Imbalance of immune homeostasis, particularly the dysregulation of pro-inflammatory/anti-inflammatory balance, is a core pathological mechanism in many acute and chronic pediatric diseases. Traditional single inflammatory biomarkers have limitations in disease prediction, clinical evaluation, and prognostic stratification, as they cannot reflect the overall dynamic balance of the immune network. This systematic review aimed to evaluate the predictive value of multi‑dimensional inflammatory biomarker ratios centered on pro‑inflammatory/anti‑inflammatory balance in pediatric inflammatory diseases, and to clarify their classification and clinical application strategies. We performed this systematic review in accordance with the PRISMA guidelines. We systematically searched PubMed, Embase, Cochrane Library, Web of Science, China National Knowledge Infrastructure (CNKI), Wanfang Data Knowledge Service Platform, and VIP Chinese Science and Technology Journal Database databases from database inception to December 31, 2025. Eligible studies were original studies that focused on multi-dimensional inflammatory biomarker ratios in children aged ≤18 years. We excluded animal studies, studies involving only adult populations, low-quality studies, conference abstracts, case reports, and irrelevant correspondence. Two independent reviewers conducted literature screening, data extraction, and methodological quality assessment using standard tools, with 32 articles finally included in this systematic review. We categorized multi-dimensional inflammatory biomarker ratios into two classes: Class A [cytokine-based ratios, e.g., interleukin (IL)-6/IL-10, tumor necrosis factor-α (TNF-α)/IL-10, IL-17A/IL-10, IL-1β/IL-1 receptor antagonist (IL-1Ra)], which have high specificity for reflecting molecular immune balance; and Class B (systemic inflammatory ratios derived from routine laboratory tests, e.g., neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, Systemic Immune-Inflammation Index, C-reactive protein-to-albumin ratio), which have high accessibility in clinical practice for rapid screening. Compared with single inflammatory biomarkers, these multi-dimensional ratios demonstrated superior predictive performance in disease prediction, severity assessment, and prognosis of pediatric sepsis, Kawasaki disease, juvenile idiopathic arthritis, and pediatric multisystem inflammatory syndrome. Multi-dimensional inflammatory biomarker ratios, centered on the pro-inflammatory/anti-inflammatory balance, overcome the limitations of single inflammatory markers. In clinical practice, Class A ratios facilitate precise immune subtyping and targeted therapy, while Class B ratios facilitate early screening and severity stratification of pediatric inflammatory diseases. The combined application of both classes can establish a stratified and precise diagnosis and treatment framework for pediatric inflammatory diseases. Future research should establish pediatric reference ranges for these ratios, conduct large-scale prospective validation studies, and integrate artificial intelligence and point-of-care testing (POCT) technologies to promote the advancement of individualized pediatric care.
To develop an online pediatric electrocardiogram (ECG) educational intervention, d to examine pediatricians' diagnostic skill development as they progressed to achieve a performance-based standard, and to determine the frequency of pediatric ECG findings at highest risk for diagnostic error. This multicenter, prospective cohort study included a convenience sample of pediatricians. There were 400 cases in the intervention, and for each case, participants first determined whether an actionable abnormality was present or absent. If present, participants categorized abnormalities as rate/rhythm, anatomical/technical, or Q-wave/repolarization, and selected the most appropriate specific diagnosis from a drop-down list of options. Immediate feedback was provided after each case, and practice continued until a minimal passing standard was achieved. A total of 345 pediatricians performed 46,649 pediatric ECG case interpretations. Initial accuracy was 82.1% in identifying ECG with actionable findings, 70.9% for correct categorization of actionable findings, and 45.2% for selecting most actionable specific diagnosis. There were learning gains for each of these diagnostic tasks: +14.6% (95% CI 13.1, 15.8), +15.0% (95% CI 13.2, 16.8), and +19.8% (95% CI 17.5, 22.1), respectively. Furthermore, 65% achieved the minimal passing standard in a median of 325 cases (IQR 198, 496) or 6.5 hours (IQR 3.4, 10.0) of practice. Among the 46,649 case interpretations, 7,675 (16.5%) were incorrect interpretations. ECG findings consistent with ventricular hypertrophy, Brugada sign, prolonged QTc, and ischemia/pericarditis were among the most challenging diagnoses. Structured practice with feedback can feasibly improve pediatrician ECG interpretation skills and derive data to identify ECG findings prone to diagnostic error.
Pediatric cardiomyopathy is a leading cause of heart failure and sudden cardiac death in children, posing a severe threat to their health and lives while imposing a heavy burden on families and society. It has become a significant public health challenge. The aim of this retrospective study is to systematically review global research articles on pediatric cardiomyopathy genetics, revealing its knowledge structure and evolutionary trajectory. Bibliometric methods and natural language processing techniques were jointly applied to analyze research articles on pediatric cardiomyopathy genetics from the Web of Science Core Collection (WOSCC) and PubMed databases. CiteSpace software was utilized to construct national collaboration networks and co-occurrence/evolution maps of keywords, while BERTopic modeling was employed for topic modeling of article abstracts. The macro-structure and micro-semantics of pediatric cardiomyopathy genetics research were systematically investigated, and finally a multi-dimensional knowledge map was constructed. Over the past 25 years, research teams from 71 countries and regions have published 1,438 articles, demonstrating fluctuating growth in publishing activity. The United States, China, and the United Kingdom are core publishing nations, with the U.S. occupying a central position in publication volume, total citations, and international collaboration networks. This study identified five core themes in pediatric cardiomyopathy genetics, including diverse disease classification and diagnostic/therapeutic mechanisms, systematically revealing the field's research trajectory toward intelligent and precision-oriented transformation. Based on keyword timeline analysis and emergence analysis, the research evolution progressed through three phases: single-gene screening to genomic sequencing (early 2000s-2010), multi-omics integration (early 2010s-2020), and precision medicine with dynamic monitoring (early 2020s-2024). This study further demonstrates that genetic research in pediatric cardiomyopathy is increasingly integrated into digital healthcare, rapidly advancing toward intelligent and precise diagnosis and treatment. The integration of multi-omics data and artificial intelligence supports personalized risk assessment, dynamic monitoring, and early warning, thereby driving the transformation toward data-driven pediatric cardiovascular health management.
Sarcopenia is a prevalent musculoskeletal complication after pediatric cancer, and its association with health-related quality of life (HRQoL) remains unknown. This study aimed to investigate health-related quality of life (HRQoL) differences according to sarcopenia status in young pediatric cancer survivors, and determined if these differences were distinct by sex. This cross-sectional study included 116 pediatric cancer survivors (12.1 ± 3.3 years old). Muscle strength was evaluated by handgrip strength test, while appendicular lean mass index (ALMI, kg/m2) was estimated via dual‑energy X‑ray absorptiometry. "Sarcopenia probable" was defined when muscle strength was ≤ decile 2 and ALMI Z-score was > -1.5 standard deviation (SD). "Sarcopenia confirmed" was defined when muscle strength was ≤ decile 2 and ALMI Z-score ≤ -1.5 SD. HRQoL was assessed using the Spanish version of the PedsQL 4.0 Generic Core Scales, and compared with age-specific reference values of healthy young population to calculate Z-scores. Only female survivors without sarcopenia had significantly better total HRQoL (-0.3 [95% CI: -0.8 to 0.2] vs. 1.5 [95% CI: -2.1 to -0.8]), physical HRQoL (-0.3 [95% CI: -0.8 to 0.2] vs. -1.6 [95% CI: -2.3 to -0.9]), psychosocial HRQoL (-0.2 [95% CI: -0.7 to 0.3] vs. -1.2 [95% CI: -1.8 to -0.6]) and school HRQoL (-0.1 [95% CI: -0.6 to 0.4] vs. -1.3 [95% CI: -2.0 to -0.7]) than female survivors with sarcopenia confirmed. Most HRQoL domains are similar across sarcopenia status in young pediatric cancer survivors. However, female survivors without sarcopenia seem to have better HRQoL Z-score than those with sarcopenia.
Elevated heart rate is associated with adverse outcomes in patients with heart failure. Heart transplant recipients often have elevated heart rate; however, the long-term associations of elevated heart rate with clinical outcomes are unknown. The records of all pediatric (≤21 years) heart transplant recipients at our institution were reviewed. Patients who died or lacked a clinic visit within 1 year following heart transplant were excluded, as were those with a permanent pacemaker. Heart rate was collected from outpatient electrocardiograms and converted to Z-scores using age-specific normative data. The cohort was divided into elevated and normal heart rate groups, with elevated heart rate defined as an average HR Z-score ≥95th percentile during the first post-transplant year. Outcomes, including survival, rejection, and cardiac allograft vasculopathy were compared between groups. A total of 110 pediatric heart transplants were included (median age 11.4 years; 62% male). Thirty-six (33%) had an elevated heart rate. The elevated heart rate group was older (15.8 vs 6.1 years, p<0.001) and had shorter follow-up (3.0 vs 4.7 years, p = 0.007). Elevated heart rate was independently associated with increased risk of death (HR 5.44, 95% CI 1.47-20.2, p = 0.011) and a composite of death or rejection (HR 3.06, 95% CI 1.30-7.20, p = 0.010), but not cardiac allograft vasculopathy. Among patients who survive the first year after pediatric heart transplantation, elevated heart rate during the first post-transplant year is independently associated with increased risk of mortality and rejection. Further work is necessary to determine whether pharmacologic modulation mitigates this risk.
To evaluate the association between ventilation strategy and catheter-tissue interaction, lesion characteristics, and procedural metrics during pediatric supraventricular tachycardia ablation. Prospective observational study. Tertiary pediatric electrophysiology center. A total of 133 consecutive patients aged < 18 lt; 18 years who underwent cardiac radiofrequency (RF) ablation between June and September 2025 were included. Patients were grouped according to ventilation strategy: high-frequency low-tidal volume (HFLTV) ventilation (n = 65), standard ventilation (SV) (n = 32), and controlled apnea (n = 36). No interventions were performed. This was a prospective observational study in which ventilation strategy was determined by the attending anesthesiologist according to routine clinical practice and individual clinical judgment. The investigators had no role in selecting or modifying the ventilation approach. For analytical purposes, ventilation modes applied during routine care were classified into 3 categories: HFLTV ventilation, standard volume-controlled ventilation, and intermittent controlled apnea. The primary variable was mean contact force (MCF). Secondary outcomes included impedance metrics, operator-rated procedural quality score (PQS, scale of 1-10), number of lesions, RF application time, and total procedure duration. Baseline demographic and arrhythmia characteristics were similar among groups. HFLTV ventilation yielded significantly higher MCF (median, 7 g) compared with SV (median, 4 g; p < 0.001) and apnea (median, 5 g; p < 0.001), as well as higher PQS (8.1 ± 1.0) than SV (7.0 ± 2.0, p < 0.001) and apnea (7.1 ± 1.0, p < 0.001). Minimum and mean impedance values were lower in the HFLTV group, suggesting more favorable catheter-tissue interaction. RF time and overall procedure duration were similar across the groups. No ventilation-related complications were observed. This prospective observational study found that different ventilation strategies influenced catheter-tissue interaction and procedural metrics during pediatric RF ablation. Notably, HFLTV ventilation was linked to higher contact force metrics. Additional research is necessary to verify these results.
To evaluate the long-term remodeling, biological adaptation, and clinical outcomes of expanded polytetrafluoroethylene (ePTFE) artificial chords (AC) used for pediatric mitral valve (MV) repair through comprehensive echocardiographic analysis. We retrospectively reviewed all patients < 18 years undergoing MV repair with ePTFE AC between 2005 and 2024. Serial echocardiographic measurements included AC length, MV coaptation geometry, regurgitation severity and stenosis, and left ventricular (LV) volumes. Forty-two patients (median operative age 5 years, interquartile [IQR] 2-12) underwent MV with AC. Median follow-up was 4.0 years (95% CI: 2.5-7.0). Median AC number was 4 (IQR 2-6). Progressive AC elongation was observed over time, with median diastolic AC length from 1.78 cm (IQR, 1.38-2.07) at discharge to 2.28 cm (IQR, 1.88-2.70) at 10 years, and median systolic length from 1.40 cm (IQR, 1.10-1.58) to 2.10 cm (IQR, 1.60-2.50). AC elongation correlated with LV volumes (end-diastolic and end-systolic, p=0.002 and p=0.018), suggesting coupling with ventricular growth. Transmitral gradients remained stable, no association between AC length and reduced coaptation length or increased tenting height were observed, supporting absence of leaflet restriction. No AC rupture or calcification occurred. At 10 years, cumulative incidence of ≥moderate MR was 45.5% (95%CI: 30.5%, 68.0%), reoperation was 20.1% (95% CI 10.8-37.4), and reprolapse with ≥moderate MR was 12.1% (95%CI: 5.3%, 27.6%). In pediatric MV repair, ePTFE-AC demonstrated excellent durability and adaptative elongation alongside LV remodeling, without inducing valve restriction. These findings support long-term safety of AC-based repair and highlight ventricular-valvular remodeling in the growing heart.
In adults with hypertrophic cardiomyopathy (HCM), left ventricular outflow tract (LVOT) obstruction on exercise stress echocardiography (ESE) and reduced left atrial (LA) strain increase heart failure and cardiac outcome risk. However, limited pediatric data exist. We evaluated the association of exercise-induced LVOT obstruction and LA strain with cardiac events in young HCM patients. Patients who underwent ESE (n = 114, 32%F, median = 17 yrs) from 2014-2022 at a single center were stratified by LVOT gradients: Group 1 (n = 44, rest/exercise gradients < 30mmHg); Group 2 (n = 41, rest < 30mmHg; exercise ≥ 30mmHg); Group 3 (n = 29, rest/exercise ≥ 30mmHg). LA reservoir (LASr), conduit (LAScd), and contractile strain were analyzed with TOMTEC AutoStrain LA. Composite cardiac event included: heart failure, non-sustained/sustained ventricular tachycardia, cardiac syncope, heart transplantation, and HCM-related death. At follow-up (median = 2.0 years), there was a greater hazard of the composite event in Group 3 vs. 2 (hazard-ratio [HR] = 4.97, p = 0.013). Severe events were rare and largely observed in Group 3. Increased HR was seen in Group 2 vs. 1 but the difference was not significant. Adjusting for LVOT gradient (Group 3), lower LASr or LAScd or increased LA stiffness (E/e' to LASr ratio) were associated with composite event (HR LASr = 1.57; LAScd = 1.70; LA stiffness = 1.31; all p < 0.01). Concordance indices were similar for these three strain/stiffness models (0.81-0.82) and higher than in models including LVOT group with traditional diastolic indices (LA volume index or E/e'; c-index = 0.76). In one of the largest pediatric HCM cohorts undergoing ESE, reduced LA strain demonstrated greater incremental value to LVOT gradient than traditional diastolic indices when evaluating association with cardiac events.
Cardiac magnetic resonance (CMR) is a key modality for diagnosing and monitoring myocarditis, including cases related to COVID-19 infection and Multisystem Inflammatory Syndrome in Children (MIS-C). While acute findings are often favorable, the persistence of abnormalities over time remains uncertain. This retrospective study included 21 pediatric patients with suspected COVID-19-related myocarditis between March 2020 and July 2023. All underwent CMR as part of clinical evaluation. Imaging findings were analyzed at early (< 30 days), mid (30-364 days), and long-term (≥ 365 days) intervals. Myocarditis was defined using the 2009 Lake Louise criteria. Of the 21 patients (mean age 13.1 years, 57% male), 8 had follow-up CMRs (median time to follow-up 347 days). Etiologies included MIS-C (57%), confirmed COVID-19 (19%), and suspected COVID-19 (24%). A total of 30 CMR studies were analyzed. Initial CMR met 2009 Lake Louise criteria in 8 patients (38%). Across temporally categorized studies, LGE declined from 46% of early studies to 25% of mid-term studies and was absent on long-term studies; pericardial effusions similarly declined from 46% to 25% to 0%. EGE remained frequent across timepoints (77%, 83%, and 80%, respectively). Among patients with serial CMR, no patient had edema on follow-up, LGE persisted in 1 of 8 (13%), pericardial effusion in 1 of 8 (13%), and only 1 of 8 (13%) continued to meet Lake Louise criteria. Biventricular systolic function remained normal on follow-up imaging. Initial LGE demonstrated marked lateral wall predominance (Cochran's Q = 13.38, p = 0.004). Median clinical follow-up was 20 months (IQR 11-38), with no documented arrhythmias, heart failure symptoms, cardiac-related rehospitalizations, or mortality. In pediatric myocarditis associated with COVID-19, most CMR abnormalities improve over time, with resolution of edema and substantial reduction in LGE and pericardial effusion. Persistent EGE remains common, although its clinical significance is uncertain. Medium-term clinical outcomes were favorable. Larger prospective studies are needed to define the long-term significance of persistent imaging abnormalities.
Mitochondrial cardiomyopathy (MCM) is a heterogeneous group of disorders characterized by abnormal myocardial structure and/or function caused by defects in genes encoding the oxidative phosphorylation chain. This review systematically summarizes molecular genetic advances regarding nuclear gene mutations associated with pediatric MCM, focusing on mutations affecting pathways including respiratory chain complex subunits and assembly factors, coenzyme Q10 biosynthesis, mitochondrial DNA maintenance and expression, lipid metabolism, iron-sulfur cluster metabolism, apoptosis regulation, and mitochondrial dynamics. These nuclear gene mutations contribute to myocardial pathological changes by disrupting key processes such as mitochondrial energy metabolism, membrane stability, and signal transduction. The review provides a theoretical basis for precise clinical diagnosis and the exploration of potential molecular targets in pediatric MCM. 线粒体心肌病（mitochondrial cardiomyopathy, MCM）是一组由编码氧化磷酸化链的基因缺陷导致心肌结构和/或功能异常的异质性疾病。该文系统综述儿童MCM相关核基因突变的分子遗传学研究进展，重点探讨影响呼吸链复合物亚基及组装因子、辅酶Q10合成、线粒体DNA维持与表达、脂质代谢、铁硫簇代谢、细胞凋亡调控及线粒体动力学等通路的核基因突变。上述核基因突变可通过干扰线粒体能量代谢、膜稳定性及信号传导等关键过程，导致心肌病理改变。该文通过总结核基因突变在儿童MCM发病机制中的作用，为临床精准诊断与潜在分子靶点的研究提供理论依据。.
Chronic thromboembolic pulmonary hypertension (CTEPH) is rare in children and published experience with pulmonary endarterectomy (PEA) is limited. We retrospectively reviewed six patients (<18 years) who underwent seven PEA procedures at our center between December 2011 and September 2025. Clinical characteristics, perioperative findings, hemodynamic outcomes, and follow-up data were analyzed. Median age was 13 years (range, 2-17), and five patients were female. Five had a history of pulmonary embolism, while one presented with hydatid cyst. Risk factors for CTEPH were hydrocephalus with ventriculoperitoneal shunt (n=2), hydatid cyst disease (n=1), infective endocarditis with left ventricular outflow tract obstruction (n=1), and chronic kidney disease with prior COVID-19 infection (n=1). Preoperative mean systolic pulmonary artery pressure (PAP) was 56 ± 23 mmHg, mean pulmonary artery pressure (mPAP) was 39 ± 15 mmHg, and mean pulmonary vascular resistance index (PVRi) was 7.51 ± 4.56 U·m². Most patients were in World Health Organization (WHO) functional class III or IV. All patients underwent PEA, with concomitant procedures required in most cases. Postoperatively, mPAP decreased to 21 ± 4 mmHg and mean PVRi to 2.36 ± 1.36 U·m². Median intensive care unit (ICU) and hospital stays were 3.5 and 9 days, respectively. There was one in-hospital mortality, all other patients recovered without major complications. PEA is feasible and effective in carefully selected pediatric patients, including those with complex comorbidities. Significant hemodynamic and functional improvements were achieved, supporting the role of PEA as a potentially curative therapy in this rare and challenging group.
Survival among children with central nervous system (CNS) tumors has improved markedly. However, long-term cardiac effects of craniospinal irradiation (CSI) remain poorly defined. Emerging data suggest an elevated risk for subclinical systolic dysfunction despite the absence of anthracycline exposure. This study aimed to evaluate longitudinal echocardiographic measures of systolic and diastolic function following CSI and identify predictors of cardiac dysfunction. We conducted a retrospective, multi-institutional cohort study of pediatric patients diagnosed with a primary CNS tumor treated with CSI between January 2000 and September 2024. Echocardiographic assessments included M-mode left ventricular ejection fraction (LVEF), LV shortening fraction (LVSF), speckle-tracking global longitudinal strain (GLS), and Doppler-based diastolic indices. Longitudinal changes were modeled using linear mixed-effects models adjusted for demographic, treatment, cardiovascular, and endocrine variables. Among 129 survivors (median age at diagnosis 8 years [IQR: 5-11]; at evaluation 23 years [IQR: 18-27]; median mean heart radiation dose 1,217 cGy [IQR: 2.340-3.600]), LVEF and LVSF declined progressively (P = 0.006 and P < 0.001). LVEF remained ≥50% in all patients, LVSF was <28% in 14 individuals. Diastolic parameters remained stable. GLS was assessed in 100 patients showing early post-CSI improvement followed by progressive decline (P < 0.001), 5 patients exhibited GLS <16%. Older age at diagnosis was associated with greater decline in LVEF and LVSF, while endocrine comorbidities correlated with lower LVEF as attained age increased. GLS trajectories differed by sex, with lower values in younger females. Progressive long-term decline in systolic function was observed in CSI-treated CNS tumor survivors despite modest cardiac radiation exposure. Age, sex, and endocrine comorbidities influence cardiac trajectories, supporting longitudinal cardiac surveillance.
Extracorporeal membrane oxygenation (ECMO) offers lifesaving support for severe cardiac or respiratory failure patients, yet thrombotic complications within the circuit remain a major challenge. Although design improvements to individual ECMO components have provided some benefits, the overall influence of full-circuit design on hemodynamics and thrombotic risk remains poorly understood. We investigated whole-circuit ECMO hemodynamics and their implications for hemolysis and thrombus formation using computational fluid dynamics (CFD). We developed a full ECMO-CFD model of our center's pediatric support circuit and validated it against in vitro measurements using blood-analog fluid circulated under clinically relevant operating conditions. The model was then used to assess the cumulative impact of circuit components and junctions on hemodynamics, and we further extended the analysis to evaluate the effect of non-Newtonian blood behavior on local flow dynamics. Flow analysis revealed largely uniform flow in the ECMO tubing, with localized disturbances near connectors and internal loops that could propagate downstream and increase thrombosis risk. While tubing and connectors experienced shear stresses below 10 Pa, ~ 0.18% of pump volume experienced 50-100 Pa, and 0.0035% exceeded 100 Pa at 1500 RPM. At 4000 RPM, these values increased ~ 23-fold, resulting in a ~ 52-fold rise in the hemolysis index, demonstrating substantial pump-driven blood trauma. Incorporating non-Newtonian effects further revealed shear and hemolysis hotspots near the pump that were overlooked under Newtonian assumptions. Our findings underscore the importance of the whole ECMO circuit in optimizing flow dynamics and reducing blood trauma, highlighting the need to consider the entire system to better predict and mitigate thrombotic complications.
Pediatric patients undergoing cardiac catheterization are at risk of major adverse events, including cardiac arrest and the need for extracorporeal membrane oxygenation support. The primary aim of this study was to characterize the use of extracorporeal cardiopulmonary resuscitation (eCPR) and its outcomes in a large pediatric cardiac catheterization laboratory (PCCL). The secondary aims were to identify key clinical and procedural risk factors associated with eCPR deployment and to assess the utility of novel risk scoring metrics in predicting the need for eCPR. Between January 1, 2000 and December 31, 2023, eCPR occurred in 39 of 29,480 (0.13%) cases in the PCCL, with a statistically significant decrease over time. Most eCPR events occurred during interventional cases and were procedure-related. Extracorporeal cardiopulmonary resuscitation was not limited to those patients with elevated patient- or procedure-specific risk scores. Survival to discharge was 61.5%. Neurological complications were present in 46.2%. Further work will need to concentrate on better understanding which patients are at risk of eCPR in the PCCL and on refining risk scoring metrics to better capture that risk. Enhanced predictive risk modeling has the potential to reduce morbidity and mortality in the pediatric congenital heart disease population.
Renal involvement is a key determinant of prognosis in IgA vasculitis (IgAV), yet its underlying immunologic mechanisms remain incompletely elucidated. The role of γδ T cells and their subsets requires clarification. The study enrolled 88 children between September 1, 2023, and September 1, 2025, categorized into three groups: IgAV with nephritis (IgAVN, n = 39), IgAV without nephritis (IgAVwoN, n = 36), and healthy controls (HC, n = 13). Flow cytometry was used to assess the percentage of peripheral blood γδ T cells, their surface phenotype (CD69, NKG2D, TLR4), and intracellular cytokines (IL-17 A, IL-10, TNF-α). Multivariable logistic regression identified independent risk factors, receiver operating characteristic curve analysis evaluated diagnostic performance, and correlation with 24-hour proteinuria was assessed. Patients with IgAVN exhibited a significantly increased frequency of total γδ T cells and an expanded CD69⁺ activated subset. TLR4 expression on γδ T cells was upregulated in IgAV patients compared to HC group, but did not differ significantly between the IgAVN and IgAVwoN groups. The IL-17 A producing γδ T cell subset was identified as an independent risk factor for IgAVN (OR = 1.202, 95% CI 1.014-1.424, P = 0.034). Its frequency positively correlated with 24-hour proteinuria levels (r = 0.575, P < 0.01). This parameter demonstrated high specificity (97.22%) and moderate diagnostic accuracy (AUC = 0.673) for distinguishing IgAVN. IL-17 A⁺ γδ T cells were an independent risk factor for IgAVN and correlated with renal injury severity. While Th17 cells are the main IL-17 source, γδ T cells may represent an additional source. Further investigation is needed.
Pulmonary arterial hypertension (PAH), a life-threatening disorder in children, necessitates palliative interventions like Potts shunt (surgical/transcatheter) for medically refractory cases. Perioperative anesthesia management remains challenging due to hemodynamic instability. This study describes our institutional anesthetic approach and summarizes the perioperative outcomes of 28 pediatric patients who underwent Potts shunt creation. A retrospective analysis was conducted on 28 consecutive children who underwent either surgical or transcatheter Potts shunt at Shanghai Children's Medical Center between 2021 and 2024. Perioperative data were extracted from structured electronic medical records. Among the 28 patients, 18 underwent surgical Potts shunt creation (100% requiring cardiopulmonary bypass [CPB], median CPB duration: 91[82-108] minutes) and 10 underwent transcatheter procedures (20% requiring CPB). Anesthesia induction predominantly utilized ciprofol-alfentanil (60.7%) and s-ketamine (28.6%). Preoperative treprostinil was universal, with 7.1% of patients requiring additional vasoactive agents. Post-shunt hemodynamic support included norepinephrine (92.9%), dopamine (28.6%) and dobutamine (28.6%). Compared with the transcatheter group, surgical patients experienced significantly longer stays in the cardiac intensive care unit (CICU), (8[7-14] vs. 5[4-9] days, p = 0.005), longer durations of mechanical ventilation (2.5[1.5-10.5] vs. 1.8[1.0-4.3] days), and longer total hospitalizations (31[23-36] vs. 25.5[19-46] days). No intraoperative cardiac arrests occurred. The in-hospital mortality rate was 14.3% (4/28). Both surgical and transcatheter reverse Potts shunt procedures represent feasible salvage therapies for children with refractory pulmonary hypertension, albeit with considerable perioperative risks. Targeted hemodynamic regulation and rigorous intraoperative emergency management are therefore essential to maintain circulatory stability, prevent pulmonary hypertensive crises, and ensure overall clinical safety.
暂无摘要（点击查看详情）
Limited data describe children with pulmonary hypertension (PH) supported on extracorporeal membrane oxygenation (ECMO). This retrospective study (2017-2023), including children less than 18 years with PH at a quaternary institution, identified associations with 1) mortality in ECMO-supported patients, 2) extracorporeal cardiopulmonary resuscitation (ECPR), and 3) inpatient death without ECMO (DWE). Among 113 children with PH supported on ECMO, the median age was 31 days (interquartile range [IQR]: 1-332), mortality was 41%, with 19% requiring ECPR. Lung disease was the most common PH-subgroup (52%). In multivariable models including pre-cannulation factors, ECPR (adjusted odds ratios [aOR]: 4.9 [1.4-17.2]) and pulmonary vein stenosis (PVS) (aOR: 7.5 [1.1-51.4]) were associated with mortality. After adding post-cannulation factors, ECPR (aOR: 9.41 [2.21-40.12]), PVS (aOR: 8.07 [1.05-61.93]), and longer ECMO duration (aOR: 3.21 [1.41-9.90]) were independently associated with mortality. Extracorporeal cardiopulmonary resuscitation was associated with cardiac intensive care unit (CICU) admission (OR: 11.7 [3.2-42.5]), single-ventricle physiology (OR: 6.6 [2.3-18.5]), and left heart disease PH-subgroup (OR: 8.9 [3.1-25.6]). Compared to ECMO-supported patients, DWE patients (n = 75) were older and more likely to have a tracheostomy, had lower pulmonary artery pressures, received fewer PH medications, and differed in PH-subgroup. While ECPR and PVS confer increased mortality risk in children with PH requiring ECMO, survival remains achievable. Incorporating patient-level data into prognostic frameworks may support individualized approaches to ECMO decision-making in this cohort.
Increasing case volume has been positively associated with procedural outcomes in many fields, but little is known about this relationship for pediatric catheter ablation. The objective of the study was to report associations between center and operator case volumes and acute success in pediatric ablations. A multicenter retrospective cohort study of patients undergoing ablation at Improving Pediatric And Adult Congenital Treatment registry sites from April 2016-March 2020 was performed evaluating the association between center or operator annual case volume (low, medium, high, or very high) and the likelihood of acute ablation success. Unadjusted comparisons and multivariable logistic regression was completed with subgroup analyses to evaluate associations in higher risk subgroups. Of 18,666 ablations at 74 hospitals, acute success was reported in 17,204 (92%). High/very high-volume centers showed marginal but statistically insignificant increases in the rates of acute success when compared to medium/low centers (92% vs 91%; P = 0.24). In multivariable models high volume centers saw increased odds of success in patients with congenital heart disease (OR: 2.3; CI: 1.21-4.4), and patients with premature ventricular contractions/ventricular tachycardia (OR: 2.2; CI: 1.23-4.1). Among 156 operators, the highest rates of acute success were seen in very high-volume operators (94%) compared to low (92.4%), medium (91.9%), and high (92.2%) operators (P = 0.015). Multivariable modeling showed no association between operator volume and acute success. Pediatric catheter ablation acute success was high across a range of center and operator volumes. Complex substrates have increased odds of success at high volume centers, and high and very high-volume centers have consistent rates of success despite increasing case complexity.