The improvement in congenital heart disease (CHD) treatment and management has increased the life expectancy in infants. However, the long-term efficacy is difficult to assess and thus, computational modelling has been applied for evaluating this. Here, we provide an overview of the applications of computational modelling in CHD based on three categories; CHD involving large blood vessels only, heart chambers only, and CHD that occurs at multiple heart structures. We highlight the advancement of computational simulation of CHD that uses multiscale and multiphysics modelling to ensure a complete representation of the heart and circulation. We provide a brief future direction of computational modelling of CHD such as to include growth and remodelling, detailed conduction system, and occurrence of myocardial infarction. We also proposed validation technique using advanced three-dimensional (3D) printing and particle image velocimetry (PIV) technologies to improve the model accuracy.

Congenital heart disease (CHD) remains the most common birth defect, with surgical intervention required in complex cases. Right ventricle (RV) function is known to be a major predictor of sustained cardiac health in these patients; thus, by elucidating the divergent profiles between CHD and the control through tissue analysis, this study aims to identify new avenues of investigation into the mechanisms surrounding reduced RV function. Transcriptomic profiling, in-silico deconvolution and functional network analysis were conducted on RV biopsies, identifying an increase in the mitochondrial dysfunction genes RPPH1 and RMPR (padj = 4.67 × 10-132, 2.23 × 10-107), the cytotoxic T-cell markers CD8a, LAGE3 and CD49a (p = 0.0006, p < 0.0001, and p = 0.0118) and proinflammatory caspase-1 (p = 0.0055) in CHD. Gene-set enrichment identified mitochondrial dysfunctional pathways, predominately changes within oxidative phosphorylation processes. The negative regulation of mitochondrial functions and metabolism was identified in the network analysis, with dysregulation of the mitochondrial complex formation. A histological analysis confirmed an increase in cellular bodies in the CHD RV tissue and positive staining for both CD45 and CD8, which was absent in the control. The deconvolution of bulk RNAseq data suggests a reduction in CD4+ T cells (p = 0.0067) and an increase in CD8+ T cells (p = 0.0223). The network analysis identified positive regulation of the immune system and cytokine signalling clusters in the inflammation functional network, as there were lymphocyte activation and leukocyte differentiation. Utilising RV tissue from paediatric patients undergoing CHD cardiac surgery, this study identifies dysfunctional mitochondrial pathways and an increase in inflammatory T-cell presence prior to reparative surgery.

To retrospectively analyze the clinical efficacy of an innovative mitral valvuloplasty strategy in the treatment of mitral regurgitation in children. From January 2018 to December 2022, 140 patients undergoing surgical treatment for mitral regurgitation in our hospital were enrolled. Ninety patients underwent three-step standardized pediatric mitral valvuloplasty (group A) and 50 patients underwent simple annuloplasty (group B). The incidence of primary and secondary study endpoint was compared between the two groups, and the independent risk factors for the primary study endpoint were determined. Our primary study endpoint was a composite endpoint of postoperative functional mitral failure, postoperative heart failure, transplantation, and/or mortality. Secondary end points were defined as perioperative complications. During the follow-up period, there was no all-cause death. Primary endpoint events occurred in 22 patients, including 12 patients in group A and 10 patients in group B. There was no significant difference in the incidence of primary and secondary endpoint events between the two groups. Multivariate Cox proportional hazards regression analysis showed that younger age and residual mitral regurgitation at discharge were independent risk factors for the primary endpoint events, while type of MV repair was not an independent risk factor. Subgroup analysis based on age showed that primary endpoint events occurred in 4 patients in group A and 7 patients in group B in patients < 1 year old. The incidence of primary endpoint events in group A was lower than that in group B (6.06% vs. 20.59%, P = 0.041). In patients ≥ 1 year old, the primary endpoint event occurred in 8 cases in group A and 3 cases in group B. There was no significant difference in the incidence of primary endpoint events between groups A and B (33.33% vs. 18.75%, P = 0.312). The degree of mitral regurgitation at discharge was significantly improved compared with that before operation in both groups (P < 0.001), and the degree of mitral regurgitation at the last follow-up was not significantly worse than that at discharge (P = 0.090). The mid-term results of mitral valvuloplasty for mitral regurgitation in children are encouraging. The perioperative recovery and postoperative outcomes of three-step standardized mitral valvuloplasty in children are not inferior to those of annuloplasty alone. Three-step standardized pediatric mitral valvuloplasty has better postoperative outcomes than simple mitral annuloplasty, especially for patients younger than 1 year old. Children with residual mitral regurgitation at discharge should be followed up regularly to be alert to the occurrence of poor prognosis.

BACKGROUND: Congenital heart disease (CHD) has been linked to impaired placental and fetal brain development. Assessing the placenta and fetal brain in parallel may help further our understanding of the relationship between development of these organs.
OBJECTIVE: 1) Placental and fetal brain oxygenation are correlated, 2) oxygenation in these organs is reduced in CHD compared to healthy controls, and 3) placental structure is altered in CHD.
METHODS: Retrospective case-control.
METHODS: Fifty-one human fetuses with CHD (32 male; median [IQR] gestational age [GA] = 32.0 [30.9-32.9] weeks) and 30 from uncomplicated pregnancies with normal birth outcomes (18 male; median [IQR] GA = 34.5 [31.9-36.7] weeks).
UNASSIGNED: 1.5 T single-shot multi-echo-gradient-echo echo-planar imaging.
RESULTS: Masking was performed using an automated nnUnet model. Mean brain and placental T2* and quantitative measures of placental texture, volume, and morphology were calculated.
METHODS: Spearman\'s correlation coefficient for determining the association between brain and placental T2*, and between brain and placental characteristics with GA. P-values for comparing brain T2*, placenta T2*, and placental characteristics between groups derived from ANOVA. Significance level P < 0.05.
RESULTS: There was a significant positive association between placental and fetal brain T2* (⍴ = 0.46). Placental and fetal brain T2* showed a significant negative correlation with GA (placental T2* ⍴ = -0.65; fetal brain T2* ⍴ = -0.32). Both placental and fetal brain T2* values were significantly reduced in CHD, after adjusting for GA (placental T2*: control = 97 [±24] msec, CHD = 83 [±23] msec; brain T2*: control = 218 [±26] msec, CHD = 202 [±25] msec). Placental texture and morphology were also significantly altered in CHD (Texture: control = 0.84 [0.83-0.87], CHD = 0.80 [0.78-0.84]; Morphology: control = 9.9 [±2.2], CHD = 10.8 [±2.0]). For all fetuses, there was a significant positive association between placental T2* and placental texture (⍴ = 0.46).
CONCLUSIONS: Placental and fetal brain T2* values are associated in healthy fetuses and those with CHD. Placental and fetal brain oxygenation are reduced in CHD. Placental appearance is significantly altered in CHD and shows associations with placental oxygenation, suggesting altered placental development and function may be related.
METHODS: 3 TECHNICAL EFFICACY: Stage 3.

Atrial septal defects are a common congenital malformation that can lead to an elevated risk for stroke due to the bypass of the lungs by deep vein thrombosis, as well as the expected repercussions of pulmonary hypertension if left untreated. Surgical intervention is definitive; however, recent advancements in treatment options, such as percutaneous intervention, represent a safer and equally effective way to treat this congenital complication. While safer, percutaneous interventions can also lead to adverse events that may force patients to present to the emergency department. Here, we present a unique case of a patient with congenital atrial septal defect status post-percutaneous intervention who developed a new-onset second-degree AV block, Mobitz type 1 Wenckebach rhythm.

UNASSIGNED: Univentricular heart disease is a relatively rare condition that affects infants, with a prevalence ranging from 0.05 to 0.1 per 1000 live births. It is characterized by an abnormality in the structure of the heart, specifically the presence of only one main pumping chamber (ventricle) instead of the usual two.
UNASSIGNED: In this particular case, a newborn male was diagnosed with double-inlet left ventricle (DILV), a specific form of univentricular heart disease. Following his birth, he exhibited symptoms of central cyanosis (a bluish tint to the skin due to poor oxygenation) and difficulties with breastfeeding. Clinical evaluation, along with a heart ultrasound, confirmed the need for palliative surgery. At the age of 6 months, the patient is scheduled to undergo the Glenn procedure, a surgical intervention that aims to redirect blood flow to the lungs and improve oxygenation.
UNASSIGNED: Given the complexity of double-inlet single ventricle anomalies, there are multiple differential diagnoses that need to be considered for accurate diagnosis, including conditions such as tricuspid atresia, large ventricular septal defect and corrected transposition of the great arteries with ventricular septal defect.
UNASSIGNED: Early intervention in the immediate postnatal period plays a crucial role in improving survival rates and reducing long-term complications. It is, therefore, essential to continue researching and refining treatment approaches.

Cardiovascular magnetic resonance (CMR) imaging is recommended in patients with congenital heart disease (CHD) in clinical practice guidelines as the imaging standard for a large variety of diseases. As CMR is evolving, novel techniques are becoming available. Some of them are already used clinically, whereas others still need further evaluation. In this statement the authors give an overview of relevant new CMR techniques for the assessment of CHD. Studies with reference values for these new techniques are listed in the supplement.

Pediatric cardiac fitness and rehabilitation programs vary widely in structure and content. The Cardiac Fitness Program (CFP) is built on traditional training pillars of aerobic, strength, and flexibility, and adds a fourth, training a positive mindset. This study assesses whether the systematic and comprehensive framework of the CFP results in broad benefits for a range of patients with congenital heart disease (CHD). Data from participants between 01/2017 and 12/2022 were analyzed. Pre- and post-CFP cardiopulmonary exercise test parameters, strength and flexibility metrics, and mindset survey results were compared overall, and by sex, age, diagnosis, and hemodynamic level. Of 62 participants (median age 15.5 years, range 8 to 23, 50% female), 3% had simple, 37% complex, 24% single ventricle CHD, and 35% arrhythmia, cardiomyopathy, or transplant. Significant improvements were noted in aerobic fitness (mean 9 ± 15% increase in % predicted peak oxygen consumption, p < 0.001). Strength metrics significantly improved (each p < 0.001), as did flexibility (p < 0.001). Patient-reported positive mindset scores did not improve significantly (mean increase 1.8 ± 5.1, p = 0.10, n = 25), whereas parents reported significant improvements (5.9 ± 10.4, p = 0.02, n = 20). Improvements were not significantly different by sex, age, diagnosis, or hemodynamic level. Comprehensive training across four pillars of fitness yields significant improvements in aerobic fitness, strength, flexibility, and parent-reported mindset scores for pediatric CHD patients, regardless of patient characteristics, diagnosis type, or severity of hemodynamic limitation. Further study is warranted into optimal standardization of training and whether a comprehensive approach amplifies individual pillars to create more than the sum of its parts.

Pediatric heart transplantation is hampered by a chronic shortage of donor organs. This problem is further confounded by graft rejection. Identification of earlier indicators of pediatric graft rejection and development of subsequent strategies to counteract these effects will increase the longevity of transplanted pediatric hearts. Heart transplant reject is due to a complex series of events, resulting in CAV, which is thought to be mediated through a host immune response. However, the earlier events leading to CAV are not very well known. We hypothesize that early events related to ischemia reperfusion injury during pediatric heart transplantation are responsible for CAV and subsequent graft rejection. Identification of the molecular markers of ischemia reperfusion injury and development of subsequent therapies to block these pathways can potentially lead to a therapeutic strategy to reduce CAV and increase the longevity of the transplanted heart. To accomplish this goal, we have developed a perfusable vascular graft model populated with endothelial cells and demonstrated the feasibility of this model to understand the early events of ischemia reperfusion injury.

Congenital heart disease (CHD) is a structural abnormality of the heart and/or great vessels and patients with CHD are at an increased risks of various morbidities throughout their lives and reduced long-term survival. Eventually, CHD may result in various complications including heart failure, arrhythmias, stroke, pneumonia, and sudden death. Unfortunately, the exact etiology and pathophysiology of some CHD remain unclear. Although the quality of life and prognosis of patients with CHD have significantly improved following technological advancement, the influence of CHD is lifelong, especially in patients with complicated CHD. Thus, the management of CHD remains a challenge due to its high prevalence. Finally, there are some disagreements on CHD among international guidelines. In this review, we provide an update of the pathophysiology, diagnosis, and treatment in most common type of CHD, including patent foramen ovale, atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus, coarctation of the aorta, transposition of the great arteries, congenitally corrected transposition of the great arteries, coronary anomalies, left and right ventricular outflow tract obstruction, tetralogy of Fallot and Ebstein anomaly. In particular, we focus on what is known and what is unknown in these areas, aiming to improve the current understanding of various types of CHD.