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.

Objective: This study compares perspectives between interventional cardiologists and cardiac surgeons on congenital heart disease management via a national survey. Methods: An online questionnaire was distributed to senior surgeons or interventional cardiologists capable of comprehensive congenital heart disease care at 118 hospitals (236 total invitations). Results: Of 162 valid responses, 90 were surgeons and 72 were cardiologists. There were no differences between surgeons and cardiologists in demographic factors. Survey results showed no statistical differences in (1) the overall relationship between interventional and surgical approaches (complementary and sequential); (2) interventional treatment for complex congenital heart disease (using the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery, simple and complex congenital heart disease were defined as lesions with category I and categories II-V); and (3) multidisciplinary team management (long-term follow-up by a consistent core team). Differences were found in the following areas: 1. In decision-making, surgeons placed emphasis on age and developmental potential (adjusted P = .016), whereas cardiologists focused on physiological status and complications (adjusted P = .008). 2. For secundum atrial septal defect (P < .001), patent ductus arteriosus (P < .001), muscular/perimembranous ventricular septal defect (P = .001), pulmonary valve stenosis (P = .010), and coarctation of the aorta (P < .001), each specialty considered their own approach as the preferred option. 3. Surgeons expressed greater demand for interdisciplinary cross-training (adjusted P = .020). Conclusions: Despite broad consensus, cardiologists and surgeons differ in decision-making priorities and training needs. These findings support structured communication, optimized management, specialty-tailored training, and hybrid care models to improve patient outcomes.

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.

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.

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)

Behçet’s disease is a multisystem inflammatory disorder, occasionally complicated by the need for aortic surgery due to vasculitis in children in whom growth potential is crucial. This report details the case of a 4-year-old girl with severe aortic regurgitation secondary to Behçet’s disease. An initial echocardiographic scan revealed a thickened, prolapsed aortic valve with severe regurgitation, leading to a confirmed diagnosis of Behçet’s disease with aortic regurgitation. Following stabilization with steroids and immunosuppressants, aortic cusp replacement surgery using autologous pericardium was performed. The surgical technique adhered to Ozaki’s method, ensuring precise pericardial preparation and interrupted suturing. The postoperative recovery was uneventful, and the patient continued anti-inflammatory treatment. At the 3-year follow-up, the patient was asymptomatic with normal activity levels, and an echocardiographic scan confirmed excellent aortic valve function. This case highlights the feasibility and long-term efficacy of using autologous pericardium for aortic cusp replacement in paediatric patients with Behçet’s disease and emphasizes the need for further studies to establish standardized protocols for this challenging patient population.

OBJECTIVES:Long-term surgical outcomes for double outlet right ventricle (DORV) continue to pose challenges for most paediatric cardiac centres. This study aimed to investigate the mid- to long-term outcomes of a large cohort of DORV patients. METHODS:DORV patients who underwent surgery guided by a refined anatomical sub-typology in our centre between August 2001 and December 2023 were retrospectively reviewed. The primary end-point was cardiac mortality and the secondary end-point was unplanned surgical reintervention. RESULTS:A total of 1135 patients were included in the study. Among them, 824 patients (72.6%) underwent biventricular repair, 258 (22.7%) received single-ventricle procedures, and 53 (4.7%) underwent palliative surgeries. The median age at surgery was 25.0 months [IQR: 9.0-72.0]. In-hospital deaths occurred in 14 cases (1.2%). Over a mean follow-up period of 8.9 years, the estimated overall survival rates at 5, 10, and 15 years were 96.3% [95% CI, 95.1-97.5], 93.0% [95% CI, 91.6-95.2], and 92.7% [95% CI, 87.7-96.9], respectively. Second end-point-free survival rates at the same time points were 95.2% [95% CI, 94.8-95.7], 89.5% [95% CI, 88.7-90.3], and 82.1% [95% CI, 80.7-83.5], respectively. Transposition of the great arteries-type (TGA-type) anatomy and palliative procedures were independently associated with increased mortality. CONCLUSIONS:Optimal outcomes in DORV can be achieved through precise anatomical evaluation and tailored surgery. TGA-type anatomy and palliative procedure were key risk predictors.

Background:The Ozaki technique demonstrated promising results in adults, but few studies reported on pediatric patients with limited follow-up time. This study aimed to evaluate the mid-term results of Ozaki technique compared with Ross operation for complex aortic valve diseases in children.Materials and methods:One hundred and seventeen children underwent either Ozaki (n=64) or Ross (n=53) operation from January 2017 to December 2023. The primary endpoint was incidence of moderate or severe regurgitation/stenosis (AR/AS) post procedure.Results:No significant difference was observed in age (6.5 +/- 3.4 vs. 7.9 +/- 4.3 years) and weight (25.9 +/- 15.5 vs. 31.0 +/- 25.9 kgs) at surgery. The Ozaki group had significantly more patients in heart failure (20.3 vs. 1.9%, P = 0.003) before surgery and more patients needed ECMO installation (6.3% vs. 0, P=0.125) after surgery. The Ozaki group were in worse status with more patients occurred heart failure (20.3 vs. 1.9%, P = 0.003) before surgery and needed ECMO installation (6.3 vs. 0, P = 0.125) after surgery. During follow-up (20.4 +/- 17.3 vs. 22.7 +/- 22.8 months, P=0.526), five patients (7.8%) in Ozaki group but no patients in Ross group required reoperations. The incidence of moderate or severe AR (28.1 vs. 3.1%) and AS (31.3 vs. 5.7%) were significantly higher than Ross group. Multivariate analysis identified lower age [HR: 1.282 (95% CI: 1.075-1.529), P=0.006] and ECMO installation [HR: 0.126 (0.018-0.887), P=0.037] to be risk factors for moderate or severe AR, and higher aortic transvalvular gradient before discharge was confirmed as the only risk factor for moderate or severe AS (>= 36 mmHg) at follow-up in Ozaki group.Conclusion:Ozaki technique may be used as a palliative procedure for complex aortic valve diseases in children, but its' mid-term results were not durable as Ross surgery, especially younger patients.

Background:Dilated cardiomyopathy (DCM) is the most common cardiomyopathy, and 40--50% of patients may die or need a heart transplant in 5 years after diagnosis. Although heart transplantation is the most effective life-saving option of end-stage DCM, scarcity of donors and series of complications prevent many patients from receiving timely treatment. Pulmonary artery banding (PAB) is recently described as an alternative strategy for end-stage DCM, with low left ventricular function (LVEF) but preserved right ventricular function, may potentially restore heart function and delay the need for heart transplantation, but current clinical evidence is still insufficient. On the other hand, the medication treatment of DCM in pediatric patients is mostly based on the experience of adults. It remains unclear whether PAB combined medication treatment could benefit infants and young children patients. The aim of this study was to assess the short-term efficacy of PAB combined with medication therapy in infants and young children with end-stage DCM, compared with medication therapy alone.Methods:This is a retrospective analysis of 18 consecutive pediatric patients aged ranging from 1 month to 44 months old who diagnosed with end-stage DCM (LVEF <= 30%) with preserved right ventricular function between 2019 and 2023 in our hospital. All patients had been treated with conventional medications for 2 months. Then they were divided in two groups: PAB surgery group (6/18), and nonsurgery group (12/18). Regardless of whether surgery was performed, both groups continued to receive medication treatment. Recovery of ventricular function was primary endpoints. Secondary endpoints included 180-day mortality and severe heart failure (LVEF <= 30%).Results:The authors found there were no differences in age, weight, height, BMI, renal function, liver function, pulmonary hypertension, tricuspid valve regurgitation, mitral valve regurgitation, and genetic abnormalities between those with and without PAB surgery. Comparing with nonsurgery group, five patients in surgery group regain the normal cardiac ejection fraction (LVEF >= 50%) (5/6, 83.3% vs. 4/12, 33.3%, P=0.131). A total of three patients had sudden death in nonsurgery group, and there was no death in surgery group (P=0.180). Five patients (5/12, 41.7%) still remain the low heart failure (LVEF <= 40%) after 6 months of enrollment only given medical therapy, and none of patients present with LVEF <= 40% in PAB surgery group (0/6, 0% vs. 8/12, 67.7%, P=0.034).Conclusion:Pulmonary artery banding is safe and effective in infants and young children with end-stage DCM with preserved right ventricular function. Combined with conventional heart failure medication therapy, it may provide short-term benefits postoperatively, decrease the cardiogenic shock, act as a bridge to recovery, and potentially reduce the need for heart transplantation. Long-term effects remain further observation, and larger randomized controlled trials would be more persuasive in validating its efficacy.

OBJECTIVES:We aimed to summarize the clinical implications and outcomes of the Ross II procedure for infants and young children. METHODS:This study retrospectively reviewed consecutive paediatric patients who underwent Ross II procedure from 2011 to 2022 at Fuwai Hospital. Baseline, operative, postoperative characteristics, and outcomes were summarized. Postoperative all-cause mortality was defined as the primary outcome, and reoperation as the secondary outcome. RESULTS:Ten patients who underwent the Ross II procedure were included in the final analysis, with 2 cases of late mortality and 3 cases of reoperation during a median follow-up of 36 months (range: 3.5 months to 9 years). The median age at surgery was 1.8 years (interquartile range [IQR]: 0.65 to 3.5), and the median mitral annulus diameter was 16 mm (IQR: 15 to 18). Patients who underwent the Ross II procedure experienced a prolonged postoperative course, with a median intensive care unit stay of 11.5 days (IQR: 8.5 to 19.75) and postoperative hospital stay of 24 days (IQR: 20.25 to 34.75). Mitral stenosis emerged as the predominant late complication following the Ross II procedure, affecting 80% patients, as evidenced by the increase in mean gradient 3 years postoperatively compared with discharge. CONCLUSIONS:The Ross II procedure may represent a safe option for infants and young children when prosthetic valves are unavailable, despite the limited growth potential of the pulmonary autograft.

This study evaluated medium and long-term postoperative results to identify perioperative factors that are associated with prolonged mechanical ventilation (PMV) and prolonged postoperative hospital stay (PPH) in pediatric patients with aortic valve disease (AVD) undergoing Ozaki procedure. We retrospectively reviewed the medical records of 129 patients who underwent the Ozaki procedure between January 2017 to July 2025. The cohort was divided into a PMV group that included patients with ≥ 75th (20.5 h) percentile for duration of mechanical ventilation and a Non-PMV group which included all other patients. Similarly, the cohort was divided into a PPH group that included patients with ≥ 75th (14d) percentile for duration of postoperative hospital stay and a Non-PPH group which included all other patients. There were 36 cases (27.91

Background: The available evidence regarding the predictive value of troponins and natriuretic peptides for early postoperative outcomes in pediatrics is limited, controversial, and based on small sample sizes. The authors aimed to investigate the association of N-terminal pro B-type natriuretic peptide (NT-proBNP) and high-sensitivity troponin T (hs-TnT) with the in-hospital adverse outcomes after congenital cardiac surgeries. Methods: A secondary analysis based on a prospective study of pediatric congenital heart disease (CHD) patients was conducted to investigate the association of NT-proBNP and hs-TnT tested within 6 h postoperatively with in-hospital adverse events. A multivariate logistic regression analysis with a minimum P value approach was used to identify the optimal thresholds of NT-proBNP and hs-TnT for risk stratification. Results: NT-proBNP and hs-TnT are positively correlated with cardiopulmonary bypass time, mechanical ventilation duration, and pediatric intensive care unit stay. The predictive performance of NT-proBNP is excellent for adverse events in both patients younger than 1 year [area under the curve (AUC): 0.771, 0.693-0.850] and those older than 1 year (AUC: 0.839, 0.757-0.922). However, hs-TnT exhibited a satisfactory predictive value solely in patients aged over 1 year. (AUC: 0.784, 0.717-0.852). NT-proBNP levels of 2000-10 000 ng/l [odds ratio (OR): 3.79, 1.47-9.76] and exceeding 10 000 ng/l (OR: 12.21, 3.66-40.80) were associated with a higher risk of postoperative adverse events in patients younger than 1 year. Patients older than 1 year, with NT-proBNP higher than 500 ng/l (OR: 15.09, 6.05-37.66) or hs-TnT higher than 1200 ng/l (OR: 5.50, 1.47-20.59), had a higher incidence of postoperative adverse events. Conclusions: NT-proBNP and hs-TnT tested within postoperative 6 h demonstrated significant predictive value for postoperative adverse events in CHD patients older than 1 year. However, among CHD patients younger than 1 year, only NT-proBNP exhibited commendable predictive performance for postoperative adverse events.

Various surgical techniques have been introduced to treat supravalvular aortic stenosis (SVAS). However, there is no consensus on the optimal approach. This study aimed to analyze the outcomes of surgical treatment of SVAS and determine the optimal strategy. The Kaplan–Meier curve was used to demonstrate the survival estimates. The Cox proportional hazard model was used to identify risk factors for residual aortic stenosis and residual stenosis-related reintervention. From December 2008 to December 2023, 98 patients with SVAS undergoing surgical repair in our institution were included [McGoon group, n = 62; Doty group, n = 36]. There were 2 in-hospital deaths and 1 late death. The survival rates at 1, 5, and 15 years were 98.0

Background The association between congenital heart disease (CHD) and non-optimal temperatures has received limited investigation. We aimed to investigate the impact of extreme temperatures on CHD mortality. Methods We reviewed the National Mortality Surveillance System of China and retrieved death records attributable to CHD from 2013 to 2021. Temperature and air pollutants data were obtained from the ERA5-Land reanalysis dataset and the ChinaHighAirPollutants database. A two-stage case-crossover study design was implemented. Sensitivity and subgroup analyses were performed to test the robustness of fi ndings and determine the vulnerable population. Findings A total of 32,168 CHD deaths were included, showing a significant association between cold and CHD mortality, while there was little effect for heat. The odd ratio (OR) ranged from 1.05 (95% confidence interval: 1.00-1.10) to 1.15 (1.03-1.29) across country, with a more pronounced impact in non-monsoon regions up to 1.67 (1.20-2.32). Cold extremes accounted for an attributable fraction of 4.09 per 1000 CHD death nationwide and 13.30 per 1000 CHD deaths in non-monsoon regions. Sensitivity analyses utilizing apparent temperature and adjusting for air pollutants confirmed the robustness of the main fi ndings. Female and pediatric CHD patients were identified as the vulnerable population to cold extremes. Interpretation For the fi rst time, this nationwide study demonstrated the significant impact of cold extremes on CHD mortality, particularly in non-monsoon regions, and among female and pediatric subgroups. These fi ndings may suggest that healthcare professionals advise CHD patients to avoid exposure to cold extremes, and provide insight into healthcare policy adjustment.

Background The prevalence and risk factors for failure to thrive (FTT) in pediatric patients with congenital heart disease (CHD) remain ambiguous. We aimed to investigate the prevalence, growth profiles, risk factors, and vulnerable subtypes of CHD associated with FTT in pediatric patients with CHD. Methods This was a cross-sectional study based on Chinese Database for Congenital Heart Surgery. FTT was defined as either stunting or underweight (height or weight standard deviation score <-2), and they were standardized by references of normal Chinese population. Risk factors was determined with logistic regression model, and growth profiles were delineated in each subgroup. Findings A total of 13,256 CHD patients were included in this study, with 3994 patients of mild CHD, 7195 patients of moderate CHD and 2067 patients of complex CHD. The prevalence of stunting, underweight and FTT was 24%, 29.3% and 36.9%, respectively. Preoperative anaemia, left ventricle systolic dysfunction, younger age, more complex CHD types, lower birth weight and genetic syndrome were found to be the risk factors for FTT in CHD patients. Interrupted aortic arch was revealed to be the most severe group associated with FTT. Interpretation FTT is ubiquitous in patients with CHD and exacerbated in high -risk subgroups. Our findings hinted the necessity of early identification and intervention for FTT in patients with CHD during daily practice of pediatrics, as it has the potential to improve outcomes and enhance their quality of life. Furthermore, we advocate for the initiation of prospective research with longitudinal data to comprehensively investigate the association between FTT and CHD across the lifespan.

Background Staged repair is common for complex conotruncal defects, often involving bidirectional Glenn (BDG) procedure. Following the cavopulmonary shunt, both Fontan completion and biventricular conversion (BiVC) serve as definitive approaches. The optimal strategy remains controversial. Methods The baseline, perioperative and follow-up data were obtained for all paediatric patients with conotruncal defects who underwent BDG procedure as palliation in Fuwai Hospital from 2013 to 2022. Patients with single ventricle were excluded. The primary outcome was mortality. The secondary outcome was reintervention, including any cardiovascular surgeries and non-diagnostic catheterisations. Results A total of 232 patients were included in the cohort, with 142 underwent Fontan (61.2%) and 90 underwent BiVC (38.8%). The median interstage period from BDG to the definitive procedure was 3.83 years (IQR: 2.72–5.42) in the overall cohort, 3.62 years (IQR: 2.57–5.15) in the Fontan group and 4.15 years (IQR: 3.05–6.13) in the BiVC group (p=0.03). The in-hospital outcomes favoured the Fontan group, including duration of cardiopulmonary bypass, aortic cross-clamp, mechanical ventilation and intensive care unit stay. Postoperative mortality was generally low and comparable, as was the reintervention rate (HR=1.42, 95% CI: 0.708 to 2.85, p=0.32). The left ventricular size was smaller at baseline and within the normal range at follow-up for both Fontan and BiVC groups; however, it was significantly larger with BiVC at follow-up. Conclusion In paediatric patients with conotruncal heart defects who underwent BDG procedure, BiVC is a feasible option, especially for patients with certain Fontan risk factors, and are not ideal candidates for successful Fontan completion.

The conventional surgery (CS) of supracardiac total anomalous pulmonary venous connection (TAPVC) is not always effective particularly in the setting of complex anatomy such as the mixed variety of TAPVC. This study aimed to analyze the outcomes of surgical treatment of supracardiac TAPVC and determine the optimal strategy. From December 2009 to December 2023, patients with supracardiac TAPVC undergoing surgical repair in our institution were included. The Kaplan–Meier curve was used to demonstrate the survival estimates. The Cox proportional hazard model was used to identify risk factors for death and postoperative pulmonary venous obstruction (PVO). One hundred and eighty-three patients with supracardiac TAPVC underwent surgical repair [CS group, n = 102; modified L-shaped incision technique (MLIT) group, n = 81]. There were 8 in-hospital deaths and 16 late deaths. The survival rates at 1, 5, and 10 years were 89.0

Introduction: The success of cardiac surgical techniques and progress of interventional therapies prolonged the life expectancy patients with congenital heart anomalies (CHA). Reduction of mortality and extension of lifespan exacerbated the burden of non-fatal comorbidities. Developmental intellectual disability (DID) is the most substantial non-fatal comorbidities of pediatric patients with CHA. However, no epidemiological study describe the global burden of DID attributable to CHA (DID-CHA). Research Question: What was the global burden, regional inequality, and age specific discrepancy of DID-CHA? Goal: The aim of this study is to address the gap of lacking epidemiological data of global burden, regional inequality, and age discrepancy of DID-CHA. Methods: This was a secondary analysis study by utilizing impairment data of GBD study 2021. Data was collected from the website of Institute for Health Metrics and Evaluation (IHME, query tool: https://ghdx.healthdata.org/gbd-2021 ). The prevalence, disability adjusted life years (DALYs), and their calculayted annual percentage changes (EAPC) across global, both sexes, seven regions, five SDI regions, and 204 countries and territories from 1990 to 2021 were investigated. Results: In 2021, the global number of DID-CHA cases was 1.05 million (95% UI: 0.83 to 1.24 million) with an age-standardized prevalence rates of 15.71 per 100,000 (95% UI: 12.36 to 18.58). From 1990 to 2021, the EAPC of prevalence was -0.15 (95% CI: -0.16 to -0.13). Regional inequality of disease burden of DID-CHA remained prevalent worldwide. Pediatric population, especially neonates and infants, had a more substatial disease burden of DID-CHA compared to adults aged over 20 years. Conclusions: This is the first study that thoroughly describe the global prevalence, regional inequality, and age discrepancy of the burden on DID-CHA, by utilizing the public data of GBD 2021. DID-CHD is believed to be a progressive issues for the whole-life management of CHD. Future efforts on resource allocation for neurodevelopmental disability in population with CHD should be comparable with the expenditure in reducing CHD mortality. Persistent regional socioeconomic disparities will definitely move to impact the discrepant burden of DID-CHA. Increasing socioeconomic resources aimed at improving DID-CHA outcomes should prioritize in neonate and infants with CHA, as they represent the most critically affected age groups.