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.

(1) Background: Exercise intolerance is a nearly ubiquitous consequence among patients who have undergone the total cavopulmonary connection (TCPC) or modified Fontan procedure; however, the specific factors influencing this condition-especially the impact of surgical timing and perioperative management-remain inadequately understood. (2) Methods: From a retrospective cohort of 255 TCPC patients, 99 who had undergone cardiopulmonary exercise testing (CPET) were included. Patients were stratified into a normal exercise tolerance group (ET group, peak VO2 ≥ 80% predicted, n = 45) and an exercise intolerance group (EI group, peak VO2 < 80% predicted, n = 54). Univariate and multivariate logistic regression analyses were performed to identify risk factors for decreased exercise tolerance. (3) Results: Patients in EI group were older at TCPC surgery (4.67 vs. 3.60 years, p = 0.004) and CPET (10.11 vs. 8.65 years, p = 0.01). They had longer operation time (312.14 vs. 267.53 min, p = 0.01), bypass time (122.26 vs. 103.59 min, p = 0.045), and higher rate of prolonged ICU stay (>3 days, 68.9% vs. 31.1%, p = 0.01). ROC analysis determined optimal surgical age cut-off at 3.81 years (AUC = 0.692, p = 0.001). Univariate analysis identified age ≥ 3.8 years, ICU stay > 3 days, longer operation/bypass times, and postoperative atrioventricular valve regurgitation as significant risk factors. Multivariate analysis confirmed age at TCPC ≥3.8 years (OR = 2.54, 95% CI: 1.01-6.42, p = 0.049) and ICU stay >3 days (OR = 2.61, 95% CI: 1.07-6.39, p = 0.04) as independent predictors of exercise intolerance. (4) Conclusions: The delayed completion (beyond 3.8 years of age) of TCPC procedure and the occurrence of early postoperative complications are associated with reduced long-term exercise capacity.

IntroductionAortic coarctation (CoA) is a prevalent congenital heart defect defined by focal aortic narrowing and progressive fibrosis, associated with substantial long-term cardiovascular morbidity. Although surgical repair improves early survival, the molecular and epigenetic mechanisms governing CoA pathogenesis remain poorly understood. N6-methyladenosine (m6A), the most abundant internal mRNA modification, regulates post-transcriptional gene expression during cardiovascular development and disease. In this study, we investigated whether dysregulated m6A epitranscriptome contributes to infantile CoA.MethodsGlobal m6A abundance was measured in stenotic aortic tissues and paired normal aortic segments from infants with CoA using dot blot and colorimetric assays. Transcriptome-wide m6A landscapes were profiled by methylated RNA immunoprecipitation sequencing (MeRIP-seq), and mRNA expression patterns were determined by RNA sequencing (RNA-seq). Quantitative real-time PCR (qRT-PCR) was used to validate key genes and m6A regulatory factors. To establish causal regulatory relationships, we further performed FTO loss-of-function knockdown experiments in human aortic smooth muscle cells (HASMCs).ResultsWe found that global m6A levels are significantly elevated in CoA tissues, with widespread hypermethylation events enriched in focal adhesion and extracellular matrix remodeling pathways. Integrated multi-omics analysis identified FTO (alpha-ketoglutarate dependent dioxygenase) downregulation as the primary driver of m6A hypermethylation in CoA. Mechanistically, reduced FTO expression increases m6A methylation of phosphatase and tensin homolog (PTEN) and G protein-coupled receptor kinase 5 (GRK5), resulting in their transcriptional repression in stenotic aortic tissue. In HASMCs, silencing FTO directly elevated m6A modification and suppressed PTEN and GRK5 expression. Functionally, diminished PTEN expression promotes aortic smooth muscle fibrosis and luminal narrowing.ConclusionOur findings uncover a previously unrecognized FTO/m6A/PTEN epitranscriptomic axis that governs infantile CoA pathogenesis, providing novel epigenetic targets for therapeutic intervention.

Single-cell and single-nucleus RNA sequencing (scRNA-seq and snRNA-seq) have transformed cardiovascular biology by resolving cellular heterogeneity and disease-specific cell states. The interpretive power of these technologies, however, hinges critically on accurate cell-type annotation, the assignment of biologically meaningful labels to clusters or individual cells. Mis-annotation risks systematic bias in mechanistic inference, obscures rare populations, and undermines cross-study reproducibility. This Review systematically integrates current conceptual frameworks, computational methodologies, and domain-specific best practices for cell-type annotation in cardiovascular single-cell research. We first examine the biological principles that underpin annotation, including the definition of cell types, the distinction between cell states and transitional phenotypes, considerations of granularity, and ontology mapping. We then delineate comprehensive analytical workflows spanning preprocessing and quality control (empty droplet filtering, doublet detection, normalization, and batch correction), dimensionality reduction (PCA, UMAP, t-SNE), and graph-based clustering. Turning to annotation strategies, we survey manual marker-based curation with canonical cardiovascular markers, reference-correlation methods (SingleR), supervised machine-learning classifiers (CellTypist, SingleCellNet, Garnett, scBERT), reference-mapping and label-transfer platforms (Azimuth, scArches, Symphony), and hybrid probabilistic frameworks (scANVI). We also summarize the software ecosystems of R/Seurat and Python/Scanpy/scvi-tools. Benchmarking evidence indicates that annotation accuracy depends more on reference data quality than on algorithmic sophistication. SingleR, CellTypist, and Azimuth have emerged as leading performers, whereas ensemble consensus approaches that integrate two or more independent methods enhance robustness. We further highlight cardiovascular-specific challenges, including modality-dependent differences in cardiomyocyte representation between scRNA-seq and snRNA-seq, vascular and stromal heterogeneity, immune cell tissue adaptation, disease-induced transitional cell states, and barriers to cross-species translation. Finally, we present a ten-step best-practice workflow, a comprehensive reporting and reproducibility checklist, and illustrative case studies drawn from the Adult Human Heart Atlas and CardioAtlas. We offer practical recommendations and discuss future directions encompassing multimodal integration, spatial transcriptomics, and AI-assisted annotation, all aimed at ensuring reproducible and interpretable annotations across laboratories [1-5].

Background This study analyzed the utility of the pulmonary artery flow study (PAFS) in prognostic assessment for children with pulmonary atresia and ventricular septal defect (PA/VSD). Methods We reviewed 121 patients with type B or C pulmonary atresia and ventricular septal defect (PA/VSD) who underwent the PAFS between August 2016 and August 2024. Demographic, operative, and follow-up data were collected. Statistical analyses included Spearman's correlation, ROC curves, and Kaplan-Meier survival analysis. Results The cohort included 60 males and 61 females, with 97 type B and 24 type C cases. Mean age was 44 months and median weight was 12.8 kg. Mean pulmonary artery pressure (mPAP) strongly correlated with postoperative right ventricular systolic pressure/left ventricular systolic pressure (RVSP/LVSP) ( P = 0.000). An mPAP cutoff of 24.5 mmHg predicted VSD closure with 79.2% sensitivity and 91.2% specificity. Mean pulmonary artery pressure and RVSP/LVSP were associated with prolonged ventilation and critical care unit stay. During follow-up, 15/121 children (12.4%) died, with significantly poorer survival in those with mPAP > 25 mmHg ( P = 0.009). Conclusions The pulmonary artery flow study is a valuable predictor of successful VSD closure and postoperative outcomes. An mPAP threshold of 25 mmHg may help identify high-risk patients requiring more intensive management.

Though remaining the optimal surgical options for children aortic valve disease, Ross and Ross-Konno procedure increase the incidence of right-sided reinterventions related to the right ventricle to pulmonary artery conduit, limiting its use in infants and small children. Here we report an 18-month-old boy who underwent a modified Ross-Konno procedure without conduit implantation. The right ventricle to pulmonary artery connection was reconstructed using the free fresh autogenetic tissue, which preserved the growth potential of the neo-pulmonary. A 1-year follow-up was finished with ideal outcomes.

Myocardial ischemia-reperfusion (I/R) injury, a major complication of reperfusion therapies for acute myocardial infarction that exacerbates myocardial necrosis, impairs cardiac function, and worsens patient prognosis. Profound metabolic disturbances drive myocardial I/R injury; however, their spatial heterogeneity remains unclear. Using MALDI mass spectrometry imaging with spatial segmentation, we mapped the spatiotemporal metabolite dynamics in mouse heart tissue across multiple time points (30 min ischemia to 7 days reperfusion), aiming to identify critical metabolic pathways and regulatory targets underlying I/R injury. Glycerophospholipids and sphingolipids exhibited pronounced heterogeneity. Glycerophospholipid metabolism remained dysregulated throughout the I/R process, with long-chain phospholipids (PA, PE, PC, and PS) exhibiting a distinct distribution gradient. They were enriched in the remote zone, downregulated in the border zone, and markedly reduced in the injured zone 6 h post-reperfusion. Sphingolipid metabolism was dynamically reprogrammed, with galactosylceramide (GalCer) accumulating and digalactosylceramide (Gal2Cer) depleting in injured and border zones. Mechanistically, α-galactosidase (GLA), which hydrolyzes Gal2Cer to GalCer, was upregulated in the border zone post-I/R. Functional validation further demonstrated that both cardiomyocyte-specific GLA knockdown (via AAV-shGla) and pharmacological inhibition (using the GLA inhibitor GR181413A) effectively reduced myocardial infarct size, alleviated pathological remodeling, and improved cardiac function in I/R-injured mice. Collectively, our spatial metabolomics revealed that GLA-mediated Gal2Cer/GalCer imbalance is a critical regulator of myocardial I/R injury, and targeting GLA represents a promising therapeutic strategy.

Backgorund: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly of coronary artery anatomy, usually diagnosed in infancy, but adults may also be affected by this deformity. Objectives: The aim of this study is to examine long-term outcomes in patients with ALCAPA and analyze the relevant factors influencing postoperative outcomes. Methods: The records of patients with ALCAPA admitted from January 2015 to December 2024 were retrospectively reviewed. Clinical data of the patients were retrieved from the records. The follow-up data included mortality rates and complications. Kaplan-Meier survival curves were used to analyze the occurrence of death and reoperation during the follow-up of patients with ALCAPA, and the log-rank test was used for comparisons between groups. A Cox regression model was used to analyze the risk factors for adverse events in patients with clinical indicators of an unfavorable prognosis. Results: 48 patients met the study criteria. Postoperative death or reoperation was defined as an unfavorable prognosis. Patients were divided into two groups: favorable prognosis (n = 41) and unfavorable prognosis (n = 7). The differences among the Kaplan-Meier survival curves for the different subgroups were statistically significant. Feeding difficulties (p < 0.001), preoperative left ventricular end-diastolic diameter (LVEDD) Z score (p = 0.049), preoperative mechanical ventilation (p < 0.001), preoperative vasoactive drugs (p < 0.001), ECMO (p < 0.001), and delayed sternal closure (p < 0.001) were associated with an unfavorable prognosis. Multivariate Cox proportional hazard analysis revealed that preoperative LVEF (HR: 0.711; 95% CI: 0.542-0.932; p = 0.013), CPB time (HR: 1.021; 95% CI: 1.003-1.04; p = 0.022) and length of stay (HR: 1.064; 95% CI: 1.015-1.116; p = 0.01) were associated with an unfavorable prognosis in patients, and age at surgery (HR: 1.092; 95% CI: 0.991-1.203; p = 0.077) was closely related to an unfavorable prognosis. Conclusions: A younger surgical age, a reduced preoperative LVEF, a longer CPB time, and a longer length of stay were associated with an unfavorable prognosis in this cohort.

Abstract Ultra-fast-track extubation (UF) improves postoperative recovery in cardiac surgery, but its cerebral effects remain unclear. This study compared UF and conventional extubation (CE) in children with congenital heart disease (CHD) after cardiopulmonary bypass (CPB), focusing on electroencephalographic (EEG) abnormalities during the initial 48 postoperative hours. Of 352 CHD patients undergoing CPB, 57 UF and 295 CE cases were propensity score-matched (PSM) (1:2), yielding 55 PSM-UF and 89 PSM-CE subjects. Intra/postoperative EEGs were analyzed for background abnormalities (sleep-wake cycle) and epileptiform discharges (seizures, spikes/sharp waves). Clinical parameters including STS-EACTS mortality risk and CPB duration were balanced. The PSM-UF group demonstrated milder background abnormalities (P = 0.02) and lower incidence of unresolved abnormalities at 48 h (7% vs. 24%, P = 0.009). Epileptiform activity was significantly reduced (0% vs. 11% seizures, P = 0.007; P = 0.008 for spikes/sharp waves). UF patients showed superior cerebral oxygen saturation (ScO2, P < 0.0001), reduced vasopressor requirements (P < 0.0001), and shorter hospital stays (2.0 ± 1.4 vs. 6.0 ± 5.6 days, P < 0.0001) with comparable CICU stay reductions (9.6 ± 4.1 vs. 13.3 ± 8.5 days, P = 0.002). UF following pediatric cardiac surgery correlates with attenuated EEG abnormalities and enhanced early recovery, supporting its neuroprotective benefits in CHD patients.

DCRV is a crucial disease that affect children heart function，which caused by abnormal developmental processes.The effect of abnormal muscles on blood flow to the heart can be fatal. Little is known about the cellular changes in abnormal muscles during the development of disease. Here，We conducted a sn-seq of the abnormal muscles and mapping of changes in cell composition, which revealed a cell type that promotes division of human heart muscle cells and captured the key genes of the cells. Our result shown key genetic information found in a cell type that promotes the regeneration of human heart muscle cells.

BackgroundLimited study has shown whether NT-proBNP is related to the prognosis of children wth ventricular septal defect (VSD) surgery. The study was conducted to determine the predictive value of NT-proBNP on outcomes of children with VSD surgery.MethodsA total of 798 children with VSD surgery were enrolled, with NT-proBNP measured at preoperatively and 24-h postoperatively. The short- and mid-term clinical outcomes were recorded. Propensity scores (PS) was performed to acquire pre-op and post NT-proBNP 24-h PS-matched cohorts for comparisons between groups.ResultsIn the pre NT-proBNP PS-matched cohort, the higher NT pro-BNP group had longer hospitalization time and lower post-op 1-month EF value compared with low NT pro-BNP group (all P < 0.05), and there wasn't significant difference of mechanical ventilation time, cardiopulmonary bypass (CPB) time, intensive care unit (CCU) stay, and ejection fraction (EF) values of 3 month to 12 months after surgery (all P > 0.05). In the post NT-proBNP PS-matched cohort, there wasn't significant difference of mechanical ventilation time, CPB time, CCU stay, hospitalization time, and EF values of 1 month to 12 months after surgery between two groups (all P > 0.05).ConclusionsVSD children with higher pre NT-proBNP level had longer hospital stays after surgery than those with lower level. Pre NT-proBNP level had no effect on mechanical ventilation time, CPB time, ACC time and CCU stay and cardiac function after 3 months postoperatively. Post-op 24-h NT pro-BNP level wasn't associated with clinical outcomes.

The present study aimed to analyze the risk factors affecting the prognosis children with fulminant myocarditis. The medical records of all patients (n = 40) who were diagnosed with fulminant myocarditis and admitted to the Cardiac Intensive Care Unit (CICU) and Pediatric Intensive Care Unit (PICU) at the Guanzhou Women and Children's Medical Center, Guangzhou Medical University between January 2014 and December 2023 were retrospectively analyzed. Patients were divided into two groups based on their in-hospital prognosis, namely, a survival group (n = 32) and an non-survival group (n = 8). Baseline demographics, laboratory findings, electrocardiograms, echocardiograms, and treatment regimens were compared between the two groups via multifactorial analysis. The median age of patients in the survival group was 7.8 years (M[5,11.5]), and the median age in the non-survival group was 9.0 years (M[6,11.5]). Compared with those in the survival group, patients in the non survival group had significantly higher levels of extracorporeal cardiopulmonary resuscitation (ECPR) use, ventricular tachycardia/ventricular fibrillation (VT/VF), peak creatine kinase isoenzyme (CK-MB), peak N-terminal B-type natriuretic peptide precursor (NT-proBNP), serum creatinine (Scr) on admission, peak serum Scr, peak aspartate aminotransferase (AST), peak alanine aminotransferase (ALT), peak cardiac troponin I (cTnI), lactate on admission, peak lactate, and extracorporeal membrane oxygenation (ECMO) use (all p < 0.05). Binary logistic regression analysis revealed that the peak lactate level was an independent risk factor for mortality in patients with fulminant myocarditis (OR = 0.661, 95

ER stress evokes various types of cell death and myocardial dysfunction. This study aimed to discern the involvement of ferroptosis in chronic Akt activation-offered benefit, if any, against ER stress-triggered cardiac remodeling and contractile anomalies. Cardiac-selective expression of active mutant of Akt (AktOE) and wild-type (WT) mice were challenged with the ER stress instigator tunicamycin (1 mg/kg, 48 h) prior to assessment of cardiac morphology and function. Tunicamycin insult prompted cardiac remodeling (interstitial fibrosis), deranged echocardiographic (higher LVESD, dropped ejection fraction and fractional shortening), cardiomyocyte mechanical and intracellular Ca2+ features alongside mitochondrial injury (collapsed mitochondrial membrane potential and ultrastructural change), oxidative stress, compromised Akt-GSK3β signaling, ER stress (upregulated GRP78 and Gadd153), carbonyl formation, apoptosis and ferroptosis (decreased GPX4, SLC7A11). Intriguingly, tunicamycin-evoked anomalies (except GRP78 and Gadd153) were abrogated by Akt activation. Chronic Akt activation negated tunicamycin-induced downregulation of ferric flavin enzyme dihydroorotate dehydrogenase (DHODH), which catalyzes the fourth step of pyrimidine ab initio biosynthesis, and conversion of dihydroorotic acid to orotate. ER stress-induced myocardial anomalies were reversed by the newly identified PI3K activator triptolide, DHODH activator menaquinone-4 and pyrimidine booster coenzyme Q. In vitro experiment revealed that Akt activation- or triptolide-evoked beneficial responses against tunicamycin-induced cardiomyocyte anomalies were cancelled off by DHODH inhibitor BAY2402234 or ferroptosis inducer erastin. These findings support that chronic Akt activation rescues ER stress-evoked myocardial derangements through DHODH-dependent control of ferroptosis and mitochondrial homeostasis.

Objective:Various clinical indicators can increase the likelihood of early identification of fulminant myocarditis, the identification of which is important for early treatment. Method:The medical records of all patients (n = 269) who were diagnosed with acute myocarditis between January 2014 and December 2023 were retrospectively analyzed. Patients were divided into two groups: the nonfulminant myocarditis group (n = 229) and the fulminant myocarditis group (n = 40). Baseline demographics, laboratory findings, electrocardiograms, echocardiograms, and treatment regimens were compared between the two groups via multifactorial analysis. A receiver operating characteristic (ROC) curve was used to explore the predictive value of related factors. Results:The median age of patients with fulminant myocarditis was significantly greater than that in the nonfulminant group (P = 0.015). The presenting symptoms at admission varied and included fever and respiratory, digestive, and circulatory symptoms. Among them, fever and hypotension were more common in the fulminant myocarditis group (P < 0.05), and vomiting was significantly more common in the nonfulminant myocarditis group (P = 0.017). Logistic regression analysis revealed that N-terminal pro-B-type natriuretic peptide (NT-proBNP), lactate (Lac), alanine aminotransferase (ALT), cardiac troponin I (cTnI), chest distress, and hypotension were early risk factors for fulminant myocarditis. ROC curve analysis demonstrated that NT-proBNP, ALT, cTnI, and Lac can serve as predictors for the early diagnosis of fulminant myocarditis. The optimal predictive values for these markers are 2,783.5 pg/ml for NT-pro BNP, 34.5 U/L for ALT, 0.2 µg/ml for cTnI, and 3.05 mmol/L for Lac. Conclusions:This study revealed that NT-proBNP, cTnI, ALT, and Lac can serve as predictive factors for the early identification of fulminant myocarditis. These findings emphasize the importance of early identification and timely diagnosis for improving the overall prognosis of patients.

BACKGROUND:We had reported that postoperative EEG background including sleep-wake cycle (SWC) and discharge (seizures, spikes/sharp waves) abnormalities were significantly correlated with adverse early outcomes in children after cardiac surgery. We aimed to analyze the relations between these EEG abnormalities and neurodevelopmental outcomes at about 2 years after cardiac surgery. METHODS:We enrolled 121 patients undergoing cardiac surgery at 3.3 months (0.03 ~ 28 months). EEG abnormalities described above during the first postoperative 48 h were evaluated. Griffiths Mental Development Scales-Chinese was used to evaluate the quotients of overall development and 5 subscales of the child's locomotor, language, personal-social, eye-hand coordination and performance skills at 16 ~ 31 months of age. RESULTS:EEG background abnormalities occurred in 59/121 (48.8%) patients and 33 (55.9%) unrecovered to normal by 48 h. Abnormal SWC occurred in 15 (12.4%) patients and 7 (5.8%) unrecovered to normal by 48 h. EEG seizures occurred in 11 (9.1%) patients with frontal lobe seizures in 4. Spikes/sharp waves occurred in 100 (82.6%). EEG background abnormalities, number of spikes/sharp waves and frontal lobe seizures were significantly associated with neurodevelopmental impairment at about 1 ~ 2 year after surgery (Ps ≤ 0.05). CONCLUSIONS:Most parameters of EEG abnormalities were significantly associated with neurodevelopmental impairment after cardiac surgery. IMPACT:Neurodevelopmental impairment in children with congenital heart disease remain poorly understood. Previous studies had reported that either EEG seizures or background abnormalities were associated with worse neurodevelopmental outcomes. Our present study showed that all the EEG background and discharge abnormalities including EEG background, seizures and spikes/sharp waves in the early postoperative period were significantly associated with neurodevelopmental impairment at about 1 ~ 2 years after cardiac surgery. Comprehensive evaluation of early postoperative EEG may provide further insights about postoperative brain injury, its relation with neurodevelopmental impairment, and guide to improve clinical management.

Aims: Multiple genes and environmental factors are known to be involved in congenital heart disease (CHD), but epigenetic variation has received little attention. Monozygotic (MZ) twins with CHD provide a unique model for exploring this phenomenon. In order to investigate the potential role of Deoxyribonucleic Acid (DNA) methylation in CHD pathogenesis, the present study examined DNA methylation variation in MZ twins discordant for CHD, especially ventricular septal defect (VSD). Methods and Results: Using genome-wide DNA methylation pro fi les, we identi fi ed 4004 differentially methylated regions (DMRs) in 18 MZ twin pairs discordant for CHD, and 2826 genes were identi fi ed. Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) analysis revealed a list of CHD-associated pathways. To further investigate the role of DNA methylation in VSD, data from 7 pairs of MZ twins with VSD were analyzed. We identi fi ed 1614 DMRs corresponding to 1443 genes associated with arrhythmogenic right ventricular cardiomyopathy, cyclic guanosine monophosphate-protein kinase G (cGMP-PKG) signaling pathway by KEGG analysis, and cell-cell adhesion, calcium ion transmembrane transport by GO analysis. A proportion of DMR-associated genes were involved in calcium signaling pathways. The methylation changes of calcium signaling genes might be related to VSD pathogenesis. Conclusion: CHD is associated with differential DNA methylation in MZ twins. CHD may be etiologically linked to DNA methylation, and methylation of calcium signaling genes may be involved in the development of VSD.

In infants with severe bronchopulmonary dysplasia (sBPD), severe pulmonary lobar emphysema may occur as a complication, contributing to significant impairment in ventilation. Clinical management of these infants is extremely challenging and some may require lobectomy to improve ventilation. However, prior to the lobectomy, it is very difficult to assess whether the remaining lung parenchyma would be able to sustain adequate ventilation postoperatively. In addition, preoperative planning and perioperative management are also quite challenging in these patients. This paper reports the utility of selective bronchial occlusion in assessing the safety and efficacy of lobectomy in a case of sBPD complicated by severe right upper lobar emphysema. Since infants with sBPD already have poor lung development and significant lung injury, lobectomy should be viewed as a non-traditional therapy and be carried out with extreme caution. Selective bronchial occlusion test can be an effective tool in assessing the risks and benefits of lobectomy in cases with sBPD and lobar emphysema. However, given the technical difficulty, successful application of this technique requires close collaboration of an experienced interdisciplinary team.

BACKGROUND:Extracorporeal membrane oxygenation (ECMO) is critical for congenital diaphragmatic hernia (CDH), who fails to achieve adequate oxygenation with conventional management. We aim to evaluate initial experiences with ECMO support in a tertiary women and children's medical center in mainland China.METHODS:We retrospectively reviewed the establishment of ECMO for CDH Program in our center and analysis of five CDH neonates, who underwent repair during ECMO between December 2016 and December 2018.RESULTS:The first ECMO for CDH Program in our institution was established and managed by a multidisciplinary team since December 2016. An alert of ECMO was prenatally created for moderate-severe pulmonary hypoplasia. Of sixteen admissions prenatally diagnosed moderate-severe CDH, eight neonates (50%) required ECMO but five (31%) received eventually. Veno-arterial ECMO was established from 3 hours to 41 hours of age (median 20 h). All the five underwent CDH repair after a stabilization period on ECMO, which ranged from 12 h to 122 h (median 58 h). There were no clotting complications, related to coagulating during CDH repair. Bleeding was the most common complication. Cannula malposition was detected in one case. Their median ECMO duration was 437 (range 85-946) hours. Neonatal survival was 80% (4/5) and 3 survived (60%) in the first three months of life.CONCLUSIONS:Multidisciplinary teamwork, precise prenatal evaluation and skillful cannulation assist the successful beginning of ECMO for CDH. Our preliminary results would encourage other institutions, whose ECMO is not well-established.