Congenital heart disease (CHD) is a common birth defect in children, and surgical intervention is the primary treatment. The traditional standard median sternotomy (MS) has drawbacks such as significant trauma and obvious scarring. The right axillary incision (RAI) has gradually become a conventional approach due to its advantages of preserving thoracic cage integrity, small incision size, rapid recovery, and hidden scarring. However, there is currently a lack of relevant guidelines and consensus for its application. This consensus adopts the international Delphi process, systematically searching domestic and foreign literature on CHD from 1982 to 2024. It uses the GRADE system for evidence grading and, through multidisciplinary expert discussions, clarifies the applicable CHD types, surgical techniques, establishment of extracorporeal circulation, organ protection strategies, management of special disease types, and approaches to common complications of RAI. Results show that RAI is strongly recommended for most simple congenital heart diseases (CHDs) (e.g., simple ventricular septal defect, atrial septal defect), weakly recommended for some complex CHDs (e.g., mild tetralogy of Fallot), and not recommended for complex CHDs such as transposition of the great arteries or in children with severe right thoracic deformity. Additionally, it standardizes key operational parameters: weight (5-30 kg as optimal), age (6 months-6 years as preferred), incision location, extracorporeal circulation cannulation, and organ protection measures. This consensus provides an evidence-based basis for standardizing the clinical application of RAI in open-heart surgery for CHD, ensuring surgical safety and efficacy.

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BACKGROUND:Several studies have confirmed the important role of progesterone in fetal and neonatal brain development. Chronic hypoxia in the fetal period may mediate neurodevelopmental and cognitive impairment in offspring by interfering with placental steroid hormone synthesis, but the mechanism is unclear. METHODS:We systematically evaluated the effects of hypoxia on placental endocrine-fetal neuro-cognitive function by constructing a model of chronic hypoxia from fetal to early childhood, combined with progesterone supplementation, multi-omics of placenta and brain samples, microglial morphological analysis, and behavioral testing. RESULTS:Chronic hypoxia significantly inhibited placental steroid synthase, leading to a concurrent decrease of progesterone levels in the fetal circulation and brain. Progesterone deficiency in the brain activates microglia, which in turn drives excessive inflammation under chronic hypoxic conditions, thereby interrupting oligodendrocyte differentiation and causing myelination deficits. Chronic hypoxia could also lead to impairment of spatial memory and learning ability shown by behavioral tests. During hypoxic pregnancy, administration of exogenous progesterone restored the progesterone gradient between the placenta and brain, inhibited abnormal activation of microglia, promoted myelination, and reversed cognitive deficits. CONCLUSION:Chronic hypoxia downregulates placenta-derived progesterone through the "placenta-neural axis", which in turn leads to cognitive impairment through the microglia-myelin pathway. Progesterone supplementation during pregnancy can provide a theoretical basis for clinical intervention.

OBJECTIVES:Group 2 pulmonary hypertension remains a highly morbid disease, yet no specific therapy exists. We sought to determine whether total pulmonary vein banding in rats could reliably display the clinical features of group 2 pulmonary hypertension and sought to investigate potential molecular and cellular determinants associated with disease progression. METHODS:Four-week-old Sprague-Dawley rats were randomized to receive total pulmonary vein banding (n = 18) or sham procedure (n = 18). Serial hemodynamic and histological assessments were performed at post-total pulmonary vein banding weeks 1, 4, and 8. At each time point, 12 rats (6 in total pulmonary vein banding vs 6 in sham group) underwent harvest for lung sectioning and transcriptomic and proteomic analysis. Weighted gene coexpression and Bayesian networks were used to explore the top hub gene associated with pulmonary hypertension development. RESULTS:Compared with the sham group, rats in the total pulmonary vein banding group developed pulmonary hypertension after surgery. Mild to severe progression of pulmonary hypertension was observed in the total pulmonary vein banding group from postsurgery week 1 to 8, including higher right ventricular systolic pressure (P < .001), impaired right ventricle-pulmonary artery coupling (P < .001), decreased tricuspid annular plane systolic excursion (P < .001), and right ventricle fractional area change (P < .001). Slc2a1 was identified as a hub gene upregulated in the lungs, which was enriched in the perivascular macrophages and associated with disease progression. CONCLUSIONS:This rodent model of total pulmonary vein banding provides a useful platform for modeling pulmonary vein congestion severe group 2 pulmonary hypertension. Slc2a1 is a key regulator of perivascular macrophage infiltration, driving the disease progression. Targeting Slc2a1-mediated perivascular inflammation might have a therapeutic potential.

Limited angiogenic potential of coronary vascular endothelial cells (cVECs) poses a significant barrier to effective therapeutic revascularization after myocardial infarction (MI). However, the underlying mechanisms of restricted vascularization post-MI remain unclear. Here, we demonstrate that both reduced density and limited proliferation of mural cells result in inadequate mural cell coverage of coronary vessels, consequently impeding coronary vascularization within the MI heart. Our findings reveal that the cell cycle inhibitor phosphatase and tensin homolog (PTEN) is expressed in mural cells from both sham-operated and MI hearts. Conditional Pten deletion in mural cells enhances mural-derived cell (MDC) proliferation, increases coronary vascular density, promotes arteriogenesis, and improves cardiac function following MI. Furthermore, Pten deficiency upregulates chemokine C-X-C motif ligand 12 expression, which potentially reduces apoptosis in cVECs and MDCs and increases vascular density. These results identify cardiac mural cells as promising therapeutic targets for enhancing coronary angiogenesis and vascular remodeling after ischemic injury.

Background:Paediatric patients who underwent surgery for mitral regurgitation (MR) have a high risk of recurrence or death; however, no prediction tool has been developed to risk-stratify this challenging subpopulation. Methods:In this multicentre cohort study, paediatric patients undergoing surgery for congenital MR in Shanghai Children's Medical Center in January 1st, 2009-December 31st, 2022 were included for analysis while those had a combination with infective endocarditis, anomalous left coronary artery from the pulmonary artery, rheumatic valvular disease, connective tissue disease, or single ventricle were excluded. A Cox regression model predictive of the primary outcome (a composite of mortality or mitral valve [MV] re-operation) was derived and converted to a point-based risk score. This score was externally validated in a cohort of patients undergoing MR surgeries between January 1st, 2009 and December, 31st, 2022 in eight independent hospitals in China. The Harrell's c index and Hosmer-Lemeshow test was used to quantify the discrimination and calibration of the risk score. Findings:In total, 2640 patients (female: 57% [n = 1505]) with a median age of 0.99 (IQR, 0.47-2.60) years were included. The primary outcome occurred in 262 patients (16.6%) over a median of 5.7-year follow-up in the derivation cohort (n = 1581; median age of 1.03 years [IQR, 0.47-2.67]) and in 130 (12.3%) over a median of 7.1-year follow-up in the validation cohort (n = 1059; median age of 0.93 years [IQR, 0.46-2.51]). The score variables included preoperative variables (age, the presence of primary MR, N-terminal pro-brain natriuretic peptide, left ventricular ejection fraction, and left atrium z score), perioperative changes in z scores of LA and left ventricular end-diastolic dimension, and the procedural variable (use of MV annuloplasty or not). This risk score, ranging from -22 to 10, yielded strong discrimination (Harrell's c index: 0.85, 95% CI, 0.83-0.87) and exhibited good calibration of postoperative 10-year primary outcome (Hosmer-Lemeshow; χ2 = 9.85; P = 0.33) in the derivation cohort, which maintained in the validation cohort (Harrell's c index: 0.86, 95% CI, 0.83-0.89; Hosmer-Lemeshow; χ2 = 23.80; P = 0.64). In addition, a simplified stratification of the score model (low-risk: -22 to -5, intermediate risk: -4 to 0, high risk: >0) showed good performances in predicting the primary outcome in both derivation and validation (Ps < 0.001). Interpretation:The scoring system represents a crucial step towards personalised management care for paediatric MR. However, this model has not been applied in clinical practice and require validation in large and diverse cohorts of patients. Further work should aim to incorporate other novel multi-modality metrics to optimise the PRIMARY score. Funding:Chinese National Natural Science Foundation of China, Science and Technology Commission of Shanghai Municipality, Science and Technology Project of Guizhou Province.

BACKGROUND:Equal access to care for patients with congenital heart disease (CHD) remains unrealized globally. The ASSIST project (Academic Medical Hospitals-Local Institutions collaboration) is an ongoing national quality initiative implemented in low-resource settings in China attempting to reduce gaps in access to CHD care. This study sought to evaluate its feasibility and effectiveness. METHODS:Shanghai Children's Medical Center, an academic medical center, has partnered with 4 local hospitals in low-resource regions to enhance local CHD programs since 2021. Comparison was made between patients receiving treatments in these 4 local hospitals before (2013-2020) versus after the ASSIST project (2021-2024). In addition, contemporaneous patients receiving treatments in Shanghai Children's Medical Center (2021-2024) were compared with the post-ASSIST cohort of patients. The primary outcome was a composite of postoperative mortality and multiorgan dysfunction. A key secondary outcome was delayed treatment, defined as an interval of more than 6 months between the time of surgery and the time when the clinicians recommended surgery at the initial presentation. RESULTS:The analysis cohort included 11 895 pediatric patients (median age, 2.0 years [25th-75th percentile 0.7-5.0]; 5933 female [49.9%]), with 3333 cases in the pre-ASSIST group, 1566 in the post-ASSIST group, and 6996 in the Shanghai Children's Medical Center group. Lower family educational attainment (odds ratio, 1.50 [95% CI 1.21-1.85]; P<0.001) and lower annual household income (odds ratio, 1.65 [95% CI, 1.14-2.38]; P=0.008) were associated with increased incidence of the primary outcome in the low-resource regions but could be mitigated by the ASSIST project in a multivariable model. There was a change in case mix of the CHDs after the ASSIST project, with a significant increase in more complex cases (35.5% versus 7.3%; P<0.001) and neonatal surgery (9.1% versus 1.5%; P<0.001). A higher portion of patients in the pre-ASSIST group had delayed treatment compared with those in the post-ASSIST group (43.2% versus 23.3%; P<0.001). Primary outcome analyses showed that patients in the post-ASSIST group and the Shanghai Children's Medical Center group had equivalent outcomes (4.4% versus 3.6%; P=0.13). CONCLUSIONS:Interhospital partnership appears to be a useful and feasible method to deliver regionalized quality care for pediatric patients with CHD in resource-limited regions.

Background Recurrent pulmonary vein stenosis (PVS) following surgical repair of total anomalous pulmonary venous connection is associated with poor prognosis. Preclinical studies have shown that use of an angiotensin receptor blocker can attenuate intimal hyperplasia; notwithstanding, its clinical utility is of uncertain benefit. Methods This single‐center study included patients undergoing total anomalous pulmonary venous connection repair in 2020 to 2021. Since August 2020, patients have been considered for valsartan therapy early after operation. Contemporaneous participants were subcategorized into study versus control groups based on valsartan exposure. Patients in the control group were treated with the same protocolized algorithm except valsartan administration. The primary end point was postoperative PVS (PPVS) progression. Results Overall, 104 patients operated on at a median age of 1.3 months were included (valsartan group: 25 versus control group: 79). The baseline characteristics were similar between the 2 groups. Within a median follow‐up of 28.6 months, 27 patients developed PPVS noted by echocardiography and computed tomography angiography, among which 22 with clinical PPVS underwent reoperations. No between‐group difference was observed in the incidence of initial PPVS (P=0.80, Cohen's h=0.06 [95% CI, −0.38 to 0.50]) and reoperation (P=0.46, Cohen's h=−0.18[ 95% CI, −0.65 to 0.29]); however, patients in the valsartan group had a significantly lower risk of PPVS progression (P=0.019, Cohen's h=−1.12 [95% CI, −1.66 to −0.57]) and subsequent PPVS progression after reoperation (P=0.011, Cohen's h=−1.71 [95% CI, −2.61 to −0.82]) compared with the control group. PPVS‐related death was observed in 9 cases (11.4%) in the control group versus none (0%) in the valsartan group. No adverse event related to valsartan occurred in this series. Conclusions Early use of valsartan after total anomalous pulmonary venous connection surgery appears to potentially be a feasible and effective adjunct to reoperation in treating pediatric acquired PVS.

OBJECTIVES:The goal of this study was to evaluate the feasibility and learning curve of a right vertical infra-axillary thoracotomy (RVIAT) in the surgical closure of a ventricular septal defect (VSD). METHODS:Clinical outcomes in paediatric patients (<18 years) undergoing VSD operations between 2018 and 2021 in 2 tertiary hospitals were reviewed retrospectively. After 1:1 propensity score matching, patients undergoing an RVIAT were compared with those undergoing a median sternotomy (MS). The learning curve that reflected the number of cases needed to achieve technical proficiency was measured using total operating time as a metric and was evaluated using a risk-adjusted cumulative sum analysis. RESULTS:Of the 3515 eligible patients, 2183 (62%) underwent an MS and 1332 (38%) underwent an RVIAT. After matching, 797 cases in the RVIAT and MS groups were recorded, respectively. Propensity weighting produced an excellent balance in patient baseline characteristics including age, weight, and VSD subtypes. There was no between-group difference in postoperative rhythm disturbances (0.6% vs 1.1%; P = 0.83), significant residual VSD (0.1% vs 0.4%, P = 0.62), and reoperation within 60 days postoperatively (0.1% vs 0.9%, P = 0.07). The RVIAT provided better cosmesis (satisfactory score: 9.21 ± 0.06 points vs 6.98 ± 1.17 points; P < 0.001), shorter median length of hospital stay (5.5 days vs 8.0 days, P < 0.001), and lower cost (8513.3 ± 3193.2 USD vs 9222.3 ± 2504.9 USD; P < 0.001). The surgeons could conquer the early learning phase of the RVIAT after performing a mean of 41 operations. CONCLUSIONS:A RVIAT can combine good outcomes with favourable cosmesis in VSD repair, and sufficient exposure to RVIAT procedures is crucial for proficiency.

IntroductionPeriodontitis has been identified as a potential risk factor for cognitive impairment associated with immune dysregulation. T helper 17 (Th17) cell-associated immune responses are involved in both diseases, while signal transducer and activator of transcription 3 (Stat3) is kown to be crucial for Th17 pathogenicity. Accordingly, in this study, we investigated how Stat3-mediated Th17 pathogenicity contributes to the link between periodontitis and cognitive impairment.MethodsLevels of Th17-related cytokines in gingival crevicular fluid (GCF) were measured in individuals with and without cognitive impairment. A periodontitis model was established in mice with conditional deletion of Stat3 in Th17 cells (Stat3fl/fl; Il17a-CreERT2, cKO) and wild type (Stat3fl/fl, WT) mice via injection of Porphyromonas gingivalis lipopolysaccharide (P. gingivalis LPS) into gingival sulcus. Cognitive function was assessed through behavioral tests. Expression of Th17-related cytokines and microglial pro-inflammatory markers was evaluated by reverse transcription-quantitative PCR (RT-qPCR), ELISA, flow cytometry, and immunohistochemistry. To evaluate effects of CD4+ T cells on microglial M1 polarization, BV2 microglia were co-cultured with primary CD4+T cells which were stimulated with P. gingivalis LPS after isolated from cKO and WT mice.ResultsCompared with cognitively normal participants, levels of Th17-related cytokines increased in participants with cognitive impairment. Significant alveolar bone resorption and cognitive impairment were observed in WT mice with periodontitis. These periodontitis-induced changes were alleviated in cKO mice, accompanied by a weakening of neuroinflammation and mitigation of Th17 immune responses. In vitro, M1 polarization and activation of the MAPK/ERK signaling pathway were inhibited in BV2 cells co-cultured with Stat3-deleted Th17 cells.ConclusionStat3-mediated Th17 pathogenicity bridged the correlation between periodontitis and neuroinflammation related to cognitive impairment, offering novel perspectives for a therapeutic target for blocking the mouth-to-brain axis.

Background Pulmonary vein stenosis (PVS) continues to be a major complication after surgical repair of total anomalous pulmonary venous connection (TAPVC). Recent studies suggest that the morphology of pulmonary venous confluence and the left atrium (LA) is associated with PVS. However, there are limited data on the prognostic value of integrating quantitative confluence-atrial morphology into risk stratification. Objectives This study sought to evaluate the prognostic impact of novel imaging metrics derived from 3-dimensional (3D) computed tomography angiography (CTA) modeling on postsurgical PVS (PPVS) in the supracardiac TAPVC (sTAPVC) setting. Methods Patients undergoing sTAPVC repair in 2017 to 2022 from 3 centers were retrospectively reviewed. Study investigators developed 3D CTA modeled geometric features to quantify confluence-atrial morphology that were analyzed with regard to PPVS. Results Of the 162 patients (median age 61 days; 55% having preoperative pulmonary venous obstruction [prePVO]) included, 47 (29%) with PPVS at a median of 1.5 months ([quartile 1-quartile 3: 1.5-3.0 months]). In the univariable analysis, the indexed total volume of the LA and confluence (iTVLC) and the ratio of the corresponding confluence length to the mean distance between the LA and confluence (CCL/mDBLC ratio) were significantly associated with PPVS. In a multivariable model adjusting for prePVO and age, the iTVLC and CCL/mDBLC ratio independently predicted PPVS (HR: 1.15; 95% CI: 1.06-1.25; and HR: 1.20; 95% CI: 1.08-1.35, respectively, all P < 0.01). Specifically, an iTVLC ≥20 cm3/m2 and a CCL/mDBLC ratio ≥7.7 were significantly associated with a reduced risk of PPVS. Conclusions Quantification of 3D confluence-atrial morphology appears to offer a deeper and better metric to predict PPVS in patients with sTAPVC.

Background: Hypertensive disorders of pregnancy (HDP) are among the primary reasons for maternal and perinatal deaths globally. Encompassing chronic hypertension, pregnancy induced hypertension (PIH), and preeclampsia, HDP are among the most frequent complications encountered during pregnancy. Considering the emerging congenital heart disease (CHD) population globally, research on HDP in pregnancies with CHD is urgently needed. Methods: Outcomes for 2220 pregnant women with CHD were evaluated retrospectively from 1993 to 2016 and prospectively from 2017 to 2019 from 7 tertiary hospitals. Maternal death, cardiac complications, obstetric and offspring complications of completed pregnancies and their demographics, cardiac characteristics, and comorbidities were collected. To identify the risk factors for HDP, univariate and multivariate analysis were performed through logistic regression. Results: Of 2220 completed pregnancies with CHD from 7 tertiary institutions, 77 women had PIH, and 76 women had preeclampsia. The main CHD diagnosis of patients with PIH was shunt lesion (including ASD, VSD, and PDA), left heart abnormality (including AS/AI, BAV, and COA), and single ventricle (SV). The main CHD diagnosis of patients with preeclampsia was shunt lesion (including ASD, VSD, and PDA), left heart abnormality (including AS/AI and MS/MI), and right heart abnormality (including TOF, PS/PAS/PI, DORV and DCRV). In parturients with PIH, the mortality and incidence of heart failure were 2.60% and 15.58%, respectively. In parturients with preeclampsia, the mortality and incidence of heart failure were 6.58% and 23.68%, respectively. Pregnancies with HDP had a significantly higher incidence of adverse maternal and offspring events than pregnancies without HDP. Independent risk factors for HDP included multiple gestation, use of medically assisted reproduction, having elevated brain natriuretic peptide (BNP) level, delayed first ANC visit, and absence of strict antenatal supervision. Conclusion: In summary, the presence of HDP in women with CHD significantly increased the risk of mortality and morbidity for both the mother and the child, particularly in cases involving preeclampsia.

Chronic hypoxia, common in neonates, disrupts gut microbiota balance, which is crucial for brain development. This study utilized cyanotic congenital heart disease (CCHD) patients and a neonatal hypoxic rat model to explore the association. Both hypoxic rats and CCHD infants exhibited brain immaturity, white matter injury (WMI), brain inflammation, and motor/learning deficits. Through 16s rRNA sequencing and metabolomic analysis, a reduction in B. thetaiotaomicron and P. distasonis was identified, leading to cholic acid accumulation. This accumulation triggered M1 microglial activation and inflammation -induced WMI. Administration of these bacteria rescued cholic acid -induced WMI in hypoxic rats. These findings suggest that gut microbiota-derived cholic acid mediates neonatal WMI and brain inflammation, contributing to brain immaturity under chronic hypoxia. Therapeutic targeting of these bacteria provides a non-invasive intervention for chronic hypoxia patients.

Objective: This study aimed to assess the efficacy of a modified exhaust method in pediatric open-heart surgery involving cardiopulmonary bypass. Method: Data from 303 cases conducted at the Department of Cardiac Surgery, Guizhou Hospital, Shanghai Children's Medical Center, between October 2023 and March 2024 were analyzed. Among these cases, 202 utilized the modified exhaust method, divided into group A (101 cases with median thoracotomy) and group C (101 cases with lateral thoracotomy), while 101 cases used the traditional exhaust method in group B (median thoracotomy). Comparative analysis included general patient data, cardiopulmonary bypass duration, aortic cross-clamp time, time for exhaust and reperfusion upon opening, post-reperfusion ST segment abnormalities on electrocardiogram, intracardiac pneumogram observations via esophageal ultrasound, relevant plasma biochemical indexes on postoperative day one, postoperative drainage volume, duration of ventilator use, and length of stay in the intensive care unit (ICU). Results: There was no difference in between-group comparisons regarding age (27.98 ± 3.57 vs. 34.05 ± 3.96 months; P = 0.401) and weight (12.23 ± 0.55vs. 12.59 ± 0.70 Kg; P = 0.563). Longer Cardiopulmonary bypass times were observed in patients undergoing median thoracotomy than those undergoing lateral thoracotomy (group B: 108.47 ± 2.30 min vs. group C: 117.03 ± 2.82 min, P = 0.002; group A: 108.91 ± 2.63 min vs. group C: 117.03 ± 2.82 min, P = 0.035). Exhaust and rebound times after opening were significantly shorter in the modified exhaust-method group compared with the traditional-method group (Group A: 52.62 ± 1.39 s vs. Group B: 65.20 ± 1.49 s, P < 0.001; Group B: 65.20 ± 1.49 s vs. Group C: 4.31 ± 1.16 s, P < 0.001). There was no statistical difference in terms of postoperative biochemical indexes, drainage volume, ventilator use time, and ICU stay time (all P > 0.05). Conclusions: The modified exhaust method demonstrates overall good immediate results in pediatric congenital heart surgery. It was superior to the traditional exhaust method in terms of reducing exhaust times and potentially minimizing the risk of local aortic injuries. Additionally, it appeared to be suitable for lateral thoracotomy surgery.