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.
Objectives To evaluate the mid-term outcomes of mechanical valve replacement of the common atrioventricular valve (CAVV) in single-ventricle pediatric patients and compare this with valvuloplasty.Methods We conducted a retrospective study of 91 single-ventricle pediatric patients who underwent CAVV surgery between 2014 and 2025. Patients were divided into two groups according to the initial surgery: valvuloplasty and mechanical valve replacement groups. Baseline and perioperative characteristics were compared between groups. Overall survival and freedom from reintervention were analyzed using Kaplan-Meier curves. Receiver operating characteristic (ROC) curve analysis and maximally selected rank statistics were used to determine the optimal cut-off values for cardiopulmonary bypass (CPB) and aortic cross-clamp (ACC) time in predicting in-hospital and late mortality.Results There were 78 cases in valvuloplasty and 13 cases in the mechanical valve replacement group. The replacement group had significantly longer CPB and ACC time (p<0.001). In-hospital mortality was 2 out of 13 (15.4%) in the replacement group and 8 out of 78 (10.3%) in the valvuloplasty group (p>0.050). The median follow-up was 39 months. Kaplan-Meier analysis showed no significant difference in overall survival (p=0.280) and freedom from reintervention (p=0.270). Reintervention for recurrent regurgitation was required in eight patients after ventriculoplasty, whereas no reintervention was observed following valve replacement. ROC analysis identified a CPB time of 173.5 min as the optimal cut-off for predicting in-hospital mortality,with an area under the curve (AUC) of 0.751. Patients with CPB duration exceeding 180 min had significantly poorer survival (p=0.003).Conclusions Mechanical valve replacement of the CAVV in single-ventricle pediatric patients provides satisfactory mid-term survival comparable to valvuloplasty, but with superior valve durability. Prolonged CPB time is associated with poorer survival and should be minimized whenever possible.
Congenital heart disease (CHD) is the most prevalent congenital abnormality worldwide and is the primary contributor to neonatal mortality. Obstetricians in China continue to face uncertainties about optimal delivery timing, appropriate delivery mode, perinatal interventions, and postnatal surgical outcomes for fetuses with complex CHD. To investigate integrated pregnancy and perinatal management strategies for fetuses diagnosed with complex CHD by prenatal ultrasound in China. A retrospective analysis was performed on 123 fetuses with complex CHD diagnosed by prenatal ultrasound at Peking University People’s Hospital between 2016 and 2023. The mean gestational age at ultrasound diagnosis was 23.7 ± 3.4 weeks. Among the 123 cases, 11 (8.9
Objective:Progressed medical techniques improved the life expectancy of congenital heart disease (CHD) population. Intellectual developmental disability (IDD) has progressively been a raised concern. This study aimed to comprehensively analyze the global burden, regional and age-specific differences, temporal trends, and economic cost of IDD attributable to CHD. Methods:This study was a secondary analysis of the Global Burden of Disease Study 2021 and World Development Indicators. The burden of IDD attributable to CHD was evaluated with prevalence, disability-adjusted life years, and estimated annual percentage change across global, socioeconomic, geographic, and age-specific subgroups. Joinpoint regression models were used to describe the temporal trends. Economic cost models were developed to estimate both direct and indirect costs. Results:In 2021, an estimated 1.05 million people lived with IDD attributable to CHD worldwide. Low-middle social-demographic index (SDI) regions were mostly affected. South Asia experienced the highest prevalence (0.30 million) among all geographic subregions. Children under the age of five were more susceptible to IDD attributable to CHD. The temporal trends varied across different SDI regions and age subgroups. The health-related expenditure of direct costs was disproportional with the burden of IDD attributable to CHD, which also contributed to a substantial income loss in the future. Conclusions and Policy Implications:Socioeconomic disadvantage and younger age are associated with a higher burden of IDD attributable to CHD. Efforts for both reducing CHD mortality and improving neurodevelopmental outcomes should be coordinately allocated.
BACKGROUND:Iron imbalance, including iron deficiency and overload, is associated with increased cardiovascular morbidity and mortality. However, the iron status and its association with postoperative adverse outcomes in pediatric patients with congenital heart disease (CHD) is unclear. This study aimed to investigate the preoperative iron status and its correlation with adverse outcomes in pediatric patients with CHD. METHODS:This is a single-center retrospective study. A total of 8065 pediatric patients (aged 1 month to 5 years) who underwent surgical treatment at Fuwai Hospital's Pediatric Cardiac Surgery Center between 2017 and 2022 were consecutively included. All patients had undergone comprehensive preoperative laboratory tests, including iron-related hematology and biochemistry examinations. The association between ferritin and adverse outcomes was examined using restricted cubic splines. A multivariable logistic regression model was used to determine the associations between preoperative iron status and postoperative adverse outcomes. RESULTS:Iron deficiency, anemia, and iron deficiency anemia were observed in 23.0% (1856/8065), 12.0% (969/8065), and 7.0% (564/8065) of patients, respectively. Patients with cyanotic CHD had a higher prevalence of iron deficiency but a lower incidence of anemia compared with patients with acyanotic CHD. A right-skewed U-shaped relationship was observed between ferritin and death and composite adverse events. Multivariable logistic regression analysis demonstrated that patients with high ferritin levels (ferritin ≥100 µg/L) had a significantly higher risk of in-hospital death (odds ratio [OR]: 8.20, 95% confidence interval [CI]: 1.61-41.86]) and composite adverse events (OR: 4.21 [95%CI: 2.65-6.68]) compared with patients with iron repletion (ferritin levels of ≥15 and <33 µg/L). In addition, patients with iron deficiency (OR: 1.80 [95%CI: 1.18-2.73]) and intermediate ferritin levels (ferritin levels of ≥33 and <100 µg/L; OR: 1.64 [95%CI: 1.11-2.44]) had a higher incidence of composite adverse events compared with those with iron repletion. CONCLUSIONS:Iron deficiency and anemia are common concern in children with CHD. Preoperative iron imbalance, including both iron deficiency and high iron load, was significantly associated with postoperative adverse outcomes in pediatric patients with CHD. Assessment of preoperative iron status might facilitate early identification of high-risk patients, and correcting iron imbalance could potentially mitigate adverse outcomes.
The comparative clinical outcomes of the modified Konno procedure (MKP) and transaortic septal myectomy (TSM) for severe left ventricular outflow tract (LVOT) obstruction in children remain unclear. A total of 177 consecutive patients (age <14 years old) underwent surgical treatments, categorized by MKP or TSM technique (MKP n = 45, TSM n = 132). After propensity-score matching, 45 MKP patients were matched 1:1 with TSM patients. At discharge, the MKP group demonstrated a significantly lower LVOT peak gradient compared to the TSM group (median, 3.2 vs 11.6 mmHg, P = 0.011), which persisted at follow-up (median, 4.8 vs 11.6 mmHg, P = 0.01) in matched data. Although postoperative atrioventricular block was more frequent in the MKP group, no significant difference was observed in permanent pacemaker implantation rate between groups (P = 0.108). During follow-up (median, 1.08 years, 0.25-2.5), the incidence of recurrent LVOT obstruction was higher in the TSM group (17.8% vs 0%). Recurrent LVOT obstruction was associated with aortic annulus size (hazard ratio [HR] = 0.811; 95% confidence interval [CI], 0.675-0.974; P = 0.025) and preoperative LVOT peak gradient (HR = 1.030, 95% CI: 1.010-1.050, P = 0.003). The smaller aortic annulus size (<12.5 mm) or severe LVOT obstruction (>82.5 mmHg) significantly decreased the risk of survival free from recurrent LVOT obstruction (P = 0.01, P < 0.001). The MKP can provide better relief of LVOT obstruction than TSM in young children, particularly with a small aortic annulus or severe obstruction. MKP may not increase the risk of permanent pacemaker implantation and mortality.
Objective To evaluate the midterm outcomes of the aortic valve neocuspidalization (AVNeo) procedure for aortic valve reconstruction in 83 pediatric patients with complex aortic valve disease and to identify risk factors associated with the outcomes. Methods A retrospective analysis was conducted on pediatric patients who underwent the AVNeo procedure between January 2017 and June 2025. The composite primary end point included all-cause mortality, reoperation, or moderate-to-severe aortic valve dysfunction (regurgitation or stenosis). Univariate and multivariate Cox proportional hazards models were used to identify risk factors. Results Among the 83 patients (mean age 7.0 ± 3.7 years; 67.5% male) included in the analysis, the predominant preoperative pathology was moderate-to-severe aortic regurgitation (88.0%). Single-leaflet reconstruction (65.1%) using autologous pericardium (94.0%) was the most common surgical approach. Over a mean follow-up of 28.4 ± 23.9 months, 27 patients (32.5%) met the composite end point, including 2 late deaths, 18 cases of moderate-to-severe regurgitation, and 9 cases of moderate-to-severe stenosis; 10 patients underwent reoperation. The probability of freedom from the primary end point was 83.6%, 64.5%, and 54.4% at 1, 3, and 6 years, respectively. Multivariable analysis identified tricuspid aortic valve morphology as an independent protective factor against the primary end point (hazard ratio, 0.408; 95% CI, 0.178-0.935, P = .034), whereas the use of autologous pericardium showed a strong protective trend (hazard ratio, 0.284; 95% CI, 0.078-1.037, P = .057). Conclusions AVNeo provides midterm outcomes that support its use as an alternative strategy. Tricuspid aortic valve morphology is a strong predictor of favorable outcomes, and the use of autologous pericardium demonstrates significant potential as a protective factor.
BACKGROUND:The impact of high left ventricular posterior wall thickness (PWT) on outcomes after septal myectomy in young children and infants with hypertrophic obstructive cardiomyopathy (HOCM) is unknown. This study sought to correlate PWT z-score with mid-term outcomes following septal myectomy in such patients with HOCM. METHODS:This cohort study included 80 consecutive children and infants who underwent septal myectomy for HOCM between January 2013 and December 2023. Postmyectomy outcomes were compared between patients with a high PWT z-score (group A) and those with a low PWT z-score (group B). The mean duration of follow-up among 97.4% (n = 75) of hospital survivors was 51.18 ± 31.83 months. RESULTS:Fifty children (62.5%) were diagnosed in infancy. The mean age at myectomy was 5.73 ± 3.87 years. Freedom from death or transplantation was 98.5% at 1 year, 96.4% at 3 years, and 88.4% at 5 years. The rate of composite events was significantly higher in group A compared to group B (35% [n = 7/20] vs 7.1% [n = 4/56]; P = .008). Six of 7 late deaths were classified as sudden cardiac death (SCD). In-hospital mortality was associated with age at procedure <2 years (hazard ratio [HR], 0.953; 95% confidence interval [CI], 1.087-14.370; P = .037), PWT z-score ≥9 (HR, 4.600; 95% CI, 1.231-17.195; P = .023) and biventricular obstruction (HR, 4.880; 95% CI, 1.325-17.978; P = .017). SCD was associated with PWT z-score ≥9 (HR, 8.800; 95% CI, 1.377-56.247; P = .022). CONCLUSIONS:Midterm survival after surgery in children with HOCM is lower than that in adult patients. A higher PWT z-score is a novel risk factor for worse postmyectomy outcomes. The most common cause of death in these patients is SCD.
Importance Infantile dilated cardiomyopathy (DCM) associated with left bundle branch block (LBBB) is a rare but life-threatening condition, especially when severe heart failure is present. Identifying effective solutions to improve the prognosis is crucial.Objective This study aims to evaluate the short-term clinical outcomes and cardiac functional changes in infants with LBBB-associated DCM treated with a combined approach of electrical and mechanical cardiac resynchronization.Methods We conducted a retrospective analysis of five infants who underwent epicardial cardiac resynchronization therapy (CRT) combined with pulmonary artery banding between 2023 and 2024. The primary endpoint was improvement in clinical functional class and cardiac function, assessed by left ventricular ejection fraction (LVEF) and N-terminal pro-B-type natriuretic peptide levels. Secondary endpoints included indicators of cardiac reverse remodeling, evaluated by LV end-diastolic dimension (LVEDd), its z-score, cardiac resynchronization, and QRS duration.Results The five enrolled infants had a median age of 6 months (range, 3-12 months). All received guideline-directed medical therapy and were followed for a median of 11 months (range, 6-24 months). All patients achieved normalization of functional class. The median LVEF increased from 26% to 65%, with improvements observed within 1 month. The median LVEDd decreased from 46 to 28 mm, and the corresponding z-score decreased from 11.2 (range, 7.6-13.2) to 0.7 (range, -1.1 to 2.3). The median QRS duration narrowed from 138 to 115 ms. Mechanical dyssynchrony was virtually resolved in all patients by the last follow-up.Interpretation The combined resynchronization strategy appears to be highly effective for treating infants with LBBB-associated DCM. Further studies are needed to differentiate the specific roles of electrical and mechanical synchronization in improving outcomes.
The objective of this study is to identify preoperative echocardiographic predictors of mitral valve (MV) repair failure in pediatric patients. Pediatric patients with mitral regurgitation (MR) grade ≥ 2 who received MV repair between January 2019 and July 2024 were retrospectively reviewed. MV repair failure was defined as a composite of postoperative functional MV failure, heart transplantation, or death. MV morphology and related parameters were assessed using two- and three-dimensional echocardiography. A total of 309 pediatric patients were included, with a median age of 15.50 (6.00, 52.30) months; 164 (53.1
The optimal timing of surgery for discrete subaortic stenosis (DSS) is controversial due to limited evidence from large-scale trials. This study aimed to identify prognostic factors and reassess current surgical thresholds. A total of 508 patients with DSS, including 375 pediatric patients, who underwent surgery at a single tertiary center between May 2018 and March 2025 were retrospectively analyzed. The primary endpoint was a composite of DSS-related adverse events, and the secondary endpoint was aortic valve (AV) dysfunction. Multivariable Cox regression and receiver operating characteristic (ROC) analyses were used to identify predictors. Over a mean follow-up period of 3.5 years, 19.2
Objectives: Humanitarian program has emerged as a critical support mechanism for the management of congenital heart disease (CHD), yet its cost-effectiveness at the hospital level receives little investigation. Methods: We reviewed 2759 patients underwent CHD surgery at Fuwai Hospital, National Center for Cardiovascular Diseases from 2012 to 2022, and received financial aid from nongovernmental organizations. Analyzed data included baseline characteristics, surgical complexity, hospitalization costs, nongovernmental organization funding proportions, and disability-adjusted life years (DALYs) averted. Cost-effectiveness was calculated as funding expenditure per DALY averted, with adjustments for natural disease history and surgical outcomes. Results: Charitable fundings covered a median of 31% of hospitalization costs (¥36,564.34/USD $5424.98 per patient), averting 50.82 DALYs per patient at a cost of ¥952.19 ($141.27) per DALY. Neonates and complex CHD cases showed higher number of DALYs averted (59.44 and 54.42, respectively) but lower charitable funding proportions (24% and 32%, respectively). Patients with single-ventricle physiology and patients with previous surgeries exhibited relatively lower cost-effectiveness (¥1627.30 and ¥1349.08 per DALY). Unexpected reinterventions correlated with higher in-hospital mortality (9.6% vs 1%) and lower funding support (19% vs 33%). Conclusions: Charitable funding reduces financial barriers for surgical CHD treatment in China, demonstrating high cost-effectiveness. Funding models should evolve to incorporate surgical complexity and patient age, ensuring more reasonable support in the future. More importantly, the demonstrated cost-effectiveness should encourage more organizations to join the effort of funding CHD surgery, which is the most pivotal action at present.
Background: Current circulating biomarkers for idiopathic pulmonary arterial hypertension (IPAH) lack specificity for preclinical detection and fail to capture the biological heterogeneity driving disease progression. Furthermore, molecular mechanisms underlying the "sex paradox" of IPAH, where females exhibit higher susceptibility but lower mortality, remain poorly understood, hindering the development of precision therapeutics. Methods: We performed an integrated proteogenomic analysis characterizing 2,920 plasma proteins from 45,811 participants in the UK Biobank. We integrated discovery-driven Cox regression with case-control verification, followed by cis-Mendelian randomization and colocalization to distinguish causal mediators from bystanders. We utilized unsupervised clustering for biological risk stratification and machine learning to deconvolute sex-dimorphic proteomic signatures, finally applying systemic drug reproposing to prioritize therapeutic candidates. Results: We identified 92 causal proteins driving IPAH incidence (e.g., NOTCH3, FLT3IG) and 15 driving mortality (e.g., REG4, CA6), with 9 proteins (e.g., EDN1, LRRN1) serving as dual determinants. Unsupervised clustering identified a high mortality-risk phenotype characterized by upregulated proteins associated with extracellular matrix-receptor interaction, transforming growth factor-β signaling, cardiac hypertrophy, and elastic fibre formation, together with reduced plasma levels of APOL1. Notably, sex-stratified analysis revealed divergent pathogenic architectures that progression of IPAH in males was predominantly linked to right ventricular dysfunction mediators (e.g., NT-proBNP, GDF15), whereas in females, it was more strongly driven by vascular dysfunction mediators (e.g., EDN1, BCL2L15). Finally, we prioritized 27 druggable targets, with genetic evidence highlighting AGRN, CLU, and DDR1 as high-confidence candidates for therapeutic intervention. Conclusions: This study delineates causal proteomic landscape of IPAH, bridging epidemiological associations with genetically supported targets. By uncovering the molecular basis of sex-dependent outcomes and prioritizing novel druggable proteins, our findings provide a robust framework for preclinical detection, innovative risk stratification, and the development of precision therapeutics. ### Competing Interest Statement The authors have declared no competing interest. ### Funding Statement This study was supported by the National Clinical Research Center for Cardiovascular Diseases, Fuwai Hospital, Chinese Academy of Medical Sciences (Grant No. NCRC2024001). The UK Biobank received ethical approval from the Northwest Multi-center Research Ethics Committee (11/NW/0382), and all participants provided written informed consent. This study was conducted under UK Biobank application number 105435. ### Author Declarations I confirm all relevant ethical guidelines have been followed, and any necessary IRB and/or ethics committee approvals have been obtained. Yes The details of the IRB/oversight body that provided approval or exemption for the research described are given below: The UK Biobank received ethical approval from the Northwest Multi-center Research Ethics Committee (11/NW/0382), and all participants provided written informed consent. This study was conducted under UK Biobank application number 105435. I confirm that all necessary patient/participant consent has been obtained and the appropriate institutional forms have been archived, and that any patient/participant/sample identifiers included were not known to anyone (e.g., hospital staff, patients or participants themselves) outside the research group so cannot be used to identify individuals. Yes I understand that all clinical trials and any other prospective interventional studies must be registered with an ICMJE-approved registry, such as ClinicalTrials.gov. I confirm that any such study reported in the manuscript has been registered and the trial registration ID is provided (note: if posting a prospective study registered retrospectively, please provide a statement in the trial ID field explaining why the study was not registered in advance). Yes I have followed all appropriate research reporting guidelines, such as any relevant EQUATOR Network research reporting checklist(s) and other pertinent material, if applicable. Yes All data produced are available online at UK biobank. (https://www.ukbiobank.ac.uk)
Cardiac fibromas are rare benign primary tumors of the heart; nearly one-third of affected patients are less than 1 year old, and only 15
OBJECTIVES:The mechanisms underlying anatomic tricuspid regurgitation in congenitally corrected transposition of the great arteries remain unclear. This study investigated the association between interventricular septal motion and tricuspid regurgitation severity. METHODS:Echocardiographic data from 406 patients with congenitally corrected transposition of the great arteries were retrospectively analyzed and stratified by tricuspid regurgitation grade (≥3 vs ≤2) at the initial evaluation. Interventricular septal motion was categorized as normal, reversed, or bidirectional. Patients who underwent pulmonary artery banding and those with tricuspid regurgitation grade 2 or less without prior surgery were analyzed as separate subgroups. RESULTS:Among 109 patients with tricuspid regurgitation grade 3 or greater, 91 (83.49%) had reversed interventricular septal motion, significantly higher than in the 2 or less group (6/297, 2.02%). In the pulmonary artery banding subgroup (n = 79), tricuspid regurgitation severity significantly decreased postprocedure (P < .001), accompanied by a shift in interventricular septal motion from reversed to normal (P < .001). In the no surgery group (n = 136), 14 patients (10.29%) developed progressive tricuspid regurgitation and right ventricular dilation secondary to the reversal of interventricular septal motion. Reversed interventricular septal motion was a strong predictor of severe tricuspid regurgitation (area under the curve, 0.77; P = .001) and independently associated with its development (hazard ratio, 28.35; P < .001). CONCLUSIONS:Reversed interventricular septal motion is significantly associated with severe tricuspid regurgitation in congenitally corrected transposition of the great arteries and may act as both a mechanistic contributor and a predictive marker of disease progression.
BACKGROUND:Thrombocytopenia is common for patients in the intensive care unit (ICU) and is associated with adverse outcomes. ICU thrombocytopenia in pediatric patients who underwent cardiac surgeries with cardiopulmonary bypass (CPB) is inadequately studied. OBJECTIVES:We aimed to investigate the incidence, risk factors, and prognostic role of ICU thrombocytopenia after congenital cardiac surgeries with CPB. METHODS:A retrospective study involving 11 761 patients was conducted. Patients were categorized into 4 groups of thrombocytopenia based on platelet counts tested during ICU: non (>150 × 109/L), mild (100-150 × 109/L), moderate (50-100 × 109/L), and severe (<50 × 109/L). Logistic and Cox regression analyses were utilized to explore the risk factors of thrombocytopenia and the association of ICU thrombocytopenia with 30-day mortality. RESULTS:ICU thrombocytopenia was observed in 4007 patients (34.1%), with mild, moderate, and severe thrombocytopenia occurring in 2773 (23.6%), 987 (8.4%), and 247 (2.1%) patients, respectively. Younger age, cyanotic congenital heart disease, CPB duration, and preoperative laboratory findings (red blood cell, thrombocytopenia, red cell distribution width, hematocrit, and coagulation disorder) were identified as independent risk factors of ICU thrombocytopenia. Patients with moderate (hazard ratio [95% CI]: 11.38 [3.02-42.87]; P < .001) and severe thrombocytopenia (hazard ratio [95% CI]: 49.54 [13.11-187.14]; P < .001) had a significantly higher risk of 30-day mortality. Furthermore, with the increase in the severity of ICU thrombocytopenia, there was an incremental increase in the incidence of postoperative critical bleeding and thrombosis, perioperative blood transfusions, length of ICU stays, and duration of mechanical ventilation. CONCLUSION:ICU thrombocytopenia occurred in one-third of children after congenital cardiac surgery with CPB, and it was associated with multiple adverse outcomes.
BACKGROUND:The applicability of four major traditional in-hospital mortality models in the Chinese setting is unclear due to disease spectrum and population heterogeneity. This study aimed to test the performance of these models in the Chinese setting and to construct and externally validate a novel model. METHODS:A total of 21,855 consecutive pediatric patients who underwent congenital heart surgery from January 2015 to December 2021 in Shanghai Children's Medical Center were enrolled. For external validation, the study additionally pooled 5,221 consecutive pediatric patients who underwent this surgical treatment from January 2020 to December 2021 in Beijing Fuwai Hospital. The performance of the Aristotle Basis Complexity (ABC) score, Risk Adjustment for Congenital Heart Surgery (RACHS)-1 categories, Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery (STAT) score, and STAT categories was tested. Independent predictors were used to develop a model. The area under the receiver operating characteristic curves (AUROCs) and Brier score were used to examine the model performance. RESULTS:The AUROCs were 0.778 for ABC score, 0.685 for RACHS-1 categories, 0.808 for STAT score, and 0.784 for STAT categories. When preoperative covariates were added to the four models, the AUROCs improved: ABC score (AUROC = 0.860), RACHS-1 categories (AUROC = 0.844), STAT score (AUROC = 0.856), and STAT categories (AUROC = 0.864). The best-performing model incorporated six variables, including age, height, oxygen support, previous cardiac operation, emergency surgery, and STAT categories. The AUROCs and Brier score were 0.864 and 0.00977 in the development cohort and 0.860 and 0.00654 in the external validation cohort. CONCLUSIONS:The four major traditional models were only moderately effective in predicting in-hospital mortality after congenital heart surgery in the Chinese setting. The novel model founded on the STAT categories in combination with preoperative covariates can serve as a useful and effective tool for predicting the risk of in-hospital mortality after congenital heart surgery in the Chinese setting.