The impact of absent ductus arteriosus in fetuses with Tetralogy of Fallot remains uncertain with concerns for the lack of prostaglandin responsiveness and compromised pulmonary blood flow. This study evaluated early postnatal outcomes in fetuses with Tetralogy of Fallot-absent ductus arteriosus compared to matched controls with a ductus arteriosus and assessed the predictive value of fetal and neonatal echocardiographic parameters for early intervention.

A retrospective matched cohort study was performed using a single-centre fetal echocardiography database (2000–2024). Fetuses with Tetralogy of Fallot-absent ductus arteriosus confirmed by neonatal echocardiography were matched 1:1 to Tetralogy of Fallot-ductus arteriosus cases by fetal pulmonary valve Z-score and gestational age. Early intervention was defined as any surgical or transcatheter procedure to augment pulmonary blood flow within six weeks of life. Comparative analyses evaluated predictors of early intervention within each cohort.

Among 253 fetuses with Tetralogy of Fallot and antegrade outflow, 27 had confirmed absent ductus arteriosus and were matched to 27 Tetralogy of Fallot-ductus arteriosus controls. Despite similar fetal pulmonary valve Z-scores, Tetralogy of Fallot-absent ductus arteriosus patients had significantly lower neonatal pulmonary valve and main pulmonary artery Z-scores and main pulmonary artery-to-aorta ratios. Early intervention occurred in 26% of Tetralogy of Fallot-absent ductus arteriosus and 41% of Tetralogy of Fallot-ductus arteriosus (p = 0.25); surgical intervention was more frequent in Tetralogy of Fallot-absent ductus arteriosus (86 vs 36%, p = 0.066). In Tetralogy of Fallot-ductus arteriosus, lower fetal and neonatal pulmonary valve/aortic valve ratios predicted early intervention. No fetal or neonatal markers were predictive in Tetralogy of Fallot-absent ductus arteriosus.

Fetal absent ductus arteriosus was not linked to higher early intervention rates in Tetralogy of Fallot, but when intervention was required, surgical palliation predominated. Conventional fetal echocardiographic predictors were not reliable in Tetralogy of Fallot-absent ductus arteriosus, complicating prenatal counselling.

Limited data exist on how trainers are trained in paediatric cardiology training centres in Europe.

A cross-sectional study employing a structured and approved questionnaire was circulated to educationalists/trainers in 95 Association for European Paediatric and Congenital Cardiology training centres.

Trainers provided complete data for 46 centres in 20 countries. The median number of trainers in each centre was 6 (range 1–16). The median number of years trainers were in a training role was 20 years (range 2–32 years). Sixty-six per cent of trainers received some training in being a trainer, most commonly a course by a local governing college (almost 50%). Almost 78% found such courses helpful as trainers. Sixty-eight per cent of trainers felt their education in training was optimal to be an effective trainer. Assessment of trainees varies from daily to monthly between centres. Workplace assessments (used by >90% trainers) with combined formative and summative feedback were the most common type of assessment. Only one-third of trainers understood or used entrustable professional activities. Time constraints in providing training were reported by 54% of trainers as the greatest challenge in providing training. The majority of trainers expressed a need for formal “training the trainers” courses and development of standards in training.

There is a marked variation in the level of training of trainers across Europe. A wide mix of assessment tools is used. Feedback is provided by the majority of trainers. Adopting a basic training programme for trainers may promote the training skills of paediatric cardiology trainers.

Patients with cyanotic CHD and those with metabolic dysfunction–associated steatotic liver disease are at risk of liver fibrosis. We compared hepatic extracellular volumes and native T1 values to better understand the burden of liver disease in these populations.

The sample comprised 136 patients in 5 groups: control (n = 23), metabolic dysfunction–associated steatotic liver disease [mild (F0–F1) and significant (F2–F4) fibrosis; n = 45], repaired tetralogy of Fallot (n = 30), and Fontan circulation (n = 38). Differences were assessed using linear regression models, with adjustment for the body mass index and sex.

The hepatic extracellular volume was significantly larger in the Fontan group (43.96% ± 4.22%) than in the other groups, even with adjustment. Patients with Fallot had significantly larger extracellular volumes (36.77% ± 5.63%) than did controls and mild liver disease (p < 0.001 and p = 0.011, respectively), although smaller extracellular volumes than patients with significant liver disease (p = 0.042). These trends were corroborated by native T1 values, which were highest in patients with Fontan (1013.7 ± 86.1 ms), although not significantly different from patients with F2–F4 steatotic liver disease.

The potential burden of CHD-related hepatic injury and steatotic liver disease highlights the importance of early identification. Given the possible additional risk of liver fibrosis in patients with coexisting metabolic dysfunction and CHD, comprehensive clinical management should prioritise regular metabolic risk assessment and the promotion of a healthy lifestyle to reduce the likelihood of liver disease development in this vulnerable population.

Children with CHD are at increased risk for feeding difficulties, yet the prevalence and predictors of paediatric feeding disorder in this population remain underexplored.

To evaluate the prevalence of paediatric feeding disorder and identify consistent predictors of feeding difficulties in children with CHD 18-<25 months of age.

A retrospective review was conducted on 159 children diagnosed with CHD. Paediatric feeding disorder was defined using consensus criteria encompassing nutritional status and feeding skill domains. Feeding outcomes were assessed at 18-<25 months, regardless of the method of feeding (oral or tube-fed). Medical history, growth, and neurodevelopmental status were analysed to identify predictors of paediatric feeding disorder.

At 18-<25 months, 58% of children met criteria for paediatric feeding disorder. Among exclusively orally fed children, 41% still qualified, indicating persistent dysfunction beyond tube dependence. Significant challenges were observed in nutrition and feeding skill domains. One-third relied on formula and overnight feeds, reflecting high energy needs and possibly inefficiencies. While 74% had transitioned to cup drinking, 21% struggled, particularly those born preterm or with neurodevelopmental delays. Texture progression was delayed: 29% had no table foods, and among those who did, 67% had chewing difficulties. Predictors of paediatric feeding disorder included medical/genetic comorbidities, low weight, prolonged hospitalisation, low maternal education, and delays in cognitive, language, and motor development.

Paediatric feeding disorder is highly prevalent in children with CHD, including those feeding orally. Early risk factors are associated with domain-specific feeding challenges, emphasising the need for individualised, developmentally informed feeding and nutrition care plans in this high-risk population.

In children with tetralogy of Fallot and associated right ventricular outflow tract obstruction, right ventricular outflow tract stenting has emerged as a viable alternative to surgical shunting, particularly in high-risk patients who are unsuitable for early definitive repair.

This prospective and retrospective observational study involved 55 symptomatic children with Fallot-type physiology who underwent right ventricular outflow tract stenting over a 30-month period at a tertiary-care centre in India. Data from pre-procedural imaging, intraoperative parameters, and post-procedural outcomes were analysed. The primary endpoints were improvement in systemic oxygen saturation and pulmonary artery growth. Secondary endpoints included complication rates, ICU stay duration, and factors associated with procedural failure.

The median age at intervention was 11 months. All patients presented with cyanosis, and 80% had documented cyanotic spells. The right internal jugular vein was used for vascular access in 75% of cases. A significant improvement was observed in systemic oxygen saturation (from 67.8 to 87.1%, p < 0.001), along with an increase in pulmonary artery Z-scores. Complications included pulmonary oedema (47%), stent migration (7.2%), and new-onset tricuspid regurgitation (26.9%). The procedure had a success rate of 83.6%. Although no significant predictors of failure or prolonged ICU stay were identified, furosemide use was associated with a longer recovery time.

Right ventricular outflow tract stenting serves as a safe, effective, and reproducible palliative option in selected neonates with Fallot physiology. It facilitates improved systemic oxygenation and promotes pulmonary artery development, thereby serving as a bridge to definitive surgical repair.

Noonan syndrome is characterised by typical facial features, short stature, CHDs, and other comorbidities, which are caused by germline mutations in genes coding for components of the Ras-mitogen-activated protein kinase pathway. Noonan syndrome is an inherited disease involving multiple systems, but ventricular arrhythmia in Noonan syndrome is rarely reported.

Here we report a 15-year-old patient with leucine zipperlike transcription regulator 1-associated Noonan syndrome, who has CHD (left ventricular hypertrophy with left ventricular outflow tract obstruction), ventricular arrhythmia, bundle branch block, pectus excavatum, costal eversion, scoliosis, myopia, growth retardation, hearing loss, chest tightness, and fatigue. Chest tightness and fatigue are the main reasons for admission of the patient. The patient was treated with spironolactone, empagliflozin, tolasemide, potassium chloride, and bisoprolol. One month after treatment, the patient has no more chest tightness or fatigue. Genetic testing revealed that the patient had a novel heterozygous variant c.313delT (p.trp105Glyfs * 42) mutation in the leucine zipperlike transcription regulator 1. We provide a review of the literature of leucine zipperlike transcription regulator 1 mutations and find that ventricular arrhythmias have been reported in leucine zipperlike transcription regulator 1-related Noonan syndrome.

Our findings expand on the Noonan syndrome phenotype and suggest that mutations in the leucine zipperlike transcription regulator 1 gene are involved in ventricular arrhythmia.

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.