There is wide variation in institutional sedation strategies in paediatric cardiac ICU. Validated tools such as State Behavioral Scale and Richmond Agitation Sedation Scale were created to help standardise sedation practices.

This is a multi-phase, multicentre, prospective project with the goal of optimising safety and comfort for paediatric cardiac ICU patients. Phase one consisted of an educational intervention with a self-paced, web-based video module on optimal sedation practices using validated sedation screening tools. Participant knowledge was assessed via a de-identified, unmatched pre- and post-test survey. Survey scores were reported as an aggregate average score and compared using a t-test.

There were 259 pre-tests, and 142 post-tests collected during the video-assisted educational intervention. There was a significant increase in mean score on the post-test compared to the pre-test for both instruments: from 4 to 4.8/10 for State Behavioral Scale (p = 0.01) and from 4.5 to 4.9 for Richmond Agitation Sedation Scale (p = 0.04). 81% of respondents who completed the Richmond Agitation Sedation Scale post-test and 88.1% of those who completed the State Behavioral Scale post-test said their practice would change based on the new knowledge acquired.

We report that our newly developed learning module intervention was effective in increasing short-term knowledge about optimal sedation and sedation scoring. Ongoing phase two efforts include evaluation of long-term compliance of validated sedation screening tools and developing an objective score to measure individual cumulative opioid dosing in the cardiac critical care unit.

Educational experience of children with CHD is often adversely impacted by factors such as medical burden, social and school functioning challenges. It is, therefore, vitally important that adequate support is provided at an early stage in order to facilitate better educational outcomes for this cohort. The role of the teacher is pivotal in supporting the overall healthy development of a child with CHD. Thus, it is important to understand how we can also support teachers to provide optimal support to this cohort. This systematic scoping review aimed to offer a comprehensive understanding of existing research in this area and identify any knowledge gaps.

The methodological framework for scoping reviews developed by Arksey and O’Malley (2005) was employed.

Children with CHD face educational challenges in cognitive, psychomotor, behavioural, and affective domains and also with school attendance. The main challenges for teachers include a lack of information around CHD and how it affects the individual child. Building a strong relationship and having frequent communication between the teacher/ parent/ child were considered key in alleviating anxiety and promoting a supportive environment.

Children with CHD often require additional support from educational professionals in the classroom. Teachers of children with CHD would benefit from condition-specific training, updated on a regular basis.

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.

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.

Adolescent and parental perceptions of the Fontan Udenafil Exercise Longitudinal Assessment Randomised Controlled Trial (FUEL) and its open-label extension were examined, to identify factors affecting future research participation.

A validated survey was administered at two time points to adolescents (12–19 years) and their parents to assess likes/dislikes of study participation, research team, study burden and benefits. A 5-point Likert scale (strongly disagree [−2] to strongly agree [ + 2]) was used, and scores were averaged. Regression models explored potential predictors. Open-ended questions queried the most/least appealing aspects of participation and considerations for future research.

Among 250 FUEL participants at 14 centres, 179 adolescent and 183 parent surveys were completed at T1 (6 months after randomisation). Perceptions of research participation were generally positive: 1.35 ± 0.45 for adolescents; 1.56 ± 0.38 (p < 0.001) for parents. There were no significant differences between females vs. males. Themes from open-ended responses included liking to help others and themselves, liking the study team, and disliking study burden. Adolescents liked the compensation and disliked study-related testing. At T2 (end of open-label extension study), 121 adolescents and 114 parents responded. Perception scores remained high at 1.39 ± 0.51 for adolescents and 1.58 ± 0.37 for parents (p = 0.001). There were no significant gender differences in perceptions between adolescents, but mothers had slightly better perceptions than fathers (p = 0.004).

Perceptions of research were positive and slightly better for parents. Study teams and compensation were key contributors to positive perceptions. Study burden and testing were viewed less favourably. Future studies should consider families’ preferences and potential barriers to participation.

Progressive ventricular remodelling in children with repaired tetralogy of Fallot may or may not result in the need for pulmonary valve replacement. We aimed to model and compare the rates of right and left ventricular adaptation over time, as assessed by cardiac MRI after surgical repair of tetralogy of Fallot, in children who did or did not require pulmonary valve replacement later in adolescence.

Single-centre, retrospective cohort study from 2000 to 2020 including patients with tetralogy of Fallot who had complete surgical repair before 24 months.

From 214 patients included in this analysis, 142 (66.3%) had pulmonary valve replacement at a median age of 12 years (interquartile range 9–15.5) during follow-up. Assessing 323 cardiac MRI studies from 201 patients commencing from a median age of 9.4 years (interquartile range 5.9–12.3), the group that required pulmonary valve replacement later during the follow-up had a steeper time-related right ventricular dilation trajectory than non-pulmonary valve replacement patients: the increment in right ventricular end-diastolic volume index was 19.4 versus 2.8 ml/m2/log2year, P < 0.001; also, right ventricular end-systolic volume index incremented more quickly, at 11.9 versus 0.8 ml/m2/log2year, P < 0.001. Left ventricular end-diastolic volume index increased more quickly in patients who eventually had pulmonary valve replacement, at 7.2 versus 1.5 ml/m2/log2year, P = 0.005; the same occurred for indexed left ventricular end-systolic volume at 3.2 versus –0.4 ml/m2/log2year, P = 0.001.

Early right and left ventricular dilation over time are identifiable by cardiac MRI in patients destined to require pulmonary valve replacement following tetralogy of Fallot repair.

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.

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.

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.

This study aimed to evaluate the association between serum magnesium levels and the risk of symptomatic patent ductus arteriosus in premature infants.

This retrospective single-centre cohort study analysed the medical records of patients. Neonates were categorised into two groups based on the presence or absence of symptomatic patent ductus arteriosus. Demographic factors including gender, gestational age, type of birth, birth weight, multiple pregnancies, medical treatment for duct closure or surgical ligation, length of hospital stay, and mortality and neonatal outcomes were compared between the groups. The relationship between serum magnesium levels at 24 hours of age and the risk of symptomatic patent ductus arteriosus and other neonatal morbidities was assessed.

This study found no significant relationship between symptomatic patent ductus arteriosus and serum magnesium levels. Additionally, no significant differences were observed between serum magnesium levels and ductal diameter, nor in the need for medical or surgical intervention for symptomatic patent ductus arteriosus. However, neonates with serum magnesium levels greater than 3 mg/dL exhibited a significantly higher incidence of respiratory distress syndrome. Conversely, the prevalence of bronchopulmonary dysplasia was significantly lower in this group, with both findings reaching statistical significance (p < 0.05)

These results suggest that while serum magnesium levels may not be a reliable marker for symptomatic patent ductus arteriosus, they could have clinical implications in the modulation of neonatal respiratory outcomes. Further research is warranted to explore the underlying mechanisms and assess the potential therapeutic role of magnesium in the management of neonatal morbidities.

B lines in lung ultrasound are useful to diagnose heart failure in adults. This study was to done to correlate lung ultrasound score with heart failure severity in CHD.

Fifty infants with left to right shunt underwent Ross scoring, measurement of NT pro BNP and lung ultrasound. Lung was divided into 14 zones (7 on each side); each zone was scored as: No B lines = 0, less than 3 B lines = 1, 3-7 B lines = 2, > 7 /or confluent B lines = 3. Total score was calculated by adding all scores. A modified ultrasound score was calculated by excluding scores of 1. Relationship between ultrasound scores and heart failure was assessed. Receiver operator curve (ROC) was generated to find a cut-off of ultrasound score to detect moderate or severe heart failure.

No correlation was found between either total or modified lung ultrasound score with the modified Ross score or NT pro BNP. A cut-off of total lung ultrasound score of 11 gave an accuracy of 68% with area under the curve (AUC) of 0.64 to detect moderate or severe heart failure.

Utility of lung ultrasound in detecting severity of heart failure in infants with left to right shunts is limited at present due to its low accuracy and lack of standardised method of scoring.

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.

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.

There is geographic disparity in the provision of Pediatric and Congenital Heart Disease (PCHD) services; Africa accounts for only 1% of global cardiothoracic surgical capacity. Methods: We conducted a survey of PCHD services in Africa, to investigate institution and national-level resources for pediatric cardiology and cardiothoracic surgery. Results were compared with international guidelines for PCHD services and institutions were ranked by a composite score for low- and middle-income PCHD services. Results: There were 124 respondents from 96 institutions in 45 countries. Eighteen (40%) countries provided a full PCHD service including interventional cardiology and cardiopulmonary bypass (CPB) cardiac surgery. Ten countries (22%) provided cardiac surgery services but no interventional cardiology service, 4 of which did not have CPB facilities. One provided interventional cardiology services but no cardiac surgery service. Ten countries (22%) had no PCHD service. There were 0.04 (interquartile range [IQR]: 0.00-0.13) pediatric cardiothoracic surgeons and 0.17 (IQR: 0.02-0.35) pediatric cardiologists per million population. No institution met all criteria for level 5 PCHD national referral centers, and 8/87 (9.2%) met the criteria for level 4 regional referral centers. Thirteen (29%) countries report both pediatric cardiology and cardiothoracic surgery fellowship training programs. Conclusions: Only 18 (40%) countries provided full PCHD services. The number of pediatric cardiologists and cardiothoracic surgeons is below international recommendations. Only Libya and Mauritius have the recommended 2 pediatric cardiologists per million population, and no country meets the recommended 1.25 cardiothoracic surgeons per million. There is a significant shortage of fellowship training programs which must be addressed if PCHD capacity is to be increased.