Surgical pulmonary valve replacement is commonly required to palliate patients with CHD affecting the right ventricular outflow tract; however, concerns remain about mid- and long-term durability. Post-operative short-term anticoagulation has been hypothesised to improve valve durability.

This is a single-centre, retrospective study of paediatric patients who underwent surgical pulmonary valve replacement and received a direct oral anticoagulant in addition to aspirin post heart valve insertion. The primary objective was a composite safety score consisting of clinically relevant non-major bleeding, major bleeding, bleeding-related readmission, and medication discontinuation.

The study analysed 34 patients with a median age 14 years (Interquartile range (IQR): 11, 15) and weight 45 kg (IQR: 35, 55). Ten patients met the composite endpoint (10/34, 29%), with 4 patients experiencing major bleeding (4/34, 12%), 6 experiencing clinically relevant non-major bleeding (6/34, 18%), and 3 patients being readmitted within 90 days of surgical pulmonary valve replacement for bleeding (3/29, 8.8%) resulting in 10 patients discontinuing medication early (10/34, 29%). Lower weight was identified as a significant risk factor for adverse event development (p = 0.04).

We observed a higher overall bleeding rate, driven predominately by clinically relevant non-major bleeding events, than other studies using short-term anticoagulation after surgical pulmonary valve replacement. Additional studies should be aimed at evaluating the dosing and safety of direct oral anticoagulants in children in the post-operative period.

Individuals with 22q11 deletion syndrome have a mutation in the TBX1 gene. This is associated with reduced left pulmonary artery/right pulmonary artery ratio in animal models and in humans with structurally normal hearts.

A retrospective analysis was undertaken of patients who underwent surgical repair of Tetralogy of Fallot, truncus arteriosus, and interrupted aortic arch between 01/2007 and 12/2022. The left pulmonary artery/right pulmonary artery ratio on initial and most recent echocardiogram and initial and subsequent intervention on the left pulmonary artery were compared between patients with and without 22q11 deletion.

There were 134 included patients; 19 patients had the deletion (22q11 positive), and 115 patients did not have the deletion (22q11 negative). Tetralogy of Fallot was present in 8/19 and 101/115 patients, truncus arteriosus in 7/19 and 7/115 patients, and interrupted aortic arch in 4/19 and 7/115 patients. Patients who were 22q11 positive had a reduced left pulmonary artery/right pulmonary artery ratio on both the initial echocardiogram [0.88 (interquartile range 0.71, 0.97) versus 1.02 (interquartile range 0.92, 1.12); p < 0.001] and most recent echocardiogram [0.66 (interquartile range 0.62, 0.91) versus 1.01 (interquartile range 0.89, 1.16); p < 0.001] and were more likely to have intervention on the left pulmonary artery at their initial surgery (36% versus 8.7%; p = 0.003).

Patients who were 22q11 positive trended towards reduced left pulmonary artery/right pulmonary artery ratios and need for early surgical intervention on the left pulmonary artery in comparison to patients without 22q11 deletion negative patients.

Acquired chylothorax is an established complication of CHD surgery, affecting 2–9% of patients. CHD places a child at risk for failure to thrive, with subsequent chylothorax imposing additional risk.

We conducted a retrospective chart review to ascertain quantitative markers of nutrition and growth in children affected by chylothorax following CHD surgery between 2018 and 2022 compared to controls.

We utilised electronic medical record system, EPIC, at Children’s Hospital, New Orleans, targeting subjects < 18 years old who underwent CHD surgery between 2018 and 2022 and developed a subsequent chylothorax. Study subjects were identified using the 10th revision of the International Classification of Diseases codes (ICD-10 codes: J94.0, I89.8, and J90.0). Each chylothorax case (n = 20) was matched by procedure type and age to a control with no chylothorax (n = 20). Data were recorded in REDCap and analysed using SPSS.

After removal of outliers, we analysed 19 total matched pairs. There was no statistical difference in growth velocity (p = 0.12), weight change (operation to discharge) (p = 0.95), weight change (admission to discharge) (p = 0.35), Z-score change (operation to discharge) (p = 0.90), Z-score change (admission to discharge) (p = 0.21), serum protein (p = 0.88), or serum albumin (p = 0.82). Among cases, linear regression demonstrated no significant association between maximum chylous output and growth velocity (p = 0.91), weight change (operation to discharge) (p = 0.15), or weight change (admission to discharge) (p = 0.98).

We did not observe statistically significant markers of growth or nutrition in children with chylothorax post-CHD surgery compared to those without chylothorax. Multisite data collection and analysis is required to better ascertain clinical impact and guide clinical practice.

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 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.

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.

Lipid levels in paediatric patients with anomalous aortic origin of a coronary artery (AAOCA) have not previously been explored. Patients with CHD have an increased risk of atherosclerotic cardiovascular disease later in life compared to the general population. We aim to characterise the lipid profiles in paediatric patients with AAOCA and explore its relation to diagnosis, race/ethnicity, and exercise.

Single institution retrospective cohort of 180 AAOCA paediatric patients (median age 13.7 years interquartile range 9.7–15.6, 66% male). Total cholesterol, HDL, LDL, triglycerides, total cholesterol to HDL ratio, and non-HDL cholesterol were evaluated across race/ethnicity, sex, type of AAOCA, documented ischaemia on imaging, exercise level, and surgery status. Normality of the data distribution for each lipid parameter was evaluated using Kolmogorov–Smirnov testing. Accordingly, Mann–Whitney U and t-tests were used to compare variables. The proportion of abnormal lipid levels by sex and race/ethnicity was calculated.

Total cholesterol was elevated in 29%, (51/177) of patients, HDL 37% (64/174), triglycerides 44% (72/165), LDL 16% (28/170), total cholesterol-HDL ratio 29%, (48/163), and non-HDL cholesterol 28% (47/165). Across subgroups categorised on the basis of surgery status, exercise level, AAOCA type, and sex, the mean and median levels for individual lipid parameters were normal. By race/ethnicity, Hispanic patients had significantly higher triglyceride (median 99, interquartile range 71–136.5, p = <0.001) and total cholesterol to HDL ratios (median 3.2, interquartile range 2.7–4.5, p = 0.014) versus non-Hispanic White and Black patients. Two-thirds of patients exercise recreationally.

Hispanic patients have significantly elevated triglycerides and total cholesterol to HDL ratios compared to others. Longitudinal follow-up evaluating differences in long-term lipid status in patients with AAOCA and risk for cardiovascular events is warranted.

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.

Cardiac intensive care providers require a comprehensive understanding of cardiac output and oxygen delivery. The estimation of cardiac output in clinical practice often relies on thermodilution and the Fick principle. Central venous saturation and lactate levels are commonly used indicators for cardiac output assessment. However, the relationship between venous lactate levels and venous oxygen saturation in paediatric cardiac intensive care patients remains unclear.

This is a single-centre retrospective pilot study aimed to investigate the correlation between venous lactate and venous oxygen saturation in paediatric patients. Data collected included venous saturation, heart rate, mean arterial blood pressure, arterial saturation by pulse oximetry, cerebral and renal near-infra-red spectroscopy values, and the presence of a functionally univentricular heart. Statistical analyses included Bayesian Pearson correlation and regression analyses.

A total of 203 data points from 37 unique patients were included in the analysis. There was no significant correlation between serum lactate and venous saturation (correlation coefficient = –0.01; Bayes factor 10 = 0.06). Serum lactate also did not correlate with other haemodynamic metrics. Venous saturation showed correlations with arterial saturation and cerebral and renal near-infra-red spectroscopy. Regression analysis revealed that parallel circulation, arterial saturation, and cerebral near-infra-red spectroscopy were predictive of venous saturation. The following equation resulted from the regression analysis: 68.0 – (12.7 x parallel circulation) – (0.8 x arterial saturation) + (0.3 x cerebral near-infra-red spectroscopy). This model had a Bayes factor 10 of 0.03 and adjusted R-squared was 0.29.

In paediatric cardiac intensive care patients, there is no significant correlation between venous lactate and venous saturation, suggesting that lactate may not be a reliable marker for assessing the adequacy of oxygen delivery in this population. Only a weak correlation could be identified once the venous saturation was 70% or lower. Additional research is needed to explore alternative markers for monitoring oxygen delivery in critically ill paediatric patients.