Dexmedetomidine is frequently used in paediatric anaesthesia. This includes use in patients with CHD, but detailed analysis of haemodynamics after administration in these patients has not yet been published. We performed a systematic review and meta-analyses examining haemodynamic changes immediately after dexmedetomidine administration in patients with CHD.

We conducted a systematic review of PubMed, Embase, and Medline from inception until May 31, 2024. Inclusion criteria were studies that contained children with CHD who received dexmedetomidine for a cardiac procedure and reported at least one haemodynamic variable before and after administration of dexmedetomidine. Exclusion criteria were studies of noncardiac procedures. We performed a meta-analysis on each haemodynamic variable that was reported by at least four studies.

We screened 5383 abstracts. We included 85 studies for review, and 16 studies were accepted for four meta-analyses (heart rate, 16 studies, n = 408; systolic blood pressure, 11 studies, n = 280; diastolic blood pressure, 10 studies, n = 276; mean arterial pressure, 5 studies, n = 130). Analysis of heart rate, systolic blood pressure, and diastolic blood pressure showed a statistically significant reduction (p < 0.001), while there was no significant change in mean arterial pressure. The clinical difference was minimal with a decrease in heart rate of 11.3 beats per minute, and a decrease in systolic blood pressure/diastolic blood pressure of 5.9 and 6.2 mmHg, respectively. Heterogeneity was high in all analyses.

Dexmedetomidine is associated with small changes in heart rate, systolic blood pressure, and diastolic blood pressure in children with CHD. Further study is warranted.

Tonsillectomy is a common paediatric procedure with potential morbidity, notably post-operative pain and bleeding. One proposed factor influencing these outcomes is the surgeon’s level of training.

To evaluate whether surgical training level affects short-term outcomes in paediatric tonsillectomy.

In a prospective randomised study (2019–2022), outcomes were compared in children undergoing cold dissection tonsillectomy performed by either an attending or a supervised resident. Measured outcomes included duration of analgesia use, time to resume oral intake, length of hospital stay and post-operative bleeding.

A total of 115 children were included in the study, of which 60 (52.2 per cent) were operated on by residents and 55 (47.8 per cent) were operated on by attending surgeons. Baseline characteristics were similar. Operations by attending surgeons were shorter (20.4 vs 29.1 minutes), but no significant differences were found in post-operative pain, diet resumption, length of stay (1.1 days) or bleeding.

Supervised residents achieve comparable outcomes to attending surgeons. Surgical training level does not impact tonsillectomy outcomes under supervision.

To determine the feasibility of developing and implementing a multidisciplinary bootcamp for early-career Paediatric Cardiac Critical Care practitioners.

A one-day pre-conference bootcamp at the Pediatric Cardiac Intensive Care Society Annual Meeting in December 2022.

Physicians, fellows, nurses, advanced practice registered nurses, and respiratory therapists who work primarily in paediatric cardiac critical care units.

A modified Delphi needs assessment with interprofessional content experts for the development of a mixed didactic and simulation-based bootcamp at the Pediatric Cardiac Intensive Care Society Annual Meeting in December 2022, with pre- and post-testing to evaluate knowledge gain and additional surveys to assess perceived value.

Eighty-three course participants came from a variety of professions and represented institutions nationally and internationally. Most participants (77%) had two or more years of professional experience, aligning with the bootcamp’s focus on advanced learners. The bootcamp received strong participant evaluations: 84.1% (37/44) strongly agreed that the bootcamp improved their clinical knowledge. Ninety-seven percent (43/44) reported increased confidence in the ability to care for paediatric cardiac critical care patients. The bootcamp demonstrated a significant improvement in participant knowledge, with pre-test scores averaging 54.9% (95% CI: 49.9–59.9) compared to post-test scores of 64.5% (95% CI: 59.7–69.2), achieving statistical significance (p < 0.05).

Our pilot bootcamp has shown an improvement in immediate knowledge retention with valuable insights gathered to enhance future bootcamps. The results of this advanced interprofessional bootcamp evaluation will inform future iterations for providers in paediatric cardiac critical care.

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.