UNASSIGNED: This study aimed to investigate the relationship between maternal serum uric acid levels in the first trimester and the incidence of congenital heart diseases (CHDs) in offspring.
UNASSIGNED: This prospective cohort study was conducted in the southeast of China and involved 21,425 pregnant women and their offspring in the final analysis between 2019 and 2022. Fasting blood samples from pregnant women participating in the Fujian birth cohort study (11.3 ± 1.40 weeks of gestation) were analyzed for serum uric acid levels. The perinatal outcome was the incidence of CHDs. All fetuses with CHDs were confirmed by echocardiography doctors and pediatric cardiologists. Logistic regression analysis and restricted cubic spline (RCS) modeling were employed to investigate the relationship between serum uric acid level and the incidence of CHDs.
UNASSIGNED: We observed that maternal log2-transformed values of serum uric acid were strongly associated with odds of CHDs in offspring (adjusted odds ratio [AOR] 1.589, 95 % CI [1.149, 2.198]). Compared to the lowest quartile, the AORs for maternal uric acid levels in the other quartiles and the corresponding risk of CHDs in offspring were 1.363 (95 % CI [1.036, 1.793]), 1.213 (95 % CI [0.914, 1.610]), and 1.472 (95 % CI [1.112, 1.949]), respectively. Hyperuricemia in the first trimester significantly increased the risk of CHDs in offspring 1.837 (95 % CI [1.073, 3.145]). Furthermore, RCS showed a linear relationship between maternal serum uric acid levels in the first trimester and the incidence of CHDs (P for nonlinearity = 0.71).
UNASSIGNED: The results of this study indicated that elevated maternal serum uric acid levels in the first trimester were associated with an increased incidence of CHDs in offspring.

Ectopia cordis is a congenital heart malformation of the sternal wall, with a prevalence of 0.1% among heart conditions and an incidence of 5.5 to 7.9 per million births. It is characterized by the heart being located outside the thoracic cavity, and it may be accompanied by other congenital anomalies such as omphalocele, Cantrell´s pentalogy, or Fallot´s tetralogy. We present a case of thoracic ectopia cordis in a male neonate. After birth, we also observed a midline thoracic malformation and respiratory difficulties with clinical and paraclinical features consistent with tetralogy of Fallot. It was decided to provide skin flap coverage, and due to the poor prognosis of the heart condition, palliative care was chosen. Unfortunately, the neonate passed away after seven days. This clinical case study contributes to understanding this rare condition and may help improve diagnosis and treatment of affected patients.
La ectopia cordis es una malformación cardíaca congénita de la pared esternal, con una prevalencia del 0,1%, e incidencia del 5,5 al 7,9 por millón de nacimientos. Se caracteriza por situar al corazón fuera de la cavidad torácica, puede acompañarse de otras anomalías congénitas como onfalocele, pentalogía de Cantrell o tetralogía de Fallot. Presentamos un caso de ectopia cordis torácica en un recién nacido de sexo masculino. Después del nacimiento, también observamos una malformación de la línea media torácica y dificultad respiratoria con características clínicas y paraclínicas compatibles con tetralogía de Fallot. Se realizó una cobertura con colgajo cutáneo, y debido al mal pronóstico, se optó por cuidados paliativos; con fallecimiento después de siete días. Este estudio de caso clínico contribuye a la comprensión de esta rara enfermedad, y puede ayudar a mejorar el diagnóstico y tratamiento de los pacientes afectados.

Background Congenital anomalies of the coronary artery anatomy (CAAs) encompass a spectrum of disorders, often asymptomatic but potentially carrying severe clinical implications such as arrhythmia, chest pain, myocardial infarction, or sudden death. The estimated prevalence of CAAs in the general population ranges from 0.3% to 1.3%, with underdiagnosis in asymptomatic individuals. Multidetector computed tomography angiography (CTA) has emerged as a vital non-invasive tool for diagnosing and characterising CAAs, offering improved visualisation and aiding in appropriate management decisions. This study aims to analyse the spectrum of CAAs in a tertiary care setting, focusing on imaging features, prevalence, and potential clinical significance, utilising data from patients who underwent multidetector CTA. Methodology A single-centre, retrospective analysis of consecutive coronary angiograms over a five-year period identified patients with CAAs, with imaging conducted using a 128-slice, single-source CT scanner. Detailed imaging evaluation was performed by experienced radiologists, with anomalies classified according to established criteria. Results Among 756 coronary CTA examinations analysed, 37 instances of anomalous coronary vessels were identified. The study revealed a diverse range of anomalies, including myocardial bridging, anomalous origin of coronary arteries, and extracardiac abnormalities. Conclusions This study contributes valuable insights into the prevalence and imaging features of CAAs, enhancing our understanding of these anomalies and guiding improved patient outcomes in cardiovascular care. Future research should focus on elucidating pathophysiological mechanisms and establishing multicenter registries to address the challenges associated with studying these infrequent but clinically significant anomalies.

Although Down syndrome (DS) is considered a risk factor for hemodynamic instabilities (mainly pulmonary hypertension-PH) following surgery for congenital cardiac communications, many DS patients do surprising well postoperatively. We prospectively analyzed perioperative factors for a possible correlation with post-cardiopulmonary bypass (CPB) inflammatory reaction and postoperative PH in pediatric subjects. Sixty patients were enrolled (age 3 to 35 months), 39 of them with DS. Clinical and echocardiographic parameters (anatomical and hemodynamic) were computed preoperatively. Pulmonary and systemic mean arterial pressures (PAP and SAP) were assessed invasively intra and postoperatively. Immediate postoperative PAP/SAP ratio (PAP/SAPIPO) and the behavior of pressure curves were selected as primary outcome. Serum levels of 36 inflammatory proteins were measured by chemiluminescence preoperatively and 4 h post CPB. Of all factors analyzed, peripheral oxygen saturation (O2Sat, bedside assessment) was the only preoperative predictor of PAP/SAPIPO at multivariate analysis (p = 0.007). Respective values in non-DS, DS/O2Sat ≥ 95% and DS/O2Sat < 95% subgroups were 0.34 (0.017), 0.40 (0.027) and 0.45 (0.026), mean (SE), p = 0.004. The difference between non-DS and DS groups regarding postoperative PAP curves (upward shift in DS patients, p = 0.015) became nonsignificant (p = 0.114) after adjustment for preoperative O2Sat. Post-CPB levels of at least 5 cytokines were higher in patients with O2Sat < 95% versus those at or above this level, even within the DS group (p < 0.05). Thus, a baseline O2Sat < 95% representing pathophysiological phenomena in the airways and the distal lung, rather than DS in a broad sense, seems to be associated with post-CPB inflammation and postoperative PH in these patients.

OBJECTIVE: Maternal obesity is a highly suggestive risk factor of offspring congenital heart diseases (CHD). However, the risk of offspring CHD associated with maternal underweight has rarely been mentioned. Therefore, this study aimed to explore the effect of preconception underweight on offspring CHD.
METHODS: From November 2017 to August 2021, 132 386 pregnant women were enrolled in a birth cohort study in China in early pregnancy, and completed follow-up until delivery (or miscarriage/termination). Offspring CHD was diagnosed by prenatal ultrasound examination in both live births and stillbirths. Log-binomial regression and restricted cubic spline were used to estimate the risk of offspring CHD associated with preconception body mass index (BMI). A generalized additive model was used to explore the modification effect of maternal age on the association between preconception BMI and offspring CHD.
RESULTS: A total of 129 096 pregnant women were included in the analysis. The incidence of CHD in the underweight, normal weight, overweight, and obesity groups were 117/17 313 (0.68%), 556/85 695 (0.65%), 128/19 936 (0.64%), 47/6152 (0.76%), respectively. Both underweight and obesity before pregnancy marginally increased the risk of offspring CHD. The association between preconception BMI and offspring CHD varied by maternal age, with low preconception BMI associated with a significantly higher risk of offspring CHD in women <24 years (RR 2.32, 95% CI: 1.07-5.01 for 17 vs 21 kg/m2).
CONCLUSIONS: Preconception underweight was associated with an increased risk of offspring CHD in young pregnant women. Therefore, weight gain is important to prevent offspring CHD, especially for young women with low preconception BMI.

The prevalence of chronic diseases in children and adolescents has risen alarmingly worldwide. Diseases such as asthma, diabetes, obesity, mental disorders, and congenital heart defects are increasingly affecting the lives of children and pose significant challenges for the healthcare system. Physical activity plays a crucial role in preventing and treating these diseases. Numerous studies have shown that regular exercise improves physical performance, increases well-being, and leads to better health in the long term. Specially tailored sports programs that meet the individual needs and abilities of the children and adolescents affected are particularly important. The KidsTUMove project addresses this by developing tailored exercise programs for children with chronic diseases\' specific needs, medical conditions, and physical abilities. Therefore, it closes the gap in care provision and can thus sustainably improve the health prospects of these children and adolescents. KidsTUMove is positioned to make a significant impact on the lives of affected children across Europe. Promotion of such programs should therefore be an integral part of future health strategies.

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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.

Background/Objectives: The right-sided aortic arch (RAA) is an uncommon variation of the aortic arch (AA), characterized by the aorta crossing over the right main bronchus. In the RAA, the descending aorta can be found on either the right or left side of the spine. The current study comprises a comprehensive retrospective computed tomography angiography (CTA) investigation into the prevalence of the RAA within the Greek population. Additionally, we will conduct a systematic review and meta-analysis to elucidate both common and rare morphological variants of the RAA. This research is significant as it sheds light on the prevalence and characteristics of the RAA in a specific population, providing valuable insights for clinical practice. Methods: Two hundred CTAs were meticulously investigated for the presence of a RAA. In addition, the PubMed, Google Scholar, and Scopus online databases were thoroughly searched for studies referring to the AA morphology. The R programming language and RStudio were used for the pooled prevalence meta-analysis, while several subgroup analyses were conducted. Results: Original study: A unique case of 200 CTAs (0.5%) was identified with an uncommon morphology. The following branches emanated from the RAA under the sequence: the right subclavian artery (RSA), the right common carotid artery (RCCA), the left common carotid artery (LCCA), and the left vertebral artery (LVA) in common origin with the aberrant left subclavian artery (ALSA). The ALSA originated from a diverticulum (of Kommerell) and followed a retroesophageal course. Systematic Review and Meta-Analysis: Sixty-two studies (72,187 total cases) met the inclusion criteria. The pooled prevalence of the RAA with a mirror-image morphology was estimated at 0.07%, and the RAA with an ALSA was estimated at <0.01%. Conclusions: AA anomalies, specifically the RAA, raise clinical interest due to their coexistence with developmental heart anomalies and possible interventional complications. Congenital heart anomalies, such as the Tetralogy of Fallot and patent foramen ovale, coexisted with RAA mirror-image morphology.

OBJECTIVE: Children with congenital tracheal stenosis born in the developing world face a high risk of mortality due to limited access to proper treatment. Patients who required preoperative respiratory support were suspected to have poor survival after slide tracheoplasty; however, this was not clearly demonstrated in the previous studies. This study aims to investigate the impact of preoperative respiratory conditions on outcomes of slide tracheoplasty.
METHODS: From 2016 to 2022, children who underwent slide tracheoplasty were retrospectively reviewed. Patients with respiratory distress requiring emergency operations (group A) were compared with patients in stable condition who were scheduled for surgery (group B).
RESULTS: Perioperative results revealed that group A (n = 43) had a longer bypass time (P < 0.001), operation time (P = 0.01), postoperative ventilation time (P < 0.001) and length of intensive care unit stay (P = 0.00125) than group B (n = 60). The early mortality rate was 7.8%, and the actuarial 5-year survival rate was 85.3%. The cumulative incidence test revealed that group A was highly significant for overall mortality [sudistribution (SHR) 4.5; 95% confidence interval (CI) 1.23-16.4; P = 0.023]. Risk factors for overall mortality were prolonged postoperative ventilation time (hazard ratio 3.86; 95% CI 1.20-12.48; P = 0.024), bronchial stenosis (hazard ratio 5.77; 95% CI 1.72-19.31; P = 0.004), and preoperative tracheal mucositis (hazard ratio 5.67; 95% CI 1.51-21.31; P = 0.01). Four patients needed reintervention during a follow-up of 28.4 months (interquartile range 15.3-47.3).
CONCLUSIONS: Preoperative respiratory distress negatively affected the outcomes of patients who required slide tracheoplasty. Therefore, early detection of congenital tracheal stenosis and aggressive slide tracheoplasty are crucial and obligatory to enhance long-term survival in this lethal congenital airway disease.