Objective To evaluate the impact of an integrated management mode of prenatal diagnosis-postnatal treatment for congenital heart disease (CHD) on perioperative and long-term outcomes of the arterial switch operation (ASO), and to analyze the efficacy of ASO in a single center. Methods This retrospective study analyzed the clinical data of 183 children who underwent ASO at Guangdong Provincial People’s Hospital from 2018 to 2024. The cohort included 106 (57.9%) patients of transposition of the great arteries with intact ventricular septum (TGA/IVS), 61 (33.3%) patients of transposition of the great arteries with ventricular septal defect (TGA/VSD), and 16 (8.7%) patients of Taussig-bing anomaly (TBA). Perioperative indicators were compared between 91 patients in the prenatal-postnatal integrated management group (an integrated group) and 92 patients in the traditional management group (a non-integrated group). Long-term survival and reoperation rates were analyzed using Kaplan-Meier curves. Results The overall perioperative mortality rate was 4.9% (9/183), showing a downward trend year by year. The primary cause of perioperative mortality was low cardiac output syndrome (LCOS), which occurred in 12 patients (6.6% incidence) with a mortality rate of 75.0%. The integrated group had a higher proportion of males (89.0% vs. 72.8%, P<0.05) and lower body weight [3.1 (2.7, 3.3) kg vs. 3.3 (3.0, 3.7) kg, P<0.05] compared to the non-integrated group. The age at surgery was significantly earlier in the integrated group [7 (3, 10) d vs. 14 (9, 48) d, P<0.05], and all children in the integrated group underwent ASO within the optimal surgical window (100.0% vs. 82.6%, P<0.05). Intraoperatively, cardiopulmonary bypass time [173 (150, 207) min vs. 186 (159, 237) min, P<0.05] and aortic cross-clamp time [100 (90, 117) min vs. 116 (97, 142) min, P<0.05] were significantly shorter in the integrated group. Although the integrated group had longer postoperative mechanical ventilation time [145 (98, 214) h vs. 116 (77, 147) h, P<0.05] and higher 48-hour maximum vasoactive inotropic score [15 (10, 21) points vs. 12 (8, 16) points, P<0.05], there was no statistically significant difference in the incidence of severe complications (LCOS, necrotizing enterocolitis, extracorporeal membrane oxygenation) or mortality rate (3.3% vs. 6.5%, P=0.51) between the two groups, despite earlier surgical intervention and a higher proportion of critically ill cases in the integrated group. The length of hospital stay in the emergency surgery group was significantly shorter than that in the elective surgery group [20 (15, 28) d vs. 25 (21, 30) d, P<0.05], suggesting that early surgery may be of potential benefit. A total of 163 patients were successfully followed up for a median of 4.7 years, with a 5-year survival rate of 95.1% and a freedom from reintervention survival rate of 95.1%. There were no late deaths, and the most common postoperative complication was pulmonary artery stenosis. Conclusion The integrated management model allowed critically ill children with lower body weights to safely undergo surgery, significantly optimizing the timing of surgery and shortening intraoperative times. The long-term risk of reoperation after ASO is primarily concentrated on pulmonary artery stenosis, necessitating long-term follow-up and monitoring.

Objective:To explore the clinical value of autologous umbilical cord whole blood(UCB) priming of the cardiopulmonary bypass(CPB) circuit in neonatal cardiac surgery for congenital heart disease(CHD).Methods:This prospective non-randomized controlled trial included neonates undergoing CHD surgery at Guangdong Provincial People’s Hospital from August 2024 to January 2025. The experimental group used autologous UCB for CPB circuit priming, while the control group used adult allogeneic blood(AAB) priming when UCB was unavailable. Preoperative characteristics, intraoperative CPB and aortic cross-clamping(ACC) times, postoperative ICU stay duration, mechanical ventilation time, and hospitalization length were compared.Results:There were no significant differences in preoperative baseline characteristics between the two groups( P>0.05). At the end of surgery, red blood cell count(RBC), hemoglobin level(Hb), and creatine kinase(CK) showed no significant differences between the groups( P> 0.05). Additionally, perioperative left ventricular ejection fraction(LVEF) demonstrated no statistically significant variations( P>0.05). At surgery completion, the UCB group exhibited lower hematocrit(HCT) and higher blood lactic acid(Lac) levels but these differences resolved by 6 hours postoperatively( P>0.05). The UCB group had higher maximum vasoactive-inotropic scores(VISmax) within 48 hours and longer ICU stays, though total hospitalization and mechanical ventilation durations showed no significant differences( P>0.05). Conclusion:Autologous UCB priming reduces AAB requirements and has minimal impact on postoperative cardiac and pulmonary function recovery, or homeostasis., which is safe and feasible. This study provides evidence supporting the clinical application of UCB priming in CPB circuits.

Objective:To explore the clinical value of autologous umbilical cord whole blood(UCB) priming of the cardiopulmonary bypass(CPB) circuit in neonatal cardiac surgery for congenital heart disease(CHD).Methods:This prospective non-randomized controlled trial included neonates undergoing CHD surgery at Guangdong Provincial People’s Hospital from August 2024 to January 2025. The experimental group used autologous UCB for CPB circuit priming, while the control group used adult allogeneic blood(AAB) priming when UCB was unavailable. Preoperative characteristics, intraoperative CPB and aortic cross-clamping(ACC) times, postoperative ICU stay duration, mechanical ventilation time, and hospitalization length were compared.Results:There were no significant differences in preoperative baseline characteristics between the two groups( P>0.05). At the end of surgery, red blood cell count(RBC), hemoglobin level(Hb), and creatine kinase(CK) showed no significant differences between the groups( P> 0.05). Additionally, perioperative left ventricular ejection fraction(LVEF) demonstrated no statistically significant variations( P>0.05). At surgery completion, the UCB group exhibited lower hematocrit(HCT) and higher blood lactic acid(Lac) levels but these differences resolved by 6 hours postoperatively( P>0.05). The UCB group had higher maximum vasoactive-inotropic scores(VISmax) within 48 hours and longer ICU stays, though total hospitalization and mechanical ventilation durations showed no significant differences( P>0.05). Conclusion:Autologous UCB priming reduces AAB requirements and has minimal impact on postoperative cardiac and pulmonary function recovery, or homeostasis., which is safe and feasible. This study provides evidence supporting the clinical application of UCB priming in CPB circuits.

Objective To introduce a modified technique of right ventricular outflow tract (RVOT) reconstruction using a handmade bicuspid pulmonary valve crafted from expanded polytetrafluoroethylene (ePTFE) and to summarize the early single-center experience. Methods Patients with complex congenital heart diseases (CHD) who underwent RVOT reconstruction with a handmade ePTFE bicuspid pulmonary valve due to pulmonary regurgitation at Guangdong Provincial People’s Hospital from April 2021 to February 2022 were selected. Postoperative artificial valve function and right heart function indicators were evaluated. Results A total of 17 patients were included, comprising 10 males and 7 females, with a mean age of (18.18±12.14) years and a mean body weight of (40.94±19.45) kg. Sixteen patients underwent reconstruction with a handmade valved conduit, with conduit sizes ranging from 18 to 24 mm. No patients required mechanical circulatory support, and no in-hospital deaths occurred. During a mean follow-up period of 12.89 months, only one patient developed valve dysfunction, and no related complications or adverse events were observed. The degree of pulmonary regurgitation was significantly improved post-RVOT reconstruction and during follow-up compared to preoperative levels (P<0.001). Postoperative right atrial diameter, right ventricular diameter, and tricuspid regurgitation area were all significantly reduced compared to preoperative values (P<0.05). Conclusion The use of a 0.1 mm ePTFE handmade bicuspid pulmonary valve for RVOT reconstruction in complex CHD is a feasible, effective, and safe technique.

Objective To analyze and summarize the early and medium-term outcomes of self-expanding interventional pulmonary valve stent (SalusTM) for right ventricular outflow tract dysfunction with severe pulmonary valve regurgitation. Methods We established strict enrollment and follow-up criteria. Patients who received interventional pulmonary valve in transthoracic implantation in Guangdong Provincial People’s Hospital from September 2, 2021 to July 18, 2023 were prospectively included, and all clinical data of patients were collected and analyzed. Results A total of 38 patients with severe pulmonary regurgitation were included, with 23 (60.5%) males and 15 (39.5%) females. The mean age was 24.08±8.12 years, and the mean weight was 57.66±13.54 kg. The preoperative mean right ventricular end-diastolic volume index (RVEDVI) and right ventricular end-systolic volume index (RVESVI) were 151.83±42.84 mL/m2 and 83.34±33.05 mL/m2, respectively. All patients successfully underwent transcatheter self-expandable pulmonary valve implantation, with 3 (7.9%) patients experiencing valve stent displacement during the procedure. Perioperative complications included 1 (2.6%) patient of postoperative inferior wall myocardial infarction and 1 (2.6%) patient of poor wound healing. The median follow-up time was 12.00 (6.00, 17.50) months. During the follow-up period, there were no deaths or reinterventions, and no patients had recurrent severe pulmonary regurgitation. Three (7.9%) patients experienced chest tightness and chest pain, and 1 (2.6%) patient developed frequent ventricular premature beats. Compared with preoperative values, the right atrial diameter, right ventricular diameter, and tricuspid annular plane systolic excursion were significantly reduced at 6 months and 1 year postoperatively, with improvement in the degree of pulmonary regurgitation (P<0.01). Compared with preoperative values, RVEDVI and RVESVI decreased to 109.51±17.13 mL/m2 and 55.88±15.66 mL/m2, respectively, at 1 year postoperatively (P<0.01). Conclusion 　Self-expanding interventional pulmonary valve in transthoracic implantation is safe and effective for severe pulmonary valve regurgitation and shows good clinical and hemodynamic results in one-year outcome.

Objective To comprehensively analyze the clinical outcomes of total cavopulmonary connection (TCPC) in the treatment of functional single ventricle combined with heterotaxy syndrome (HS). Methods A retrospective analysis was conducted on the patients with functional single ventricle and HS who underwent TCPC (a HS group) in Guangdong Provincial People's Hospital between 2004 and 2021. The analysis focused on postoperative complications, long-term survival rates, and identifying factors associated with patient survival. Early and late postoperative outcomes were compared with matched non-HS patients (a non-HS group). Results Before propensity score matching, 55 patients were collected in the HS group, including 42 males and 13 females, with a median age of 6.0 (4.2, 11.8) years and a median weight of 17.0 (14.2, 28.8) kg. Among the patients, there were 53 patients of right atrial isomerism and 2 patients of left atrial isomerism. Eight patients underwent TCPC in one stage. TCPC procedures included extracardiac conduit (n=39), intracardiac-extracardiac conduit (n=14), and direct cavopulmonary connection (n=2). Postoperative complications included infections in 27 patients, liver function damage in 19 patients, and acute kidney injury in 11 patients. There were 5 early deaths. The median follow-up time was 94.7 (64.3, 129.8) months. The 1-year, 5-year, and 10-year survival rates were 87.2%, 85.3%, and 74.3%, respectively. After propensity score matching, there were 45 patients in the HS group and 81 patients in the non-HS group. Compared to the non-HS group, those with HS had longer surgical and mechanical ventilation time, higher infection rates (P<0.05), and a 12.9% lower 10-year survival rate. Multivariate Cox regression analysis identified asplenia was a risk factor for mortality (HR=8.98, 95%CI 1.86-43.34, P=0.006). Conclusion Compared to non-HS patients, patients with HS have lower survival rates after TCPC, and asplenia is an independent risk factor for the survival of these patients.

Objective To develop an inpatient assessment form for geriatric syndromes,aiming to quickly and accurately determine whether elderly inpatients are at risk of geriatric syndromes.Methods From May 2021 to December 2023,an initial item pool for the scale was proposed through literature review.Sixteen experts from four fields of geriatric nursing,nursing management,nursing education,and geriatric medicine were selected from seven institutions in six provinces and cities across the country.Two rounds of Delphi expert consultation were conducted to inquire about the assessment form.Results The enthusiasm coefficient of the two rounds of expert consultation was 100.00%,and the expert authority coefficient was 0.88.In the first round of expert consultation,the importance ratings of the three-level indicators ranged from 4.06 to 4.75,with a coefficient of variation of 0.09 to 0.25,a full score rate of 43.75%to 81.25%,and a Kendall coordination coefficient W of 0.210.In the second round of expert consultation,the importance ratings of the three-level indicators ranged from 4.63 to 4.94,with a coefficient of variation of 0.05 to 0.13,a full score rate of 62.50%to 93.75%,and a Kendall coordination coefficient W of 0.419.After two rounds of expert consultation,the inpatient assessment form for geriatric syndromes was finally determined,including 5 first-level indicators(past history,physical function,common geriatric symptoms,mental and psychological status,socioeconomic status),26 second-level indicators,and 41 third-level indicators.Conclusion The inpatient assessment form for geriatric syndromes developed in this study is scientifically and practically feasible and can be used to quickly identify the risk of geriatric syndromes in elderly inpatients.

Objective:To investigate the clinicopathological features and the prognosis of IgA nephropathy (IgAN) in children with massive proteinuria.Methods:It was a retrospective cohort study. Clinical data of IgAN children with massive proteinuria admitted to the Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics from January 2008 to December 2021 were retrospectively analyzed. Patients were divided into effective group and ineffective group according to whether urine protein turned negative after 6 months of initial treatment. The follow-up endpoint event was defined as a reduction in proteinuria of less than 50% or end-stage renal disease (ESRD) achievement. MedCalc software was used to perform Kaplan-Meier survival analysis, and Log-rank test was used to compare the difference of renal survival between the two groups.Results:A total of 127 patients were diagnosed as primary IgAN by renal biopsy, of whom 57 patients with IgAN showed massive proteinuria. These 57 IgAN patients with macroproteinuria accounted for 44.9% of the total IgAN patients and were enrolled in the study. Among the 57 cases, 33 cases (57.9%) were Lee's grade Ⅲ, 11 cases (19.3%) were below Lee's grade Ⅲ, and 13 cases (22.8%) were above Lee's grade Ⅲ. The follow-up time was 4.0 (3.0,5.8) years. In the initial treatment, among 57 patients, 46 (80.7%) were effective (effective group) and 11 (19.3%) were ineffective (ineffective group). Compared with the effective group, the ineffective group had a higher proportion of concurrent AKI at the onset of disease and longer recovery time of renal function, with significant difference (7/11 vs. 13/46, χ2=4.878, P=0.027). Compared with the effective group, the proportion of Lee grade Ⅲ or above was higher in the ineffective group, and the difference was statistically significant (5/11 vs. 8/46, χ2=3.971, P=0.046). There were significant differences in endocapillary hypercellularity (E1), segmental glomerulosclerosis or adhesion (S1) and cellular/fibrocellular crescents (C2) of Oxford classification between IgAN children with Lee grade Ⅲ or below and those over Lee grade Ⅲ (11/13 vs. 20/44, χ2=6.204, P=0.013; 12/13 vs. 17/44, χ2=11.566, P=0.001; 9/13 vs. 7/44, χ2=14.131, P=0.001). Among 57 patients, endpoint events occurred in 2 patients who both were urinary protein unmitigated, and none of the children progressed to ESRD. There was no significant difference in cumulative renal survival between the two groups by Kaplan-Meier survival analysis and Log-rank test ( χ2=0.537, P=0.460) after addition of calcineurin inhibitors (CNIs) to the initial treatment ineffective group. Conclusions:Macroproteinuria is the prominent manifestation of IgAN in children. The pathological type is mainly Lee grade Ⅲ. Children with macroproteinuria have a good prognosis in the short and medium term after active treatment. For IgAN with macroproteinuria that does not respond well to initial treatment, AKI is more common at onset, and renal function recovery time is longer. The application of CNIs may have a certain effect on improving the renal outcome of IgAN with massive proteinuria.

Objective:This study aimed at reviewing surgical experiences and exploring risk factors for mortality and postoperative complications in patients with infracardiac total anomalous pulmonary venous connection (TAPVC).Methods:This retrospective study included 74 infants who underwent conventional repair (28 cases) and sutureless repair (46 cases) in one hospital from February 2009 to December 2022. Clinical data were reviewed to assess risk factors for mortality and postoperative pulmonary venous obstruction (PVO). Kaplan- Meier curves and cox regressions were applied to analyze the overall survival. Cumulative incidence curve and sub-distribution hazard models were used to evaluate postoperative PVO. Results:There were 4 early deaths and 3 late deaths, and the overall survival rate was 90.5%. A total of 12 patients complicated postoperative PVO. The median follow-up was 39.4 months ( IQR: 13.3 to 73.7 months). The overall survival rate was higher in the sutureless group than the conventional group ( P=0.003). The incidence rate of postoperative PVO in the conventional group was higher than that in the sutureless group ( P= 0.008). Risk factors for recurrent PVO included longer cardiopulmonary bypass time, the increase of direct bilirubin level and international normalized ratio level before surgical repair. Conclusion:Both sutureless and conventional repairs for patients with infracardiac TAPVC can achieve acceptable postoperative outcomes. Sutureless repair has a higher survival rate and a lower incidence of re-stenosis in pulmonary veins and anastomosis.

Proximity labeling catalyzed by promiscuous enzymes,such as APEX2,has emerged as a powerful approach to characterize multiprotein complexes and protein-protein interactions.How-ever,current methods depend on the expression of exogenous fusion proteins and cannot be applied to identify proteins surrounding post-translationally modified proteins.To address this limitation,we developed a new method to label proximal proteins of interest by antibody-mediated protein A-ascorbate peroxidase 2(pA-APEX2)labeling(AMAPEX).In this method,a modified protein is bound in situ by a specific antibody,which then tethers a pA-APEX2 fusion protein.Activation of APEX2 labels the nearby proteins with biotin;the biotinylated proteins are then purified using streptavidin beads and identified by mass spectrometry.We demonstrated the utility of this approach by profiling the proximal proteins of histone modifications including H3K27me3,H3K9me3,H3K4me3,H4K5ac,and H4K12ac,as well as verifying the co-localization of these iden-tified proteins with bait proteins by published ChIP-seq analysis and nucleosome immunoprecipi-tation.Overall,AMAPEX is an efficient method to identify proteins that are proximal to modified histones.