Studying the effect of interleukin-17 (IL-17) on the mechanism of CD4+ T-cell immune regulation and the Janus kinase/signal transducer and activator of transcription (JAK/STAT) pathway may offer new ideas and methods for the therapy of dilated cardiomyopathy.

Naive CD4+ T cells were isolated from mice using a magnetic bead sorting reagent and manipulated by overexpression or knockdown of IL-17. Protein levels of Janus kinase 2 (JAK2), phosphorylated JAK2 (p-JAK2), signal transducer and activator of transcription 3 (STAT3), phosphorylated STAT3 (p-STAT3), matrix metalloproteinase-2 (MMP-2), and matrix metalloproteinase-9 (MMP-9) were determined by Western blotting. Quantitative polymerase chain reaction was used to assess the levels of JAK2, STAT3, MMP-2, and MMP-9. Expression of tumour necrosis factor-alpha (TNF-α), interleukin-1 beta (IL-1β), interleukin-4 (IL-4), and interferon-gamma (IFNγ) was determined by enzyme-linked immunosorbent assay test kits. TNF-α, IL-1β, IL-4, and IFNγ secretion was measured by flow cytometry.

In CD4+ T cells, IL-17 overexpression increased TNF-α, IL-1β, IL-4, IFNγ, p-JAK2, p-STAT3, MMP-2, MMP-9 levels, and apoptosis. Knockdown of IL-17 reduced the levels of TNF-α, IL-1β, IL-4, IFNγ, p-JAK2, p-STAT3, MMP-2, and MMP-9, as well as the level of apoptosis.

Through regulation of IL-17 expression in CD4+ T cells, this study reveals its crucial role in regulating the secretion of inflammatory factors, activation of the JAK/STAT signaling pathway, expression of matrix metalloproteinases, and apoptosis of CD4+ T cells.

The COVID-19 pandemic presented unique challenges to global healthcare. Face-to-face outpatient care was dramatically reduced. This study implemented a remote consultation service via a mobile app (Pexip) to monitor patients with major CHD.

Study design was quasi-experimental and prospective.

Remote consultations were carried out at a tertiary paediatric cardiology centre in Northern Ireland.

Children with major CHD aged 0–16years in Northern Ireland.

The intervention was a Pexip-enabled remote consultation.

Primary outcome measures included the number of attendances to hospital both initiated and avoided via remote consultation. Remote consultations were conducted by doctor and/or cardiac specialist nurse or by specialist nurse alone (52% vs. 48%).

In the study, 32 patients enrolled; three were non-responders and a further two excluded. There were 201 remote consultations delivered (mean = 7.4). There were 12 admissions to hospital resulting from the remote consultation; the commonest indication was abnormal oxygen saturations (42%). There were 38 hospital attendances avoided, predominantly related to infant feeding and medication advice (both 42%).

A significant number of unnecessary hospital attendances were avoided (n = 38). Remote consultation technology proved a user-friendly and valuable adjunct to the provision of ongoing specialist patient care in challenging circumstances. There was a reduction in parental anxiety, and both parents and clinicians found this initiative beneficial to patient care. There was prompt identification of unwell children on remote consultations.

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.

High takeoff of the right coronary artery suspected by echocardiography is widely considered a normal variant. However, in our experience, some patients initially thought to have a high takeoff of the coronary artery were later found to have an anomalous coronary origin with high-risk features. The aim of this study was to test the hypothesis that high takeoff of the right coronary artery suspected by echocardiography may indicate the presence of an anomalous coronary artery lesion with an intramural course requiring further investigation.

A retrospective chart review was performed of patients evaluated at the University of Florida Congenital Heart Center from January 2010 through September 2015. Charts of all 62 patients who were noted to have an anomalous coronary artery or concern for an anomalous coronary artery were reviewed to identify those who were initially identified as having simply a high takeoff of the right coronary artery by initial echocardiogram. A total of 24 patients met these criteria.

Out of 24 patients identified as having high takeoff of the right coronary artery on their initial echocardiogram, 20 had confirmatory computerized tomographic angiography. On review of these patients, 9 had a right coronary origin from the left. This included 3 patients with an anomalous right coronary artery from the left coronary sinus and 6 with an anomalous right coronary artery origin just above the left coronary sinus. Six of these 9 patients had an intramural course. The remaining patients had high takeoff above the right coronary sinus or normal coronary origins.

Additionally, on review of all patients with computerized tomographic angiographic confirmation of high takeoff of the coronary artery, those with high takeoff above the left coronary sinus were more likely to have an intramural course (6 out of 9). Meanwhile, none of the 6 patients with high takeoff above the right coronary sinus confirmed by computerized tomographic angiography had an intramural course.

Accurate identification of the coronary origin and course of the anomalous coronary artery is difficult by echocardiogram. Correct diagnosis of origin and course is important for appropriate risk stratification and treatment decisions. Therefore, patients with high takeoff of the right coronary artery suspected by echocardiography should undergo additional evaluation to assess for the presence of a potentially malignant course.

Ebstein’s anomaly represents 40% of congenital tricuspid valve abnormalities. Studies about paediatric Ebstein’s anomaly patients are limited.

To evaluate clinical characteristics, treatment (medical/arrhythmia ablation/surgical) results, and outcome of Ebstein’s anomaly patients, and to determine factors affecting arrhythmia presence and mortality.

Clinical data, echocardiography, treatment results, and outcomes of patients followed in our centre between 2000 and 2017 were retrospectively evaluated.

A total of 79 patients (61 children, median diagnosis age: 1.5 years [1 day–24 years]) were included. Eight patients (10.1%) were deceased during the study period. Common associated anomalies were atrial septal defect/patent foramen ovale (56.9%), mitral regurgitation (25.3%), pulmonary stenosis/atresia (17.7%), and ventricular septal defect (16.5%). Genetic diseases/congenital anomalies were present in 5/3.8%. Tricuspid regurgitation was present in 75.9%, and severe in 50%. Arrhythmias were detected in 30.4%, and accessory pathway-mediated re-entrant tachycardia was the most common (67%). Wolff-Parkinson-White syndrome was present in 12.7%. Twenty-one ablation procedures (radiofrequency ablation [85.7%]/cryoablation [14.3%]) were performed in 16 patients (median age: 13.3 years [4.9–17]). Acute success/recurrence rates: 87.5/25%. Surgery was performed in 31.6% (median age: 6.5 years [4 days–29 years]), 7.6% were operated during the first month, and 12.6% during the first year. Second surgery was required in 28%. Perioperative mortality rate was 12%, and median mortality age was 25 days (1 day–17 years). Median follow-up period was 5.3 years (1 day–32 years). Older diagnosis age (p = 0.005) and mild-moderate mitral regurgitation (p = 0.036) were associated with arrhythmias. Younger age at diagnosis (p = 0.012), younger age at first surgery (p = 0.004), surgery before age three years (p = 0.037), and presence of pulmonary atresia (p = 0.000014) were associated with mortality. Gender, diagnosis age, congenital anomalies/genetic disorders, tricuspid regurgitation, arrhythmias, and surgery history did not have an independent effect on survival.

In children and young adults presenting with Ebstein’s anomaly, younger age at presentation and at surgery, surgery before age three years, pulmonary atresia were associated with death. Ablation procedures can be successfully performed but recurrence rate is still high.

The COVID pandemic has had deleterious effects on the mental health of the global population. Parents of children with CHD were particularly vulnerable to negative mental health outcomes such as depression, anxiety, and perceived stress. A better understanding of the CHD parent experiences, needs, and concerns while navigating the healthcare system during a pandemic is needed.

Online survey responses from 71 parents of young children with CHD representing families across the United States of America and Canada were analysed. Qualitative data were collected one year into the COVID pandemic. Thematic analysis was used to examine responses to the open-ended question “What would you like healthcare professionals (doctors, nurses) to know about your experience of being a parent with a child with CHD during the COVID-19 pandemic?.”

Two major themes with subthemes and an umbrella theme emerged from the parents’ responses (1) Pandemic Parenting: The Emotional Toll of Hospital Visitation Restrictions, Dealing with Social Distancing, Feeling Isolated, Decision Making in Uncertainty, and Playing it Safe versus Returning to Normal and (2) Unmet Expectations of Care: Needing Information, Wanting Empathy, Requesting Respect, Questioning Care Quality, and the umbrella theme of: Our Lives were Turned Upside Down.

CHD parents describe a negative impact of healthcare-related challenges during the COVID pandemic. These findings may offer insight to how healthcare professionals can better support the mental health and care burden of CHD parents during future pandemics.

The Wolff–Parkinson–White pattern is a delta wave frequently detected in school-based cardiovascular screening programs in Japan. Although most children with Wolff–Parkinson–White pattern are asymptomatic, initial symptoms may include syncope or sudden death, necessitating accurate diagnosis and management. Delta waves can also indicate a fasciculoventricular pathway, which poses no risk and does not require management.

We reviewed the medical records of patients referred to our hospital between April 2008 and March 2022 to evaluate the electrocardiographic signs of the Wolff–Parkinson–White pattern. The existence of Wolff–Parkinson–White syndrome and fasciculoventricular pathway were determined based on atrioventricular block and QRS waveform changes after adenosine administration during sinus rhythm.

The study cohort included 127 children (65 males; median age: 12.8 years, resting heart rate: 75 beats/min, PR interval: 109 ms, and QRS duration: 101 ms). The adenosine administration test revealed a fasciculoventricular pathway, Wolff–Parkinson–White syndrome, and indeterminate findings in 64, 54, and 9 children, respectively. More than 60% of children with a QRS duration ≤ 120 ms had a fasciculoventricular pathway. Age ≤ 12 years, QRS duration >120 ms, and type A category (children with R/S ratios >1 in lead V1) were identified as independent risk factors for Wolff–Parkinson–White syndrome. No adverse events were observed in any child.

The adenosine administration test is safe and feasible for differentiating Wolff–Parkinson–White syndrome from fasciculoventricular pathways and can reduce the unnecessary management of children with fasciculoventricular pathways.

We sought to examine the relative importance of surgical lesion complexity versus the presence of genetic/syndromic/extracardiac anomalies (GSAs) in determining survival, morbidity or need for reinterventions following repair for aortic arch hypoplasia.

A single-centre, retrospective cohort study of infants undergoing biventricular aortic arch repair via sternotomy from 2010 to 2021 was conducted. Survival analysis was performed using Kaplan–Meier methods, with additional Bayesian survival modelling for subgroups. Composite morbidity comprised respiratory, renal, neurologic, or sepsis-related complications.

Of 83 included infants, n = 13/83 (15.7%) had complex repairs; 27/83 (32.5%) were GSA+. Operative mortality was significantly higher in GSA+ versus GSA− patients (18.5% vs. 1.8%; p = 0.01), though not for complex versus non-complex repairs. Overall 10-year Kaplan–Meier survival was 86.7%. Bayesian modelling suggested equivalent post-discharge attrition in non-complex/GSA+ and complex/GSA− patients, with the poorest outcomes in complex/GSA+ patients; non-complex/GSA− patients had 100% survival. GSA+ patients exhibited higher composite morbidity (44.4% vs. 7.1% in GSA− p < 0.001), with their mode of death seemingly related to a high incidence of respiratory and neurological morbidity, notably in Dandy–Walker syndrome. The 10-year freedom from arch reinterventions was 87.7%; neither complexity, GSA status, nor post-repair peak arch velocity predicted the need for arch reinterventions.

Whilst anatomic complexity may have been somewhat neutralised as a risk factor for operative mortality, in contrast to GSA+ status, there is further post-discharge attrition attributable to complexity or GSA+ status, with additive risk effects. Morbidity directly related to certain syndromes underlies some of this risk. Non-anatomic substrates represent a persistent limitation to outcomes of surgical aortic arch repair in infants.

Risk stratification is recommended for patients with ventricular pre-excitation, particularly when sports eligibility is required. Few studies have examined the changes in the electrophysiological properties of the accessory pathway during growth. This study investigates the evolution of electrophysiological properties of the ventricular pre-excitation in young athletes referred for sports eligibility.

Between January 2011 and July 2022, 44 paediatric patients (32 males; mean age, 10 ± 2.42) with ventricular pre-excitation underwent an electrophysiological study, both at rest and during adrenergic stress at two different times (T0 and T1) within a minimal interval of 2 years. Transcatheter ablation was not performed between the two electrophysiological studies. Electrophysiological data were collected and compared.

Electrophysiological study under basal conditions showed a significant decrease in the anterograde accessory pathway effective refractory period and 1:1 conduction over the accessory pathway from T0 to T1. The shortest pre-excited R-R interval during atrial fibrillation did not significantly change at the basal condition; however, it decreased during the stress test. Furthermore, six patients (13.6%) changed the risk profile of their accessory pathway: two “high-risk” patients at T0 became “low-risk” and four “low-risk” patients became “high-risk” at T1. Atrioventricular re-entry tachycardia inducibility did not differ significantly between the two electrophysiological studies.

This study highlights the importance of repeating electrophysiological study (transesophageal or intracardiac) in paediatric athletes with ventricular pre-excitation because significant and clinically relevant changes in the conduction and refractoriness of accessory pathway can occur. This could influence risk stratification for sports eligibility and the correct indication and timing for accessory pathway ablation.

To evaluate the influence of pre-existing maternal diabetes mellitus on fetal myocardial performance index and systolic-to-diastolic duration ratio.

Prospective cohort study included 179 pregnant women between 20 and 36w6d, divided into 3 groups: Group 1 (120, normal), Group 2 (31, type 1 diabetes mellitus), and Group 3 (28, type 2 diabetes mellitus). Systolic-to-diastolic duration ratio was calculated as the sum of isovolumic contraction time and ejection time divided by the sum of isovolumic relaxation time and ventricular filling time. Spectral Doppler was used to assess left ventricle systolic-to-diastolic duration ratio. Tissue Doppler was used to assess right ventricular filling time. Using spectral Doppler, left ventricle myocardial performance index was calculated as the sum of isovolumic contraction time and isovolumic relaxation time divided by ejection time.

Pre-existing maternal diabetes mellitus had a significant influence on fasting glucose levels (p < 0.001), left ventricle isovolumic contraction time (p < 0.001), left ventricle ejection time (p = 0.025), and left ventricle myocardial performance index (p < 0.001). Group 2 had higher left ventricle isovolumic contraction time (0.036 vs. 0.031 sec, p = 0.001) and left ventricle myocardial performance index (0.487 vs. 0.453, p = 0.003) compared with Group 1. Group 3 showed higher left ventricle myocardial performance index (0.492 vs. 0.449, p = 0.006) and lower left ventricle ejection time (0.161 vs. 0.169 sec, p = 0.038) than Group 1. Left ventricle systolic-to-diastolic duration (p = 0.704), right ventricle systolic-to-diastolic duration ratio’ (p = 0.757), left ventricle isovolumic contraction time (p = 0.163), left ventricle ejection time (p = 0.093), and left ventricle myocardial performance index (p = 0.087) were not useful parameters in predicting composite neonatal outcomes.

Pre-existing maternal diabetes mellitus had significant influence on fetal left ventricle myocardial performance index, but no effect on systolic-to-diastolic duration ratio. Systolic-to-diastolic duration ratio was not useful in predicting adverse perinatal outcomes.

Northwest axis on an electrocardiogram is conventionally thought to be associated with CHD, but there is a paucity of data regarding the types and incidence of CHD associated with this finding. The purpose of this study was to report the types and incidence of CHD found at our institution to determine the efficacy of electrocardiograms as a screening test in infants.

Retrospective, single-centre study of all infants ≤ 60 days of age who underwent a first-time electrocardiogram from 2015 to 2021. Our inclusion criteria included those found to have a northwest axis on electrocardiogram and who also had an echocardiogram performed. Data were described using standard summary statistics.

Two hundred sixty-eight infants met inclusion criteria (median age 4 days, interquartile range 2–24). The most common reason for an electrocardiogram to be obtained was the presence of a heart murmur (n = 102, 38%). One hundred thirty-one infants (49%) were found to have significant CHD. Cardiac defects included ventricular septal defect (n = 36, 28%), severe pulmonary valve stenosis (n = 17, 13%), atrioventricular canal defect (n = 16, 12%), and single ventricle disease (n = 16, 12%).

In this study, nearly half (49%) of these infants with northwest axis on electrocardiogram were found to have significant CHD. Electrocardiograms remain an important screening tool for infants with findings associated with potential cardiac disease and echocardiogram is warranted for infants found to have northwest axis on a first-time electrocardiogram.

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.

This research seeks to ascertain the prevalence and determinants of mirror-image dextrocardia in fetuses

With December 2022 as the reference point, we compiled colleted data on pregnant women who carried fetuses with mirror-image dextrocardia in Xi’an, Shaanxi Province: September–October 2022, November 2022, and December 2022–January 2023. An online questionnaire was distributed to 209 pregnant across China who had contracted COVID-19. The case group comprised women whose final menstrual cycle occurred in November 2022 and who had a fetus with mirror-image dextrocardia. Women with a November 2022 final menstrual period and a fetus without this condition made up the control group. To identify the risk factors associated with fetal mirror-image dextrocardia, both univariate and multivariate logistic regression analyses were employed.

A significant difference was noted in the gestational age at COVID-19 infection women with a September to October 2022 and December 2022 to January 2023 final menstrual period who did not bear a fetus with mirror-image dextrocardia, and those with a November 2022 final menstrual period whose fetus exhibited this condition. The univariate and multivariate analyses conducted on pregnant women with a final menstrual period in November 2022 who had contracted COVID-19 revealed significant differences in the presence and duration of fever between those bearing fetuses with mirror-image dextrocardia and those without (P = 0.000).

The findings suggest two critical factors to the increased prevalence of fetal mirror-image dextrocardia: 1) the infection timing which occurs between the 4th and 6th week of pregnancy and 2) the presence of fever and its prolonged duration.

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.

Alternate electrocardiogram acquisition with fewer leads lacks systematic evaluation in children. This study aims to determine if electrocardiograms with fewer leads maintain diagnostic accuracy in paediatrics.

This is a single-centre review of 200 randomly selected standard 12-lead electrocardiograms from our hospital database (2017–2020) for patients aged 2 weeks to 21 years. An overlay technique generated 8-lead (limb + V1/V6) and 6-lead (limb only) variations of the 12-lead tracings, resulting in a total of 600 electrocardiograms, which were then interpreted by two independent paediatric electrophysiologists.

In total, 18% (35/200) of the baseline electrocardiograms were abnormal. Intervals were measured in lead II for all electrocardiograms. Comparing 12-lead to 6- and 8-lead electrocardiograms, there was almost perfect agreement for specific rhythm identification (97.5–100%, κ 0.85-1). The 8-lead showed substantial agreement with 12-lead electrocardiograms when identifying specific electrocardiogram patterns (97.5–100%, κ 0.66–1). A similar degree of agreement was not demonstrated with the 6-lead variant. Utilising the 12-lead electrocardiogram as the gold standard, sensitivity and specificity of the 8- and 6-lead electrocardiogram were > 89% for specific rhythm identification. Specificity for specific pattern recognition was > 99% while sensitivity was < 90% for certain variables for both 6- and 8-lead electrocardiogram, likely due to smaller sample size and fewer abnormal electrocardiograms. There was high percent reader agreement (92.5–100%).

8-lead electrocardiograms provide comparable diagnostic accuracy to 12-lead electrocardiograms for children. This information holds potential for future technological advancements in electrocardiogram acquisition tailored specifically for paediatrics. Additional studies are required to further refine conventional electrocardiogram acquisition.