OBJECTIVE: Currently, nearly 90% of patients with congenital heart disease (CHD) reach adulthood in relatively good health. Structured transition programs have emerged to support adolescents and young adults in transitioning to adult care structures, improve their autonomy, and limit healthcare ruptures. The TRANSITION-CHD randomized controlled trial aimed to assess the impact of a transition program on health-related quality of life (HRQoL) in adolescents and young adults with CHD.
METHODS: From January 2017 to February 2020, 200 subjects with a CHD, aged 13-25 years, were enrolled in a prospective, controlled, multicenter study and randomized in two balanced groups (transition program vs. standard of care). The primary outcome was the change in PedsQL self-reported HRQoL score between baseline and 12-month follow-up, using an intention-to-treat analysis. The secondary outcomes were the change in disease knowledge, physical health (cardiopulmonary fitness, physical activity), and mental health (anxiety, depression).
RESULTS: The change in HRQoL differed significantly between the transition group and the control group (mean difference = 3.03, 95% confidence interval (CI) = [0.08; 5.98]; p = .044; effect size = 0.30), in favor of the intervention group. A significant increase was also observed in the self-reported psychosocial HRQoL (mean difference = 3.33, 95% CI = [0.01; 6.64]; p = .049; effect size = 0.29), in the proxy-reported physical HRQoL (mean difference = 9.18, 95% CI = [1.86; 16.51]; p = .015; effect size = 0.53), and in disease knowledge (mean difference = 3.13, 95% CI = [1.54; 4.72]; p < .001; effect size = 0.64).
CONCLUSIONS: The TRANSITION-CHD program improved HRQoL and disease knowledge in adolescents and young adults with CHD, supporting the generalization and systematization of similar preventive interventions in pediatric and congenital cardiology.

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.

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.

Experimental evidence has indicated a correlation between in-utero exposure to neonicotinoid pesticides (NEOs) and adverse birth outcomes in mammals. However, the distribution of NEO exposure during human pregnancy, as well as its association with congenital heart diseases (CHDs), the most common birth defects, are unclear. Our purpose was to explore the distribution of and contributing factors to NEO exposure in pregnant women during early-mid pregnancy and to assess the associations between NEOs and CHDs. This nested case-control study was conducted within an ongoing prospective birth cohort study and enrolled 141 CHD singletons and their 282 individually matched controls. Six \"parent\" NEOs and three NEO metabolites were measured in maternal serum collected at an average gestational age of 16 weeks, using liquid chromatography-tandem mass spectrometry. Logistic regression was used to quantify the NEOs-CHDs associations and explore potential contributing factors to serum NEO levels in controls. N-desmethyl acetamiprid (N-dm-ACE) and imidacloprid (IMI) were the most frequently detected NEOs, found in 100% and 20% of maternal sera, respectively. We did not find a statistically significant association between total NEOs and overall CHDs. However, there was a trend towards a higher risk of septal defects with greater serum NEOs (ORs ranged from 1.80 to 2.36), especially nitro-containing NEOs represented by IMI. Pregnant women with lower education had elevated serum total NEOs compared to women with higher education (OR = 48.39, 95% CI: 23.48-99.72). Pregnant women were primarily exposed to N-dm-ACE and IMI during early-mid pregnancy. Gestational exposure to NEOs may be associated with an increased risk of septal defects, but the evidence is limited at present. Education is a potential contributing factor to NEO exposure in pregnant women. Larger and more precise studies with longitudinal biospecimen collection, are recommended to validate our exploratory findings.

OBJECTIVE: Patients with congenital heart disease (CHD) increasingly live into adulthood, often requiring cardiac reoperation. We aimed to assess the outcomes of adults with CHD (ACHD) undergoing repeat sternotomy at our institution.
METHODS: Review of our institution\'s cardiac surgery database identified 1960 ACHD patients undergoing repeat median sternotomy from 1993 to 2023. The primary outcome was early mortality, and the secondary outcome was a composite end point of mortality and significant morbidity. Univariable and multivariable logistic regression models were used to determine factors independently associated with outcomes.
RESULTS: Of the 1960 ACHDs patient undergoing repeat sternotomy, 1183 (60.3%) underwent a second, third (n = 506, 25.8%), fourth (n = 168, 8.5%), fifth (n = 70, 3.5%), and sixth sternotomy or greater (n = 33, 1.6%). CHD diagnoses were minor complexity (n = 145, 7.4%), moderate complexity (n = 1380, 70.4%), and major complexity (n = 435, 22.1%). Distribution of procedures included valve (n = 549, 28%), congenital (n = 625, 32%), aortic (n = 104, 5.3%), and major procedural combinations (n = 682, 34.7%). Overall early mortality was 3.1%. Factors independently associated with early mortality were older age at surgery, CHD of major complexity, preoperative renal failure, preoperative ejection fraction, urgent operation, and postoperative blood transfusion. In addition, sternotomy number and bypass time were independently associated with the composite outcome.
CONCLUSIONS: Despite the increase in early mortality with sternotomy number, sternotomy number was not independently associated with early mortality but with increased morbidity. Improvement strategies should target factors leading to urgent operations, early referral, along with operative efficiency including bypass time and blood conservation.

Neonates who require surgery for congenital heart disease (CHD) frequently have difficulty with oral feeds post-operatively and may require a feeding tube at hospital discharge. The purpose of this study was to determine the effect of oral or nasal intubation route on feeding method at hospital discharge. This was a non-blinded randomized control trial of 62 neonates who underwent surgery for CHD between 2018 and 2021. Infants in the nasal (25 patients) and oral (37 patients) groups were similar in terms of pre-operative risk factors for feeding difficulties including completed weeks of gestational age at birth (39 vs 38 weeks), birthweight (3530 vs 3100 g), pre-operative PO intake (92% vs 81%), and rate of pre-operative intubation (22% vs 28%). Surgical risk factors were also similar including Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery category (3.9 vs 4.1), shunt placement (32% vs 41%), cardiopulmonary bypass time (181 vs 177 min), and cross-clamp time (111 vs 105 min). 96% of nasally intubated patients took full oral feeds by discharge as compared with 78% of orally intubated infants (p = 0.05). Nasally intubated infants reach full oral feeds an average of 3 days earlier than their orally intubated peers. In this cohort of patients, nasally intubated infants reach oral feeds more quickly and are less likely to require supplemental tube feeding in comparison to orally intubated peers. Intubation route is a potential modifiable risk factor for oral aversion and appears safe in neonates. The study was approved by the University of Virginia Institutional Review Board for Health Sciences Research and was retrospectively registered on clinicaltrials.gov (NCT05378685) on May 18, 2022.

The European-funded Health Effects of Cardiac Fluoroscopy and Modern Radiotherapy in Pediatrics (HARMONIC) project is a multicenter cohort study assessing the long-term effects of ionizing radiation in patients with congenital heart disease. Knowledge is lacking regarding the use of ionizing radiation from sources other than cardiac catheterization in this cohort.
This study aims to assess imaging frequency and radiation dose (excluding cardiac catheterization) to patients from a single center participating in the Norwegian HARMONIC project.
Between 2000 and 2020, we recruited 3,609 patients treated for congenital heart disease (age < 18 years), with 33,768 examinations categorized by modality and body region. Data were retrieved from the radiology information system. Effective doses were estimated using International Commission on Radiological Protection Publication 60 conversion factors, and the analysis was stratified into six age categories: newborn; 1 year, 5 years, 10 years, 15 years, and late adolescence.
The examination distribution was as follows: 91.0% conventional radiography, 4.0% computed tomography (CT), 3.6% diagnostic fluoroscopy, 1.2% nuclear medicine, and 0.3% noncardiac intervention. In the newborn to 15 years age categories, 4-12% had ≥ ten conventional radiography studies, 1-8% underwent CT, and 0.3-2.5% received nuclear medicine examinations. The median effective dose ranged from 0.008-0.02 mSv and from 0.76-3.47 mSv for thoracic conventional radiography and thoracic CT, respectively. The total effective dose burden from thoracic conventional radiography ranged between 28-65% of the dose burden from thoracic CT in various age categories (40% for all ages combined). The median effective dose for nuclear medicine lung perfusion was 0.6-0.86 mSv and for gastrointestinal fluoroscopy 0.17-0.27 mSv. Because of their low frequency, these procedures contributed less to the total effective dose than thoracic radiography.
This study shows that CT made the largest contribution to the radiation dose from imaging (excluding cardiac intervention). However, although the dose per conventional radiograph was low, the large number of examinations resulted in a substantial total effective dose. Therefore, it is important to consider the frequency of conventional radiography while calculating cumulative dose for individuals. The findings of this study will help the HARMONIC project to improve risk assessment by minimizing the uncertainty associated with cumulative dose calculations.

UNASSIGNED: The study aimed to monitor fetuses with tetralogy of Fallot (TOF) after prenatal counseling and how it influenced the decision of parents to terminate the pregnancy.
UNASSIGNED: Fetuses with isolated TOF diagnosed between January 2019 and December 2021 were prospectively enrolled. The follow-up period extended until termination or 6 months after the operation.
UNASSIGNED: Of the 1,026 fetuses diagnosed with cardiac defects, 129 were identified to have isolated TOF and completed the follow-up. A total of 55 (42.6%) fetuses were terminated, with larger maternal age (odds ratio: 0.893, 95% confidence interval: 0.806-0.989, P = 0.031) as the protective factor. The maternal anxiety score, gestational weeks, and pulmonary-to-aortic-diameter ratio lost significance in multivariate analysis. Subjectively, the two most common reasons for terminating the pregnancy were worries about the prognosis (41.8%) and concerns about the possible suffering of the unborn child (18.2%). The prenatal diagnosis was accurate in 73 of the 74 (98.6%) live births. Out of the 64 live births that underwent surgical repair in our center, 57 (89.1%) received primary repair, with a median age of 104 days, and 49 (76.6%) underwent valve-sparing repair. No perioperative death occurred.
UNASSIGNED: Termination for fetuses with TOF remains common in China. Live births with TOF can be safely and effectively managed.

Cardiac malformations are sporadically diagnosed in domestic species; however, little literature is available for this group of developmental anomalies in goats. We performed a retrospective study to catalog congenital cardiac conditions in goats submitted to the University of California-Davis, Veterinary Medical Teaching Hospital, Anatomic Pathology Autopsy Service. From 2000 to 2021, of 1,886 goat autopsies, 29 cases of cardiac malformations were identified (1.5%). Thirteen were ≤ 2-wk-old, 8 were 1-6-mo-old, and 8 were adults 2-9-y-old. The most common malformations were ventricular septal defect (VSD; 21 of 29), atrial septal defect or persistent foramen ovale (10 of 29), and double-outlet right ventricle (3 of 29). Nine cases had > 1 malformation, typically including a VSD. Conditions that had not been reported in the goat included double-outlet right ventricle (3), tetralogy of Fallot (1), cor triatriatum sinister (1), and mitral valve dysplasia (1). Two adult cases were incidental and not suspected clinically. Cardiac malformations occur not uncommonly in goats and should be considered in a wide age range.