Decision-making in congenital cardiac care, although sometimes appearing simple, may prove challenging due to lack of data, uncertainty about outcomes, underlying heuristics, and potential biases in how we reach decisions. We report on the decision-making complexities and uncertainty in management of five commonly encountered congenital cardiac problems: indications for and timing of treatment of subaortic stenosis, closure or observation of small ventricular septal defects, management of new-onset aortic regurgitation in ventricular septal defect, management of anomalous aortic origin of a coronary artery in an asymptomatic patient, and indications for operating on a single anomalously draining pulmonary vein. The strategy underpinning each lesion and the indications for and against intervention are outlined. Areas of uncertainty are clearly delineated. Even in the presence of “simple” congenital cardiac lesions, uncertainty exists in decision-making. Awareness and acceptance of uncertainty is first required to facilitate efforts at mitigation. Strategies to circumvent uncertainty in these scenarios include greater availability of evidence-based medicine, larger datasets, standardised clinical assessment and management protocols, and potentially the incorporation of artificial intelligence into the decision-making process.

The Editorial Board of Cardiology in the Young has recently discussed the need for a Bioethics Forum and has given authorisation to proceed with its creation. Herein, we provide the organisational structure and launch process to introduce properly this interesting and timely endeavour. By this communication, we are establishing this Bioethics Forum of Cardiology in the Young. We hope to attract manuscripts concerning timely bioethical subjects and to offer the readership the opportunity to respond to these topics with supporting or opposing views as appropriate. New articles regarding timely topics will be written by the readership, as well as by invited authors, and these articles will be published. We hope to stimulate interactive discussion concerning the published manuscripts, and these manuscripts and the associated discussions will be open to all interested parties.

The Society of Thoracic Surgeons Congenital Heart Surgery Database and the Vermont Oxford Network Expanded Database are both large, international, well-established quality and outcomes databases with high penetration in their respective fields of congenital heart surgery and neonatology. Previous studies have shown the value of combining large databases for research purposes. Our aim was to examine the feasibility and value of combining these databases on a local level.

We included patients from both databases, cared for at our centre and born from 2015–2020, who had cardiac surgery as neonates or during the birth hospitalisation. We examined the number of patients from each database and overlap between the two. We compared cardiac diagnoses, surgeries performed, pre-operative factors, mortality, and length of stay between databases.

Of the 255 patients meeting criteria, 209 (81.9%) had records in both databases. The most common diagnoses in both were hypoplastic left heart syndrome, coarctation, and transposition of the great arteries. Surgical data were incompletely recorded in Vermont Oxford. Gestational age, birth weight, multiple gestation, mortality, and length of stay did not differ significantly between the databases, while the percentage of patients with an extracardiac malformation or genetic syndrome recorded was higher in the Society for Thoracic Surgeons group.

Larger-scale matching and comparison studies using these databases are feasible and desirable; for some variables, a record with data from both databases may be more complete. Specific attention should be given to inclusion criteria, reconciling different schema of diagnoses, and formulating questions relying on each database’s relative strengths.

We honour a great man and a true giant. Lodewyk H.S. van Mierop (March 31, 1927 – October 17, 2021), known as Bob, was not only a Paediatric Cardiologist but also a dedicated Scientist. He made many significant and ground-breaking contributions to the fields of cardiac anatomy and embryology. He was devoted as a teacher, spending many hours with medical students, Residents, and Fellows, all of whom appreciated his regularly scheduled educational sessions. Those of us who were fortunate to know and spend time with him will always remember his great mind, his willingness to share his knowledge, and his ability to encourage spirited and fruitful discussions. His life was most productive, and he will long be remembered by many through his awesome and exemplary scientific contributions.

His legacy continues to influence the current and future generations of surgeons and all providers of paediatric and congenital cardiac care through the invaluable archive he established at University of Florida in Gainesville: The University of Florida van Mierop Heart Archive. Undoubtedly, with these extraordinary contributions to the fields of cardiac anatomy and embryology, which were way ahead of his time, Professor van Mierop was a true giant in Paediatric Cardiology. The invaluable archive he established at University of Florida in Gainesville, The University of Florida van Mierop Heart Archive, has been instrumental in teaching medical students, Residents, Medical Fellows, and Surgical Fellows. Only a handful of similar archives exist across the globe, and these archives are the true legacy of giants such as Dr. van Mierop. We have an important obligation to leave no stone unturned to continue to preserve these archives for the future generations of surgeons, physicians, all providers of paediatric and congenital cardiac care, and, most importantly, our patients.

In the December 2021 issue of Cardiology in the Young, Hubrechts and colleagues, from Brussels and Leuven in Belgium, describe their experience in which the pulmonary veins were normally connected to the morphologically left atrium. By virtue of the presence of a shelf dividing the morphologically left atrium, however, the venous return was to the morphologically right atrium, with no evidence of formation of the superior interatrial fold, meaning that there was no obstruction of flow into the systemic venous circulation. The question posed by the Belgian authors is whether the shelf dividing the morphologically left atrium is a deviated primary atrial septum, as the arrangement has previously been interpreted. As they discuss, it is currently impossible to arbitrate this conundrum. In our commentary, we discuss the background to the dilemma. We point out that, as yet, it is not possible to code accurately this congenital cardiac malformation within The International Paediatric and Congenital Cardiac Code (IPCCC), nor within the newly produced 11th Revision of the International Classification of Diseases (ICD-11).

Professor Liesl Zühlke is the focus of our fifth in a series of interviews in Cardiology in the Young entitled, “Global Leadership in Paediatric and Congenital Cardiac Care”. Professor Zühlke (nee Hendricks) was born in Cape Town, South Africa. She would attend medical school in her hometown at University of Cape Town, graduating in 1991. Professor Zühlke then went on to complete a Diploma in Child Health at College of Medicine in Cape Town followed by completion of her Paediatric and Paediatric Cardiology training in 1999 and 2007, respectively. She would subsequently complete her Masters of Public Health (Clinical Research Methods) at the University of Cape Town, completing her dissertation in 2011 on computer-assisted auscultation as a screening tool for cardiovascular disease, under the supervision of Professors Landon Myer and Bongani Mayosi.

Professor Zühlke began her clinical position as a paediatric cardiologist in the Department of Paediatrics and Child Health at the Red Cross War Memorial Children’s Hospital in Cape Town, South Africa in 2007. In this role, she has been instrumental in developing a transitional clinic at the paediatric hospital, is a team member of the combined cardio-obstetric and grown-up congenital heart disease clinics, each of which are rare in South Africa, with very few similar clinics in Africa. Professor Zühlke would continue her research training, completing her Doctorate at the University of Cape Town in 2015, with her dissertation on the outcomes of asymptomatic and symptomatic rheumatic heart disease under the supervision of Professor Bongani Mayosi and Associate Professor Mark Engel. In 2015, in affiliation with the University of Cape Town and the Department of Paediatrics and the Institute of Child Health, she established The Children’s Heart Disease Research Unit, with the goals to conduct, promote and support paediatric cardiac research on the African continent, facilitate Implementation Science and provide postgraduate supervision and training in paediatric cardiac research. In 2018, she would subsequently complete her Master of Science at the London School of Economics in Health Economics, Outcomes and Management of cardiovascular sciences. Professor Zühlke currently serves as the acting Deputy-Dean of Research at the Faculty of Health Sciences, University of Cape Town.

Professor Zühlke has achieved the highest leadership positions within cardiology in South Africa, including President of the Paediatric Cardiac Society of South Africa and President of the South African Heart Association. She is internationally regarded as a leader in research related to rheumatic heart disease. Professor Zühlke’s work includes patient, family and health advocacy on a global scale, being involved in the development of policies that have been adopted by major global organisations such as the World Health Organization. In addition to her clinical and research efforts, she is highly regarded by students, colleagues and graduates as an effective teacher, mentor and advisor. This article presents our interview with Professor Zühlke, an interview that covers her experience as a thought leader in the field of Paediatric Cardiology, specifically in her work related to rheumatic heart disease, Global Health and paediatric and congenital cardiac care in resource-limited settings.

We present the case of a 13-year-old male with a complex congenital cardiac history who was supported with extracorporeal membrane oxygenation for 394 days while awaiting cardiac transplantation. The patient underwent successful cardiac transplantation after 394 days of support with veno-arterial extracorporeal membrane oxygenation and is currently alive 2 years after cardiac transplantation. We believe that this case represents the longest period of time that a patient has been supported with extracorporeal membrane oxygenation as a bridge to cardiac transplantation.

We also review the literature associated with prolonged support with extracorporeal membrane oxygenation. This case report documents many of the challenges associated with prolonged support with extracorporeal membrane oxygenation, including polymicrobial bacterial and fungal infections, as well as renal dysfunction. It is possible to successfully bridge a patient to cardiac transplantation with prolonged support with extracorporeal membrane oxygenation of over 1 year; however, multidisciplinary collaboration is critical.

The growing unmet demand for suitable organ donors increases each year. Despite relative contraindications for thoracic organ donation after previous cardiac surgery, experienced programmes and surgeons can successfully utilise the lungs from select donors who have undergone prior cardiac surgery. This is the first reported case of double lung en bloc procurement from a donor who had a previous arterial switch operation as an infant.

Children with cardiac diseases suffer from significant morbidity and mortality secondary to thromboembolic complications. Anticoagulant agents currently used for thromboprophylaxis have many limitations, including subcutaneous administration (low molecular weight heparins) and requirement for frequent monitoring via venipuncture (vitamin K antagonists). Edoxaban is an oral direct factor Xa inhibitor without need of monitoring. In the treatment of venous thromboembolism in adults, edoxaban has shown to be effective and safe.

This manuscript summarises the rationale and design of a phase 3, open-label, randomised controlled trial to evaluate and compare the safety and efficacy of edoxaban against standard of care (namely, vitamin K antagonist and low molecular weight heparin) in children with cardiac diseases.

A goal of 150 children with cardiac diseases at risk of thromboembolic complications who need primary or secondary anticoagulant prophylaxis will be recruited. Eligible children between 6 months and <18 years of age will be randomised in a ratio of 2 to 1 for edoxaban versus standard of care. Randomisation will be stratified based on underlying cardiac disease and concomitant use of aspirin for patients other than Kawasaki disease. The primary outcome will be safety, comprised of major and clinically relevant non-major bleeding in first 3 months of treatment. Bleeding beyond 3 months, symptomatic and asymptomatic thromboembolic events, and pharmacokinetic and pharmacodynamic parameters will be evaluated as secondary outcomes.

Randomised controlled anticoagulation trials are challenging in children. This study will evaluate a potentially valuable alternative of oral anticoagulant prophylactic use in children with cardiac diseases.

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.

The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.

The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.

Neurodevelopmental and psychosocial impairments negatively impact health-related quality of life for survivors with CHD and complicate the transition to independent adulthood. Risk for neurodevelopmental and psychosocial impairments is influenced by a complex interplay among genetic, foetal, surgical, perioperative, family, and social factors, requiring a multi-pronged approach to neuroprotection and intervention. To ensure future research can ultimately reduce the burden of CHD for individuals, families, and society, the most pressing issues in cardiac neurodevelopment requiring scientific investigation must be identified.

Through funding from an R13 Grant from the National Heart, Lung, and Blood Institute of the National Institutes of Health of the United States of America, the Cardiac Neurodevelopmental Outcome Collaborative convened a two-day meeting of international experts in cardiac neurodevelopmental and psychosocial research, clinical care, and health disparities, including patient and family stakeholders, to define the cardiac neurodevelopmental and psychosocial outcomes research agenda for the next decade. Seven multidisciplinary working groups were formed to address key domains crucial to the advancement of cardiac neurodevelopmental and psychosocial outcomes research: 1) Foetal Brain Development and Neuroprotection, 2) Surgical/Perioperative Neuroprotection and Neurodevelopment, 3) Characterization of Neurodevelopmental and Psychological Outcomes, 4) Neurodevelopmental and Psychosocial Intervention, 5) Parent Mental Health and Family Functioning, 6) Neurodevelopmental Education, Outreach and Advocacy, and 7) Health Disparities and Neurodevelopmental Outcomes. Working groups identified significant gaps in knowledge and critical questions that must be answered to further knowledge, policy, care, and outcomes. The development of a research agenda in cardiac neurodevelopmental and psychosocial outcomes is critical for informing collaborative initiatives and allocation of funding for research to scientific inquiries of highest value to key stakeholders.

This series describes three adolescent females who presented with chest pain and ventricular dysfunction related to acute coronary ischemia secondary to Takayasu’s arteritis with varied courses of disease progression leading to a diverse range of therapies including cardiac transplantation. While Takayasu’s arteritis is rare in childhood, it should be strongly considered in any adolescent female presenting with systemic inflammation and chest pain consistent with myocardial infarction. A high index of suspicion can lead to early detection and aggressive management of the underlying vasculitis reducing associated morbidity and mortality. The purpose of this report is to describe the challenges in the clinical diagnosis and management of Takayasu’s arteritis with myocardial infarction. We also seek to enhance awareness about unique presentations of Takayasu’s arteritis within the paediatric community.

Chest radiography compares left ventricular decompression in the same patient supported with extracorporeal membrane oxygenation with atrial septal fenestration and subsequently supported with left ventricular assist device with apical cannulation.

Dr. Katarina Hanséus is the focus of our fourth in a series of interviews in Cardiology in the Young entitled, “Global Leadership in Paediatric and Congenital Cardiac Care”. Dr. Hanséus was born in Malmö, Sweden. She attended undergraduate school in her home town in Malmö, Sweden, graduating in 1974. Dr. Hanséus then went on to complete medical school at University of Lund in Lund, Sweden, graduating in 1980, where additionally she completed a Doctoral Dissertation in the evaluation of cardiac function and chamber size in children using Doppler and cross-sectional echocardiography. Under the Swedish Board of National Welfare, Dr. Hanséus completed her authorisation as a paediatrician in 1986, followed by her authorisation as a paediatric cardiologist in 1988, at University of Lund. She was appointed head of Paediatric Cardiology in 2000 at the Children’s Heart Center, Skane University Hospital, Lund, Sweden. The programme at Lund serves as one of the two national referral centres for comprehensive paediatric and congenital cardiac care, including paediatric cardiac surgery, in Sweden. From 2006 to 2013, she served as the clinical and administrative head of the Department of Neonatology, Paediatric Surgery, Paediatric Intensive Care, Paediatric Cardiology, and Paediatric Cardiac Surgery, returning as the head of Paediatric Cardiology in 2013, for which she currently holds the position.

Dr. Hanséus is a recognised leader in the field of Paediatric Cardiology and has been involved in leadership within the Swedish Pediatric Society, the Swedish Association for Pediatric Cardiology, and the Association for European Paediatric and Congenital Cardiology throughout her career. Within the Association for European Paediatric and Congenital Cardiology, she served as the Secretary General from 2011 to 2016, the President Elect in 2018, and is the current President serving from 2019 until 2022. This article presents our interview with Dr. Hanséus, an interview that covers her experience as a leader in the field of Paediatric Cardiology, including the history and goals of the Association for European Paediatric and Congenital Cardiology, and her role and vision as their current President.