Background: In the 1990s, there were few multicenter research collaborations and pediatric cardiovascular clinical trials. The National Heart, Lung, and Blood Institute at the National Institutes of Health established the Pediatric Heart Network (PHN) in 2001 to stimulate multi-center collaboration and clinical studies in children and adults with congenital heart disease (CHD) and pediatric acquired heart disease. Methods: The PHN developed a flexible infrastructure for multi-center collaborative clinical research in children and adults with CHD and pediatric acquired heart disease. The objectives of the PHN are to improve health outcomes in individuals of all ages with CHD and pediatric acquired heart disease, to disseminate findings to improve treatment options and standards of care, to train and educate new investigators, and to support families during the conduct of clinical research. Results: To date, the PHN has conducted 30 studies, including 13 clinical trials, across over 60 sites and has enrolled over 10,000 participants. PHN studies have impacted clinical practice and guidelines in CHD and have supported the career development of young investigators, research nurses, and study coordinators. None of this would have been possible without the many partnerships with patient advocacy organizations, the U.S. Food and Drug Administration, a variety of industry collaborators and clinical registries. PHN studies have leveraged registry data to improve efficiency, minimize burden and reduce errors in data collection. Conclusion: The PHN’s success is due to fostering collaboration across pediatric cardiology centers, creating a clinical research infrastructure that can adapt to different types of studies, and emphasizing career development of young investigators and research coordinators. This paper will summarize the PHN’s history, partnerships, use of clinical registries, future directions, and ways to get involved.
Atherosclerotic cardiovascular disease remains the leading cause of global mortality, with low-density lipoprotein cholesterol established as a primary causal risk factor. Despite widespread implementation of statin therapy, substantial interindividual variability in treatment response persists, necessitating precision medicine approaches to optimize therapeutic outcomes. This comprehensive narrative review synthesizes current understanding of pharmacogenomic determinants influencing lipid-lowering therapy efficacy, examines mechanisms underlying residual cardiovascular risk, and evaluates emerging therapeutic modalities targeting previously unexploited pathways in lipid metabolism. Genetic variants in key genes including 3-hydroxy-3-methylglutaryl-CoA reductase, apolipoprotein E, low-density lipoprotein receptor, and proprotein convertase subtilisin/kexin type 9 demonstrate significant associations with differential treatment responses, with specific polymorphisms conferring enhanced efficacy or increased intolerance risk. Beyond traditional statin therapy, novel therapeutic approaches targeting proprotein convertase subtilisin/kexin type 9, angiopoietin-like protein 3, apolipoprotein C-III, and ATP citrate lyase offer substantial low-density lipoprotein cholesterol reductions of 50–80%, while RNA-based therapies including antisense oligonucleotides and small interfering RNA provide precise molecular targeting capabilities. Despite intensive lipid-lowering interventions, residual cardiovascular risk persists through four principal mechanisms: triglyceride-rich lipoproteins, lipoprotein(a), inflammatory processes, and suboptimal treatment adherence. Integration of pharmacogenomic insights with emerging therapeutic modalities enables personalized risk stratification and treatment selection, representing a paradigm shift toward precision medicine in cardiovascular disease prevention and management.
Background: Four-dimensional (4D) ultrasound is increasingly being used for prenatal diagnosis of congenital heart disease (CHD). We aimed to perform a systematic review and meta-analysis to evaluate its diagnostic accuracy for fetal CHD. Methods: This systematic review was conducted in accordance with the PRISMA-DTA guidelines. We systematically searched eight databases for studies published up to July 22, 2025. Data were extracted to calculate diagnostic accuracy metrics, study quality was assessed using QUADAS-2, and a bivariate random-effects model was used for the meta-analysis. Results: A total of 49 studies were included, comprising 45 retrospective and 4 prospective studies, which were mainly (91.8%) conducted in China. These studies involved 23,397 fetuses, among which 2115 were diagnosed with congenital heart disease. The pooled sensitivity of 4D ultrasound for diagnosing fetal congenital heart disease was 0.91 (95% CI: 0.89–0.93), the pooled specificity was 0.98 (95% CI: 0.97–0.99), and the area under the summary receiver operating characteristic (SROC) curve (AUC) was 0.98 (95% CI: 0.96–0.99). Although 4D ultrasound technology can be implemented as early as 11 weeks of gestation, its diagnostic sensitivity and specificity reached a superior level and stabilized at an average of 20 weeks of gestation (range 14–28 weeks). Meta-regression indicated that sample size and prior suspicion of fetal CHD were significant contributors to heterogeneity (p < 0.05). Conclusion: Four-dimensional ultrasound has high diagnostic efficacy for fetal CHD and is suitable for prenatal screening of fetal CHD, and the diagnostic effect is optimal and stable at an average gestational age of 20 weeks (range 14–28 weeks).
Background: During the surgical repair of complex congenital heart disease (CCHD), a subset of patients is unable to tolerate abrupt postoperative hemodynamic shifts, which can lead to significant complications. To mitigate this risk, certain abnormal venous channels are deliberately left open at the conclusion of surgery to provide a decompressive route, thereby reducing the likelihood of pulmonary hypertensive crises. Nevertheless, the continued patency of these vessels may induce chronic hemodynamic disturbances, often requiring subsequent treatment. This study was designed to assess the safety and efficacy of transcatheter intervention for such persistent anomalous systemic veins in CCHD patients following initial corrective operation. Methods: We performed a retrospective review of 14 CCHD patients who underwent transcatheter closure of residual anomalous systemic veins-including azygos, hemiazygos, and vertical veins-after prior corrective surgery at Fuwai Hospital from December 2007 to September 2019. Results: All procedures were completed successfully. Following closure of the azygos or hemiazygos pulmonary arterial pressure (mPAP) rose slightly but statistically significantly post-intervention, although it remained within normal limits (12.3 f 2.7 mmHg vs. 10.8 f 3.3 mmHg, p = 0.027). In the two patients undergoing vertical vein closure, SFAO2 also improved markedly (Case 13: 98% vs. 86%; Case 14: 99% vs. 88%). Over a mean follow-up period of 26.3 f 13.9 months, all patients remained clinically stable without major adverse events. Conclusions: Transcatheter closure of residual anomalous systemic veins after corrective surgery for CCHD is a safe and effective therapeutic option, associated with high procedural success and favorable short- to mid-term clinical outcomes.
Uhl’s anomaly is an exceedingly rare (fewer than 1 in 1,000,000 live births) and often fatal congenital heart disease characterized by the near-complete absence of the right ventricular (RV) myocardium. Although typically considered sporadic, we report a familial case suggesting an inherited etiology. A 12-year-old boy presented with exertional chest pain and a decade-long history of an abnormal cardiac silhouette. Comprehensive imaging revealed apical RV wall thinning, aneurysmal bulging with trabeculations, and severely impaired RV function, with a Tricuspid Annular Plane Systolic Excursion (TAPSE) of 10 mm and a Fractional Area Change (FAC) of 35%. These findings are consistent with a Uhl-like phenotype. Family screening identified similar, though less severe, RV structural anomalies in the patient’s father and sister, supporting an autosomal dominant inheritance pattern. Whole-exome sequencing revealed a rare heterozygous TTN variant (NM_003319:exon154:c.C56156T:p.T18719M) that co-segregated with the disease phenotype. The proband was treated with medical therapy targeting heart failure and remained clinically stable at discharge. To our knowledge, this is the first reported case of familial Uhl’s anomaly associated with a TTN gene mutation. These findings support a possible genetic basis for Uhl’s anomaly and highlight the importance of genetic screening in patients with familial cardiac structural abnormalities.
Background: An increasing number of patients with Fontan circulation are reaching adulthood; however, long-term outcomes remain limited by Fontan failure, which is characterized by elevated central venous pressure (CVP) and reduced cardiac output. Red blood cell distribution width (RDW), a readily available hematological parameter, is a known prognostic marker of heart failure. However, its relationship with invasive hemodynamics in adolescent and adult Fontan patients has not been fully examined. Objectives: To clarify the association between RDW and invasive hemodynamic indices in adolescent and adult Fontan patients and assess the utility of RDW as a noninvasive circulatory marker. Methods: This single-center retrospective study included consecutive Fontan patients aged >= 16 years who underwent routine cardiac catheterization >= 5 years after surgery, between June 2014 and July 2025. Laboratory data and catheter-derived hemodynamics were also analyzed. The primary endpoint was the correlation between RDW and CVP, and the secondary endpoint was the correlation between RDW and central venous oxygen saturation (ScvO(2)). Results: Forty patients (median age: 22 years) were analyzed. The median RDW was 13.3%, and the median CVP was 11.0 mmHg. RDW correlated positively with CVP (rho = 0.57, p < 0.001) and negatively with ScvO(2) (rho = -0.66, p < 0.001) and the cardiac index (rho = -0.34, p = 0.03). Patients with elevated RDW (>14.5%) had higher CVP (14.5 vs. 10.5 mmHg, p < 0.001) and lower ScvO(2) (63.8% vs. 76.1%, p < 0.001), compared with those with normal RDW. Multivariable analysis identified RDW as an independent predictor of ScvO(2) (p < 0.001). Conclusions: In adolescents and adults after the Fontan procedure, RDW was significantly associated with elevated CVP and reduced ScvO(2) and independently predicted impaired oxygen delivery. RDW is inexpensive, widely accessible, and may serve as a practical noninvasive biomarker for the early detection of Fontan failure and the optimization of invasive testing and interventions during long-term follow-up.
Background: We evaluated surgical outcomes of biventricular repair for atrioventricular septal defect (AVSD) with tetralogy of Fallot (TOF) or double-outlet right ventricle (DORV). Methods: This retrospective pilot study included 12 patients who underwent biventricular repair of AVSD with TOF (n = 6) or DORV (n = 6) between 2004 and 2023. Right ventricular outflow tract (RVOT) reconstruction was performed using transannular patch (TAP, n = 4) or pulmonary valve preservation (PVP, n = 8). Clinical outcomes, including longitudinal pulmonary valve growth, RVOT obstruction, and pulmonary regurgitation, were reviewed descriptively, with particular focus on the feasibility of PVP. Results: The median age and body weight at the time of surgery were 11.7 (8.3-18.8) months and 8.6 (7.3-10.5) kg, respectively. The median follow-up duration was 67.9 (58.7-174.3) months. The two-patch technique (n = 10) was most commonly used for AVSD repair. There were no early mortalities and one late mortality. At discharge, significant (>= 36 mmHg) RVOT obstruction was observed in two patients who underwent PVP. During follow-up, one patient required reoperation for significant (moderate or greater) atrioventricular valve regurgitation, and two patients in the PVP group underwent transcatheter intervention for significant RVOT obstruction. At the last follow-up, significant RVOT obstruction was present in two patients. Significant (moderate or greater) pulmonary regurgitation occurred in three patients in the TAP group. In patients who underwent PVP, the pulmonary valve annulus z-score remained within acceptable ranges, and the RVOT pressure gradient predominantly decreased to acceptable levels during follow-up. Conclusions: Mid-term outcomes of PVP in the biventricular repair of AVSD with TOF or DORV are acceptable. In selected patients, the PVP strategy may be considered a reasonable and feasible option for RVOT reconstruction.
Obstructed infradiaphragmatic total anomalous pulmonary venous return (TAPVR) in premature infants presents significant management challenges due to the high surgical risk in low-birth-weight, preterm neonates. We present strategies for managing this condition in a 10-day old 1.3 kg ex-32-week premature infant including late umbilical venous access, use of wire-snare rail for stable stent deployment, and monitoring for progressive multi-level obstruction. Long-term follow-up demonstrated spontaneous stent fracture and occlusion. This approach successfully bridged to definitive repair with excellent outcomes.
Background: We aimed to explore the value of echocardiography plus cardiopulmonary exercise testing (CPET) for predicting the functional status and adverse outcomes of adult patients with congenital heart disease (CHD), and to develop a multivariate prediction model. Methods: Subjects (135 in total) in this single-center prospective cohort study were enrolled from adult patients suffering from CHD treated in this hospital during January 2021 and August 2023. Standardized echocardiography and CPET were conducted on all subjects at enrollment, with such indicators as left ventricular ejection fraction (LVEF), right ventricular function parameters, peak oxygen uptake (peak VO2), and carbon dioxide ventilatory equivalent slope [ventilation (VE)/carbon dioxide output (VCO2) slope] recorded. Results: In contrast with the non-event group, the event group exhibited higher levels of N-terminal pro-B-type natriuretic peptide (NT-proBNP) and lower LVEF and peak VO2 (p < 0.05). Four key predictors (age, NT-proBNP, LVEF and peak VO2) were acquired after Least Absolute Shrinkage and Selection Operator (LASSO) regression analysis. According to Cox analysis, age and NT-proBNP served as risk factors, while LVEF and peak VO2 acted as protective factors (p < 0.05). As to the Logistic model, its area under curve (AUC) was 0.920, the mean AUC in cross-validation was 0.919, and the SHAPLEY Additive exPlanations (SHAP) analysis results were in line with the Cox model. Conclusions: Echocardiography plus CPET is capable of effectively forecasting the functional status and adverse outcomes of adult CHD patients, and the key independent predictors are age, NT-proBNP, LVEF and peak VO2.
Background: Coronary artery disease (CAD) is a major global health concern requiring efficient and accurate diagnostic methods. Manual interpretation of coronary computed tomography angiography (CTA) images is time-consuming and prone to interobserver variability, underscoring the need for automated segmentation and stenosis detection tools. Methods: This study presents a hybrid multi-scale 3D segmentation framework utilizing both 3D U-Net and Enhanced 3D U-Net architectures, designed to balance computational efficiency and anatomical precision. Processed CTA images from the ImageCAS dataset underwent data standardization, normalization, and augmentation. The framework applies ensemble learning to merge coarse and fine segmentation masks, followed by advanced post-processing techniques, including connected component analysis and centerline extraction, to refine vessel delineation. Stenosis regions are detected using the Enhanced 3D U-Net and morphological operations for accurate localization. Results: The proposed pipeline achieved near-perfect segmentation accuracy (0.9993) and a Dice similarity coefficient of 0.8539 for coronary artery delineation. Precision, recall, and F1 scores for stenosis detection were 0.8418, 0.8289, and 0.8397, respectively. The dual-model approach demonstrated robust performance across varied anatomical structures and effectively localized stenotic regions, indicating clear superiority over conventional models. Conclusion: This hybrid framework enables highly reliable and automated coronary artery segmentation and stenosis detection from 3D CTA images. By reducing reliance on manual interpretation and enhancing diagnostic consistency, the proposed method holds strong potential to improve clinical workflows for CAD diagnosis and management.
Background: There has been an increasing number of studies documenting the application of the right axillary thoracotomy (RAT) approach for the repair of congenital heart diseases. However, no research has reported the RAT approach in repairing the anomalous aortic origin of a coronary artery (AAOCA). This study aims to investigate the feasibility and safety of the RAT approach for repairing AAOCA in children. Methods: We performed a retrospective study at the Children’s Hospital of Chongqing Medical University between January 2024 and October 2025 to investigate the clinical outcomes of the RAT approach for repairing AAOCA in children. Results: A total of twelve patients with AAOCA were operated on via a RAT approach. Conventional unroofing was performed in eight cases, and a modified unroofing procedure was performed in four cases. Simultaneous repair of associated cardiac defects was conducted in eight cases. Postoperative pneumonia occurred in one patient. There were no early deaths during the hospitalization. The postoperative ostial diameter was significantly larger than the preoperative diameter (p = 0.0005). No patients were lost to follow-up, and aortic valve insufficiency was not observed during the follow-up. There were no late deaths reported following discharge, and ischemic signs were also not documented. Conclusions: Surgical repair via the RAT approach may serve as a safe and effective alternative for children with AAOCA. However, surgical strategies should be carefully determined based on preoperative assessment of the anomalous coronary arteries.
Objective: The objective of this study was to understand intraoperative risk factors for post-Norwood extracorporeal membrane oxygenation (ECMO) in patients with hypoplastic left heart syndrome (HLHS). Methods: We conducted a retrospective, single-institution review of all patients with HLHS who underwent a Norwood procedure (nadir cardiopulmonary bypass temperature <= 22 degrees C) over a 12-year period with quantitative and qualitative analysis. Results: Of 102 Norwood patients, 14 (13.7%) required ECMO. ECMO patients had longer median cardiopulmonary bypass (CPB) times (276 vs. 172 min, p < 0.001) and myocardial ischemic times (98.5 vs 83 min, p = 0.021). Longer CPB time was associated with ECMO (OR 1.04, p = 0.001); the converse was true for myocardial ischemic time (OR 0.94, p = 0.029). For patients with long CPB times (>205 min), 41.9% (13/31) required ECMO. A narrative review for patients with long CPB times revealed suboptimal surgical management in 76.9% (10/13) of ECMO cases, with incorrect problem assessment leading to unnecessary revisions being most common. Conclusion: The qualitative analysis of prolonged CPB time and ECMO highlighted critical surgical decision-making, including consideration for extension of ischemic vs non-ischemic approaches to optimize surgical repair.
Background: Psychosocial functioning and quality of life (QoL) are strongly associated with outcomes in pediatric heart transplant recipients. The data in pediatric transplantation, however, is limited. This study aims to investigate the associations of perioperative anxiety and depression with postoperative complications, sociodemographic and clinical characteristics. Methods: This observational, analytical, longitudinal study included 42 pediatric participants aged 8 to 16 years old. Preoperative psychological assessments were completed by 36 children, the remaining 6 were unable to participate due to invasive ventilation, extracorporeal membrane oxygenation (ECMO), and physical debilitation. Postoperatively, all 42 subjects completed the psychosocial evaluations. Data on recipients characteristics, family characteristics and clinical parameters were collected. Anxiety and depression were assessed using the Screen for Child Anxiety Related Emotional Disorders (SCARED) and the Depression Self-Rating Scale for Children (DSRSC). The Short Form-36 Health Survey (SF-36) was applied to assess the health-related QoL. Results: Before transplantation, 91.7% (33/36) of the children exhibited symptoms of anxiety, and an identical proportion (91.7%, 33/36) showed signs of depression. After transplantation, the rates of anxiety and depression decreased to 35.7% (15/42) and 11.9% (5/42), respectively. Longer disease course (p = 0.042), preoperative hypoalbuminemia (p = 0.032), older age (p = 0.024), postoperative hypertension (p = 0.011), and postoperative CRRT (p = 0.015) result in depression symptoms. Preoperative hypoalbuminemia (p = 0.032) was also more common in the anxiety group. Additionally, children with psychosocial risks had significantly lower QoL scores in general health (p = 0.008) and mental health (p = 0.015). Conclusions: Perioperative anxiety and depression are highly prevalent among pediatric heart transplant recipients. Although significant improvements in psychosocial risks were observed at posttransplantation, approximately 40% children continued to experience psychosocial challenges.
Background: The life-course management of children with tetralogy of Fallot (TOF) has focused on demonstrating brain structural alterations, developmental trajectories, and cognition-related changes that unfold over time. Methods: We introduce an magnetic resonance imaging (MRI) dataset comprising TOF children who underwent brain MRI scanning and cross-sectional neurocognitive follow-up. The dataset includes brain three-dimensional T1-weighted imaging (3D-T1WI), three-dimensional T2-weighted imaging (3D-T2WI), and neurodevelopmental evaluations using the Wechsler Preschool and Primary Scale of Intelligence-Fourth Edition (WPPSI-IV). Results: Thirty-one children with TOF (age range: 4-33 months; 18 males) were recruited and completed corrective surgery at the Children's Hospital of Nanjing Medical University, Nanjing, China. Aiming to promote the neurodevelopmental outcomes in children with TOF, we have meticulously curated a comprehensive dataset designed to dissect the complex interplay among risk factors, neuroimaging findings, and adverse neurodevelopmental outcomes. Conclusion: This article aims to introduce our open-source dataset on neurodevelopment in children with TOF, which covers the data types, data acquisition and processing methods, the procedure for accessing the data, and related publications.
Persistent left superior vena cava (PLSVC) is a rare congenital anomaly that may complicate cardiac procedures when associated with a dilated coronary sinus (CS) and conduction disturbances. We report the case of a 27-year-old male with Wilson’s disease who presented with complete heart block. Echocardiography showed biatrial enlargement and severe CS dilation, while contrast-enhanced computed tomography (CT) confirmed PLSVC draining into the CS without a bridging vein. Anatomical constraints prevented cardiac resynchronization therapy, and dual-chamber pacemaker implantation proved technically challenging due to lead placement difficulties. This case highlights the importance of thorough preoperative assessment and individualized pacing strategies in patients with PLSVC, in order to anticipate anatomical challenges and optimize outcomes.
Background: Congenital heart disease (CHD) is the most common congenital anomaly, but whether the COVID-19 pandemic affects its prevalence is unknown. We aimed to compare the incidence of CHD during the COVID-19 pandemic with that before the pandemic in China. Methods: This multicenter retrospective observational study involved all newborns in seven representative cities of China between 01 September 2019, and 31 December 2021. All the newborns underwent pulse oximetry monitoring combined with cardiac murmur auscultation in the first 6 h to 72 h after birth for CHD screening. We defined fetuses born in and beyond September 2020 as the exposed group, and before as the non-exposed group. The incidence of CHD and specific heart abnormalities, including atrial septal defect (ASD) and ventricular septal defect (VSD), before and during the COVID-19 pandemic were compared. Results: The study included 492,662 newborns; 217,003 newborns born before September 2020 and 275,659 newborns born in and beyond September 2020. There were 3115 patients with CHD in total during the whole study period. Of those, 1055 (September 2019 to August 2020) and 2060 (September 2020 to December 2021) were less and more affected by the pandemic, respectively. There was a significant increase in the incidence of CHD in the early stage of the COVID-19 pandemic (7.78 per 1000 births) compared to that before the pandemic (4.86 per 1000 births) (p < 0.001). The birth prevalence of ASD and VSD significantly increased during the pandemic from 3.991 per 1000 births to 4.717 per 1000 births (p = 0.008) and from 1.650 per 1000 births to 3.508 per 1000 births (p < 0.001), respectively. Conclusions: The incidence of CHD increased during the COVID-19 pandemic, which was possibly related to the reallocation of medical resources, increased psychological pressure, and increased socioeconomic deprivation, though underlying mechanisms remain unclear.
Background: Cardiac implantable electronic devices (CIEDs) are essential for preventing sudden cardiac death in patients with cardiovascular diseases, but implantation procedures carry risks of complications such as infection, hematoma, and bleeding, with incidence rates of 3-4%. Previous studies have examined individual risk factors separately, but integrated predictive models are lacking. We compared the predictive performance and interpretability of artificial neural network (ANN) and logistic regression models to evaluate their respective strengths in clinical risk assessment. Methods: This retrospective study analyzed data from 180 patients who underwent cardiac implantable electronic device (CIED) implantation in Taiwan between 2017 and 2018. To address class imbalance and enhance model training, the dataset was augmented to 540 records using the Synthetic Minority Oversampling Technique (SMOTE). A total of 13 clinical risk factors were evaluated (e.g., age, body mass index (BMI), platelet count, left ventricular ejection fraction (LVEF), prothrombin time/international normalized ratio (PT/INR), hemoglobin (Hb), comorbidities, and antithrombotic use). Results: The most influential risk factors identified by the ANN model were platelet count, PT/INR, LVEF, Hb, and age. In the logistic regression analysis, reduced LVEF, lower hemoglobin levels, prolonged PT/INR, and lower BMI were significantly associated with an increased risk of complications. ANN model achieved a higher area under the curve (AUC = 0.952) compared to the logistic regression model (AUC = 0.802), indicating superior predictive performance. Additionally, the overall model quality was also higher for the ANN model (0.93) than for logistic regression (0.76). Conclusions: This study demonstrates that ANN models can effectively predict complications associated CIED procedures and identify critical preoperative risk factors. These findings support the use of ANN-based models for individualized risk stratification, enhancing procedural safety, improving patient outcomes, and potentially reducing healthcare costs associated with postoperative complications.
Background: Low cardiac output syndrome (LCOS) is a frequent and serious complication after pediatric cardiac surgery. Endothelial glycocalyx (EG) degradation, indicated by elevated syndecan-1, contributes to microvascular dysfunction and postoperative instability. The relationship between syndecan-1 dynamics and surgical risk categories remains unclear. Objective: To examine the association between perioperative syndecan-1 levels and clinical outcomes across Risk Adjustment for Congenital Heart Surgery (RACHS-1) categories. Methods: We analyzed 106 children (RACHS-1 categories 2-4) undergoing elective cardiac surgery with cardiopulmonary bypass (CPB). Syndecan-1 was measured at baseline (T0), 4 h (T4), and 72 h (T72). Outcomes included LCOS, vasoactive inotropic score (VIS), Pediatric Logistic Organ Dysfunction (PELOD-2), pediatric intensive care unit (PICU) stay, and mortality. Analyses used Kruskal-Wallis, Bonferroni post hoc tests, Spearman correlation, and multivariable regression adjusted for CPB duration, cross-clamp time, and pre-PICU status. Results: Syndecan-1 differed significantly across RACHS groups at T0 (p = 0.044) and T72 (p = 0.015). RACHS score was weakly correlated but significant with syndecan-1 at T72 (r = 0.238, p = 0.019) and decline from T4-T72 (r = 0.249, p = 0.013), indicating delayed recovery at higher risk. RACHS-4 patients had the highest VIS and PELOD-2 scores and longer PICU stay. In adjusted models, RACHS-3 was associated with higher syndecan-1 at T72 ((3 = +51.9, p = 0.016), higher VIS 0-4 h ((3 = +4.9, p = 0.008), and increased LCOS risk (OR 5.99, 95% CI 1.61-25.70, p = 0.010). RACHS-4 showed greater organ dysfunction but LCOS risk was attenuated (OR 0.19 vs. RACHS-3, p = 0.035). Mortality was highest in RACHS-4 (17.6%) but not statistically significant (p = 0.368). Conclusion: Higher RACHS categories are linked with delayed EG recovery, greater vasoactive support, and more severe organ dysfunction. Syndecan-1 kinetics at 72 h, alongside VIS and LCOS, may serve as adjunctive markers for postoperative risk stratification in pediatric cardiac surgery.
