Quantification of 3-Dimensional Confluence-Atrial Morphology in Supracardiac Total Anomalous Pulmonary Venous Connection.

Background: Pulmonary vein stenosis (PVS) continues to be a major complication after surgical repair of total anomalous pulmonary venous connection (TAPVC). Recent studies suggest that the morphology of pulmonary venous confluence and the left atrium (LA) is associated with PVS. However, there are limited data on the prognostic value of integrating quantitative confluence-atrial morphology into risk stratification.

Gut microbiota-derived cholic acid mediates neonatal brain immaturity and white matter injury under chronic hypoxia.

Chronic hypoxia, common in neonates, disrupts gut microbiota balance, which is crucial for brain development. This study utilized cyanotic congenital heart disease (CCHD) patients and a neonatal hypoxic rat model to explore the association. Both hypoxic rats and CCHD infants exhibited brain immaturity, white matter injury (WMI), brain inflammation, and motor/learning deficits.

Surgical repair of neonatal total anomalous pulmonary venous connection: A single institutional experience with 241 cases.

Objective: Challenges persist in surgery for neonatal total anomalous pulmonary venous connection (neoTAPVC), with the high mortality risk not mitigated over time.

Methods: A prospectively collected single-center database containing all neonates with TAPVC undergoing biventricular repair in 2012 to 2020 was retrospectively reviewed. The primary outcome was death or postoperative pulmonary venous obstruction (PPVO).

Novel dominant-negative mutation in a family with heterotaxy plus mouse model.

Background: Primary ciliary dyskinesia (PCD) is a clinically heterogeneous group of autosomal or, less frequently, X-chromosomal recessive inheritance syndrome of motile cilia dysfunction characterized by neonatal respiratory distress, oto-sino-pulmonary disease, infertility and situs inversus. Recently, type 43 PCD (CILD43, OMIM#618699) was established by autosomal-dominant loss-of-function mutations identified in Forkhead box J1 (). However, the functional validation of mutations in humans and mice has not been fully performed.

Review of surgical experience in 188 infants and young children with Ebstein anomaly.

Objectives: This study sought to review our single-institutional surgical experience in paediatric Ebstein anomaly (EA).

Methods: We retrospectively reviewed the paediatric patients with EA undergoing operation between 2004 and 2020. The time-to-event analysis was studied using Kaplan-Meier estimates.

Anatomical attention-based prediction of postoperative pulmonary venous obstruction via CTA images.

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease, with which some patients suffer from postoperative pulmonary venous obstruction (PPVO), requiring particular follow-up strategies and treatments. PPVO prediction has important clinical significance, while building a PPVO prediction model is challenging due to limited data and class imbalance distribution. Inspired by the anatomical evidence of PPVO, which is related to the structure of the left atrium (LA) and pulmonary vein (PV), we design an effective multi-task network for PPVO classification.

Inattention and hyperactivity in children and adolescents with repaired D-transposition of the great arteries: Prevalence, perioperative risk factors, and clinical outcomes.

Objective: The present study objectives were to determine the prevalence of attention-deficit/hyperactivity disorder symptoms (ADHD-like symptoms) in children and adolescent with d-transposition of great artery (D-TGA) after arterial switch operation (ASO) and examine associated risk factors and adverse personal, family dysfunctions.

Methods: This cohort study included 103 patients with D-TGA who underwent ASO in early infancy at Shanghai Children's Medical Center between 2011 and 2016 and then follow-up. Data analysis was conducted from September 2020 to April 2022.

COVID-19 in a Child With Transposition of the Great Arteries S/P Fontan Palliation: A Case Report and Literature Review.

Background: Although adult patients with cardiovascular disease are at higher risk of adverse outcomes such as death or severe infection, limited data exist regarding pediatric patients with congenital heart disease. We would like to report our experience with COVID-19 in a pediatric patient with Fontan circulation. Furthermore, we present a review of patients with Fontan palliation and COVID-19 previously reported in the literature to summarize the clinical characteristics of this population.

Risk factors for postoperative pulmonary venous obstruction after surgical repair of total anomalous pulmonary venous connection: a systemic review and meta-analysis.

Objectives: A meta-analysis was performed to investigate the risk factors for postoperative pulmonary venous obstruction (PVO) after surgical repair of total anomalous pulmonary venous connection (TAPVC).

Methods: Data bases including PubMed, Embase, Web of Science and Cochrane Library were searched systematically. The goal was to discuss the risk factors for postoperative PVO after TAPVC.

Cardiac-type total anomalous pulmonary venous return is not benign.

Objective: The objective of this study was to investigate the association between morphological variation and postsurgical pulmonary vein (PV) stenosis (PPVS) in patients with cardiac total anomalous pulmonary venous connection (TAPVC).

Methods: This single-center, retrospective study included 168 pediatric patients who underwent surgical repair of cardiac TAPVC from 2013 to 2019 (connection to the coronary sinus [CS], n = 136; connection directly to the right atrium [RA], n = 32). Three-dimensional computed tomography modeling and geometric analysis were performed to investigate the morphological features; their relevance to the PPVS was examined.

Surgical options for proximal and distal transverse arch hypoplasia in infants with coarctation.

Background: Although various surgical techniques have been reported for aortic arch reconstruction for proximal and distal transverse arch (PDTA) hypoplasia, no consensus has been reached on a surgical option for initial arch reconstruction. This study was undertaken to review various arch reconstruction options for PDTA hypoplasia in Chinese infants.

Methods: A retrospective review of 121 infants who underwent initial arch reconstruction of the proximal and distal aortic arches between 2010 and 2020 was performed.

Building a Risk Prediction Model for Postoperative Pulmonary Vein Obstruction via Quantitative Analysis of CTA Images.

Total anomalous pulmonary venous connection (TAPVC) is a rare but mortal congenital heart disease in children and can be repaired by surgical operations. However, some patients may suffer from pulmonary venous obstruction (PVO) after surgery with insufficient blood supply, necessitating special follow-up strategy and treatment. Therefore, it is a clinically important yet challenging problem to predict such patients before surgery.

Review of surgical experience in 61 patients with mixed total anomalous pulmonary venous connection.

Objectives: Prior studies have reported a high mortality and incidence of post-repair pulmonary venous obstruction (PVO) in mixed total anomalous pulmonary venous connection (TAPVC). This study sought to review the surgical outcomes in this entity.

Methods: A review of 61 patients undergoing surgical repair of mixed TAPVC was conducted.

Impact of early Coronavirus Disease 2019 pandemic on pediatric cardiac surgery in China.

Objective: This study aimed to provide an insight into the impact of the early outbreak of the novel Coronavirus Disease 2019 on the care management for patients with congenital heart disease.

Methods: This study respectively enrolled a cohort of surgical patients who underwent surgery in 2018 (group I), 2019 (group II), and 2020 (group III) and a cohort of follow-up patients who had follow-up in 2017 (group A), 2018 (group B), and 2019 (group C) in 13 children hospitals.

Results: During the Coronavirus Disease 2019 era, there was a significant decrease in total surgical volume and a change in case mix in terms of an increase in the proportion of emergency operations.

Artificial intelligence-assisted auscultation in detecting congenital heart disease.

Aims: Computer-assisted auscultation has become available to assist clinicians with physical examinations to detect congenital heart disease (CHD). However, its accuracy and effectiveness remain to be evaluated. This study seeks to evaluate the accuracy of auscultations of abnormal heart sounds of an artificial intelligence-assisted auscultation (AI-AA) platform we create.

Single-institution outcomes of surgical repair of infracardiac total anomalous pulmonary venous connection.

Objective: This contemporary study sought to describe the outcomes of patients undergoing biventricular repair of infracardiac total anomalous pulmonary venous connection.

Methods: A retrospective study was performed on patients with infracardiac total anomalous pulmonary venous connection who underwent sutureless technique or conventional repair between 2006 and 2018. Risk factors for survival and post-repair pulmonary vein stenosis (PVS) were assessed with Cox regression model.

15q11.2 deletion is enriched in patients with total anomalous pulmonary venous connection.

Introduction: CNV is a vital pathogenic factor of congenital heart disease (CHD). However, few CNVs have been reported for total anomalous pulmonary venous connection (TAPVC), which is a rare form of CHD. Using case-control study, we identified 15q11.

The therapeutic impact of human neonatal BMSC in a right ventricular pressure overload model in mice.

Objective: To determine the impact of donor age on the therapeutic effect of bone marrow-derived mesenchymal stem cells (BMSCs) in treating adverse remodeling as the result of right ventricle (RV) pressure overload.

Methods: BMSCs were isolated from neonatal (< 1 month), infant (1 month to 1 year), and young children (1 year to 5 years) and were compared in their migration potential, surface marker expression, VEGF secretion, and matrix metalloprotein (MMP) 9 expression. Four-week-old male C57 mice underwent pulmonary artery banding and randomized to treatment and untreated control groups.

Human cardiac extracellular matrix-chitosan-gelatin composite scaffold and its endothelialization.

The present study developed a cardiac extracellular matrix-chitosan-gelatin (cECM-CG) composite scaffold that can be used as a tissue-engineered heart patch and investigated its endothelialization potential by incorporating CD34 endothelial progenitor cells (EPCs). The cECM-CG composite scaffold was prepared by blending cardiac extracellular matrix (cECM) with biodegradable chitosan-gelatin (CG). The mixture was lyophilized using vacuum freeze-drying.

Hemodynamic Effects of A Simplified Venturi Conduit for Fontan Circulation: A Pilot, In Silico Analysis.

Objectives: To study the effects of a self-powered Fontan circulation in both idealized Fontan models and patient-specific models.

Methods: In silico, a conduit with a nozzle was introduced from ascending aorta into the anastomosis of superior vena cava and pulmonary artery. Computational fluid dynamics (CFD) simulation was applied to calculate the fluid fields of models.

Integrated model for the prenatal diagnosis and postnatal surgical treatment of total anomalous pulmonary venous connection: A multidisciplinary collaborative experience and preliminary results.

Background: This study aimed to evaluate an integrated model for the prenatal diagnosis and postnatal treatment of total anomalous pulmonary venous connection (TAPVC).

Methods: From January 2014 to December 2018, 11 patients were considered as a prenatally diagnosed group, who would accept the integrated model for prenatal diagnosis and postnatal treatment of TAPVC. Besides, 25 patients as postnatally diagnosed group underwent emergency surgery during the corresponding period at the same age.

Intermediate-Term Outcomes of Slide Tracheoplasty in Pediatric Patients With Ring-Sling Complex.

Background: This study was intended to determine the intermediate-term outcomes after slide tracheoplasty (STP) in patients with ring-sling complex.

Methods: All children undergoing STP between 2009 and 2018 were included. The patients' baseline characteristics, perioperative management details, and follow-up evaluations were reviewed retrospectively.