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.

A series of webinars covering widespread knowledge on paediatric cardiology and cardiac surgery topics was initiated by Association for European Paediatric and Congenital Cardiology, serving towards preparation for the Association for European Paediatric and Congenital Cardiology certification in paediatric and congenital cardiology. This study investigated the impact of webinars as educational tools for junior paediatric cardiologists in the post-COVID-19 pandemic era.

A cross-sectional survey design study using an online survey as a tool for the assessment of trainees. An open and closed-ended SurveyMonkey questionnaire was used to document the learners’ opinions on webinars. Results were reported using descriptive statistical analyses.

Twenty-seven Association for European Paediatric and Congenital Cardiology junior members participated in the online survey from twelve different countries. Most of the participants were trainees in paediatric cardiology (56%), and the remainder were junior consultants in paediatric cardiology. Approximately 70% found no difficulties in participating in the webinars. The webinars were appreciated by participants, who found the webinars interactive and highly educational with contents highly applicable to everyday clinical practice. Significant heterogeneity emerged in training programmes across Europe and worldwide in terms of programme duration, number of fellows, teaching approach, and assessments. Training opportunities such as courses, grants, and more webinars were suggested as tools to support continuous learning by the Association for European Paediatric and Congenital Cardiology.

The Association for European Paediatric and Congenital Cardiology webinar series has confirmed the crucial role of online-based learning resources in the new generation of junior paediatric cardiologists. Association for European Paediatric and Congenital Cardiology webinars and the examination in paediatric cardiology may help standardise training across Europe, promoting the highest standards in patient care.

Procedure duration is an important predictor of patient outcomes in surgery. However, the relationship between procedure duration and adverse events in congenital cardiac catheterization is largely unexplored.

All cases entered into the Congenital Cardiac Catheterization Project on Outcomes from 2014 to 2017 were included. Cases were ordered from shortest to longest case length, minus time spent managing adverse events, for each case type. The outcomes, Level 3bc/4/5 and 4/5 adverse event rates, were calculated for cases above and below the 75th percentile for case length. To identify an independent relationship between case length and outcomes, the case length percentile was added to the CHARM II risk model.

Among 14,704 catheterizations, longer cases (>75th percentile for case length) had Level 4/5 rates that were 2.2% and 2.7% compared to cases ≤75th percentile with adverse event rates of 0.9% and 1.4% for diagnostic and interventional cases, respectively. Level 3bc/4/5 rates were 5.0% and 8.4% in longer cases compared to 2.4% and 5.4% for diagnostic and interventional cases, respectively. After adding case length to the CHARM II risk model, case length 50th–75th percentile had an odds ratio (OR) of 1.4, 75th–90th percentile an OR of 1.56, and >90th percentile an OR of 2.24 as compared to cases with case length <50th percentile (p ≤ 0.001 for all).

Longer case lengths are associated with clinically important and life-threatening adverse events in congenital cardiac catheterization, even after accounting for known risk factors. Case length may be an important target for future quality improvement work.

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 delivery of paediatric cardiac care across the world occurs in settings with significant variability in available resources. Irrespective of the resources locally available, we must always strive to improve the quality of care we provide to our patients and simultaneously deliver such care in the most efficient and cost-effective manner. The development of cardiac networks is used widely to achieve these aims.

This paper reports three talks presented during the 56th meeting of the Association for European Paediatric and Congenital Cardiology held in Dublin in April 2023.

The three talks describe how centres of congenital cardiac excellence can be developed in low-income countries, middle-income countries, and well-resourced environments, and also reports how centres across different countries can come together to collaborate and deliver high-quality care. It is a fact that barriers to creating effective networks may arise from competition that may exist among programmes in unregulated and especially privatised health care environments. Nevertheless, reflecting on the creation of networks has important implications because collaboration between different centres can facilitate the maintenance of sustainable programmes of paediatric and congenital cardiac care.

This article examines the delivery of paediatric and congenital cardiac care in resource limited environments, well-resourced environments, and within collaborative networks, with the hope that the lessons learned from these examples can be helpful to other institutions across the world. It is important to emphasise that irrespective of the differences in resources across different continents, the critical principles underlying provision of excellent care in different environments remain the same.

To assess the prevalence of obesity and investigate any changes in body mass index in children with CHD compared to age-matched healthy controls, in Southwestern Ontario.

The body mass index z-scores of 1259 children (aged 2–18) with CHD were compared with 2037 healthy controls. The body mass index z-scores of children who presented to our paediatric cardiology outpatient clinic from 2018 to 2021 were compared with previously collected data from 2008 to 2010. A longitudinal analysis of patients with data in both cohorts was also completed.

In total, 21.4% of patients with CHD and 26.6% of healthy controls were found to be overweight or obese (p < 0.001). The 2018–2021 cohort of CHD patients and controls had significantly higher body mass index z-scores compared to the 2008–2010 cohort (p < 0.001). Longitudinal analysis showed that body mass index z-scores significantly increased over time for CHD patients with data in both cohorts (2018–2021: M = 0.59, SD = 1.26; 2008–2010: M = −0.04, SD = 1.05; p < 0.001).

The prevalence of obesity in all children, irrespective of CHD, is rising. The coexistence of obesity and CHD may pose additional cardiovascular risks and complications.

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.

A subgroup of CHDs can only be treated palliatively through a Fontan circulation. In case of a failing Fontan situation, serum proteins are lost unspecifically and can also lead to a loss of vaccine antibodies. In a failing Fontan situation, heart transplantation may be the only feasible option.

We describe a 17-year-old patient born with a hypoplastic left heart complex, who underwent Fontan completion at the age of 4 years and developed a failing Fontan physiology. Therefore, a Fontan takedown with creation of a reverse 1½-circulation was performed. Multiple exacerbations of protein losing enteropathy occurred with a hypoproteinaemia, hypalbuminaemia, and hypogammaglobulinaemia. The patient was hospitalised several times and treated with intravenous application of immunoglobulins and albumin for symptom control. Before one of this substitutions, the immunoglobulin G against measles, mumps, and rubella was determined: the patient’s serum demonstrated a positive measles and rubella, but mumps was negative. After administration of the iv-therapy, the lacking antibodies were replenished, and there was a positive test for mumps.

Serum samples were analysed by neutralisation test and enzyme-linked immunosorbent assay (ELISA).

Although the patient had been vaccinated according to national guidelines, we saw an intermittent immune deficiency for mumps, but not for rubella and measles. For patient with a failing Fontan circulation, we recommend to test to vaccine antibodies for mumps, measles, and rubella with an ELISA an if its negative with a neutralisation test, especially in view of a possible heart transplantation to find a possible immune deficiency.

This study explored the prospective use of the Ages and Stages Questionnaires-3 in follow-up after cardiac surgery.

For children undergoing cardiac surgery at 5 United Kingdom centres, the Ages and Stages Questionnaires-3 were administered 6 months and 2 years later, with an outcome based on pre-defined cut-points: Red = 1 or more domain scores >2 standard deviations below the normative mean, Amber = 1 or more domain scores 1–2 standard deviations below the normal range based on the manual, Green = scores within the normal range based on the manual.

From a cohort of 554 children <60 months old at surgery, 306 participated in the postoperative assessment: 117 (38.3%) were scored as Green, 57 (18.6%) as Amber, and 132 (43.1%) as Red. Children aged 6 months at first assessment (neonatal surgery) were likely to score Red (113/124, 85.6%) compared to older age groups (n = 32/182, 17.6%). Considering risk factors of congenital heart complexity, univentricular status, congenital comorbidity, and child age in a logistic regression model for the outcome of Ages and Stages score Red, only younger age was significant (p < 0.001). 87 children had surgery in infancy and were reassessed as toddlers. Of these, 43 (49.2%) improved, 30 (34.5%) stayed the same, and 13 (16.1%) worsened. Improved scores were predominantly in those who had a first assessment at 6 months old.

The Ages and Stages Questionnaires results are most challenging to interpret in young babies of 6 months old who are affected by complex CHD.

Extubation failure after neonatal cardiac surgery is associated with increased intensive care unit length of stay, morbidity, and mortality. We performed a quality improvement project to create and implement a peri-extubation bundle, including extubation readiness testing, spontaneous breathing trial, and high-risk criteria identification, using best practices at high-performing centers to decrease neonatal and infant extubation failure by 20% from a baseline of 15.7% to 12.6% over a 2-year period.

Utilising the transparency of the Pediatric Cardiac Critical Care Consortium database, five centres were identified as high performers, having better-than-expected neonatal extubation success rates with the balancing metric of as-expected or better-than-expected mechanical ventilation duration. Structured interviews were conducted with cardiac intensive care unit physician leadership at the identified centers to determine centre-specific extubation practices. Data from those interviews underwent qualitative content analysis which was used to develop a peri-extubation bundle. The bundle was implemented at a single-centre 17-bed cardiac intensive care unit. Extubation failure, defined as reintubation within 48 hours of extubation for anything other than a procedure, ventilator days and bundle compliance was tracked.

There was a 41.4% decrease in extubation failure following bundle implementation (12 failures of 76 extubations pre-implantation; 6 failures of 65 extubations post-implementation). Bundle compliance was 95.4%. There was no difference in ventilator days (p = 0.079) between groups.

Implementation of a peri-extubation bundle created from best practices at high-performing centres reduced extubation failure by 41.4% in neonates and infants undergoing congenital heart surgery.