Specialised training opportunities in paediatric cardiology are rare for advanced practice providers, creating an educational gap for novice practitioners. Standardised curricula have been cited as a beneficial intervention to optimally prepare these providers for highly specialised fields. We sought to understand the current onboarding practices of advanced practice providers in paediatric acute care cardiology to identify opportunities for curricular improvement.

A survey developed by a task force by the Pediatric Acute Care Cardiology Collaborative (PAC3) was distributed across PAC3 programmes in May 2023 to evaluate the onboarding practices of advanced practice providers at paediatric heart centres nationwide.

Survey responses reflected orienting practices at 19 paediatric heart centres representing varying programme and team sizes. Of the respondents, 32% felt their current model does not meet all the needs of the new team member. Key successful onboarding elements included a structured curriculum with goals and objectives, dedicated education time and materials, standardised assessments, and individualised learning in the presence of a supportive team. All respondents agreed that an online curriculum would be beneficial.

There is no national standardised educational pathway for advanced practice providers entering paediatric acute care cardiology practice. There are opportunities to develop a formalised curriculum with structured learner assessment at a national level, which could be modified at the institution or learner level to enhance current onboarding practices.

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.

To enumerate the experience in different parameters, including demographic, anatomic, procedural, ICU care, and outcome of ductal stenting procedures in duct-dependent pulmonary circulation in a resource-limited setup.

Stenting the arterial duct emerged as an alternative to a variety of surgical interventions in the early 1990s. Ductal stenting in neonates with duct-dependent pulmonary circulation may maintain duct patency reliably for several months as an alternative to systemic-to-pulmonary shunts.

This is a retrospective review from a single centre with a developing paediatric cardiology unit and limited paediatric cardiac surgical scope. Eighty-nine symptomatic patients who needed stable ductal flow for adequate pulmonary circulation underwent the procedure from October 2018 to December 2022.

We had a procedural success rate of 98.8% (one case failed out of 89) and a successful discharge rate of 96.6% (85 discharged out of 88; 3 immediate mortalities). At the six-month follow-up, the success rate was 93% (6 mortalities in 88 patients). No patient underwent a rescue surgical shunt, but an elective Blalock-Taussig shunt was needed in 1 patient (1.1%). In the remaining 82 cases, 65 are now in follow-up, among them 22 already underwent single-ventricle palliation, and 8 underwent biventricular repair successfully.

Ductal stenting is less invasive well-accepted alternative for first-stage palliation. Our focus in this study is to practice this even in resource-limited settings as an alternative to surgical shunts with good short- and mid-term outcomes.

In paediatric cardiac patients requiring staged palliation, superior cavopulmonary anastomosis is common. Pain control is a crucial aspect of postoperative care as agitation, untreated pain, and hypoventilation can cause increased pulmonary vascular resistance reduction and pulmonary blood flow.

This was a large volume single-centre, retrospective cohort study evaluating the impact of gabapentin on opioid and benzodiazepine exposure in infants undergoing superior cavopulmonary anastomosis between January 2018 and December 2022. The primary endpoint was opioid exposure in morphine milligram equivalents per kilogram in infants receiving gabapentin compared to no gabapentin.

The study analysed 85 infants, 40 of which received perioperative gabapentin. Other than there being more males in the gabapentin group (70% versus 47%; p = 0.03), there was no difference in baseline characteristics. Opioid use, measured in morphine milligram equivalents per kilogram, was similar in the no gabapentin group compared to the gabapentin group during the first 5 POD’s (2.66 (interquartile range1.76, 3.30) versus 2.27 (interquartile range R 1.75, 3.40); p = 0.93. However, there was a lower benzodiazepine exposure, measured in midazolam equivalents per kilogram, in the gabapentin group both on POD 2 (0.05 (interquartile range 0.00, 0.11) versus 0 (interquartile range 0.00, 0.08); p = 0.031) and cumulative (0.15 (interquartile range 0.00, 0.35) versus 0.05 (interquartile range 0.00, 0.15); p = 0.031).

Gabapentin did not significantly reduce opioid exposure; however, its use was associated with modest reduction in benzodiazepine exposure. There were no differences in adverse events. Our findings suggest gabapentin is safe in infants undergoing superior cavopulmonary anastomosis; however, additional studies should be conducted to evaluate optimal gabapentin dosing.

Current research suggests that a small pulmonary artery can cause adverse events and reduce exercise capacity after the Fontan procedure. This study aimed to evaluate the impact of pulmonary artery size on early haemodynamic and laboratory variables after total cavopulmonary connection.

We reviewed all patients who underwent staged Fontan between 2012 and 2022. Pulmonary artery index before bidirectional cavopulmonary shunt and before total cavopulmonary connection was calculated according to Nakata and colleagues. We sought to analyse the impact of the pulmonary artery index on early haemodynamic and laboratory variables, including pulmonary artery pressure and mean arterial pressure 12 hours after extubation and lactate levels 6 hours after extubation.

A total of 263 patients were included. Median age and weight at total cavopulmonary connection were 2.2 (interquartile ranges: 1.8–2.7) years and 11.7 (interquartile range: 10.7–13.3) kg, respectively. Before that, all patients underwent bidirectional cavopulmonary shunt at a median age of 4.1 (interquartile range: 3.2–5.8) months. In the multivariable analysis, pre-bidirectional cavopulmonary shunt pulmonary artery index (p = 0.016, odds ratio 0.993), with a cut-off value of 154 mm2/m2 was an independent risk factor for a higher pulmonary artery pressure (> 17 mmHg). No variable was identified as a significant risk factor for lower mean arterial pressure (< 57 mmHg). Regarding lactate levels (> 4.5 mg/dl), pre-bidirectional cavopulmonary shunt right pulmonary artery index (p < 0.001, odds ratio 0.983), with a cut-off value of 70 mm2/m2 was identified as an independent risk factor.

In patients with staged Fontan palliation, a small pulmonary artery size before bidirectional cavopulmonary shunt and total cavopulmonary connection was a determinant factor associated with unfavourable early postoperative haemodynamics after total cavopulmonary connection.

Pulmonary oedema is a common complication after paediatric cardiac surgery, and it is linked to increased morbidity. Lung ultrasound has been recognised as an alternative to chest radiography, offering rapid and accurate diagnosis without exposure to ionising radiation.

The study aimed to investigate the association between the severity of pulmonary oedema identified through a B-lines score—a lung ultrasound score used to assess the severity of pulmonary oedema—and ventilatory outcomes in the postoperative period of cardiac surgery. It was a prospective single-centre cohort study conducted at a quaternary paediatric hospital. Patients up to 18 years old who underwent cardiac surgery for CHD were included. The primary outcome was extubation failure within 48 hours after surgery, and the secondary outcome was mechanical ventilation time.

Among the 131 included patients, 116 were extubated with an extubation failure rate of 18.3%. Patients with extubation failure were younger and had a longer duration of mechanical ventilation. A robust association between higher B-lines score on lung ultrasound within the first 24 hours after surgery and extubation failure was observed (p < 0.001). The correlation between B-lines score and mechanical ventilation time was positive, with stronger correlation preoperatively.

Severe pulmonary oedema detected through lung ultrasound in the first postoperative day of paediatric cardiac surgery show better accuracy to predict patients at greater risk of extubation failure and prolonged mechanical ventilation.

We sought to assess the degree to which environmental risk factors affect CHD prevalence using a case–control study.

A hospital-based study was conducted by collecting data from outpatients between January 2016 and January 2021, which included 31 CHD cases and 72 controls from eastern China. Risk ratios were estimated using univariate and multivariate logistic regression models and mediating effect analysis.

Residential characteristics (usage of cement flooring, odds ratio = 17.04[1.954–148.574], P = 0.01; musty smell, odds ratio = 3.105[1.198–8.051], P = 0.02) and indoor total volatile organic compound levels of participants’ room (odds ratio = 31.846[8.187–123.872, P < 0.001), benzene level (odds ratio = 7.370[2.289–23.726], P = 0.001) increased the risk of CHDs in offspring. And folic acid plays a masking effect, which mitigates the affection of the total volatile organic compound (indirect effect = -0.072[−0.138,-0.033]) and formaldehyde (indirect effect = −0.109[-0.381,-0.006]) levels on the incidence of CHDs. While food intake including milk (odds ratio = 0.396[0.16–0.977], P = 0.044), sea fish (odds ratio = 0.273[0.086–0.867], P = 0.028), and wheat (odds ratio = 0.390[0.154–0.990], P = 0.048) were all protective factors for the occurrence of CHDs. Factors including women reproductive history (history of conception control, odds ratio = 2.648[1.062–6.603], P = 0.037; history of threatened abortion, odds ratio = 2.632[1.005–6.894], P = 0.049; history of dysmenorrhoea (odds ratio = 2.720[1.075–6.878], P = 0.035); sleep status (napping habit during daytime, odds ratio = 0.856[0.355–2.063], P = 0.047; poor sleep quality, odds ratio = 3.180[1.037–9.754], P = 0.043); and work status (working time > 40h weekly, odds ratio = 2.882[1.172–7.086], P = 0.021) also influenced the CHDs incidence to differing degrees.

Diet habits, nutrients intake, psychological status of pregnant women, and residential air quality were associated with fetal CHDs. Indoor total volatile organic compound content was significantly correlated with CHDs risk, and folic acid may serve as a masking factor that reduce the harmful effects of air pollutants.

Membranous subaortic stenosis is a CHD with high recurrence-rate despite surgical treatment. This study investigated the outcome of operated patients and possible predictors for recurrence.

Retrospective review of all patients (n = 38) ≤ 18 years of age operated for membranous subaortic stenosis between 1994–2019 at Sahlgrenska University Hospital. The primary outcomes were recurrence, reintervention, and mortality. Predictors of recurrence and reintervention were secondary outcomes.

Median age (range) at diagnosis, initial intervention, and last follow-up were 2.3 (0.003–17.2), 5.3 (0.03–17.5) and 17.5 (3.6–20.4) years, respectively. Median follow-up time was 9.9 (0.01–19.5) years. 61% were males, and 53% had other associated CHD. 19 patients (56%) developed recurrence and 7 (21%) underwent reintervention. One patient died peri-operatively. Age <5 years at first intervention increased the likelihood of reintervention. Postoperative peak/mean gradients were higher in patients with disease recurrence.

The median echocardiographic peak-/mean gradients at initial diagnosis, pre-, postoperative, and at last follow-up were 61/36, 83/50, 16/8, and 19/17 mmHg respectively (p < 0.0001 pre/post). Pre-/postoperative peak gradients were linearly correlated, decreasing by 80% pre-/postoperatively (p < 0.01). Presence of symptoms and the preoperative peak gradient were positively associated (p < 0.001) with a peak gradient threshold value of > 90 mmHg. The distance between the subaortic stenosis membrane and the aortic valve was inversely correlated to the preoperative peak-gradient (p < 0.01).

Reintervention following surgical intervention of membranous subaortic stenosis is common. A positive correlation exists between high pre- and postoperative peak-gradient. A low postoperative peak gradient may be important in avoiding recurrence.

Medical advances in the management of CHD have shifted the focus from childhood mortality to life-long morbidity; therefore, clinical research in paediatric cardiology tends to replace mortality outcomes with functional and patient-reported outcomes. Despite these advances, the stratification of disease severity using a simple and reproducible CHD classification has not been established. The aim was to determine which classification best predicts functional status in children with CHD, in terms of cardiopulmonary fitness.

This retrospective cohort study was assessed from a cohort study of 296 children (mean age 11.3 ± 3.1 years, 129 female), who underwent a baseline and final cardiopulmonary exercise tests with a mean follow-up of 4.1 ± 1.6 years.

Seven CHD classifications were identified, assessing anatomical, physiological, therapeutic, or functional parameters in foetal, paediatric, or adult CHD populations. The four-stage paediatric CHD disease severity classification established by Uzark et al. best predicted functional status at the final cardiopulmonary exercise test assessment, with an area under the receiver operating characteristics curves of 0.62 (0.55–0.69) for impaired cardiopulmonary fitness (maximum oxygen uptake (VO2max) or ventilatory anaerobic threshold < −1.64 Z-score). The best inter-judge classification agreement was also observed for Uzark classification, with a Kappa coefficient of 0.88 (0.78–0.98).

The CHD classification by Uzark is reliable in terms of functional status prediction and reproducibility in children with CHD. This simple classification may contribute to identifying children with CHD most at risk of cardiopulmonary fitness impairment and initiating early preventive cardiovascular interventions in paediatric cardiology.

Left ventricular function after arterial switch operation for d-transposition of the great arteries is notoriously compromised because of abnormal coronary artery anatomy or altered loading conditions. We sought to longitudinally investigate the performance of the left ventricle in a cohort of d-transposition of the great artery patients after arterial switch operation, by using advanced echocardiographic deformation imaging and grouping patients according to pre- and post-surgery variables, labelled as risk factors.

Longitudinal single-centre study involving 53 d-transposition of the great artery patients (81.1% male) after arterial switch operation, the latter being performed as unique surgical procedure in 39 patients (76.5%). Median follow-up was 59 months [23.5–72].

Selected patients were split into two groups according to risk factors. Fifteen patients (30.6%) were grouped into high-risk class (<3 risk factors). Echocardiographic variables such as tricuspid annular plane systolic excursion, ejection fraction, and global longitudinal strain were compared between the two groups. Only global longitudinal strain reached statistical significance (−17.56 ± 2.26 versus −19.82 ± 1.97 %; p < 0.001). To discriminate high- versus low-risk patients, a receiver operating characteristic (ROC) curve identified a global longitudinal strain cut-off value of −17.75% (sensitivity 57.1%, specificity 97%, AUC 80%).

Several neonatal and post-surgical variables might conditionate long-term follow-up of d-transposition of the great artery patients after arterial switch operation, and global longitudinal strain best conveys the overall risk profile of these patients.

To assess short- and medium-term outcomes of VenusP-valve implantation in the pulmonary position in the paediatric population.

Percutaneous pulmonary valve implantation is now an established alternative to surgical pulmonary valve replacement, especially in those with conduits in the right ventricular outflow tract. The VenusP-valve™ (Venus Medtech, Shanghai, China) has demonstrated early efficacy in the adult population with larger conduit-free right ventricular outflow tracts. However, its use in children has not been well described.

Retrospective review of patients under 18 years of age undergoing VenusP-valve implantation at a single institution between June 2015 and February 2023.

Fifteen patients under the age of 18 years underwent VenusP-valve™ implantation. All had severe pulmonary regurgitation and fulfilled accepted criteria for pulmonary valve implantation. Mean age at valve implantation was 14.1 (range 9.8–17.9) years, and mean weight was 54.9 (34.0–98.5) kg. The valve was deployed successfully in all the patients. The valve diameter and length ranged between 28–36 mm and 25–35 mm, respectively. Mean follow-up was 3.4 (0.5–8.1) years. At follow-up, twelve patients have undergone magnetic resonance imaging MRI as part of the regular surveillance. Indexed right ventricular end-diastolic volume improved from 157.8 (140.0–197.0) ml/m2 to 117.6 (91.0–152.0) ml/m2 (p = 0.004). Pulmonary regurgitation fraction had reduced from a mean of 44.3 (31.0–60.0) % to 3.6 (0.0–15.0) % (p = 0.003).

This study demonstrates the safety and feasibility of the VenusP-valve implantation in children. Medium-term follow-up suggests that valve implantation is associated with a reduction in the degree of pulmonary regurgitation and right ventricular end-diastolic volume.