Exercise capacity (VO2peak) predicts mortality in adult patients with CHD. There is a lack of paediatric exercise capacity data based on specific CHD lesions, limiting the ability to contextualise interpretation based on expected performance during testing. The primary aim of this study was to establish VO2peak percentiles for paediatric patients with repaired CHD undergoing treadmill-based cardiopulmonary exercise testing (CPET).

Retrospective analysis of CPET data from 2004 to 2022. CPETs were analysed for patients with CHD aged 6–18 years. Patients with repaired CHD were categorised based on their most haemodynamically significant CHD lesion. Percentiles and age-based trends were plotted for each group.

A total of 887 patients were included. CHD patients were divided into ten diagnostic subgroups. The mean percent expected VO2peak for each of the subgroups were as follows: Atrial and ventricular septal defect (94.5 ± 25.1%), pulmonary valve repair (88.1 ± 18.4%), aortic valve repair (92.7 ± 16.4%), tricuspid and mitral valve repair (81.3 ± 20.4%), coarctation of the aorta (93.6 ± 18.8%), transposition of the great arteries (90.5 ± 19.4%), double outlet right ventricle and truncus arteriosus (80.5 ± 16.2%), tetralogy of Fallot (85.6 ± 20.9%), left ventricle dominant Fontan (74.7 ± 18.3%), and right ventricle dominant Fontan (75.7 ± 16.7%).

There is a varying degree of reduced exercise capacity in paediatric patients with repaired CHD. Univentricular hearts and tricuspid and mitral valve repair have the lowest VO2peak. These CHD-specific percentiles may help providers risk-stratify and counsel patients with CHD.

This study aimed to identify echocardiographic predictors of successful weaning from extracorporeal membrane oxygenation in paediatric and congenital heart disease patients.

We retrospectively analyzed pediatric patients who underwent venoarterial extracorporeal membrane oxygenation for cardiogenic shock or postoperative support between March 2018 and September 2023. Clinical and echocardiographic variables assessed at the time of weaning evaluation were compared between patients who were successfully weaned and those who were not.

Among the 46 enrolled patients, 31 were successfully weaned from extracorporeal membrane oxygenation. The mean age at extracorporeal membrane oxygenation initiation was 9.6 ± 13.9 years, and the mean duration of support was 12.3 ± 12.1 days. Patients in the successfully weaned group had significantly higher left ventricular ejection fraction (50.9 ± 16.4% vs. 27.3 ± 18.7%, p < 0.001) and higher velocity time integral at the left ventricular outflow tract (12.3 ± 8.0 cm vs. 4.1 ± 3.6 cm, p = 0.001) compared with the unsuccessfully weaned group. The cutoff values for predicting successful weaning were a left ventricular ejection fraction of 43.03% (sensitivity, 74.2%; specificity, 86.7%) and a velocity time integral of 4.45 cm (sensitivity, 92.0%; specificity, 66.7%).

Left ventricular ejection fraction and velocity time integral provide valuable echocardiographic information for predicting successful weaning from extracorporeal membrane oxygenation in pediatric patients and may support clinical decision-making during weaning assessments.

There is currently no established normative data for cardiopulmonary exercise parameters in the semi-supine position. There is conflicting data regarding the impact of a semi-supine body position on the semi-supine recumbent ergometer on cardiopulmonary exercise parameters. The goal of the current study was to match semi-supine recumbent ergometer tests completed in children/adolescents with those completed on the cycle ergometer and treadmill to identify differences in cardiopulmonary exercise parameters between devices.

Maximal semi-supine recumbent ergometer tests were matched by demographics (age, race/ethnicity, sex, height, weight, and body mass index) to tests completed on the cycle ergometer and treadmill. Groups were compared with two-sample T-tests for numeric variables and Fisher’s exact tests for categorical variables.

There was no difference in demographics between groups. Peak cardiopulmonary exercise parameters (watts, oxygen consumption, heart rate, blood pressure, oxygen saturation, minute ventilation, respiratory exchange ratio, respiratory rate, and anaerobic threshold) were unchanged between semi-supine recumbent ergometer and cycle ergometer, but the ventilatory equivalent of carbon dioxide was higher on the cycle ergometer versus semi-supine recumbent ergometer. Anaerobic threshold, peak oxygen consumption, and peak minute ventilation were lower on the semi-supine recumbent ergometer than on the treadmill.

The uniformity in nearly all cardiopulmonary exercise parameters between the semi-supine recumbent ergometer and cycle ergometer suggests that normative data for the cycle ergometer are a reasonable surrogate for normative data on the semi-supine recumbent ergometer until semi-supine recumbent ergometer-specific normative data are developed.

This study aimed at investigating the clinical, individual, and systemic factors influencing paediatricians’ and family physicians’ clinical decision-making process in the vaccination of children during infection from the physician’s perspective.

A qualitative study through semi-structured in-depth interviews was conducted among 10 paediatricians and 10 family physicians working in Ankara, Türkiye. The audio-recorded interviews were translated into written texts, and the obtained data was analysed using the thematic analysis method proposed by Braun and Clarke.

Four main themes were identified in of thematic analysis: (I) Impact of clinical conditions on vaccination decisions, (II) attitudes of families and their communication processes with physicians, (III) impact of practice settings and institutional factors, and (IV) vaccine postponement and compensation approaches. It was observed that the decision-making processes of the paediatricians were mainly based on the clinical evaluation criteria, while family physicians considered the expectations of the families and institutional conditions. Also, the importance of establishing effective communication with vaccine-hesitant families has been emphasized by both groups of physicians.

In the immunization of infected children, decision-making is shaped in addition to medical facts in relation to the parental attitude, organizational factors within health institutions, and personal experiences of medical staff. Decisions of paediatricians are largely grounded in medical facts, whereas family practitioners assess that social and organizational factors are of higher importance. Improved adherence to medical guidelines and communication competencies of medical professionals can contribute towards medical practice consistency.

Infective endocarditis is a leading cause of morbidity and mortality in children and adolescents with underlying CHD. Appropriate diagnostic workup and management in the inpatient setting can be challenging in this patient population due to the spectrum of disease complexity and the dynamic nature of the field. Therefore, the Paediatric Acute Care Cardiology Collaborative has undertaken the creation of this clinical practice guideline.

A panel of paediatric cardiologists, infectious disease specialists, intensivists, advanced practice practitioners, pharmacists, cardiothoracic surgeons, and a dentist was convened. The literature was systematically reviewed for relevant articles on the management of infective endocarditis in patients with CHD. Using the modified Delphi technique, recommendations were generated and put through iterative Delphi rounds to achieve consensus for inclusion.

Based on 127 articles that met the inclusion criteria, 82 recommendations were generated, 50 of which achieved consensus for inclusion and are included in this guideline. They address risk factors specific to CHD lesion type and prior interventions including implanted material, diagnostic considerations, management strategies, and recommendations on counselling other healthcare providers, patients, and families. Of the 50 consensus recommendations, 36 are strong recommendations, though 20 have low or very low quality of evidence.

A central theme in this guideline is that an individual’s specific CHD lesion and prior interventions must be carefully considered for risk stratification, diagnostic approach, and management. While most are strong recommendations, many are supported by low quality of evidence, emphasising the need for further research in this subject.

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.