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UNASSIGNED: Congenital heart disease (CHD), a developmental abnormality of the heart and vessels, is encountered in the pediatric age group frequently. Brachial artery flow-mediated dilation (FMD) and carotid intima-media thickness (CIMT) are indicators of subclinical cardiovascular disease and are used as surrogate measures of subclinical atherosclerosis. The present study aimed to compare CIMT and FMD between children with acyanotic congenital heart disease (ACHD) and healthy controls.
UNASSIGNED: A case-control study on 50 children with ACHD and 43 healthy individuals was done in Isfahan, Iran, between 2021 and 2022. The case group was selected via non-random sampling, and healthy controls were recruited from the relatives of the patients. A checklist, including age, sex, body mass index, and blood pressure, was filled out for all the participants. Then, FMD and CIMT were measured with brachial and carotid artery ultrasonography.
UNASSIGNED: Fifty children with ACHD and 43 healthy individuals (controls) under 18 years old participated in this study. Of these, 44 (47.3%) were girls and 49 (52.7%) were boys. The mean FMD was significantly higher in the ACHD group than in the control group (0.084±0.027 vs 0.076±0.042; P=0.021; 95% CI, 007 to 0.122;). CIMT was significantly higher in the ACHD group than in the control group (0.39±0.12 vs 0.34±0.1; P=0.037; 95% CI, 0.009 to 0.102;). However, systolic and diastolic blood pressure did not show differences between the groups.
UNASSIGNED: Based on our results, CIMT and FMD assessment may help detect early changes in peripheral vessels associated with atherosclerosis in the future in ACHD. Further studies are needed to confirm our findings.

BACKGROUND: Pretricuspid shunts have been associated with poorer survival rates in patients with Eisenmenger syndrome compared with postricuspid shunts and complex lesions. However, the risk stratification for persistent pulmonary hypertension (PH) in this population remains uncertain.
RESULTS: We retrospectively enrolled 103 patients with pretricuspid shunts with high total pulmonary resistance >4.5 Wood units (estimated pulmonary vascular resistance ≥3 Wood units). During a mean±SD follow-up of 20.95±24.84 months, 32 patients developed postoperative persistent PH after shunt correction. We identified 3 significant predictors of postoperative persistent PH, including mean pulmonary artery pressure after inhaled oxygen ≥40.5 mm Hg (odds ratio [OR], 7.78 [95% CI, 2.02-30.03]; P<0.01), total pulmonary resistance after inhaled oxygen ≥6.5 Wood units (estimated pulmonary vascular resistance ≥5 Wood units; OR, 12.23 [95% CI, 2.12-70.46]; P<0.01), and artery oxygen saturation at rest <95% (OR, 3.34 [95% CI, 1.07-10.44]; P=0.04). We established the prediction model with the C-statistics of 0.85 (95% CI, 0.77-0.93; P<0.01), and the C-statistic was 0.83 (95% CI, 0.80-0.86) after bootstrapping 10 000 times with a good performance of the nomogram calibration curve for predicting persistent PH.
CONCLUSIONS: Our study presents a multivariable risk stratification model for persistent PH after shunt correction in adults with pretricuspid shunts. This model, based on 3 hemodynamic predictors after inhaled oxygen, may assist in identifying individuals at higher risk of persistent PH after shunt correction.

Introduction Congenital heart defects (CHD) are one of the most common congenital anomalies, and their association with assisted reproductive technology (ART) is controversial in different populations. The purpose of this study was to evaluate this association and to provide information about the necessity of specialized echocardiography during pregnancy with ART. Methods This retrospective study was performed on all pregnancies conceived by ART and referred for fetal echocardiography to the Rasoul Akram and Akbar Abadi hospitals in Tehran, Iran. A total of 109 patients were enrolled in the study (56 in the ART group and 53 in the non-ART). Two-dimensional and color Doppler echocardiography were performed on all patients to identify heart problems and anomalies and medical records of the patients were reviewed. The outcome was considered the presence of functional and structural heart defects on echocardiography. Results The study groups were similar in terms of maternal age and GA. The ART group consisted of 31 singletons (55%) and 25 multiples (45%). All pregnancies in the non-ART group were singletons. Following in vitro fertilization (33%), ovulation induction (25%) was the next most used method. The findings of echocardiography were one atrial septal defect (ASD) in ART and one in non-ART, six ventricular septal defects (VSD) in ART and three in non-ART, and one ASD and VSD in the ART group. These structural abnormalities showed no difference in the two groups (P value = 0.58). There was no significant difference in rhythm between the two groups (P = 0.51). Echocardiographic indices of both groups did not differ statistically except in the TR-PG index (P value = 0.02). Conclusions The structural defects of the two groups were not different, and no heart dysfunction was observed in ART fetuses. There was no association between ART and CHD in our study. We concluded that echocardiography by pediatric cardiologists is not necessary for these fetuses.

Genetic testing with exome sequencing and genome sequencing is increasingly offered to infants and children with cardiovascular diseases. However, the rates of positive diagnoses after genetic testing within the different categories of cardiac disease and phenotypic subtypes of congenital heart disease (CHD) have been little studied. We report the diagnostic yield after next-generation sequencing in 500 patients with CHD from diverse population subgroups that were enrolled at three different sites in the Clinical Sequencing Evidence-Generating Research consortium. Patients were ascertained due to a primary cardiovascular issue comprising arrhythmia, cardiomyopathy, and/or CHD, and corresponding human phenotype ontology terms were selected to describe the cardiac and extracardiac findings. We examined the diagnostic yield for patients with arrhythmia, cardiomyopathy, and/or CHD and phenotypic subtypes of CHD comprising conotruncal defects, heterotaxy, left ventricular outflow tract obstruction, septal defects, and \"other\" heart defects. We found a significant increase in the frequency of positive findings for patients who underwent genome sequencing compared to exome sequencing and for syndromic cardiac defects compared to isolated cardiac defects. We also found significantly higher diagnostic rates for patients who presented with isolated cardiomyopathy compared to isolated CHD. For patients with syndromic presentations who underwent genome sequencing, there were significant differences in the numbers of positive diagnoses for phenotypic subcategories of CHD, ranging from 31.7% for septal defects to 60% for \"other\". Despite variation in the diagnostic yield at each site, our results support genetic testing in pediatric patients with syndromic and isolated cardiovascular issues and in all subtypes of CHD.

UNASSIGNED: Improve the efficiency of an inpatient clinical decision support tool (CDS) for patients with adult congenital heart disease (ACHD).
UNASSIGNED: The efficiency of a CDS was evaluated across two time periods and compared.
UNASSIGNED: An academic, tertiary care center.
UNASSIGNED: ACHD patients roomed in an inpatient setting.
UNASSIGNED: Plan-Do-Study-Act (PDSA) methods were applied starting in 2021 and included refinement of diagnostic codes and the addition of department encounter codes.
UNASSIGNED: True positive and false positive CDS alerts.
UNASSIGNED: Baseline data from 2017 had a median (IQR) of 38 (17) and 2019 baseline data had 65 (19) total alerts per month. Combining both baseline data years, the median true positive CDS alerts was 47.3 %. There were 71 (6) total alerts per month for the 2021-2022 time period and with ongoing PDSA cycles and optimization in the CDS the true positive alerts improved substantially resulting in a shifting of the median to 78.9 % within 9 months.
UNASSIGNED: CDS can efficiently notify providers when an ACHD patient is encountered. The use of ICD 10 codes alone to identify ACHD patients has limited accuracy with a high proportion of false positives. Ongoing revision of the CDS system methods is important to improving efficiency and minimizing provider alert fatigue.

Hypoplastic left heart syndrome (HLHS) is a congenital malformation commonly treated with palliative surgery and is associated with significant morbidity and mortality. Risk stratification models have often relied upon traditional survival analyses or outcomes data failing to extend beyond infancy. Individualized prediction of transplant-free survival (TFS) employing machine learning (ML) based analyses of outcomes beyond infancy may provide further valuable insight for families and healthcare providers along the course of a staged palliation. Data from both the Pediatric Heart Network (PHN) Single Ventricle Reconstruction (SVR) trial and Extension study (SVR II), which extended cohort follow up for five years was used to develop ML-driven models predicting TFS. Models incrementally incorporated features corresponding to successive phases of care, from pre-Stage 1 palliation (S1P) through the stage 2 palliation (S2P) hospitalization. Models trained with features from Pre-S1P, S1P operation, and S1P hospitalization all demonstrated time-dependent area under the curves (td-AUC) beyond 0.70 through 5 years following S1P, with a model incorporating features through S1P hospitalization demonstrating particularly robust performance (td-AUC 0.838 (95% CI 0.836-0.840)). Machine learning may offer a clinically useful alternative means of providing individualized survival probability predictions, years following the staged surgical palliation of hypoplastic left heart syndrome.

BACKGROUND: Infertility affects about 80 million individuals worldwide and 10-15% of couples at reproductive age will seek medical assistance. There is increasing evidence that pregnancies after Assisted Reproduction Techniques (ART) are associated with pre-term birth, low birthweight, congenital defects, and increased mortality rates. The aim of this review is to assess all the published literature and provide an updated review on the effect of assisted conception and perinatal and neonatal outcomes.
METHODS: Comprehensive research on Pubmed/Medline, Scopus, and Google scholar electronic databases was conducted from July 2023 up to September 2023, using the terms assisted reproductive techniques, ART, in vitro fertilization, IVF, intracytoplasmic sperm injection, ICSI, preterm birth, PTB, low birth weight, LBW, chromosomal defects, congenital defects, and hypospadias. In total, 87 full text articles were retrieved and after a careful evaluation, 31 studies were selected for data extraction.
RESULTS: Our review demonstrated a higher risk of congenital and chromosomal defects, and a higher incidence of male genital tract defects and heart defects in ART pregnancies. Regarding pre-term birth, our results were contradictory.
CONCLUSIONS: Although assisted reproduction techniques are associated with increased risks, they are safe regarding perinatal outcomes and couples should not be discouraged from utilizing them. Our results aim to alert clinicians to these specific outcomes and offer more personalized care and counseling to infertile couples and their children.

UNASSIGNED: Following prenatal diagnosis of critical congenital heart disease (CCHD), parents encounter emotional distress while facing caregiving challenges. Supportive psycho-educational interventions using mobile health (mHealth) can make care more accessible.
UNASSIGNED: We tested a novel nurse-guided mHealth care program, Preparing Heart and Mind™ (PHM™), with the objectives of examining feasibility and estimating the effect of the intervention on parents\' emotional distress.
UNASSIGNED: This pilot study design randomized participants using a 2:1 intervention to control ratio. Analysis involved description of retention, and intervention attendance and engagement, and adjusted linear mixed models to estimate group differences in depressive (CES-D), anxiety (STAI-S), and traumatic stress (IES-r) symptoms.
UNASSIGNED: The sample included 55 parents (n=38 PHM™ group, n=17 control). Complete retention of 37 (67%) parents included 29 (76%) in the PHM™ group and 8 (47%) control. Most attrition was due to infant death (7 parents), transplant referral (2 parents), or postnatal diagnostic ineligibility (4 parents). For the PHM™ group, ≥96% of parents attended pre- and postnatal sessions and most (65%) messaged with the nurse. mHealth engagement was highest prenatally, with handling uncertainty the most viewed topic (average 94% pages viewed). In linear mixed models analyses, the PHM™ group had on average 4.84 points lower depression (95% CI: -10.68-1.04), 6.56 points lower anxiety (-14.04-0.92), and 6.28 points lower trauma (-14.44-1.88) scores by study end.
UNASSIGNED: Findings suggest that a nurse-guided mHealth approach is feasible and may contribute to a clinically important reduction in parents\' emotional distress.

The interventions aimed at improving the levels of physical activity (PA) in children and adolescents diagnosed with heart disease did not produce the expected outcomes. Safe participation in sport activities proposed based on actual recommendations could be a solution to promote PA in this population. The aims of this study were to discover a causal diagram of sport participation in children and youth with heart disease and establish the factors that affect and are affected thereof through the use of questionnaires. Furthermore, the study aims to qualitatively assess the reliability of the constructed diagram in comparison with existing medical knowledge. The Greedy Fast Causal Inference method was employed to conduct a data-driven search of the directed acyclic graph that represents the causal relationships within the provided observational data. This causal discovery was performed using the Tetrad software. The analysis involved a cohort of 121 Caucasian patients (50 females) diagnosed with heart disease. The age range of the patients included in the study was 8-17 years. The study findings indicate that the participants engaged in sports presented significantly higher values of health-related quality of life (QoL) and motives for participating in physical and leisure activities. Age appears to be a cause of sport participation. Sport participation appears to be a cause of participation in physical education classes, which in turn appears to be a cause of higher enjoyment. Higher enjoyment appears to be a cause of other motives for participating in physical and leisure activities, as well as a higher score in terms of physical health. The causal diagram provided a graphical representation of the causal relationship between sport participation and better QoL with potential confounders for children and adolescents with heart disease that nearly coincided with the existing literature. Clinical trials should be designed to validate clinical utility of the presented causal diagram.