Paediatric coronary artery bypass surgery with internal thoracic artery grafting is the optimal choice for left main coronary artery atresia; we report successful reconstruction in a patient with bypass graft obstruction. The pulmonary trunk was transected to expose the left main coronary stem, which was opened beyond the obstruction and the incision extended through the left main coronary artery to the circumflex bifurcation and left anterior descending artery. A funnel-shaped coronary ostium was created with a glutaraldehyde-treated autologous pericardium onlay patch. Surgical reconstruction is a good alternative for left main coronary artery atresia with graft obstruction and preserves the remaining internal thoracic artery for future use.

Aneurysm and pseudoaneurysm development is a known, albeit uncommon, complication after right ventricular outflow tract surgical reconstruction. Large right ventricular outflow tract aneurysms and pseudoaneurysms have not been extensively described in recent literature and we report our experience with this unusual complication in five patients at our institution over the last 8 years. Although uncommon, this complication has potentially important clinical implications. Thus, clinicians should be aware of its potential, particularly in certain anatomic conditions.

Hybrid cardiac surgery involves simultaneous catheter based and surgical treatment of heart diseases. We report a complete repair of dextro-transposition of the great arteries and perimembranous and muscular ventricular septal defects in a 5-month-old girl. This included an arterial switch operation followed by an off-bypass, echocardiographically guided per-ventricular muscular ventricular septal defect closure.

Transposition of the great arteries combined with totally anomalous pulmonary venous connection is extremely rare outside of heterotaxy syndrome. Most reported cases have been treated by a modified atrial switch operation. We report the successful treatment of a neonate with this rare association, repaired by arterial switch operation and connection of the pulmonary venous return to the left atrium.

The ductus arteriosus (DA) connects the pulmonary artery to the aorta to bypass the pulmonary circulation in utero. It normally closes within 24–72 hours after birth due to increased pulmonary resistance from an increase in oxygen partial pressure with the baby’s first breath. Medical treatment can help close the DA in certain situations where closure is delayed. However, in duct-dependent cardiac defects, the presence of the DA is crucial for survival and as such medical and surgical techniques have evolved to prevent closure. This review aims to outline the two main management options for keeping a ductus arteriosus patent. This includes stenting the PDA and shunting via a modified Blalock–Taussig shunt. Whilst both techniques exist, multicentre trials have found equal mortality end points but significantly reduced morbidity with stenting than shunting. This is also reflected by shorter recovery times, reduced requirement for extracorporeal membrane oxygenation (ECMO), and improved quality of life, although stent longevity remains a limiting factor.

Improved survival of patients with paediatric and congenital cardiac disease has led to a heightened awareness of the cognitive, neurodevelopmental, psychosocial, and physicalfunctioning deficits that limit health, academic functioning, adaptive functioning, behavioural and emotional outcomes, health-related quality of life, and well-being for children and adults with congenital heart disease. The Cardiac Neurodevelopmental Outcome Collaborative was founded in 2016; its mission is to determine and implement best practices of neurodevelopmental and psychosocial services for individuals with paediatric and congenital heart disease and their families through clinical, quality improvement, and research initiatives.

Cardiology in the Young is devoted to cardiovascular issues affecting the young, and older patients with congenital heart disease, or with other cardiac diseases acquired during childhood. The journal serves the interests of all professionals concerned with these topics. By design, the journal is international and multidisciplinary in its approach, and members of the editorial board take an active role in its mission, helping to make it an indispensable reference for paediatric and congenital cardiac care. All aspects of paediatric and congenital cardiac care are covered in the journal. The content includes original articles, brief reports, editorials, reviews, and papers devoted to continuing professional development. High-quality colour figures are published on a regular basis, and without charge to the authors. Regular supplements are published containing the abstracts of the annual meetings of the Association for European Paediatric and Congenital Cardiology, along with other occasional supplements. These supplements are supplied free to subscribers.

The vision of Cardiology in the Young is to use print and electronic media to improve paediatric and congenital cardiac care. The mission of Cardiology in the Young is to be a premier global journal for paediatric and congenital cardiac care – an essential journal that spans the domains of patient care, research, education, and advocacy, and also spans geographical, temporal, and subspecialty boundaries.

Cardiology in the Young and the Cardiac Neurodevelopmental Outcome Collaborative are pleased to announce that Cardiology in the Young will serve as the official journal of the Cardiac Neurodevelopmental Outcome Collaborative. This new partnership between the Cardiac Neurodevelopmental Outcome Collaborative and Cardiology in the Young will facilitate the dissemination of knowledge and recommendations to the scientific, clinical, and patient advocacy communities, ultimately improving paediatric and congenital cardiac care and outcomes for individuals with paediatric and congenital heart disease and their families.

Compared to the general population, individuals with complex congenital heart disease are at increased risk for deficits in cognitive, neurodevelopmental, psychosocial, and physical functioning, resulting in a diminished health-related quality of life. These deficits have been well described over the past 25 years, but significant gaps remain in our understanding of the best practices to improve neurodevelopmental and psychosocial outcomes and health-related quality of life for individuals with paediatric and congenital heart disease. Innovative clinical, quality improvement, and research opportunities with collaboration across multiple disciplines and institutions were needed to address these gaps. The Cardiac Neurodevelopmental Outcome Collaborative was founded in 2016 with a described mission to determine and implement best practices of neurodevelopmental and psychosocial services for individuals and their families with paediatric and congenital heart disease through clinical, quality improvement, and research initiatives. The vision is to be a multi-centre, multi-national, multi-disciplinary group of healthcare professionals committed to working together and partnering with families to optimise neurodevelopmental outcomes for individuals with paediatric and congenital heart disease through clinical, quality, and research initiatives, intending to maximise quality of life for every individual across the lifespan. This manuscript describes the development and organisation of the Cardiac Neurodevelopmental Outcome Collaborative.

Over the last two decades, heart centres have developed strategies to meet the neurodevelopmental needs of children with congenital heart disease. Since the publication of guidelines in 2012, cardiac neurodevelopmental follow-up programmes have become more widespread. Local neurodevelopmental programmes, however, have been developed independently in widely varying environments. We sought to characterise variation in structure and personnel in cardiac neurodevelopmental programmes. A 31-item survey was sent to all member institutions of the Cardiac Neurodevelopmental Outcome Collaborative. Multidisciplinary teams at each centre completed the survey. Responses were compiled in a descriptive fashion. Of the 29 invited centres, 23 responded to the survey (79%). Centres reported more anticipated neurodevelopment visits between birth and 5 years of age (median 5, range 2–8) than 5–18 years (median 2, range 0–10) with 53% of centres lacking any standard for routine neurodevelopment evaluations after 5 years of age. Estimated annual neurodevelopment clinic volume ranged from 85 to 428 visits with a median of 16% of visits involving children >5 years of age. Among responding centres, the Bayley Scales of Infant and Toddler Development and Wechsler Preschool and Primary Scale of Intelligence were the most routinely used tests. Neonatal clinical assessment was more common (64%) than routine neonatal brain imaging (23%) during hospitalisation. In response to clinical need and published guidelines, centres have established formal cardiac neurodevelopment follow-up programmes. Centres vary considerably in their approaches to routine screening and objective testing, with many centres currently focussing their resources on evaluating younger patients.

This paper provides specific guidelines for the neurodevelopmental evaluation of children aged birth through 5 years with complex congenital heart disease. There is wide recognition that children with congenital heart disease are at high risk for neurodevelopmental impairments that are first apparent in infancy and often persist as children mature. Impairments among children with complex congenital heart disease cross developmental domains and affect multiple functional abilities. The guidelines provided are derived from the substantial body of research generated over the past 30 years describing the characteristic developmental profiles and the long-term trajectories of children surviving with complex congenital heart conditions. The content and the timing of the guidelines are consistent with the 2012 American Heart Association and the American Academy of Pediatrics scientific statement documenting the need for ongoing developmental monitoring and assessment from infancy through adolescence. The specific guidelines offered in this article were developed by a multidisciplinary clinical research team affiliated with the Cardiac Neurodevelopmental Outcome Collaborative, a not-for-profit organisation established to determine and implement best neurodevelopmental practices for children with congenital heart disease. The guidelines are designed for use in clinical and research applications and offer an abbreviated core protocol and an extended version that expands the scope of the evaluation. The guidelines emphasise the value of early risk identification, use of evidence-based assessment instruments, consideration of family and cultural preferences, and the importance of providing multidimensional community-based services to remediate risk.

In 2012, the American Heart Association and the American Academy of Paediatrics released a scientific statement with guidelines for the evaluation and management of the neurodevelopmental needs of children with CHD. Decades of outcome research now highlight a range of cognitive, learning, motor, and psychosocial vulnerabilities affecting individuals with CHD across the lifespan. The number of institutions with Cardiac Neurodevelopmental Follow-Up Programmes and services for CHD is growing worldwide. This manuscript provides an expanded set of neurodevelopmental evaluation strategies and considerations for professionals working with school-age children with CHD. Recommendations begin with the referral process and access to the evaluation, the importance of considering medical risk factors (e.g., genetic disorders, neuroimaging), and the initial clinical interview with the family. The neurodevelopmental evaluation should take into account both family and patient factors, including the child/family’s primary language, country of origin, and other cultural factors, as well as critical stages in development that place the child at higher risk. Domains of assessment are reviewed with emphasis on target areas in need of evaluation based on current outcome research with CHD. Finally, current recommendations are made for assessment batteries using a brief core battery and an extended comprehensive clinical battery. Consistent use of a recommended assessment battery will increase opportunities for research collaborations, and ultimately help improve the quality of care for families and children with CHD.

Bronchogenic cysts are considered rare airway anomalies that can have a significant impact on the well-being of infants. In this case report, we present a rare presentation of bronchogenic cyst presenting with early neonatal respiratory distress due to airway and vascular compression. Surgical excision was curative with an excellent prognosis.

Exercise capacity is a modifiable factor in patients with CHD that has been related to surgical outcomes in adults. We hypothesised that this was true for children undergoing surgical pulmonary valve replacement; therefore, the relationship of preoperative percent predicted peak oxygen consumption to surgical outcomes as measured by total hospital length of stay was explored.

Pulmonary artery aneurysms are rare. They are characterised by an aneurysmatic dilatation of the pulmonary vascular bed, including the main pulmonary artery or the accompanying pulmonary artery branches. Increases in pulmonary flow and pulmonary artery pressure increase the risk of rupture: when these conditions are detected, surgical intervention is necessary.

This study is a retrospective analysis of 33 patients treated in our paediatric cardiac surgery clinic from 2012 to 2020. Aneurysms and pseudoaneurysms in patients who were patched for right ventricular outflow tract reconstruction and corrected with a conduit were excluded from the study. Seventeen (51.5%) of the patients included in the study were female and 16 (48.5%) were male. The patients were aged between 23 and 61 years (mean 30.66 ± 12.72 years). Graft interpositions were performed in 10 patients (30.3%) and pulmonary artery plications were performed in 23 patients (69.7%) to repair aneurysms. There was no significant difference in mortality between the two groups (p > 0.05).

Pulmonary artery aneurysm interventions are safe, life-saving treatments that prevent fatal complications such as ruptures, but at present there is no clear guidance regarding surgical timing or treatment strategies. Pulmonary artery interventions should be performed in symptomatic patients with dilations ≥5 cm or asymptomatic patients with dilations ≥8 cm; pulmonary artery pressure, right ventricular systolic pressure, and pulmonary artery aneurysm diameter must be considered when planning surgeries, their timing, and making decisions regarding indications. Experienced surgical teams can achieve satisfactory results using one of the following surgical techniques: reduction pulmonary arterioplasty, plication, or graft replacement.

Surgical repair of Tetralogy of Fallot has excellent outcomes, with over 90% of patients alive at 30 years. The ideal time for surgical repair is between 3 and 11 months of age. However, the symptomatic neonate with Tetralogy of Fallot may require earlier intervention: either a palliative intervention (right ventricular outflow tract stent, ductal stent, balloon pulmonary valvuloplasty, or Blalock-Taussig shunt) followed by a surgical repair later on, or a complete surgical repair in the neonatal period. Indications for palliation include prematurity, complex anatomy, small pulmonary artery size, and comorbidities. Given that outcomes after right ventricular outflow tract stent palliation are particularly promising – there is low mortality and morbidity, and consistently increased oxygen saturations and increased pulmonary artery z-scores – it is now considered the first-line palliative option. Disadvantages of right ventricular outflow tract stenting include increased cardiopulmonary bypass time at later repair and the stent preventing pulmonary valve preservation. However, neonatal surgical repair is associated with increased short-term complications and hospital length of stay compared to staged repair. Both staged repair and primary repair appear to have similar long-term mortality and morbidity, but more evidence is needed assessing long-term outcomes for right ventricular outflow tract stent palliation patients.

Pulmonary pressure is one of the most important parameters in the postoperative follow-up of patients who have undergone the Glenn procedure. Platelet activation markers, which are inexpensive and easily accessible blood count parameters, have been shown to be associated with the aetiology and pathogenesis of primary pulmonary artery hypertension. We examined the relationship between platelet activation markers and pulmonary pressures in the early postoperative period of patients who underwent the Glenn procedure.

Eighty-five patients who underwent the Glenn procedure in our clinic between January 2011 and March 2020 were included in the study retrospectively. Fifty-one patients were male and 34 were female, and age varied from 4 to 416 months, with a mean of 28.64 ± 51 months.

Patients with increased pulmonary blood flow on palliation before Glenn surgery had higher mean platelet volume values. However, no correlation was found between pulmonary pressures and platelet activation markers in the early postoperative period.

There was not similar study evaluating platelet activation markers in the paediatric age group before and after postoperative Glenn surgery in the literature. Therefore, even if platelet activation markers provide information about the pulmonary bed, they may be misleading due to other reasons that trigger bleeding and inflammatory processes in the early postoperative period.

Aortopulmonary window is a rare congenital heart defect. Left main coronary artery extrinsic compression by an enlarged pulmonary artery is a rare complication and a potential cause for chest pain and sudden cardiac death in patients with pulmonary hypertension. Here, we present the case of a 14-year-old boy with a large aortopulmonary window who was planned for a device closure, but during the procedure, he developed ST-T segment changes while the device was being deployed, and hence the procedure was abandoned. The boy subsequently underwent a successful surgical closure thereafter.