Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. As a critical component of surgical repair, reconstruction of the right ventricular outflow tract (RVOT) serves as a primary determinant of postoperative hemodynamics and long-term prognosis. With advancements in surgical techniques, early postoperative survival rates for TOF have significantly improved, shifting clinical focus toward addressing long-term complications such as right ventricular dysfunction and reduced exercise tolerance. However, challenges persist regarding optimal timing of surgery in relation to adverse long-term outcomes, balancing RVOT reconstruction strategies, and selecting appropriate repair materials for RVOT reconstruction, with a lack of unified high-level evidence-based guidelines. To address these issues and standardize the application of radical corrective surgery for TOF to better serve patients, the National Society for Congenital Heart Diseases has systematically reviewed existing high-level evidence. Consequently, we have registered and formulated the "Protocol for Chinese expert consensus on right ventricular outflow tract reconstruction in tetralogy of Fallot (2026 edition)", which details the methodology and development process of this upcoming consensus.

Objective To investigate the efficacy of leaflet augmentation technique to repair the recurrent mitral valve (MV) regurgitation after mitral repair in children. Methods A retrospective analysis was conducted on the clinical data of children who underwent redo MV repair for recurrent regurgitation after initial MV repair, using a leaflet augmentation technique combined with a standardized repair strategy at Fuwai Hospital, Chinese Academy of Medical Sciences, from 2018 to 2022. The pathological features of the MV, key intraoperative procedures, and short- to mid-term follow-up outcomes were analyzed. Results A total of 24 patients (12 male, 12 female) were included, with a median age of 37.6 (range, 16.5–120.0) months. The mean interval from the initial surgery was (24.9±17.0) months. All children had severe mitral regurgitation preoperatively. The cardiopulmonary bypass time was (150.1±49.5) min, and the aortic cross-clamp time was (94.0±24.2) min. There were no early postoperative deaths. During a mean follow-up of (20.3±9.1) months, 3 (12.5%) patients developed moderate or severe mitral regurgitation (2 severe, 1 moderate). One (4.2%) patient died during follow-up, and one (4.2%) patient underwent a second MV reoperation. The left ventricular end-diastolic diameter was significantly reduced postoperatively compared to preoperatively [ (43.5±8.6) mm vs. (35.8±7.8)mm, P<0.001]. Conclusion The leaflet augmentation technique combined with a standardized repair strategy can achieve satisfactory short- to mid-term outcomes for the redo mitral repair after previous MV repair. It can be considered a safe and feasible technical option for cases with complex valvular lesions and severe pathological changes.

Two pediatric heart failure patients were treated with pulmonary artery banding(PAB)at Fuwai Hospital,from December 2021 to January 2022.In the first case,an 8-month-old patient presented with left ventricular non-compaction cardiomyopathy(LVNC),left ventricular systolic dysfunction,ventricular septal defect,and atrial septal defect.The second case was a 4-month-old patient with LVNC,left ventricular systolic dysfunction,and coarctation of the aorta.After PAB,the left ventricular function and shape of both patients were significantly improved,without serious surgery-related complications.In these individual cases of pediatric heart failure,pulmonary artery banding exhibited a more satisfactory efficacy and safety compared to pharmacological treatment,especially for those with unsatisfactory medication results.Future clinical data are needed to promote the rational and broader application of this therapeutic option for indicated patients.

Objective:To evaluate the role of nuclear factor kappa B (NF-κB)/NOD-like receptor thermoprotein structural domain-associated protein 3 (NLRP3) signaling pathway in hippocampal microglia in dexmedetomidine-induced improvement in long-term cognitive function after multiple sevoflurane anesthesia in newborn mice.Methods:One hundred SPF healthy male C57BL/6 mice, aged 6 days, weighing 3-5 g, were divided into 5 groups ( n=20 each) by a random number table method: control group (group C), multiple sevoflurane anesthesia group (group S), multiple sevoflurane anesthesia+ NF-κB inhibitor pyrrolidine dithiocarboxylate group (group SP), multiple sevoflurane anesthesia + dexmedetomidine group (group SD), and multiple sevoflurane anesthesia + NF-κB agonist phorbol 12-myristate 13-acetate (PMA) + dexmedetomidine group (group SPD). Anesthesia was induced by inhalation of 3% sevoflurane and maintained by inhalation of 60% oxygen and 3% sevoflurane for 2 h for 3 consecutive days in S, SP, SD and SPD groups. At 30 min before each sevoflurane inhalation, pyrrolidine dithiocarbamate 100 mg/kg was intraperitoneally injected in group SP, and dexmedetomidine 20 μg/kg was intraperitoneally injected in group SD. NF-κB agonist PMA 15 μg/kg was intraperitoneally injected at 1 h before each sevoflurane inhalation, and 30 min later dexmedetomidine 20 μg/kg was intraperitoneally injected in group SPD. The mice were sacrificed at the age of 11 days, and the hippocampal tissues were isolated for determination of the expression of phosphorylated NF-κB p65 (p-NF-κB p65) and NLRP3 (by Western blot). The co-staining area of NLRP3 and microglia-specific ionized calcium binding adaptor molecule 1 (Iba-1) double positive cells (NLRP3 + -Iba-1 + cells) was calculated by immunofluorescence. The open field test and novel object recognition test were performed at 29 days of age, and the Morris water maze test was performed at 30-34 days of age. Results:There were no significant differences in each parameter of the open field test among the five groups ( P>0.05). Compared with group C, the expression of p-NF-κB p65 and NLRP3 in hippocampal tissues was significantly up-regulated, the co-staining area of NLRP3 + -Iba-1 + cells was increased, the percentage of novel object exploration and discrimination index were decreased, the escape latency was prolonged, and the frequency of crossing the original platform was reduced in group S ( P<0.05). Compared with group S, the expression of p-NF-κB p65 and NLRP3 in hippocampal tissues was significantly down-regulated, the co-staining area of NLRP3 + -Iba-1 + cells was reduced, the percentage of novel object exploration and discrimination index were increased, the escape latency was shortened, and the frequency of crossing the original platform was increased in SP, SD and SPD groups ( P<0.05). Compared with SD group, the expression of p-NF-κB p65 and NLRP3 was significantly up-regulated, the co-staining area of NLRP3 + -Iba-1 + cells was increased, the percentage of novel object exploration and discrimination index were decreased, the escape latency was prolonged, and the number of crossing the original platform location was decreased in SPD group ( P<0.05). Conclusions:The mechanism by which dexmedetomidine improves long-term cognitive function after multiple sevoflurane anesthesia may be related to inhibiting the activation of NF-κB/NLRP3 signaling pathway in the hippocampal microglia of newborn mice.

Objective:To evaluate the protective effect of pressure-controlled volume-guaranteed ventilation (PC-VG) combined with dexmedetomidine on the lung of pediatric patients undergoing laparoscopic surgery.Methods:Forty-eight pediatric patients of either sex, aged 2-6 yr, weighing 8-21 kg, scheduled for elective laparoscopic pyeloplasty, were divided into 3 groups ( n=16 each) using a random number table method: volume-controlled ventilation (VCV) group (V group), PC-VG group (P group), and PC-VG combined with dexmedetomidine group (PD group). In PD group, dexmedetomidine was intravenously infused for 15 min at a loading dose of 0.5 μg/kg starting from the time point before anesthesia induction followed by a continuous infusion of 0.2-0.5 μg·kg -1·h -1 until the end of operation. VCV mode was used in group V, and PC-VG mode was used in P and PD groups, ventilator settings were adjusted to the mode with a tidal volume 6-8 ml/kg, respiratory rate 15-25 breaths/min, inspiratory/expiratory ratio 1∶2, oxygen flow rate 2 L/min, fraction of inspired oxygen 60%, and P ETCO 2 was maintained at 35-40 mmHg during mechanical ventilation in three groups. At 5 min before pneumoperitoneum (T 0), 10, 60 and 120 min of pneumoperitoneum (T 1-3) and 10 min after release of pneumoperitoneum pressure (T 4), peak airway pressure (Ppeak), mean airway pressure (Pmean), compliance of lung (C L) and airway resistance (Raw) were recorded, alveolar-arterial oxygen partial pressure difference (PA-aO 2), oxygenation index (OI), and respiratory index (RI) were recorded. The occurrence of pulmonary complications was recorded within 7 days after operation. Results:Compared with V group, the Ppeak and Raw were significantly decreased and C L was increased at T 1-4, and PA-aO 2 and RI were decreased and OI was increased at T 3, 4 in P group and PD group ( P<0.05). Compared with P group, no significant change was found in the parameters of respiratory mechanics mentioned above at each time point ( P>0.05), and PA-aO 2 and RI were decreased and OI was increased at T 3, 4 in PD group ( P<0.05). There was no significant difference in the incidence of postoperative pulmonary complications among the three groups ( P>0.05). Conclusions:PC-VG combined with dexmedetomidine has a certain lung-protective effect in pediatric patients undergoing laparoscopic surgery.

Objective    To report the short-term outcomes of a standardized, simplified and reproducible strategy of mitral valvuloplasty (MVP), which was focused on leaflet foldoplasty and anatomic anomalies of congenital mitral regurgitation (MR). Methods    Consecutive 74 patients who underwent MVP by our standardized strategy in our institution from 2016 to 2018 were included retrospectively. There were 30 males and 44 females with a median age of 18.5 (6-146) months and weight of 15.4 (7-51) kg. Results    Anatomic anomalies of MR included: (1) subvalvular apparatus: 72 (97.3%) patients with mal-connected chordae tendineae, 31 (41.9%) with absent chordae tendineae and 14 (18.9%) with fused or dysplastic papillary muscle; (2) leaflet: 10 (13.5%) patients with cleft of anterior leaflet, 61 (82.4%) with leaflet prolapse including 56 (91.8%) with anterior leaflet prolapse; (3) annulus: 71 (95.9%) patients with annular dilatation. Leaflet foldoplasty was performed in 61 (82.4%) patients with leaflet prolapse. All patients were successfully discharged and 4 (5.4%) patients were with moderate MR. The follow-up time was 22.0 (9.1-41.8) months. During the follow-up period, 3 patients had moderate MR and 1 patient had reoperation for severe MR. All patients were in normal cardiac function with a mean left ventricular ejection fraction of 66.0%±6.1%. In addition, the mean left ventricular end-diastolic dimension was 31.8±6.0 mm, which was significant smaller than that before the operation (t=6.090, P<0.000 1). Conclusion    The standardized leaflet foldoplasty with resection of mal-connected chordae tendineae and posterior annuloplasty technique is safe and feasible with favorable short-term outcomes in MR patients.

Objective    To summarize the reoperation experience for complete atrioventricular septal defect (CAVSD) with severe left atrioventricular valve regurgitation (LAVVR) by standardized mitral repair-oriented strategy. Methods    From 2016 to 2019, 11 CAVSD patients underwent reoperation for severe LAVVR by standardized mitral repair-oriented strategy at Fuwai Hospital, including 5 males and 6 females with a median age of 56 (22-152) months. The pathological characteristics of severe LAVVR, key points of repair technique and mid-term follow-up results were analyzed. Results    The interval time between the initial surgery and this surgery was 48 (8-149) months. The aortic cross-clamp time was 54.6±21.5 min and the cardiopulmonary bypass time was 107.4±38.1 min, ventilator assistance time was 16.4±16.3 h. All patients recovered smoothly with no early or late death. The patients were followed up for 29.0±12.8 months, and the echocardiograph showed trivial to little mitral regurgitation in 5 patients, little regurgitation in 5 patients and moderate regurgitation in 1 patient. The classification (NYHA) of cardiac function was class Ⅰ in all patients. Conclusion    Standardized mitral repair-oriented strategy is safe and effective in the treatment of severe LAVVR after CAVSD surgery, and the mid-term results are satisfied.

Objective    To summarize the clinical outcomes and experience of surgical treatment for patients with complete atrioventricular septal defect (CAVSD) above the optimal age for surgery. Methods    We retrospectively reviewed clinical data of 163 simple type CAVSD patients less than 7 years who underwent operations in Fuwai Hospital from 2002 to 2013. The patients were divided into a normal group (n=84, including 37 males and 16 females with an average age of 7.6±2.7 months) and an over-age group (n=79, including 30 males and 49 females with an average age of 34.6±19.6 months) according to whether the age was more than 1 year. Results    The average aortic cross clamp time (88.3±24.4 min vs. 106.1±35.4 min, P<0.001) and cardiopulmonary bypass time (123.6±31.1 min vs. 142.6±47.1 min, P=0.003) were statistically different between the two groups. During the follow-up period (the normal group 53.3±43.9 months, the over-age group 57.2±48.2 months), there was no statistical difference in all-cause mortality (10.7% vs. 8.9%, P=0.691), the incidence of moderate or severe left atrioventricular valve regurgitation (16.7% vs. 21.5%, P=0.430) and reintervention rate (3.6% vs. 0.0%, P=0.266) between the two groups. No left ventricular outflow tract obstruction and complete atrioventricular block occurred in both groups. Conclusion    For CAVSD children above the optimal age, rational surgical treatments can also achieve satisfying results.

To analyze the mid-long-term outcomes of surgical balloon valvuloplasty (SBV) for right ventricular decompression in the treatment of pulmonary atresia with intact ventricular septum (PA/IVS). Methods    Clinical data of consecutive 91 patients who were diagnosed with PA/IVS and underwent SBV in our institution from January 2005 to December 2017 were retrospectively analyzed, including 52 (57.1%) males and 39 (42.9%) females. The median age was 3 months (1 d, 24 months) and the median weight was 4.1 (2.5, 12.0) kg. Results    The SBV was performed in all patients, and 62 of whom received other simultaneous surgeries, including ligation of patent ductus arteriosus (PDA, 33 patients), ligation of PDA with modified Blalock-Taussig shunt (23 patients), ligation of PDA with bidirectional Glenn shunt (6 patients). There was no early postoperative death. The median follow-up time was 8.8 (2.5, 13.4) years, 4 patients were lost. There were 7 (8.0%) deaths and 1 (1.1%) patient with a re-SBV for pulmonary stenosis. The one and a half ventricular repair was performed in 5 (5.7%) patients and Fontan procedure in 2 (2.3%) patients. In addition, the mean Z-value of tricuspid valve annulus was −1.7±1.5, which was significant bigger than that before the operation (t=5.587, P<0.001). Conclusion    SBV via right ventricular outflow tract for right ventricular decompression in the treatment of PA/IVS is safe and reliable. The majority of patients can receive biventricular repair instead of single ventricular palliation by SBV with individually customized shunt.

Objective To study the relationship between intrauterine infection and early neonatal sepsis.Method From October 2015 to September 2016,the clinical data of pregnant mothers and their newborns in Shenzhen Longhua District Central Hospital were collected,and data of Shenzhen People's Hospital from January 2016 to June 2016 were collected.100 pairs of pregnant mothers and their newborns with confirmed or suspected intrauterine infection were selected as the observation group,and another 100 pairs without intrauterine infection during the same period as the control group.The ratio of term infants vs.premature infants was 1∶ 1.The complete blood count (CBC),CD64,procalcitonin (PCT) and C-reactive protein (CRP) were measured in peripheral blood of all mothers on the day of delivery.The CBC,CD64,CRP,PCT,blood culture of both umbilical venous blood and peripheral blood in neonates were examined and the pathological examination of placenta was performed.Result The positive rate of placental pathology and umbilical cord blood culture in observation group were significantly higher than that in the control and the positive rate in preterms was higher than the terms in observation group (P ＜ 0.05).No significant differences existed between term and premature newborns on the positive rate of peripheral blood culture (P ＞ 0.05).The positive rate of blood culture from umbilical cord blood was higher than peripheral blood in observation group (P ＜ 0.05),but no significant difference in control group (P ＞ 0.05).The incidence of septicemia in term and premature newborns in observation group was significantly higher than the control group (P＜ 0.05).The CD64,PCT in mother's peripheral blood and umbilical cord blood,and CRP in mother's blood were all higher than the control group,the differences were statistically significant (P ＜ 0.05),but CRP in umbilical cord blood in both group were similar (P ＞ 0.05).The area under ROC curve of CD64 and PCT in mother's peripheral blood,CD64 and PCT in umbilical cord blood to diagnose early-onset septicemia in newborns was 0.755,0.793,0.852 and 0.811,respectively.Conclusion The risk of neonatal infections is significantly increased because of intrauterine infection.Combination of peripheral and umbilical blood cultures can increase the accuracy of sepsis diagnosis.Both CD64 and PCT in umbilical cord blood and maternal blood can be used as indicators of intrauterine infection with a predictive value in the diagnosis of early-onset neonatal sepsis.