Bleeding in the perioperative period of congenital heart surgery with cardiopulmonary bypass is associated with increased morbidity and mortality both from the direct effects of haemorrhage as well as the therapies deployed to restore haemostasis. Perioperative bleeding is complex and multifactorial with both patient and procedural contributions. Moreover, neonates and infants are especially at risk. The objective of this review is to summarise the evidence regarding bleeding management in paediatric surgical patients and identify strategies that might facilitate appropriate bleeding management while minimising the risk of thrombosis. We will address the use of standard and point-of-care tests, and the role of contemporary coagulation factors and other novel drugs.

A newborn with supracardiac total anomalous pulmonary venous connection vein presented the small left ventricle with z score of −7.5, retrograde blood supply in the transverse arch, and the dutcus-dependent systemic circulation. The patient underwent the repair of the anomalous pulmonary vein and bilateral pulmonary arterial banding soon after the birth and then transcatheter pulmonary arterial debanding at the age of 10 months because of an appropriate growth of the left ventricle.

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.

Fontan fenestration allows right-to-left shunting increasing cardiac output and oxygen delivery. Increased shunting occurs as cardiac function and ventricular end-diastolic pressures improve, potentially decreasing oxygen saturation. Complete closure may result in impaired Fontan haemodynamics and low cardiac output; however, there are no dedicated devices to reduce fenestration size. We describe Fontan fenestration size reduction using the Atrial Flow Regulator.

A newborn with hypoplastic left heart underwent a Norwood operation. Obstruction of the Blalock–Thomas–Taussig shunt was treated with a stent. During resuscitation, this was compressed, which contributed to a fatal outcome.

Improved survival of patients with paediatric and congenital cardiac disease has led to a heightened awareness of the cognitive, neurodevelopmental, psychosocial, and physical functioning 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.

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.

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.

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.

Premature ductal constriction was diagnosed in a 30-week gestation fetus with hypoplastic left heart syndrome. The fetus developed right ventricular hypertrophy and mild tricuspid regurgitation. Foetal neuroimaging showed slowed growth of the brain and increased lactate. We describe the imaging and clinical findings. Fetuses with critical CHD and in utero ductal constriction are at increased risk of morbidity and mortality, and require appropriate counselling.

We report three-dimensional imaging of a rare finding of Ebstein’s anomaly of the tricuspid valve in a patient with hypoplastic left heart syndrome, which has been previously reported only by two-dimensional echocardiography. A fetal echocardiogram was performed at 19 weeks that showed a moderately hypoplastic left ventricle, severely hypoplastic mitral valve, a severely hypoplastic aortic valve, and a dysplastic tricuspid valve. Post Caesarean delivery at 40 weeks of gestation, a transthoracic echocardiogram confirmed the findings seen on the fetal echocardiogram. A three-dimensional echocardiogram was then performed, which demonstrated an Ebstein tricuspid valve with apical displacement of the septal leaflet, chordal attachments of the valve to the right ventricular outflow tract, and moderate tricuspid regurgitation. The patient underwent a successful heart transplantation.

Survival for hypoplastic left heart syndrome patients following the Norwood procedure is 71–90%. Mortality in patients with Turner’s syndrome and hypoplastic left heart syndrome after conventional palliation (Norwood operation) has been reported as high as 80%. This questions the approach of traditional staged palliation. Here, we report a patient with hypoplastic left heart syndrome and Turner’s syndrome bridged to orthotopic heart transplantation following a hybrid procedure.