Maternal Oxidative Balance Score during Pregnancy and Congenital Heart Defects.

The relationship between maternal oxidative balance score (OBS) in pregnancy, representing overall oxidative balance status by integrating dietary and lifestyle factors, and congenital heart defects (CHD) remains unclear; therefore, this study attempted to explore their associations among the Chinese population. We conducted a case-control study including 474 cases and 948 controls in Northwest China. Pregnant women were interviewed to report diets and lifestyles in pregnancy by structured questionnaires. Logistic regression models were used to estimate the adjusted ORs (95%CIs). Maternal OBS ranged from 6 to 34 among cases, and 5 to 37 among controls. Comparing the highest with the lowest tertile group, the adjusted OR for CHD was 0.31 (0.19-0.50). The CHD risk was reduced by 7% (OR = 0.93, 95%CI = 0.90-0.95) in association with per 1 higher score of OBS during pregnancy. The inverse relationship between maternal OBS and CHD risk appeared to be more pronounced among participants in urban areas (OR = 0.89, 95%CI = 0.86-0.93). Maternal OBS during pregnancy showed good predictive values for fetal CHD, with the areas under the receiver operating characteristic curve 0.78 (0.76-0.81). These findings highlighted the importance of reducing oxidative stress through antioxidant-rich diets and healthy lifestyles among pregnant women to prevent fetal CHD.

Survival and Risk Factors for Mortality in Infants With Congenital Heart Disease in South Korea.

BACKGROUND/AIM: The survival of patients with congenital heart disease (CHD) has dramatically improved over recent decades. However, a disparity exists depending on the country and medical system. This study aimed to analyze the survival of infants with CHD until the age of 18 years using large-scale population data in South Korea and investigate the effect of neonatal conditions at birth. PATIENTS AND METHODS: We retrospectively extracted the Korean National Health Insurance Service claims data from January 2002 to December 2020. We included patients diagnosed with CHD who were less than one year of age. The follow-up duration was until their death or until they were censored before the age of 18 years. The CHD lesions were classified hierarchically (conotruncal, severe non-conotruncal, coarctation of the aorta, ventricular septal defect, atrial septal defect, and others). Several neonatal conditions were adopted as risk factors. RESULTS: Overall, 127,958 infants had been diagnosed with CHD and 2,275 died before the age of 18 years. The survival rate of infants with CHD during childhood was 97.9%. The highest childhood mortality rate was associated with non-conotruncal defects (19.7%), followed by conotruncal defects (10.2%). The significant risk factors for childhood mortality were complex CHD, pulmonary hypertension, birth asphyxia, small for gestational age, respiratory distress, pulmonary hemorrhage, bronchopulmonary dysplasia, and convulsions. CONCLUSION: The survival of infants with CHD has been favorable in South Korea. Several neonatal conditions are risk factors for childhood mortality. Individualized risk assessment and optimal treatment strategies may help improve their survival rate.

Impact of veno-venous collaterals on outcome after the total cavopulmonary connection.

OBJECTIVE: To evaluate the prevalence of veno-venous collaterals (VVCs) after total cavopulmonary connection (TCPC) and analyze their impact on outcomes. METHODS: Patients undergoing TCPC between 1994 and 2022 were evaluated. VVCs were identified using angiograms of cardiac catheterizations and their impact on outcomes was analyzed. RESULTS: A total of 635 patients were included. Median age at TCPC was 2.3 (interquartile ranges (IQR): 1.8-3.3) years. The most frequent diagnosis was hypoplastic left heart syndrome in 173 (27.2%) patients. Prior bidirectional cavopulmonary shunt was performed in 586 (92.3%) patients at a median age of 5.3 (3.6-9.9) months. VVCs were found in 94 (14.8%) patients at a median of 2.8 (0.1-11.8) years postoperatively. The prevalence of VVCs was similar between the dominant right and left ventricle (14.7 vs. 14.9%, p = 0.967). Mean pulmonary artery pressure (16.2 vs. 16.0 mmHg, p = 0.902), left atrial pressure (5.5 vs. 5.7 mmHg, p = 0.480), transpulmonary gradient (4.0 vs. 3.8 mmHg, p = 0.554) and oxygen saturation (81.4 vs. 82.6%, p = 0.103) before TCPC were similar between patients with and without VVCs. The development of VVCs did not affect survival after TCPC (p = 0.161). Nevertheless, VVCs were a risk for the development of plastic bronchitis (PB, p < 0.001). Interventional closure of VVCs was performed in 60 (9.4%) patients at a median of 8.9 (0.6-15.1) years after TCPC, and improvement of oxygen saturation was observed in 66% of the patients. CONCLUSIONS: The prevalence of VVCs after TCPC was 15%. VVCs had no impact on survival following TCPC but were associated with a high prevalence of PB.

Incidence and predictive factors of mortality of infective endocarditis in adults with congenital heart disease: A systematic review and meta-analysis.

INTRODUCTION: Congenital heart diseases (CHD) with abnormal turbulent blood flow are associated with the highest risk of infective endocarditis (IE). Despite advancement in diagnostics and treatment, the mortality rate of IE remains high due the life-threatening complications. Our study aims to assess the incidence and mortality rates of IE and predictive factors for mortality among adults CHD (ACHD). METHODS: A systematic literature search was conducted on PubMed, SCOPUS, and Ovid SP to retrieve relevant studies. The pooled estimates and predictors of mortality were calculated using the random-effects generic inverse variance method using R programming. RESULTS: 12 studies involving 3738 ACHD patients were included in this meta-analysis. The overall incidence of IE in ACHD was 1.26 per 1000 patient-years (95% CI 0.55-1.96). 60% (95% CI 46-72%) of patients had surgical management for IE. The mortality rate of IE was 9% (95% CI 7-12%). The predictors of mortality were conservative management (OR: 5.07, 95% CI: 4.63-5.57), renal dysfunction (OR: 4.15, 95% CI: 2.92-5.88), cerebral complications (OR: 3.59, 95% CI: 1.78-7.23), abscesses/valve complications (OR: 2.67, 95% CI: 1.71-4.16), Staphylococcus aureus infection (OR: 2.32, 95% CI: 1.33-4.06), emboli (OR: 2.03, 95% CI: 1.47-2.79), body mass index (OR: 1.10, 95% CI: 1.01-1.21), age (OR: 1.02, 95% CI: 1.00-1.04), and previous IE (OR: 1.02, 95% CI: 1.00-1.04). CONCLUSION: The mortality rate of IE in ACHD is low. However, conservative management is associated with the highest risk of mortality.

Beyond the heart: Cognitive and verbal outcomes in Arab children with congenital heart diseases.

BACKGROUND: This study investigates how congenital heart diseases (CHD) characteristics and interventions affect cognitive and verbal skills in Arab children, while also uncovering previously unexplored connections between these skills and the quality of life (QoL) scores as perceived by both children and parents. METHODS: A cross-sectional study was conducted in Jordan, involving 62 children with CHD aged 6-16. Data were collected through standardized intelligence tests (namely The Raven's Progressive Matrices Test and The Wechsler Intelligence Scale for Children) and QoL assessments. RESULTS: Sex, disease severity, cyanosis, CHD defect status, conducted operations, and types of interventions did not significantly influence cognitive scores. However, a significant difference was observed in Wechsler's scores between cyanotic and non-cyanotic children (p < .01) and between severe and moderate cases (p = .01). Further, a significant positive correlation was identified between Wechsler's Scores and QoL reported by parents (r = 0.33, p < .01). This correlation was particularly pronounced in the social and school functioning dimensions of QoL. CONCLUSIONS: This study highlights the need for personalized care approaches for children with CHDs based on their individual characteristics. While cognitive abilities did not directly correlate with children's QoL reports, a significant positive correlation between verbal skills and QoL reported by parents underscores the importance of effective communication in assessing a child's overall well-being. Future research should further examine the cognitive development in this population, employing neurocognitive investigations and longitudinal studies to gain a deeper understanding of their cognitive profiles and trajectories.

Zebrafish Congenital Heart Disease Models: Opportunities and Challenges.

Congenital heart defects (CHDs) are common human birth defects. Genetic mutations potentially cause the exhibition of various pathological phenotypes associated with CHDs, occurring alone or as part of certain syndromes. Zebrafish, a model organism with a strong molecular conservation similar to humans, is commonly used in studies on cardiovascular diseases owing to its advantageous features, such as a similarity to human electrophysiology, transparent embryos and larvae for observation, and suitability for forward and reverse genetics technology, to create various economical and easily controlled zebrafish CHD models. In this review, we outline the pros and cons of zebrafish CHD models created by genetic mutations associated with single defects and syndromes and the underlying pathogenic mechanism of CHDs discovered in these models. The challenges of zebrafish CHD models generated through gene editing are also discussed, since the cardiac phenotypes resulting from a single-candidate pathological gene mutation in zebrafish might not mirror the corresponding human phenotypes. The comprehensive review of these zebrafish CHD models will facilitate the understanding of the pathogenic mechanisms of CHDs and offer new opportunities for their treatments and intervention strategies.

Association of congenital heart defects (CHD) with factors related to maternal health and pregnancy in newborns in Puerto Rico.

Background: Given the pervasive issues of obesity and diabetes both in Puerto Rico and the broader United States, there is a compelling need to investigate the intricate interplay among BMI, pregestational, and gestational maternal diabetes, and their potential impact on the occurrence of congenital heart defects (CHD) during neonatal development. Methods: Using the comprehensive System of Vigilance and Surveillance of Congenital Defects in Puerto Rico, we conducted a focused analysis on neonates diagnosed with CHD between 2016 and 2020. Our assessment encompassed a range of variables, including maternal age, gestational age, BMI, pregestational diabetes, gestational diabetes, hypertension, history of abortion, and presence of preeclampsia. Results: A cohort of 673 patients was included in our study. The average maternal age was 26 years, within a range of 22 to 32 years. The mean gestational age measured 39 weeks, with a median span of 38 to 39 weeks. Of the 673 patients, 274 (41%) mothers gave birth to neonates diagnosed with CHD. Within this group, 22 cases were linked to pre-gestational diabetes, while 202 were not; 20 instances were associated with gestational diabetes, compared to 200 without; and 148 cases exhibited an overweight or obese BMI, whereas 126 displayed a normal BMI. Conclusion: We identified a statistically significant correlation between pre-gestational diabetes mellitus and the occurrence of CHD. However, our analysis did not show a statistically significant association between maternal BMI and the likelihood of CHD. These results may aid in developing effective strategies to prevent and manage CHD in neonates.

The Significance of Congenital Portosystemic Shunts in Congenital Heart Disease and the Bizarre Phenomenon of Alternating Portosystemic and Systemic-Portal Shunting.

Congenital portosystemic shunts (CPSSs) are rare vascular anomalies characterized by abnormal connections between the portal/splanchnic veins and the systemic veins. CPSSs often occur as an isolated congenital anomaly, but they can also coexist with congenital heart disease (CHD). Owing to their myriad consequences on multiple organ systems, familiarity with CPSS is of tremendous importance to the care of patients with CHD. The rationale and timing for interventions to embolize CPSS in this scenario are discussed. Specific shunt embolization techniques are beyond the scope of this article.

Transcatheter Interventions in Adult Congenital Heart Disease.

Congenital heart disease (CHD) is the most common congenital birth defect with an incidence of 1 in 100. Current survival to adulthood is expected in 9 out of 10 children with severe CHD as the diagnostic, interventional, and surgical success improves. The adult CHD (ACHD) population is increasingly diverse, reflecting the broad spectrum of CHD and evolution of surgical techniques to improve survival. Similarly, transcatheter interventions have seen exponential growth and creativity to reduce the need for repeat sternotomies. This article focuses on newer data and evolving techniques for transcatheter interventions specific to certain ACHD populations.

Transcatheter Pulmonary Valve in Congenital Heart Disease.

Over the last 2 decades, experience with transcatheter pulmonary valve replacement (TPVR) has grown significantly and has become an effective and reliable way of treating pulmonary valve regurgitation, right ventricular outflow (RVOT) obstruction, and dysfunctional bioprosthetic valves and conduits. With the introduction of self-expanding valves and prestents, dilated native RVOT can be addressed with the transcatheter approach. In this article, the authors review the current practices, technical challenges, and outcomes of TPVR.

Current Understanding of Indications, Technical Aspects and Outcomes of Fetal Cardiac Interventions.

With the improvement in the detection of congenital heart disease in fetal life, fetal cardiac interventions are pushing the envelope in hopes of either altering the natural history of disease or improving survival in certain high-risk lesions. These interventions include fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome, fetal atrial septoplasty with or without atrial septal stenting for hypoplastic left heart syndrome and variants with intact or severely restrictive atrial septum, and fetal pulmonary valvuloplasty for severe pulmonary stenosis or pulmonary atresia with intact ventricular septum. This review discusses their indications, technical aspects, and outcomes based on available literature.

Lymphatic Interventions in Congenital Heart Disease.

Lymphatic disorders in congenital heart disease can be broadly classified into chest compartment, abdominal compartment, or multicompartment disorders. Heavily T2-weighted noninvasive lymphatic imaging (for anatomy) and invasive dynamic contrast magnetic resonance lymphangiography (for flow) have become the main diagnostic modalities of choice to identify the cause of lymphatic disorders. Selective lymphatic duct embolization (SLDE) has largely replaced total thoracic duct embolization as the main lymphatic therapeutic procedure. Recurrence of symptoms needing repeat interventions is more common in patients who underwent SLDE. Novel surgical and transcatheter thoracic duct decompression strategies are promising, but long-term follow-up is critical and eagerly awaited.

Effects of antibiotics, hospitalisation and surgical complications on self-reported immunological vulnerability following paediatric open-heart surgery and thymectomy: a single-centre retrospective cohort study.

BACKGROUND: Partial or complete thymectomy is routinely performed in paediatric open-heart surgeries when treating congenital heart defects. Whether or not thymectomised children require systematic immunological monitoring later in life is unknown. The objective of this study was to investigate the effects of preoperatively and postoperatively used antibiotics, hospitalisation and surgical complications on self-reported immunological vulnerability in paediatric patients with early thymectomy to better recognise the patients who could benefit from immunological follow-up in the future. METHODS: We conducted a retrospective cohort study, including 98 children and adolescents aged 1-15 years, who had undergone an open-heart surgery and thymectomy in infancy and who had previously answered a survey regarding different immune-mediated symptoms and diagnoses. We performed a comprehensive chart review of preoperative and postoperative factors from 1 year preceding and 1 year following the open-heart surgery and compared the participants who had self-reported symptoms of immunological vulnerability to those who had not. RESULTS: The median age at primary open-heart surgery and thymectomy was 19.5 days in the overall study population (60% men, n=56) and thymectomies mainly partial (80%, n=78). Broad-spectrum antibiotics were more frequently used preoperatively in participants with self-reported immunological vulnerability (OR=3.05; 95% CI 1.01 to 9.23). This group also had greater overall use of antibiotics postoperatively (OR=3.21; 95% CI 1.33 to 7.76). These findings were more pronounced in the subgroup of neonatally operated children. There was no statistically significant difference in the duration of intensive care unit stay, hospitalisation time, prevalence of severe infections, surgical complications or glucocorticoid use between the main study groups. CONCLUSION: Antimicrobial agents were more frequently used both preoperatively and postoperatively in thymectomised children with self-reported immunological vulnerability after thymectomy. Substantial use of antimicrobial agents early in life should be considered a potential risk factor for increased immunological vulnerability when evaluating the significance of immune-mediated symptom occurrence in thymectomised paediatric patients.

Challenges of managing anomalous mitral arcade with severe mitral regurgitation and hydrops fetalis in infants.

Anomalous mitral arcade (MA) is a rare congenital anomaly. We report a case of MA in a newborn who presented with hydrops fetalis due to severe mitral regurgitation. After birth, he developed severe respiratory failure, congestive heart failure and airway obstruction because an enlarged left atrium from severe mitral regurgitation compressed the distal left main bronchus. There is limited experience in surgical management of this condition in Thailand, and the patient's mitral valve was too small for replacement. Therefore, he was treated with medication to control heart failure and supported with positive pressure ventilation to promote growth. We have followed the patient until the current time of writing this report at the age of 2 years, and his outcome is favourable regarding heart failure symptoms, airway obstruction, growth and development. This case describes a challenging experience in the non-surgical management of MA with severe regurgitation, which presented at birth.

Similarity-driven motion-resolved reconstruction for ferumoxytol-enhanced whole-heart MRI in congenital heart disease.

A similarity-driven multi-dimensional binning algorithm (SIMBA) reconstruction of free-running cardiac magnetic resonance imaging data was previously proposed. While very efficient and fast, the original SIMBA focused only on the reconstruction of a single motion-consistent cluster, discarding the remaining data acquired. However, the redundant data clustered by similarity may be exploited to further improve image quality. In this work, we propose a novel compressed sensing (CS) reconstruction that performs an effective regularization over the clustering dimension, thanks to the integration of inter-cluster motion compensation (XD-MC-SIMBA). This reconstruction was applied to free-running ferumoxytol-enhanced datasets from 24 patients with congenital heart disease, and compared to the original SIMBA, the same XD-MC-SIMBA reconstruction but without motion compensation (XD-SIMBA), and a 5D motion-resolved CS reconstruction using the free-running framework (FRF). The resulting images were compared in terms of lung-liver and blood-myocardium sharpness, blood-myocardium contrast ratio, and visible length and sharpness of the coronary arteries. Moreover, an automated image quality score (IQS) was assigned using a pretrained deep neural network. The lung-liver sharpness and blood-myocardium sharpness were significantly higher in XD-MC-SIMBA and FRF. Consistent with these findings, the IQS analysis revealed that image quality for XD-MC-SIMBA was improved in 18 of 24 cases, compared to SIMBA. We successfully tested the hypothesis that multiple motion-consistent SIMBA clusters can be exploited to improve the quality of ferumoxytol-enhanced cardiac MRI when inter-cluster motion-compensation is integrated as part of a CS reconstruction.

Perinatal deaths attributable to congenital heart defects in Hunan Province, China, 2016-2020.

OBJECTIVE: To explore the association between demographic characteristics and perinatal deaths attributable to congenital heart defects (CHDs). METHODS: Data were obtained from the Birth Defects Surveillance System of Hunan Province, China, 2016-2020. The surveillance population included fetuses and infants from 28 weeks of gestation to 7 days after birth whose mothers delivered in the surveillance hospitals. Surveillance data included demographic characteristics such as sex, residence, maternal age, and other key information, and were used to calculate the prevalence of CHDs and perinatal mortality rates (PMR) with 95% confidence intervals (CI). Multivariable logistic regression analysis (method: Forward, Wald, α = 0.05) and adjusted odds ratios (ORs) were used to identify factors associated with perinatal deaths attributable to CHDs. RESULTS: This study included 847755 fetuses, and 4161 CHDs were identified, with a prevalence of 0.49% (95%CI: 0.48-0.51). A total of 976 perinatal deaths attributable to CHDs were identified, including 16 (1.64%) early neonatal deaths and 960 (98.36%) stillbirths, with a PMR of 23.46% (95%CI: 21.98-24.93). In stepwise logistic regression analysis, perinatal deaths attributable to CHDs were more common in rural areas than urban areas (OR = 2.21, 95%CI: 1.76-2.78), more common in maternal age <20 years (OR = 2.40, 95%CI: 1.05-5.47), 20-24 years (OR = 2.13, 95%CI: 1.46-3.11) than maternal age of 25-29 years, more common in 2 (OR = 1.60, 95%CI: 1.18-2.18) or 3 (OR = 1.43, 95%CI: 1.01-2.02) or 4 (OR = 1.84, 95%CI: 1.21-2.78) or > = 5 (OR = 2.02, 95%CI: 1.28-3.18) previous pregnancies than the first pregnancy, and more common in CHDs diagnosed in > = 37 gestional weeks (OR = 77.37, 95%CI: 41.37-144.67) or 33-36 gestional weeks (OR = 305.63, 95%CI: 172.61-541.15) or < = 32 gestional weeks (OR = 395.69, 95%CI: 233.23-671.33) than diagnosed in postnatal period (within 7 days), and less common in multiple births than singletons (OR = 0.48, 95%CI: 0.28-0.80). CONCLUSIONS: Perinatal deaths were common in CHDs in Hunan in 2016-2020. Several demographic characteristics were associated with perinatal deaths attributable to CHDs, which may be summarized mainly as economic and medical conditions, severity of CHDs, and parental attitudes toward CHDs.