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.

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.

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.

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.

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.

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.

The Pediatric Acute Care Cardiology Collaborative (PAC3) previously showed decreased postoperative chest tube duration and length of stay in children undergoing 9 Society of Thoracic Surgeons benchmark operations. Here we report how these gains were sustained over time and spread to 8 additional centers within the PAC3 network.

Patient data were prospectively collected across baseline and intervention phases at the original 9 centres (Pioneer) and 8 new centres (Spread). The Pioneer baseline phase was 6/2017–6/2018 and Spread was 5/2019–9/2019. The Pioneer intervention phase was 7/2018–7/2021 and Spread 10/2019–7/2021. The primary outcome measure was postoperative chest tube duration in hours, with the aim of 20% overall reduction. Balancing measures included chest tube reinsertion and readmission for pleural effusion. Statistical process control methods and traditional statistics were used to analyse outcomes over time.

Among 5,042 patients at 17 centres, demographics were comparable. The Pioneer cohort (n = 3,383) sustained a 22.6% reduction in mean chest tube duration (from 91.9 hours to 70.5 hours), while the Spread cohort (n = 1,659) showed a 9.7% reduction (from 73.1 hours to 66.0 hours) in the first 13 months following intervention. Across both cohorts, rates of reinsertion (2.0% versus 2.1%, p = 0.869) and readmission for effusion did not change (0.3% versus 0.5%, p = 0.285).

This multicenter prospective quality improvement study demonstrated sustained reduction in chest tube duration at 9 centres while successfully spreading improvement to 8 additional centres. This project serves as a model for post-operative multicentre quality improvement across a large cohort of congenital cardiac surgery patients.

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.

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.

Ventricular tachycardia is a rare condition in the fetus and neonate with a paucity of data regarding the management and outcomes.

We reviewed the clinical histories, associated CHD and syndromes, diagnostic investigations, management strategies, and outcomes for all fetuses and neonates with ventricular tachycardia encountered between 2005 and 2020.

Five fetal and 8 neonatal cases of ventricular tachycardia were encountered. Two (40%) fetal cases, compared to 5 (62%) neonatal cases had CHD (p = 0.59), and only 1 fetus had cardiomyopathy with findings suggesting restriction before ventricular tachycardia onset. The median age at ventricular tachycardia presentation was 32 (26–37) weeks for fetal and 11 (1–27) days for neonatal cases. Of the fetal cases, 2 were initially treated trans-placentally with propranolol and 2 with amiodarone. Two fetuses (40%) had ventricular tachycardia suppression prenatally that recurred during the neonatal period necessitating propranolol therapy, 2 (40%) had resolution before birth with no postnatal recurrence, and the cardiomyopathy case never achieved full control, developed hydrops and demised. Of the neonatal cases, 4 received intravenous antiarrhythmics on admission: 3 amiodarone and 1 esmolol, and 2 of these were converted to propranolol and 2 to sotalol. Three other neonates initially received propranolol, with 1 discharged on propranolol, 1 on sotalol, and 1 on mexiletine after failed propranolol and sotalol treatment. The 8th neonate was discharged on no medication.

Most fetal and neonatal ventricular tachycardia is manageable with pharmacologic therapy. Given its rarity, larger studies are needed to identify optimal management strategies.

The Child Opportunity Index is an index of 29 indicators of social determinants of health linked to the United States of America Census. Disparities in the treatment of Wolff–Parkinson–White have not be reported. We hypothesise that lower Child Opportunity Index levels are associated with greater disease burden (antiarrhythmic use, ablation success, and Wolff–Parkinson–White recurrence) and ablation utilisation.

A retrospective, single-centre study was performed with Wolff–Parkinson–White patients who received care from January 2021 to July 2023. Following exclusion for <5 years old and with haemodynamically significant CHD, 267 patients were included (45% high, 30% moderate, and 25% low Child Opportunity Index). Multi-level logistic and log-linear regression was performed to assess the relationship between Child Opportunity Index levels and outcomes.

Low patients were more likely to be Black (p < 0.0001) and to have public insurance (p = 0.0006), though, there were no significant differences in ablation utilisation (p = 0.44) or time from diagnosis to ablation (p = 0.37) between groups. There was an inverse relationship with emergency department use (p = 0.007). The low group had 2.8 times greater odds of having one or more emergency department visits compared to the high group (p = 0.004).

The Child Opportunity Index was not related with ablation utilisation, while there was an inverse relationship in emergency department use. These findings suggest that while social determinants of health, as measured by Child Opportunity Index, may influence emergency department utilisation, they do not appear to impact the overall management and procedural timing for Wolff–Parkinson–White treatment.

Bicuspid aortic valve is considered to have a multifactorial origin. Some research suggests a defect in neural crest cell signalling may increase the risk of developing bicuspid aortic valve, and also impact on the proximal conduction system.

To examine electrocardiographic parameters in unselected newborns from the general population diagnosed with bicuspid aortic valve within 30 days after birth.

This is a substudy of the Copenhagen Baby Heart Study; a multicentre, prospective, population-based cohort study with prenatal inclusion. Cardiac examination, including transthoracic echocardiography and electrocardiography, were obtained within 30 days after birth. Newborns diagnosed with bicuspid aortic valve were matched 1:4 with newborns with structurally normal hearts based on age, sex, gestational age, weight, and length at examination.

A total of 127 newborns with bicuspid aortic valve (84 boys, median age 11 days) and 508 controls (336 boys, median age 11 days) were included. Newborns with bicuspid aortic valve had a significantly longer PR-interval (100 vs 96 ms, p = 0.011) and QRS duration (56 vs 54 ms, p = 0.042), and a significantly lower R-wave amplitude in V6 (759 vs 906 µV, p = 0.047) compared to controls. However, when correcting for multiple testing none of the results were significant.

Newborns from the general population with bicuspid aortic valve demonstrated a slightly longer PR-interval, a longer QRS duration, and a lower maximum R-wave amplitude in V6 than matched controls, although non-significant after correcting for multiple testing. This may represent early signs of conduction abnormalities, but longitudinal follow-up will provide further clarification.

The Takeuchi procedure remains an important surgical option for treating anomalous origin of the left coronary artery from the pulmonary artery, particularly in cases where direct coronary reimplantation is not feasible. However, long-term outcome data in paediatric patients are limited.

We retrospectively reviewed 18 paediatric patients who underwent Takeuchi repair between 2007 and 2023. Clinical characteristics, echocardiographic data, and outcomes—including survival, ventricular function, mitral regurgitation, and reintervention—were analysed. Kaplan–Meier analysis was used to assess survival and freedom from reintervention, and paired comparisons were evaluated using the Wilcoxon signed-rank test.

The median age at surgery was 6 months (range: 25 days to 12 years). Preoperative left ventricular ejection fraction was significantly depressed (median 23.5%), and mitral regurgitation was present in all patients. There were two early deaths (11.1%), both in patients with severe heart failure. No late mortality was observed during a maximum follow-up of 10.9 years. All survivors achieved New York Heart Association class I status. Left ventricular ejection fraction improved significantly postoperatively (p < 0.0001), and mitral regurgitation grade also decreased significantly (p < 0.001), with 94.4% showing only mild residual mitral regurgitation. Reintervention occurred in three patients (16.7%) for pulmonary artery stenosis or baffle leak. Freedom from reintervention at 10.9 years was 66.7%.

The Takeuchi procedure offers excellent survival and functional recovery in paediatric anomalous origin of the left coronary artery from the pulmonary artery patients when coronary translocation is not feasible. Although late complications such as pulmonary artery stenosis or baffle leak can occur, outcomes remain favourable with appropriate follow-up.

To describe the experience of a series of paediatric patients with Fontan-associated liver dysfunction.

A retrospective study including 12 patients with Fontan-associated liver disease. Patients were selected from the single-ventricle program at a high-complexity centre in Colombia between 2001 and 2024.

During the study period, 108 patients were in the Fontan stage. Among them, 12 met the criteria for Fontan-associated liver disease (11.1%). The median age at extracardiac Fontan completion was 3.9 years, while the median age at Fontan-associated liver disease diagnosis was 14.5 years. Concomitant protein-losing enteropathy and/or plastic bronchitis were present in 33% of cases. Echocardiographic follow-up showed systolic dysfunction in 41% and diastolic dysfunction in 16% of patients. All patients exhibited some degree of valvular insufficiency, mild in 83.3% and moderate in 16%. Haemodynamic variables at the time of diagnosis did not show significant abnormalities.

Liver disease is a persistent concern in paediatric patients with Fontan physiology, posing diagnostic and therapeutic challenges. Healthcare professionals managing these patients should be aware of its early identification and appropriate treatment.

Persistent pulmonary hypertension of the newborn is a serious disease with significant morbidity and mortality. Magnesium sulphate has been proposed as a potential treatment for persistent pulmonary hypertension of the newborn, but its efficacy remains unclear. The article aims to evaluate the effects of magnesium sulphate on persistent pulmonary hypertension of the newborn.

A comprehensive search of PubMed, Web of Science, Embase, and Cochrane Library was conducted to identify relevant studies. The primary outcomes were pulmonary artery pressure and oxygenation index, while secondary outcomes included mean blood pressure, alveolar-arterial oxygen difference, arterial partial pressure of oxygen (PaO2), arterial partial pressure of carbon dioxide (PaCO2), and arterial oxygen saturation. Statistical analysis was performed by Cochrane Review Manager 5.3.

The study analysed twelve studies involving 380 patients. Results indicated that magnesium sulphate treatment significantly reduced pulmonary artery pressure levels (MD −24.96, 95% CI −28.19 to −21.73, P < 0.0001) and mean blood pressure (MD −3.11, 95% CI −3.91 to −2.32, P < 0.0001) compared to pretreatment. Additionally, it led to a notable decrease in oxygenation index (P < 0.00001) and alveolar-arterial oxygen difference (P < 0.0001), while increasing PaO2 (P < 0.0001) and arterial oxygen saturation (P < 0.001). However, there was no significant effect on PaCO2 levels compared to pretreatment.

Magnesium sulphate is a valuable therapy for persistent pulmonary hypertension of the newborn. It markedly reduced pulmonary artery pressure, alveolar-arterial oxygen difference, and oxygenation index, while enhancing PaO2, and arterial oxygen saturation, with no impact on PaCO2 levels. Magnesium sulphate may also reduce mean blood pressure following a 2-hour treatment. Additional studies are necessary to further clarify its efficacy and potential side effects.

This study was registered with PROSPERO (CRD42024578092).