Aortic arch obstruction and/or coarctation of aorta is uncommon (5–20%) in balanced atrioventricular septal defects. Although technically challenging, prenatal diagnosis of aortic arch obstruction in atrioventricular septal defect is critical for delivery planning and improves prenatal counselling regarding the timing of cardiac surgery. We sought to identify prenatal predictors of coarctation of aorta in atrioventricular septal defect.

Retrospective review of patients prenatally diagnosed with atrioventricular septal defect at two institutions.

Ninety-five fetuses with atrioventricular septal defect were identified and sufficient outcome data and diagnostic acoustic windows were available in 62. Six patients (10%) had coarctation of aorta after birth. Among the 38 patients with Trisomy 21, four (11%) had coarctation of aorta. On multivariable analysis, the proximal transverse aortic arch z score and ratio of left:right atrioventricular valve were independent predictors of coarctation of aorta with good interobserver reproducibility. Either proximal transverse aortic arch z score < −2, or ratio of left:right atrioventricular valve <0.7, predicted aortic arch obstruction with 100% sensitivity and 89% specificity.

Proximal transverse aortic arch z score and lower ratio of left:right atrioventricular valve diameter are independent predictors of postnatal coarctation of aorta in fetal patients with atrioventricular septal defect. The next step is the prospective application of these parameters to create an algorithm directing fetal counselling in terms of delivery location, and expected timing of surgical interventions.

Paediatric patients with heart failure requiring ventricular assist devices are at heightened risk of neurologic injury and psychosocial adjustment challenges, resulting in a need for neurodevelopmental and psychosocial support following device placement. Through a descriptive survey developed in collaboration by the Advanced Cardiac Therapies Improving Outcomes Network and the Cardiac Neurodevelopmental Outcome Collaborative, the present study aimed to characterise current neurodevelopmental and psychosocial care practices for paediatric patients with ventricular assist devices.

Members of both learning networks developed a 25-item electronic survey assessing neurodevelopmental and psychosocial care practices specific to paediatric ventricular assist device patients. The survey was sent to Advanced Cardiac Therapies Improving Outcomes Network site primary investigators and co-primary investigators via email.

Of the 63 eligible sites contacted, responses were received from 24 unique North and South American cardiology centres. Access to neurodevelopmental providers, referral practices, and family neurodevelopmental education varied across sites. Inpatient neurodevelopmental care consults were available at many centres, as were inpatient family support services. Over half of heart centres had outpatient neurodevelopmental testing and individual psychotherapy services available to patients with ventricular assist devices, though few centres had outpatient group psychotherapy (12.5%) or parent support groups (16.7%) available. Barriers to inpatient and outpatient neurodevelopmental care included limited access to neurodevelopmental providers and parent/provider focus on the child’s medical status.

Paediatric patients with ventricular assist devices often have access to neurodevelopmental providers in the inpatient setting, though supports vary by centre. Strengthening family neurodevelopmental education, referral processes, and family-centred psychosocial services may improve current neurodevelopmental/psychosocial care for paediatric ventricular assist device patients.

To examine feasibility, acceptability, and preliminary effectiveness of a novel group-based telemedicine psychoeducation programme aimed at supporting psychological well-being among adolescents with Fontan-palliated CHD.

A 5-week telemedicine psychoeducation group-based programme (WE BEAT) was developed for adolescents (N = 20; 13–18 years) with Fontan-palliated CHD aimed at improving resiliency and psychological well-being. Outcome measures included surveys of resilience (Connor–Davidson Resilience Scale), benefit finding (Benefit/Burden Scale for Children), depression, anxiety, peer relationships, and life satisfaction (National Institutes of Health Patient-Reported Outcomes Measurement Information System scales). Within-subject changes in these outcomes were compared pre- to post-intervention using Cohen’s d effect size. In addition, acceptability in the form of satisfaction measures and qualitative feedback was assessed.

Among eligible patients reached, 68% expressed interest in study participation. Of those consented, 77% have been scheduled for a group programme to date with 87% programme completion. Twenty adolescents (mean age 16.1 ± SD 1.6 years) participated across five WE BEAT group cohorts (range: 3–6 participants per group). The majority (80%) attended 4–5 sessions in the 5-session programme, and the median programme rating was a 9 out of 10 (10 = most favourable rating). Following WE BEAT participation, resiliency (d = 0.44) and perceptions of purpose in life increased (d = 0.26), while depressive symptoms reduced (d = 0.36). No other changes in assessed outcome measures were noted.

These findings provide preliminary support that a group-based, telemedicine delivered psychoeducation programme to support psychological well-being among adolescents with CHD is feasible, acceptable, and effective. Future directions include examining intervention effects across diverse centres, populations, and implementation methods.

The microRNA-200 family plays a key role in inflammatory and vascular processes, making it a relevant target for Kawasaki disease, a vasculitis with coronary complications. This study aimed to evaluate the diagnostic potential of urinary exosomal microRNA-200 family members in Kawasaki disease patients.

Urine samples from 15 Kawasaki disease patients and 15 healthy controls underwent total exosome isolation and high-throughput sequencing. Differential expression of microRNA-200 family members was validated using quantitative real-time polymerase chain reaction. Diagnostic potential was assessed via receiver operating characteristic analysis, and correlations with clinical parameters were evaluated using Spearman correlation.

High-throughput sequencing identified upregulation of microRNA-429, microRNA-200b-3p/5p, microRNA-141-3p, microRNA-200a-3p/5p, and microRNA-200c-3p in Kawasaki disease patients. We confirmed significant upregulation of microRNA-200a-3p/5p, microRNA-200b-3p/5p, and microRNA-429, with receiver operating characteristic analysis showing high diagnostic potential for these microRNAs (area under the curves of 0.844, 0.791, 0.942, 0.842, and 0.898, respectively) and a combined analysis yielding a perfect area under the curve of 1.000. MicroRNA-141 and microRNA-200c-3p/5p, however, showed no significant diagnostic value. MicroRNA-200a-3p and microRNA-200a-5p were positively correlated with white blood cells, platelet counts, and C-reactive protein, while microRNA-200b-3p and microRNA-429 were positively correlated with white blood cells, platelet counts, erythrocyte sedimentation rate, and C-reactive protein. microRNA-200b-5p showed moderate correlations with platelet counts and erythrocyte sedimentation rate.

Urinary exosomal microRNA-200 family members, especially microRNA-200a-3p/5p, microRNA-200b-3p/5p, and microRNA-429, demonstrate strong diagnostic potential for Kawasaki disease, correlating with key inflammatory markers. MicroRNA-141 and microRNA-200c did not demonstrate significant diagnostic utility.

Giant coronary artery aneurysms and myocardial fibrosis after Kawasaki disease may lead to devastating cardiovascular outcomes. We characterised the vascular and myocardial outcomes in five selected Kawasaki disease patients with a history of giant coronary artery aneurysms that completely regressed.

Five patients were selected who had giant coronary artery aneurysm in early childhood that regressed when studied 12–33 years after Kawasaki disease onset. Coronary arteries were imaged by coronary CT angiography, and coronary artery calcium volume scores were determined. We used endocardial strain measurements from CT imaging to assess myocardial regional wall function. Calprotectin and galectin-3 (gal-3) as biomarkers of inflammation and myocardial fibrosis were measured by enzyme-linked immunosorbent assay.

The five selected patients with regressed giant coronary artery aneurysms had calcium scores of zero, normal levels of calprotectin and gal-3, and normal appearance of the coronary arteries by coronary computed tomography angiography. CT strain demonstrated normal peak systolic and diastolic strain patterns in four of five patients. In one patient with a myocardial infarction at the time of Kawasaki disease diagnosis at the age of 10 months, CT strain showed altered global longitudinal strain, reduced segmental peak strain, and reduced diastolic relaxation patterns in multiple left ventricle segments.

These patients illustrate that regression of giant aneurysms after Kawasaki disease is possible with no detectable calcium, normal biomarkers of inflammation and fibrosis, and normal myocardial function. Individuals with regressed giant coronary artery aneurysm still require longitudinal surveillance to assess the durability of this favourable outcome.

To assess whether isolated very low QRS voltage of ≤0.3 mV in the frontal leads might be a marker for diagnosing paediatric vasovagal syncope and the risk of recurrence.

We included 118 children with vasovagal syncope, comprising 70 males and 48 females in our retrospective analysis. All patients underwent head-up tilt test and supine 12-lead electrocardiography. Furthermore, the QRS voltage was measured from each one of the 12 leads on basal electrocardiography. Patients were followed up for 6–24 months (average, 16 months).

Eighty-six patients (73%) patients displayed isolated very low QRS voltage in frontal leads. Furthermore, the patients were classified into two groups based on the presence or absence of isolated very low QRS voltage. Enhanced syncopic spells over the past 6 months, and the positive rates of head-up tilt test were observed in patients having isolated very low QRS voltage in the frontal leads than those without isolated very low QRS voltage (p < 0.05). The single factor and time-to-event analyses also showed an increased syncope recurrence rate in patients with isolated very low QRS voltage in frontal leads when compared with those without isolated very low QRS voltage (p < 0.01).

Isolated very low QRS voltage in frontal leads is correlated with the positive response of the head-up tilt test in children who experience syncope and its recurrence post-treatment. Hence, isolated very low QRS voltage in frontal leads might become a novel diagnostic indicator and a risk factor for syncope recurrence in children with vasovagal syncope.

Giant coronary artery aneurysms are rare but potentially fatal complications of Kawasaki disease. The lack of evidence-based recommendations on their management and treatment cause guidelines and practices to differ. We aimed to assess these variations.

An anonymous online survey regarding surveillance, imaging, pharmacological management, and interventional practices was distributed among 134 physicians attending to Kawasaki disease patients worldwide. A p-value of <0.05 was deemed significant.

The majority (60%) of respondents were general paediatric cardiologists, and 29% interventional specialists. The average years in practice was 15 ± 9.6. Physicians from Asia had the most experience with giant coronary artery aneurysms. American practitioners preferred combining anticoagulants with aspirin. Beta-blockers and statins were more likely used in teenagers versus younger children. Cardiac catheterisation was most (52%) chosen for coronary surveillance in patients with echocardiogram anomalies, followed by Coronary CT-angiography. The indications for coronary intervention were split among respondents, regardless of geographic region or experience. The preferred treatment of coronary stenosis was percutaneous intervention (69%) versus bypass surgery. For thrombosis, thrombolytics (50%) were preferred over percutaneous (39%) and surgical (11%) interventions. Most (92%) preferred intervening in young children in a paediatric facility but were split between a paediatric and adult facility for older children. Most chose combined management by adult and paediatric specialists for either age-scenarios (70, 82%).

As identified by our study, the lack of large studies and evidence-based recommendations cause uncertainty and ambivalence towards certain treatments. International collaborative efforts are needed to provide more robust evidence in the management of these patients.

Endomyocardial biopsy remains the gold standard for cardiac cellular rejection surveillance after heart transplantation. We studied a novel non-invasive index of left ventricular relaxation to detect cardiac cellular rejection in paediatric heart transplant patients.

This is a single-centre retrospective study of paediatric heart transplant patients who underwent endomyocardial biopsy from June 2014 to September 2021. Left ventricular relaxation index was calculated as the sum of diastolic tissue Doppler imaging velocities (E) of the left ventricular lateral, septal, and posterior walls divided by the percentage of the left ventricular posterior wall thinning by M-mode. Statistical analysis included t-tests and Mann-Whitney tests to compare means and medians between treatment and non-treatment groups. We used the cut-off with the maximum Youden index to compare the sensitivity and specificity of left ventricular relaxation index to detect rejection.

The study included 65 patients who underwent 246 cardiac catheterizations and endomyocardial biopsies. Out of 246, 192 procedures were included and 54 were excluded due to recent transplants or lack of echocardiographic data. A total of 114 demonstrated Grade 0R, 68 Grade 1R, 8 Grade 2R, and 2 Grade 3R allograft rejection. The difference in mean left ventricular relaxation index between treatment versus non-treatment groups (2R, 3R vs. 0R, 1R) was not statistically significant (p = 0.917). A left ventricular relaxation index cut-off of 0.73 had the highest Youden index with good sensitivity (100%) and poor specificity (23%) for detecting rejections with grades 2R and 3R.

Left ventricular relaxation index, a novel index of left ventricular relaxation, was not a sensitive or specific predictor of cardiac cellular rejection in paediatric heart transplants.

The functional roles of ventricular dominance and additional ventricular chamber after Fontan operation are still uncertain. We aim to assess and correlate such anatomical features to late clinical outcomes.

Fontan patients undergoing cardiac MRI and cardiopulmonary exercise test between January 2020 and December 2022 were retrospectively reviewed. Clinical, cardiac MRI, and cardiopulmonary exercise test data from the last follow-up were analysed.

Fifty patients were analysed: left dominance was present in 29 patients (58%, median age 20 years, interquartile range:16–26). At a median follow-up after the Fontan operation was 16 years (interquartile range: 4–42), NYHA classes III and IV was present in 3 patients (6%), 4 (8%) underwent Fontan conversion, 2 (4%) were listed for heart transplantation, and 2 (4%) died. Statistical analysis showed that the additional ventricular chamber was larger (>20 mL/m2) in patients with a right dominant ventricle (p = 0.01), and right dominance was associated with a higher incidence of post-operative low-cardiac output syndrome (p = 0.043). Left ventricular dominance was associated with a better ejection fraction (p = 0.04), less extent of late gadolinium enhancement (p = 0.022), higher metabolic equivalents (p = 0.01), and higher peak oxygen consumption (p = 0.033). A larger additional ventricular chamber was associated with a higher need for post-operative extracorporeal membrane oxygenation support (p = 0.007), but it did not influence functional parameters on cardiac MRI or cardiopulmonary exercise test.

In Fontan patients, left ventricular dominance correlated to better functional outcomes. Conversely, a larger additional ventricular chamber is more frequent in right ventricular dominance and can negatively affect the early post-Fontan course.

In recent years, the importance of telemedicine has increased significantly. Especially in the field of echocardiography, virtual reality glasses offer the possibility of real-time data transmission without restrictions in the examination process. In particular, the care of critically ill newborns with suspected CHD might be improved by allowing a specialized paediatric cardiologist to remotely guide an echocardiographic examination. The current study aims to prove whether novices, under Google Glass guidance by a paediatric cardiologist, can perform an appropriate neonatal echocardiography.

The current study is a prospective monocentric single-blinded pilot study. Participants were supposed to perform two test runs: The first test run was “unguided” and the second test run was instructed via Google Glass. A validated training simulator for neonatal echocardiography “EchocomNeo, Echocom GmbH” was used. The study took place at the Leipzig Heart Center, Department of Pediatric Cardiology from April 2022 to November 2022.

A total of 21 medical students were enrolled. In total 252 views (126 views in each test run) were recorded. The overall performance was significantly higher in the Google Glass guided test run compared to “unguided” (structure score: 77.6% vs. 63.2%. p < 0.001 and quality score: 58.7% vs. 47.2%, p < 0.001). Also, the time was significantly lower in the Google Glass guided test run than in the unguided test run, p = 0.014.

Google Glass guidance by a paediatric cardiologist could optimize the performance of novices in echocardiography using a standardized neonatal echo-simulator with structural normal cardiac anatomy.

Lack of psychological safety within surgical teams and fear of damaging one’s career or reputation may counteract the effect of formal reporting as a tool for preventing bullying. Also, personality traits of cardiothoracic surgeons may promote bullying. In the current study, we evaluate these factors and their association with bullying in cardiothoracic surgical teams.

Forty-four cardiothoracic surgeons filled in the Negative Acts Questionnaire-Revised, Safety Attitude Questionnaire, and Big Five Inventory.

Of the 44 participants, 18 (40.90%) experienced bullying during their careers. Psychological safety was negatively associated with work-related bullying and positively associated with the agreeableness and openness personality factors.

Bullying within the cardiothoracic surgical teams is a prominent problem that is negatively associated with psychological safety. Psychological safety is positively associated with agreeableness and openness, and interventions focusing on promoting an organizational culture based on such factors may improve the effectiveness of such interventions.

Interstage monitoring programs for single ventricle disease have been developed to reduce morbidity and mortality. There is increased use of telemedicine and mobile application monitoring. It is unknown if there are disparities in use based on patient socio-demographic factors.

We conducted a retrospective cohort study of patients enrolled in the single ventricle monitoring program and KidsHeart application at a single centre from 4/21/2021 to 12/31/2023. We investigated the association of socio-demographic factors with telemedicine usage, mobile application enrollment and usage. We assessed resource utilisation and weight changes by program era.

There were 94 children in the cohort. Patients with Norwood and ductal stent had higher mean telemedicine visits per month (1.8 visits, p = 0.004), without differences based on socio-demographic factors. There were differences in application enrollment with more Black patients enrolled compared to White patients (p = 0.016). There were less Hispanic patients enrolled than Non-Hispanic patients (p = 0.034). There were no Spaish speaking patient’s enrolled (p = 0.0015). There were no patients with maternal education of less than high school enrolled and all those with maternal education of advanced degree were enrolled (p = 0.0016). There was decreased mobile application use in those from neighbourhoods in the lowest income quartile. There were decreased emergency department visits with mobile application monitoring. Mean weight-for-age z-scores had increased from start to completion of the program in all eras.

Differences were seen in mobile application enrollment and usage based on socio-demographic factors. Further work is needed to ensure that all patients have access to mobile application usage.