data of 41 children with residual issues after congenital aortic valve disease surgery, who were treated at the Department of Cardiovascular and Thoracic Surgery, Shanghai Children's Medical Center, between January 2013 and August 2024. Among them, 25 were male and 16 were female, with a mean age of(116.8±45.1)months and a mean weight of(40.0±20.2)kg. The mean interval for secondary intervention was(60.0±36.4)months. The residual issues included aortic valve insufficiency(20 cases), aortic valve stenosis(11 cases), and combined aortic valve insufficiency and stenosis(10 cases).Results:Secondary interventions included aortic valve repair(7 cases), mechanical valve replacement(20 cases), Ross procedure(13 cases), and Ozaki procedure(1 case). Additionally, 5 cases with left ventricular outflow tract stenosis underwent concomitant Konno surgery.One case of early postoperative in-hospital death occurred, with a mortality rate of 2.4%, primarily due to acute left heart failure and multiple organ dysfunction. The average follow-up period for surviving children was( 64.6±34.5) months(5-147 months), with no mid- to long-term mortality. Follow-up results showed that In surviving children, the left heart showed significant reduction in size postoperatively, with the LVEDD Z-score decreasing from 1.24±2.23 before surgery to -0.97±1.48( P=0.001). Postoperative LVEF 0.66±0.06 showed no significant difference compared to pre-reintervention 0.68±0.10( P=0.140). In aortic stenosis(AS) patients, the peak transvalvular pressure gradient decreased from(69.5±19.8) mmHg(1 mmHg=0.133 kPa) preoperatively to(25.1±10.9) mmHg postoperatively( P=0.003). In aortic insufficiency(AI) patients, the width of the aortic regurgitation jet decreased from(5.8±1.1) mm preoperatively to(2.7±1.1) mm postoperatively( P=0.012). 97.5% of children maintained mild-to-moderate aortic regurgitation(jet width <4 mm), with only 1 case of moderate regurgitation(jet width 4.4 mm)remaining under close observation.Two children underwent reoperation on the aortic valve, with a tertiary intervention rate of 5.0%. One case underwent redo commissurotomy 21 months after aortic valve repair due to recurrent stenosis, and the other underwent mechanical valve replacement 34 months after the Ozaki procedure due to leaflet calcification and infective endocarditis. Mid-term follow-up revealed good cardiac function recovery in surviving children, with 87.5% achieving NYHA functional class Ⅰ/Ⅱ. Conclusion:Due to the demands of growth and development and the degenerative nature of valve tissue, residual issues are inevitable in the mid- to long-term following congenital aortic valve disease surgery in children, often necessitating secondary intervention. Given the lack of suitable autologous valve tissue, the use of aortic valve replacement has significantly increased in secondary interventions. The Ross procedure, as an ideal secondary intervention, demonstrates satisfactory mid-term follow-up outcomes. For children with high-risk factors for the Ross procedure, mechanical valve replacement also yields favorable prognoses.

The complement component 4 binding protein alpha(C4BPA)gene is the alpha chain of complement binding protein 4.As a plasma protein involved in the complement and coagulation systems,it can influence immune responses and lipid metabolism.In order to study the polymor-phism of C4BPA gene and its correlation with milk quality traits in Chinese Holstein cows,genom-ic DNA was extracted from blood samples of 92 Chinese Holstein cows,and the target fragment of C4BPA gene was amplified by PCR,and the association analysis was performed by using direct se-quencing to obtain the SNP loci and milk quality traits.The results showed that among the four SNPs found at the third intron of the C4BPA gene,I3-11 G＞A was highly significantly correlated with milk protein and urea nitrogen(P＜0.05),I3-291 T＞G was significantly correlated with lac-tose(P＜0.05),I3-374 C＞T was highly significantly correlated with lactose and urea nitrogen(P＜0.05),and I3-375 T＞G was highly significantly correlated with lactose(P＜0.05),milk pro-tein and urea nitrogen.The chi-square test values for each point indicated that the population was in genetic equilibrium.Individuals of haplotype combination H1 H1 had the highest lactose content,and haplotype combination H1H2 can be used as the best haplotype combination in the molecular selection work of dairy cows.

Objective:To analyze and explore the selection of secondary intervention strategies for residual issues following congenital aortic valve disease surgery in children, as well as to evaluate their prognostic efficacy.Methods:A retrospective analysis was conducted on the clinical data of 41 children with residual issues after congenital aortic valve disease surgery, who were treated at the Department of Cardiovascular and Thoracic Surgery, Shanghai Children's Medical Center, between January 2013 and August 2024. Among them, 25 were male and 16 were female, with a mean age of(116.8±45.1)months and a mean weight of(40.0±20.2)kg. The mean interval for secondary intervention was(60.0±36.4)months. The residual issues included aortic valve insufficiency(20 cases), aortic valve stenosis(11 cases), and combined aortic valve insufficiency and stenosis(10 cases).Results:Secondary interventions included aortic valve repair(7 cases), mechanical valve replacement(20 cases), Ross procedure(13 cases), and Ozaki procedure(1 case). Additionally, 5 cases with left ventricular outflow tract stenosis underwent concomitant Konno surgery.One case of early postoperative in-hospital death occurred, with a mortality rate of 2.4%, primarily due to acute left heart failure and multiple organ dysfunction. The average follow-up period for surviving children was( 64.6±34.5) months(5-147 months), with no mid- to long-term mortality. Follow-up results showed that In surviving children, the left heart showed significant reduction in size postoperatively, with the LVEDD Z-score decreasing from 1.24±2.23 before surgery to -0.97±1.48( P=0.001). Postoperative LVEF 0.66±0.06 showed no significant difference compared to pre-reintervention 0.68±0.10( P=0.140). In aortic stenosis(AS) patients, the peak transvalvular pressure gradient decreased from(69.5±19.8) mmHg(1 mmHg=0.133 kPa) preoperatively to(25.1±10.9) mmHg postoperatively( P=0.003). In aortic insufficiency(AI) patients, the width of the aortic regurgitation jet decreased from(5.8±1.1) mm preoperatively to(2.7±1.1) mm postoperatively( P=0.012). 97.5% of children maintained mild-to-moderate aortic regurgitation(jet width <4 mm), with only 1 case of moderate regurgitation(jet width 4.4 mm)remaining under close observation.Two children underwent reoperation on the aortic valve, with a tertiary intervention rate of 5.0%. One case underwent redo commissurotomy 21 months after aortic valve repair due to recurrent stenosis, and the other underwent mechanical valve replacement 34 months after the Ozaki procedure due to leaflet calcification and infective endocarditis. Mid-term follow-up revealed good cardiac function recovery in surviving children, with 87.5% achieving NYHA functional class Ⅰ/Ⅱ. Conclusion:Due to the demands of growth and development and the degenerative nature of valve tissue, residual issues are inevitable in the mid- to long-term following congenital aortic valve disease surgery in children, often necessitating secondary intervention. Given the lack of suitable autologous valve tissue, the use of aortic valve replacement has significantly increased in secondary interventions. The Ross procedure, as an ideal secondary intervention, demonstrates satisfactory mid-term follow-up outcomes. For children with high-risk factors for the Ross procedure, mechanical valve replacement also yields favorable prognoses.

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.

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.

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.

Objective:To summarized the experience of 149 cases of critical pediatric cardiac surgery in a single-center during the epidemic period, for providing a safe and feasible management strategy.Methods:Based on the epidemiological characteristics of COVID-19, a strategy consisting of 14 days of isolation was established for the arrangement of cardiac surgery in children during the epidemic period. Retrospective analysis of clinical data of 149 cases of critical cardiac surgery performed from January 23, 2020 to March 20, 2020 under the guidance of this strategy. The primary composite endpoint was death and suspected or confirmed COVID-19.Results:The median age of the children undergoing surgery was 136 days; 73(49.0%) cases were male. Twenty-one cases (14.1%) came from Shanghai, one case (0.7%) came from Hubei Province, and 127 cases (85.2%) came from areas other than Hubei and Shanghai. One patient (0.7%) outside Shanghai who had been isolated for less than 14 days carried emergency surgery under special protection; other 148 patients (99.3%) underwent elective early repair procedure. One patient (0.7%) died, and no COVID-19 was confirmed or suspected.Conclusion:During the COVID-19 epidemic, pediatric cardiac surgery can be safely performed using a specific management strategy, which can be used as a reference when major public health events occur.

Objective    To evaluate the effect of off-pump pulmonary valvulotomy for the patients with pulmonary atresia with intact ventricular septum (PA/IVS). Methods    The clinical data of 61 PA/IVS patients who underwent off-pump right ventricular decompression surgery in our hospital from January 2013 to September 2019 were retrospectively analyzed, including 37 males and 24 females, with an average age of 29.7 (2.0-86.0) d and weight of 4.1 (2.5-6.9) kg. Thirty-nine patients received off-pump pulmonary valvulotomy (an open-view valvulotomy group) and 22 patients received balloon valvuloplasty through the right ventricle pulmonary valve (a hybrid therapy group). The postoperative mortality, early re-intervention, and completion of final operation of the two groups were compared. Results    There were 2 deaths in the study with a mortality rate of 3.3% (2/61), and the mortality rate of the two groups was not significantly different (2.6% vs.4.5%, P=0.68). The rate of early re-intervention in the two groups was 5.3% and 19.0%, respectively (P=0.09). There was no statistical difference in intubation time (56.0±25.9 h vs. 62.0±28.9 h, P=0.41), ICU retention time (4.7±2.9 d vs. 5.5±2.2 d, P=0.23) and postoperative hospital stay time (3.9±0.9 d vs. 4.3±1.1 d, P=0.38) between the two groups. The follow-up time was 45.3 (4.0-84.0) months. There were 5 patients lost to follow-up. During the follow-up period, in the open-view valvulotomy group, 17 patients did not need further operation, 13 patients completed the final operation. In the hybrid therapy group, 7 patients did not need further operation, 8 patients completed the final operation. Heart function classification of all patients was in New York Heart Association class Ⅰ-Ⅱ. Conclusion    Compared with the hybrid therapy, off-pump pulmonary valvulotomy for PA/IVS also has the advantages of simple operation, short operation time and high survival rate, and it may be easier to be promoted in clinical application because of its more economic benefits and relatively lower re-intervention rate.