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.

Background Double outlet right ventricle (DORV) is a complex congenital heart disease with significant anatomical variations. Patients with atrioventricular discordance have traditionally been managed with univentricular palliation strategies. However, advances in surgical concepts and techniques have enabled biventricular or one-and-a-half ventricle repair in selected patients, even after prior palliation. Case presentation We report a case of a 32-year-old woman with DORV and atrioventricular discordance who successfully underwent conversion to a one-and-a-half ventricle circulation using a combined Hemi-Mustard/Rastelli procedure with preservation of a pre-existing bidirectional Glenn shunt, performed 18 years after initial palliation. Conclusion This case provides insight into the surgical decision-making and technical considerations for managing similarly complex congenital anomalies.

Background Left ventricular (LV) reverse remodeling has been linked to long‐term prognosis and life quality of pediatric patients with LV outflow tract obstruction. However, a lack of suitable animal model limits further study of LV reverse remodeling in young hearts. This study reports on the development and mechanism exploration of an animal model mimicking pediatric LV reverse remodeling. Methods A reversible neonatal ascending aorta constriction mice model using absorbable suture was established to simulate pediatric LV reverse remodeling. Cardiac hypertrophy, myocardial fibrosis, angiogenesis, exercise tolerance, and myocardial reserve throughout the reverse remodeling process were evaluated. Multiple‐time points RNA sequencing identified key genes and pathways involved. Cardiomyocyte‐specific stimulator of interferon genes (STING) knockout mice were generated for mechanism study. Results In the reversible neonatal ascending aorta constriction model, hydrolysis of the absorbable suture reduced cardiac afterload after ventricular remodeling, initiating reverse remodeling at 4 weeks postsurgery. Reverse remodeling young hearts displayed improved function, resolution of fibrosis, but persistent hypertrophy, reduced exercise capacity, and myocardial reserve. Multiple‐time points RNA sequencing and in vivo experiments revealed a gradual upregulation of mitophagy during reverse remodeling, along with suppression of the mitochondrial DNA (mtDNA)‐cyclic GMP‐AMP synthase (cGAS) in which the STING gene is flanked by loxP sites‐STING pathway. Cardiomyocyte‐specific STING knockout enhanced early reverse remodeling. The early application of urolithin A promoted mitophagy and suppressed the mtDNA‐cGAS‐STING pathway, accelerating reverse remodeling. Conclusions This study introduces a novel model of pediatric LV reverse remodeling in male mice, delineating the trajectory and transcriptional footprints during the reverse remodeling process. The Mitophagy‐mtDNA‐cGAS‐STING axis plays a vital role and holds therapeutic potential to promote young heart recovery.

The optimal reoperation strategy and long-term outcomes of pediatric patients with congenital aortic stenosis (AS) have not been well elucidated. This study aimed to evaluate the reintervention outcomes and long-term prognosis in patients with isolated AS following their initial aortic valve (AoV) repair. A retrospective analysis was conducted on the clinical data of all patients with isolated AS who underwent initial AoV repair between 2013 and 2024. The primary outcome was the rate of freedom from reoperation after the initial procedure and secondary surgeries. A total of 203 patients who underwent initial AoV repair were included. The median age at initial surgery was 2.4 (0.6, 4.7) years. The 30-day mortality rate was 0.5

Chronic heart failure (HF) remains a global health challenge due to the lack of therapies that effectively disrupt the pathological fibro-inflammatory networks driving disease progression. While current nanomedicine strategies often target intracellular pathways in isolated cell types, they overlook the multicellular crosstalk central to HF. Here, we develop scalably synthesized Prussian blue (PB) nanoparticles that selectively intercept the CCL2-CCR2 chemokine axis, a key pathway in fibroblast-macrophage communication. Single-nucleus RNA sequencing of murine and human failing hearts identifies a conserved pro-fibroinflammatory cardiac fibroblast subpopulation (POSTNhi CCL2hi) that recruits CCR2+ macrophages via CCL2 secretion. PB nanoparticles exhibit ultrahigh affinity (KD = 1.11 × 10-10 m) for free CCL2, inducing conformational distortion in its N-terminal domain via specific C≡N interface interactions with CRS1 residues, thereby blocking CCR2 engagement, a mechanism distinct from conventional nanomaterials. Although ineffective in monocultures, PB nanoparticles robustly improve cardiac function and remodeling in murine and translational porcine pressure-overload HF models, reducing left ventricular end-diastolic volume by 56.2% and fibrosis by 40.5%, while selectively depleting CCR2+ macrophages without systemic immunosuppression. Supported by scalable production (> 100 g/batch), long-term stability, and biosafety, this work establishes a cell communication-targeting nanomedicine strategy for network-driven diseases like HF.

Mechanical sensation has become a recent focus and key pathological driver of cardiac fibrosis. However, the crosstalk between major mechanosensitive cell and fibroblasts remains to be elucidated. In this study, single-cell temporal atlas of mechanosensitive ion channels is depicted in transverse aortic constriction male mouse heart. Piezo1 is the most abundant mechanosensitive ion channel and exhibits the highest expression in epicardial cells. Epicardial-specific Piezo1 knockout mice exhibit decreased fibrosis and cardiac dysfunction. Piezo1 mediates epithelial-mesenchymal transition only contributes to subepicardial fibrosis. Further functional experiments and human samples validation reveal that Piezo1 facilitates intraventricular fibroblast activation via the Chemerin-Cmklr1 paracrine signaling pathway. The activation of cardiac fibroblasts is mediated by Pi3k-Akt1-Pou3f1 pathway. The Cmklr1 inhibitor α-NETA effectively mitigates myocardial fibrosis and dysfunction, holding therapeutical potential. Collectively, this study untangles the global landscape of mechanosensitive channels and the spatiotemporal mechanism of epicardial cells to activate fibroblast via the Chemerin-Cmklr1 paracrine signaling pathway.

Background Right ventricle (RV) decompression for pulmonary atresia with intact ventricular septum (PA/IVS) could be achieved surgically or percutaneously, yet transcatheter valvotomy is not widely accessible in remote regions and classic on-pump pulmonary valvotomy procedure carries risks of by pass in neonatal patients. We aim to summarize our 15-year's surgical experience treating PA/IVS with on-pump valvulotomy, off-pump valvulotomy and hybrid techniques. Methods From January 2010 to March 2025, 104 PA/IVS children who underwent off-pump, on-pump pulmonary valvulotomy or hybrid therapy in our hospital were retrospectively reviewed, including off-pump group (n = 56), on-pump group (n = 34) and hybrid group (n = 14). The postoperative mortality, operation time, early and mid-term follow-up of the three groups were analyzed. Results Both the off-pump and hybrid groups exhibited significantly shorter operative times, reduced vasoactive drug support, shorter intubation times, and shorter intensive care unit (ICU) and postoperative hospital stays compared to the on-pump group (P < 0.001). Cerebral, intestinal and renal oxygen monitor suggested that no obvious hypoxia was caused by clamping in off-pump procedure (P > 0.05). At final follow-up, RV hypoplasia, tricuspid Z score and pulmonary regurgitation were not significant among groups (P > 0.05). However, the hybrid group demonstrated a significantly higher rate of restenosis. Conclusions The off-pump pulmonary valvotomy is an effective, safe and technical-friendly operation. It provides superior early outcomes compared to on-pump surgery and a lower restenosis rate relative to hybrid techniques, highlighting its value for broader adoption across medical centers.

INTRODUCTION:Cartilage defects (CDs) are orthopedic conditions with limited regenerative potential. This study aimed to identify endoplasmic reticulum (ER) stress-related biomarkers and construct a diagnostic model to enhance the early detection of CD. METHODS:This study analyzed the transcriptomic dataset GSE129147 to identify ER stressrelated differentially expressed genes (ERSRDEGs) between CD and control tissues using the limma package (version 3.58.1). Kyoto Encyclopedia of Genes and Genomes (KEGG) and Gene Ontology (GO) analyses were employed for functional enrichment. Immune infiltration was assessed using cell-type identification, which involved estimating the relative subsets of RNA transcripts and single-sample gene set enrichment analysis. Diagnostic models were constructed using logistic regression, support vector machine, and least absolute shrinkage and selection operator regression. RESULTS:Twenty ERSRDEGs were identified, with CYBB, ATP6V1A, and TNFRSF12A significantly upregulated in CD samples. GO and KEGG analyses highlighted oxidative stress response and extracellular matrix remodeling as key mechanisms in CD pathogenesis. Immune analysis revealed an increase in regulatory T cells and a reduction in CD8⁺ T cells. TNFRSF12A showed strong immune associations and, together with TWIST1 and ATP6V1A, formed the final preliminary diagnostic model. The preliminary LASSO model achieved satisfactory predictive accuracy (AUC: 0.7-0.9). DISCUSSION:These findings suggest that ER stress and immune imbalance jointly contribute to cartilage degeneration. The identified genes, particularly TNFRSF12A, TWIST1, and ATP6V1A, not only serve as potential biomarkers but also provide preliminary evidence for new mechanistic insights into stress-immune crosstalk in CD. CONCLUSION:This study reveals the key roles of ER stress and immune dysregulation in CDs. Moreover, the ERSRDEG-based diagnostic model provides preliminary bioinformatics evidence and potential molecular indicators for targeted diagnostics and therapies.

An anomalous aortic origin of the coronary artery (AAOCA) is a rare congenital heart disease. Some high-risk anatomical structures are at risk of inducing cardiogenic shock or even sudden death. This article summarizes our surgical experience with AAOCA in paediatric patients. We retrospectively analysed the clinical data of 27 paediatric AAOCA patients admitted to the Department of Cardiothoracic Surgery in our hospital from July 2015 to June 2023 and summarized the surgical treatment experience and follow-up results. A total of 27 patients were included in this study, including 14 patients with an anomalous left coronary artery (ALCA) and 13 patients with an anomalous right coronary artery (ARCA). A comparison of clinical data between ALCA and ARCA patients revealed that the preoperative left ventricular ejection fraction (LVEF) in ALCA patients was significantly lower than that in ARCA patients (p < 0.05). There were significantly more patients with preoperative complications, such as major adverse cardiovascular events (MACEs) and mitral regurgitation (MR), in the ALCA group than in the ARCA group (p < 0.05). No postoperative adverse events such as severe bleeding, mediastinitis, central nervous system complications, the need for reoperation, pacemaker implantation, pleural effusion complications occurred after operation.The duration of follow-up was 58.5 (31.5, 77.3) months. During the follow-up period, none of the patients presented symptoms such as chest tightness or chest pain, and cardiac CTA revealed unobstructed coronary arteries. Compared with the preoperative LVEF, the LVEF significantly improved at the last follow-up (p < 0.05). Patients with an AAOCA should be taken seriously, and surgical treatment should be considered for these patients. Surgery should be considered for patients with ALCA as well as patients with ARCA with symptoms of myocardial ischaemia or a positive diagnosis of myocardial ischaemia or ventricular arrhythmia. For patients with other congenital heart defects that require surgical treatment, if the AAOCA is a high-risk anatomical structure, simultaneous surgery should be considered. The surgical method should be tailored to the coronary artery anatomy of the individual patient.

Tracheal replacement is a promising approach for treating tracheal defects that are caused by conditions such as stenosis, trauma, or tumors. However, slow postoperative epithelial regeneration often leads to complications, such as infection and granulation tissue formation. Ferroptosis, which is an iron-dependent form of regulated cell death, limits the proliferation of tracheal basal cells (TBCs), which are essential for the epithelialization of tissue-engineered tracheas (TETs). This study explored the potential of ferrostatin-1 (FER-1), which is a ferroptosis inhibitor, to increase TBC proliferation and accelerate the epithelialization of 3D-printed TETs. TBCs were isolated from rabbit bronchial mucosal tissues and cultured in vitro. Ferroptosis was induced in TBCs at passage 2, as shown by increased reactive oxygen species (ROS) levels, Fe2⁺ accumulation, decreased ATP contents, and mitochondrial damage. TBCs were treated with FER-1 (1 μM) for 48 h to inhibit ferroptosis. The effects on ROS levels, Fe2⁺ levels, ATP contents, and mitochondrial morphology were measured. For in vivo experiments, FER-1-treated TBCs were seeded onto 3D-printed polycaprolactone (PCL) scaffolds, which were implanted into rabbits with tracheal injury. Epithelial regeneration and granulation tissue formation were evaluated 6 months after surgery. FER-1 treatment significantly reduced ferroptosis marker levels in vitro; that is, FER-1 treatment decreased ROS and Fe2⁺ accumulation, ameliorated mitochondrial structures, and increased ATP levels. TBC proliferation and viability were increased after ferroptosis inhibition. In vivo, the group that received 3D-printed scaffolds seeded with TBCs exhibited accelerated TET epithelialization and reduced granulation tissue formation compared with the control groups. These results suggest that inhibiting ferroptosis with FER-1 improves TBC function, leading to more efficient tracheal repair. Ferrostatin-1 effectively inhibits ferroptosis in tracheal basal cells, promoting their viability and proliferation. This results in faster epithelialization of tissue-engineered tracheas, offering a promising strategy for improving tracheal reconstruction outcomes and reducing complications such as infection and granulation tissue formation. Future studies are needed to further investigate the molecular mechanisms underlying ferroptosis in TBCs and its potential clinical applications.

Cardiac fibrosis under chronic pressure overload is an end-stage adverse remodeling of heart. However, current heart failure treatments barely focus on anti-fibrosis and the effects are limited. We aimed to seek for a cardiac abundant and cardiac fibrosis specific piRNA, exploring its underlying mechanism and therapeutic potential. Whole transcriptome sequencing and the following verification experiments identified a highly upregulated piRNA (piRNA-000691) in transverse aortic constriction (TAC) mice, TAC pig, and heart failure human samples, which was abundant in heart and specifically expressed in cardiac fibroblasts. CFRPi was gradually increased along with the progression of heart failure, which was illustrated to promote cardiac fibrosis by gain- and loss-of-function experiments in vitro and in vivo. Knockdown of CFRPi in mice alleviated cardiac fibrosis, reversed decline of systolic and diastolic functions from TAC 6 weeks to 8 weeks. Mechanistically, CFRPi inhibited APLN, a protective peptide that increased in early response and became exhausted at late stage. Knockdown of APLN in vitro notably aggravated cardiac fibroblasts activation and proliferation. In vitro and in vivo evidence both indicated Pi3k-AKT-mTOR as the downstream effector pathway of CFRPi-APLN interaction. Collectively, we here identified CFPPi as a heart abundant and cardiac fibrosis specific piRNA. Targeting CFRPi resulted in a sustainable increase of APLN and showed promising therapeutical prospect to alleviate fibrosis, rescue late-stage cardiac dysfunction, and prevent heart failure.

ObjectiveThis study aims to assess the early to mid-term clinical efficacy of expanded polytetrafluoroethylene (ePTFE) trileaflet valved conduits in pediatric right ventricular outflow tract reconstruction for congenital heart disease.MethodsWe conducted a retrospective analysis of pediatric patients who underwent right ventricular outflow tract (RVOT) reconstruction using ePTFE trileaflet valved conduits at two cardiac centers in China, between January 2017 and June 2023. The main assessment criterion was the functionality of the prosthetic pulmonary valve conduit.ResultsA total of 162 pediatric patients were included, with follow-up periods ranging from 0.1 to 5 years post-discharge, and a median follow-up duration of 1 year (interquartile range: 1, 2). Three patients (1.9%) required re-operation due to conduit obstruction. During follow-up, pulmonary valve flow velocities were recorded as <3 m/s in 134 patients (82.7%), between 3 and 4 m/s in 24 patients (14.8%), and >4 m/s in 4 patient (2.5%). Mild pulmonary valve regurgitation was noted in 148 patients (91.4%), and moderate pulmonary valve regurgitation was noted in 14 patients (8.6%), with no instances of more than moderate pulmonary valve regurgitation.ConclusionThe ePTFE trileaflet valved conduit, known for its accessibility and simplicity in manufacturing, demonstrates favorable early to mid-term clinical outcomes in pediatric RVOT reconstruction.

肺静脉再狭窄是肺静脉畸形术后常见并发症，需行再次手术干预。基于解剖修补术(anatomic-based repair)是由波士顿儿童医院报道治疗肺静脉异位引流和肺静脉狭窄的手术方法，目前国内尚无应用先例。本文报道应用基于解剖修补术成功治疗1例部分性肺静脉异位引流术后右肺静脉再狭窄的16个月女患儿，术后效果良好。

Objective:To apply different strategies of pulmonary artery (PA) plasty to seek an optimal treatment for Tetralogy of Fallot (TOF) with unbalanced PA branches.Methods:From January 2006 to December 2017, retrospective review was conducted for 18 consecutive surviving TOF infants with imbalanced PA branches.Regular follow-ups were performed.The relevant clinical data were processed with software SPSS 19.0.Results:Among them, PA plasty was primary (n=14) and secondary (n=4). After an initial PA plasty, ratio of balanced PA branches was much higher in NATIVE group (100%) than that in PATCH & EXTENSION group (100% vs 0, P<0.05); After secondary PA plasty, only 25% of them attained a decent development and balanced PA braches, 28% with patch enlargement had PA branch atresia. Conclusions:For TOF with imbalanced PA branches, leaving HPA in a native state without patch enlargement may be a better strategy.

Objective:To summarize the outcomes of neurologic development disorders after surgery with selective cerebral perfusion (SCP) in children of aortic arch malformations (including coarctation of aorta & interruption of aortic arch).Methods:From May 2019 to June 2021, the relevant clinical data were retrospectively reviewed for 58 children operated for aortic arch malformation.According to whether or not SCP was applied, they were assigned into two groups of SCP (n=22) and circulatory arrest (n=11). Basic profiles of age, gender, weight, clinical manifestations, hospitalization stay, intraoperative bypass duration, aortic occlusion duration, postoperative blood levels of lactate & procalcitonin and postoperative complications were retrospectively reviewed.Thirty-three of them underwent cranial magnetic resonance imaging (MRI). Griffiths mental development assessment score (GDS-C) was assessed at 1 year postoperatively.It was expressed as developmental quotient (DQ). DQ values between two groups were compared by independent t-test.Results:There were 34 boys and 24 girls with an age range of (1-2 052) day.They were followed up for 1 to 3 years after discharge and weighting from 2.5 to 21.4 kg.Postoperative lactate value was (2.41±1.08) mmol/l and (3.19±1.22) mmol/l in SCP and circulatory arrest groups respectively ( P<0.05). GDS-C test results indicated that average DQ scores of hand-eye coordination energy area was (94.51±14.53) and (81.95±18.16) in SCP and circulatory arrest groups respectively ( P<0.05). However, scores of DQ in sports, personal-social, hearing and language areas were higher in SCP group than those in circulatory arrest group.The differences were not statistically significant.The percentage of individuals with DQ ≥70 in hearing and language areas was equal in two groups.However, percentage ratio in other areas was higher in SCP group than that in circulatory arrest group. Conclusions:Mild hypothermia with SCP may be applied with fewer neurological complications.As a feasible, safe and effective cerebral protection measure, SCP provides effective cerebral perfusion and reduces the risk of central nervous system complications due to cerebral ischemia of traditional arch surgery in children.

The second edition of the expert consensus on pediatric nutrition was formed based on a global update of pediatric nutrition guidelines or consensus worldwide, the management of congenital heart disease, and the results of multi-center clinical nutrition research for congenital heart disease following the first Chinese consensus edition of 2016. The consensus was also shaped by the results of three discussion sessions and two questionnaires conducted by the 13-member collaboration group. This process was informed by both clinical guidelines and expert consensus. The quality of literature, both in English and Chinese, and the level of recommendations were evaluated using the Grading of Recommendations Assessment, Development, and Evaluations (GRADE) system.

Objective:To analyze the coronary angiographic (CAG) characteristics of coronary artery lesion (CAL) in children with Kawasaki disease (KD), and to clarify the necessity of CAG in the diagnosis and treatment of KD combined with CAL in children.Methods:It was a retrospective study to analyze the clinical data, electrocardiogram, echocardiography, time and findings of CAG in children with KD and CAL who underwent CAG in Shanghai Children′s Medical Center of Shanghai Jiao Tong University School of Medicine from January 2013 to August 2022.The distribution, type, severity, and prognosis of CAL were analyzed.Results:A total of 117 children with KD and CAL were included in the analysis.The onset age of KD was from 2 months to 12.8 years old, and the age of performing CAG was from 8 months to 18.1 years old.A total of 234 coronary artery lesions were detected in 117 cases.Among them, CAL in the right coronary artery (RCA), left anterior descending branch (LAD), left main coronary artery and left circumflex artery were detected in 96 branches(41.1%), 78 branches(33.3%), 44 branches(18.8%), and 16 branches(6.8%), respectively.Unilateral coronary artery involvement was detected in 43 cases (36.8%), of which LAD was the dominant; while bilateral involvement was detected in 74 cases (63.2%), among which, LAD and RCA were the most involved arteries.Stratified by the degree of coronary involvement, large coronary aneurysms and severe coronary stenosis were most frequently occurred in the RCA and LAD.In contrast, 10 cases (13.6%), 20 cases (24.3%), 55 cases (45.8%) and 37 cases (67.3%) of intraluminal lesions were found in small, medium and large coronary aneurysms, and stenosis or occlusion, respectively.The incidence of intraluminal lesions tended to be higher in the site of severe lesions.CAG showed stenosis or occlusion in a total of 55 cases, and collateral circulation at varying degrees was found in cases of severe stenosis or occlusion.Conclusions:CAL in children with KD are complex and varied.Although clinical symptoms, routine electrocardiogram and cardiac ultrasound may indicate severe CAL.Their applications are limited by the diagnosis of the type (especially stenosis), degree, and extent of CAL, as well as the detection of extracoronary lesions.CAG is of great significance to identify vascular lesions and guide clinical management of KD combined with CAL in children.