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.

This study investigated the prevalence of malnutrition, time to achieve caloric goals, and nutritional risk factors after surgery for CHD in a cardiac ICU.

This retrospective study included patients with CHD (1 month-18 years old) undergoing open-heart surgery (2021–2022). We recorded nutritional status, body mass index-for-age z-score, weight-for-length/height z-score, cardiopulmonary bypass and aortic cross-clamp time, Paediatric Risk of Mortality-3 score, Paediatric Logistic Organ Dysfunction-2 score, vasoactive inotropic score, total duration of mechanical ventilation, length of stay in the cardiac ICU, mortality, and time to achieve caloric goals.

Of the 75 included patients, malnutrition was detected in 17% (n= 8) based on the body mass index-for-age z-score and in 35% (n= 10) based on the weight-for-length/height z-score. Sex, mortality, cardiopulmonary bypass and aortic cross-clamp time, Paediatric Risk of Mortality-3, Paediatric Logistic Organ Dysfunction-2, and vasoactive inotropic score, duration of mechanical ventilation, and length of cardiac ICU stay were similar between patients with and without malnutrition. Patients who achieved caloric goals on the fourth day and those who achieved them beyond the fourth day showed statistical differences in mortality, maximum vasoactive inotropic score, duration of mechanical ventilation, cardiopulmonary bypass and aortic cross-clamp time, Paediatric Risk of Mortality-3, Paediatric Logistic Organ Dysfunction-2, and length of cardiac ICU and hospital stay (p< 0.05). Logit regression analysis indicated that the duration of mechanical ventilation, Paediatric Logistic Organ Dysfunction-2 and Paediatric Risk of Mortality-3 score was a risk factor for achieving caloric goals (p< 0.05).

Malnutrition is prevalent in patients with CHD, and concomitant organ failure and duration of mechanical ventilation play important roles in achieving postoperative caloric goals.

Infants who require cardiopulmonary bypass for surgical repair of CHD are at high risk for secondary infections, which cause significant death and disability. The risk of secondary infection is increased by immune dysfunction. The intestinal microbiome calibrates immune function. Infants with CHD have substantial changes in their intestinal microbiome. We performed this scoping review to describe the current understanding of the relationship between the intestinal microbiome and immune function after pediatric cardiac surgery with cardiopulmonary bypass.

We searched the PubMed, Cumulative Index to Nursing and Allied Health Literature, Cochrane, and Scopus databases with the assistance of a medical librarian. We included trials that analysed intestinal microbiome composition and immune function after cardiac surgery with cardiopulmonary bypass in infants.

We found two observational cohorts and two interventional trials describing composition of intestinal microbiome and some measures of immune function after heart surgery with cardiopulmonary bypass in infants. A total of 114 children were analysed. Three trials were exclusively in infants, and one was in older children and infants. All trials found a differential composition of the intestinal microbiome in infants with CHD compared to those without CHD, and one described a robust correlation between composition of the intestinal microbiome with cytokine profile and adverse outcomes.

Despite robust preclinical data and data from other disease states, there is minimal data about the correlation between immune function and intestinal microbiome composition in infants with CHD after cardiopulmonary bypass.

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.

Data on arterial thromboembolism in children undergoing cardiac surgery are limited. We sought to characterise, and estimate rates of, incident and recurrent arterial thromboembolism, and describe antithrombotic therapies for treatment in a large multinational population of children with CHD undergoing cardiac surgery.

We queried the TriNetX global electronic health record (derived real-world data research platform) from 2017 to 2024 for patients less than 18 years of age and an index arterial thromboembolism within 1 year of congenital cardiac surgery. Data were descriptively analysed.

Of 20,102 children who underwent an index cardiac surgery for CHD, 206 (1.1%) developed an index arterial thromboembolism within 1 year of surgery: 111 (53.9%) had only arterial thromboembolism and 95 (46.1%) had concomitant venous thromboembolism. The most common anatomic site for arterial thromboembolism was the lower extremity (n = 141, 68.4%), and the most common surgery was the Glenn procedure (n = 35, 17%). Unfractionated heparin was utilised in 136 (67 %) and aspirin in 91 (44.2%) patients. Recurrent thromboembolism occurred in 36 (17.5%) patients within 1 year of the index thromboembolism.

Among children undergoing congenital cardiac surgery, arterial thromboembolism was rare (1% of patients), but the 1-year risk of recurrent thromboembolism was high, at 17.5%. Multicentre prospective cohort studies are warranted to further evaluate risk factors for recurrent thromboembolism, to facilitate future risk-stratified interventional trials designed to reduce the high thromboembolism recurrence risk in these children.

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.

Acute rheumatic fever commonly causes carditis in children. It is critical to have biomarkers for diagnostic and prognostic purposes. We aimed to investigate the expressions of miR-101, miR-1183, and miR-1299 in children with acute rheumatic fever and rheumatic heart disease and determine their potential as diagnostic biomarkers.

This cross-sectional study was conducted on 69 paediatric acute rheumatic fever patients and 27 gender and age-matched controls. The clinical findings were noted from medical records, echocardiography was repeated, and plasma miR-101, miR-1183, and miR-1299 expressions were studied using RT-PCR.

Out of 54 patients with carditis, 36 developed rheumatic heart disease with significant left-sided heart involvement. No significant demographic difference was present between rheumatic heart disease patients and controls (p > 0.05). miR-101 and miR-1183 expressions significantly reduced by 4.5-fold in acute rheumatic fever patients compared to controls (p : 0.029 and p : 0.024, respectively). Family history was found to be significantly associated with 9-fold reduced miR-1183 expression in acute rheumatic fever patients (p : 0.005). The expression of miR-1183 in acute rheumatic fever children with carditis in general and severe carditis was significantly reduced 4.5- and 9-fold, respectively (p : 0.024 and p : 0.007, respectively). In acute rheumatic fever cases with moderate-severe carditis and patients who did not develop rheumatic heart disease, miR-1183 expression significantly reduced 8.3-fold and 9-fold, respectively (p : 0.015 and p : 0.019, respectively). The ROC analysis revealed that miR-101 and miR-1183 were statistically significant for acute rheumatic fever diagnosis (AUC: 0.64 for both miRNAs).

Significantly lower plasma expressions of miR-101 and miR-1183 in acute rheumatic fever patients, especially in cases with moderate-severe carditis, might indicate the potential use of these miRNAs as diagnostic biomarkers.

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.

To demonstrate safety and efficacy of using the AmplatzerTM vascular plug II device for perimembranous ventricular septal defect closure with retrograde approach and show the follow-up in all patients.

At present, there is no FDA-approved device for transcatheter closure of perimembranous ventricular septal defects. Small studies and case reports have shown the use of various catheter-based devices in an off-label management; however, there are no large studies to show their efficacy. The second generation of AmplatzerTM vascular plug seems to offer a safe and attractive alternative for this procedure. Besides, a retrograde approach might decrease procedure time and radiation exposure time.

Patients with congenital perimembranous ventricular septal defects who underwent transcatheter closure using Amplatzer vascular plug II devices were included. Primary end point was to determine efficacy and safety of this generation of devices and the incidence of complications at follow-up (complete heart block and aortic/tricuspid/mitral regurgitation). Forty-five patients underwent perimembranous ventricular septal defect closure at a median age of 6 years (9 months–17 years). During the catheterization, there were only minor complications and at follow-up of 48 ± 25.7 months (up to 96 months). Closure rate was high of 93.3% and freedom from atrioventricular block was 100%.

The second generation of the AmplatzerTM vascular plug II seems to offer a safe and attractive alternative for percutaneous closure of the perimembranous ventricular septal defects.

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.

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.