Xuming Mo

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.

Objective: This study aims to evaluate the neurodevelopmental outcomes in children with congenital heart disease (CHD) using magnetic resonance imaging (MRI), and to assess the role of brain volume metrics as predictors of neurodevelopmental abnormalities. Methods: In this retrospective cohort study, 160 children with CHD treated at Children’s Hospital of Nanjing Medical University from January 2020 to December 2023 were analyzed. Patients were classified into normal (DQ ≥ 70, n=106) and abnormal neurodevelopment (DQ < 70, n=54) groups based on Developmental Quotient (DQ) scores. MRI scans were used to measure total brain volume, cortical gray matter, deep gray matter, white matter, and cerebrospinal fluid volumes. Neurodevelopmental assessments focused on adaptive behavior, motor skills, language, and personal-social behavior. ROC analysis was performed to determine the predictive value of brain volume metrics for neurodevelopmental abnormalities. Results: Total brain volume in the normal group (341.82 ± 10.43 mL) was significantly higher than in the abnormal group (323.92 ± 10.24 mL) (P < 0.05). Cortical gray matter volume in the normal group (131.47 ± 4.02 mL) was also significantly greater than in the abnormal group (121.63 ± 6.91 mL) (P < 0.05). No significant differences were observed in white matter, deep gray matter, or cerebrospinal fluid volumes. Children in the abnormal group scored significantly lower in all developmental domains (P < 0.05). ROC analysis showed that total brain volume (AUC = 0.968) and cortical gray matter volume (AUC = 0.936) were strong predictors of neurodevelopmental abnormalities (P < 0.001). Conclusion: Total brain volume and cortical gray matter volume, as measured by MRI, are effective predictors of neurodevelopmental abnormalities in children with CHD and can serve as valuable tools for early neurodevelopmental assessment.

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.

Complex pediatric cardiac disease is associated with brain impairment and neurodevelopmental disorders, particularly in patients requiring cardiac surgery for aortic arch anomalies. This study examines the incidence, risk factors, and outcomes of perioperative brain injury in children undergoing aortic arch repair who had aortic arch anomalies. A total of 145 children with aortic arch anomalies in our center undergoing aortic arch repair between January 2014 and December 2022 were enrolled. There were 129 (89.0%) with coarctation of the aorta (COA) and 16 (9.7%) with interrupted aortic arch (IAA). Risk factor analysis of brain injuries was done using perioperative imaging and included symptoms of hemorrhagic stroke, arterial ischemic stroke, white matter injury, cerebral sinus venous thrombosis, and other pathologies. Preoperatively, 50/145 (34.5%) patients had brain injuries. Multivariate analysis showed that an increased risk of hemorrhagic stroke was associated with newborns (odds ratio [OR], 2.09 [95% CI 0.08–3.50]), isolated COA (OR, 3.69 [95% CI 1.23–7.07]), mechanical ventilation (MV) ([OR, 2.56 [95% CI 1.25–4.03]), and sepsis (OR, 1.73 [95% CI 0.46–3.22]). Newborns ([OR, 1.91 [95% Cl 0.58–3.29]) and weight-for-age z score ([OR, −0.45 [95% CI −0.88 to −0.1]) were associated with an increased risk of white matter injury. New postoperative brain injuries were present in 12.9% of the patients (16/124). Deep hypothermic circulatory arrest (DHCA) was associated with new postoperative brain injuries compared with deep hypothermic low-flow (DHLF) plus antegrade cerebral perfusion (ACP) (([OR, 2.67 [95% CI, 0.58–5.75])). Isolated COA was almost associated with new postoperative brain injuries (OR, 1.13 [95% CI, −0.04 to 2.32]). Children diagnosed with isolated COA appeared to have a higher risk of perioperative brain injury, but the underlying mechanisms are still unclear. We focused on the intrinsic mechanism by which changes in hemodynamics caused by COA result in perioperative brain injury. Further research will be needed to optimize the personalized treatment and cerebral perfusion techniques for complex pediatric cardiac surgery.

Background: Fulminant myocarditis (FM) is an inflammatory process of the myocardium and an important cause of cardiac dysfunction in children; it is characterized by rapid onset, acute progression, and high mortality. The study sought to describe the clinical characteristics and prognostic factors in children with FM.Methods: The study population consists of 37 consecutive patients admitted from May 2014 to December 2021 with a diagnosis of FM. According to the prognosis of children with FM during hospitalization, they were divided into “survival” group (25 cases) and “death” group (12 cases). A multivariate logistic regression analysis was performed to identify the independent predictors of in-hospital mortality in the patients, and receiver operating characteristic (ROC) curve was used to explore the predictive value of related factors.Results: The 37 children with FM had an average age of 8.35 ± 4.36 years old. Twenty-five of the patients survived and 12 died. Twenty-five of the children were discharged from the hospital after a series of active rescue treatments such as nutritional myocardial drugs, high-dose intravenous immunoglobulin (IVIG), glucocorticoids (GCs), temporary pacemaker (TP), extracorporeal membrane oxygenation (ECMO), and continuous renal replacement therapy (CRRT).Twelve of the children were classified into the death group because the resuscitation failed. The levels of procalcitonin (PCT), creatine kinase (CK), and myoglobin (MYO) in the death group were all higher than in the survival group (all P < 0.05), and the left ventricular ejection fraction (LVEF) in the death group was significantly lower than in the survival group (P = 0.002). The binary logistic regression analysis revealed that MYO [OR:1.006; 95%CI:(1–1.012); P = 0.045] and LVEF [OR: 0.876; 95% CI: (0.785–0.978); P = 0.019] were independent predictors of FM. ROC curve analysis showed that the area under ROC curve (AUC) of MYO and LVEF was [AUC:0.957; 95%CI:0.897~1] and [AUC:0.836; 95%CI:0.668~1], and the area under the combined ROC curve for MYO + LVEF was significantly higher than that for MYO or LVEF alone (P < 0.05), indicating that the MYO + LVEF combined diagnosis had a higher predictive value for FM.Conclusion: The levels of MYO and LVEF can be markers for prognosis of FM and can effectively evaluate the disease severity. Their combination can improve forecast accuracy; thus, the detection of the above-mentioned indexes possesses a higher value for clinical applications.

Background: Inflammatory myofibroblastic tumor (IMFT) is a rare neoplasm mainly affecting children and young adults. We conducted a retrospective study to evaluate the clinical features and treatment alternatives of childhood inflammatory myofibroblastic tumors.Methods: A total of 19 patients who were pathologically diagnosed with IMT between December 2008 and October 2018 were included. Collected data were demographic information, main complaints, tumor characteristics, treatment, pathological results, immunohistochemical analysis, and prognosis.Results: The male/female ratio was 13:6. The mean age at disease onset was 44.9 ± 33.9 months (range 4 to 111 months). The mean tumor size was 6.5 ± 4.0 cm (range 1.2 to 17.0 cm). The most common site was the abdomen (13/19). The most commonly used detection tool was CT. Eleven patients (57.9%) had aggressive tumor growth, including eight receiving extensive resection and three receiving palliative resection due to high local invasiveness and postoperative chemotherapy. Eight cases whose tumors were completely enveloped received complete resection. Immunohistochemistry was performed for 17 patients and ALK positivity was found in 11 patients. Despite three children lost to follow-up, sixteen patients were followed up for 6 to 132 months (average 63.9 months, median 66 months). Of which, twelve children survived with no evidence of IMT, and four cases (21%) showed local recurrences (two of them died). No distant metastasis was detected.Conclusions: IMT is rare in children with various locations, mostly appearing in the abdomen. Whether the tumor could be completely removed, the location and the invasiveness of surrounding tissues might be highly prognosis-related.

Apparent diffusion coefficients (ADC) can provide phenotypic information of brain lesions, which can aid the diagnosis of brain alterations in neonates with congenital heart diseases (CHDs). However, the corresponding clinical significance of quantitative descriptors of brain tissue remains to be elucidated. By using ADC metrics and texture features, this study aimed to investigate the diagnostic value of single-slice and multi-slice measurements for assessing brain alterations in neonates with CHDs. ADC images were acquired from 60 neonates with echocardiographically confirmed non-cyanotic CHDs and 22 healthy controls (HCs) treated at Children's Hospital of Nanjing Medical University from 2012 to 2016. ADC metrics and texture features for both single and multiple slices of the whole brain were extracted and analyzed to the gestational age. The diagnostic performance of ADC metrics for CHDs was evaluated by using analysis of covariance and receiver operating characteristic. For both the CHD and HC groups, ADC metrics were inversely correlated with the gestational age in single and multi-slice measurements (P < 0.05). Histogram metrics were significant for identifying CHDs (P < 0.05), while textural features were insignificant. Multi-slice ADC (P < 0.01) exhibited greater diagnostic performance for CHDs than single-slice ADC (P < 0.05). These findings indicate that radiomic analysis based on ADC metrics can objectively provide more quantitative information regarding brain development in neonates with CHDs. ADC metrics for the whole brain may be more clinically significant in identifying atypical brain development in these patients. Of note, these results suggest that multi-slice ADC can achieve better diagnostic performance for CHD than single-slice.

Ischemia/reperfusion-derived myocardial dysfunction is a common clinical scenario in patients after cardiac surgery. In particular, the sensitivity of cardiomyocytes to ischemic injury is higher than that of other cell populations. At present, hypothermia affords considerable protection against an expected ischemic insult. However, investigations into complex hypothermia-induced molecular changes remain limited. Therefore, it is essential to identify a culture condition similar to in vivo conditions that can induce damage similar to that observed in the clinical condition in a reproducible manner. To mimic ischemia-like conditions in vitro, the cells in these models were treated by oxygen/glucose deprivation (OGD). In addition, we applied a standard time-temperature protocol used during cardiac surgery. Furthermore, we propose an approach to use a simple but comprehensive method for the quantitative analysis of myocardial injury. Apoptosis and expression levels of apoptosis-associated proteins were assessed by flow cytometry and using an ELISA kit. In this model, we tested a hypothesis regarding the effects of different temperature conditions on cardiomyocyte apoptosis in vitro. The reliability of this model depends on strict temperature control, controllable experimental procedures, and stable experimental results. Additionally, this model can be used to study the molecular mechanism of hypothermic cardioprotection, which may have important implications for the development of complementary therapies for use with hypothermia.

In children with tetralogy of Fallot (TOF), there is a risk of brain injury even if intracardiac deformities are corrected. This population follow-up study aimed to identify the correlation between cerebral morphology changes and cognition in postoperative school-aged children with TOF. Resting-state functional magnetic resonance imaging (rs-fMRI) and the Wechsler Intelligence Scale for Children–Chinese revised edition (WISC-CR) were used to assess the difference between children with TOF and healthy children (HCs). Multiple linear regression showed that the TOF group had a lower verbal intelligence quotient (VIQ, 95.000 ± 13.433, p = 0.001) than the HC group and that VIQ had significant positive correlations with the cortical thickness of both the left precuneus (p < 0.05) and the right caudal middle frontal gyrus (p < 0.05) after adjustment for preoperative SpO2, preoperative systolic blood pressure (SBP), preoperative diastolic blood pressure (DBP) and time of aortic override (AO). Our results suggested that brain injury induced by TOF would exert lasting effects on cortical and cognitive development at least to school age. This study provides direct evidence of the relationship between cortical thickness and VIQ and of the need for strengthened verbal training in school-aged TOF patients after corrective surgery.