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.

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.

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.

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.

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.

Ostium secundum atrial septal defects are mostly closed in the cardiac catheterisation laboratories using either Amplatzer® (Abbott Laboratories, IL) atrial septal occluder, Gore® Cardioform septal occluder and more recently using the recently approved (US FDA approval June 2019) Gore® Cardioform atrial septal defect occluder (W. L. Gore & Associates, AZ). Similar to any new device in the market, there is a learning curve to the deployment of this device. We therefore aim to report the key features about this new Gore Cardioform atrial septal defect occluder device with special emphasis on technical aspects that can be employed during transcatheter closure of challenging ostium secundum atrial septal defects using this device.

On 15 October, 2020, Dr. Paul M. Weinberg, a true giant in the field of paediatric cardiology, succumbed to a prolonged illness. Dr. Weinberg had a 43-year career and was described as a pillar of The Children’s Hospital of Philadelphia and the spirit of the Division of Cardiology, a cherished and beloved teacher, and an outstanding clinician. His impact on the field and on the careers of his students will be remembered for generations to come.

In 2019, Dr. Weinberg wrote for Jefferson Medical School’s 50th year reunion memory book: “In the true spirit of Hippocrates, I seek to mentor the next generation as I was mentored by the last, without expectation of reward. I am forever indebted to these educators for all the knowledge they imparted to me and for the wisdom that I acquired under their tutelage.” These words are a true reflection of his unassuming dedication to teaching the next generation of paediatric cardiologists. His legacy will continue to live on through these trainees and impact the field for generations to come.

Cardiac surgery for CHD was pioneered in Washington, DC by Charles Hufnagel and Edgar Davis working at Georgetown University and Children’s Hospital of the District of Columbia. Children’s Hospital, now Children’s National Hospital, had been established just 5 years after the end of the Civil War. In the 1950s, Davis and Hufnagel undertook many open-heart operations using the technique of surface cooling, hypothermia, and circulatory arrest. Hufnagel and Lewis Scott, who founded the cardiology department at Children’s, were trained in Boston by Gross and Nadas. Judson Randolph, also a trainee of Gross, introduced cardiac surgery using cardiopulmonary bypass and established the General Pediatric Surgery department at Children’s in the 1960s. The transition of hospital staffing from community-based private physicians to full-time hospital employees was often controversial but was complete by the turn of the millennium. The 21st century has seen continuing growth of the new Children’s National Heart Institute and consolidation of several congenital cardiac programmes in Washington, DC.

The Neurodevelopmental and Psychological Outcomes Working Group of the Cardiac Neurodevelopmental Outcome Collaborative was formed in 2018 through support from an R13 grant from the National Heart, Lung, and Blood Institute with the goals of identifying knowledge gaps regarding the neurodevelopmental and psychological outcomes of individuals with CHD and investigations needed to advance science, policy, clinical care, and patient/family outcomes. Accurate characterisation of neurodevelopmental and psychological outcomes in children with CHD will drive improvements in patient and family outcomes through targeted intervention. Decades of research have produced a generalised perspective about neurodevelopmental and psychological outcomes in this heterogeneous population. Future investigations need to shift towards improving methods, measurement, and analyses of outcomes to better inform early identification, prevention, and intervention. Improved definition of underlying developmental, neuropsychological, and social-emotional constructs is needed, with an emphasis on symptom networks and dimensions. Identification of clinically meaningful outcomes that are most important to key stakeholders, including patients, families, schools and providers, is essential, specifically how and which neurodevelopmental differences across the developmental trajectory impact stakeholders. A better understanding of the discontinuity and patterns of neurodevelopment across the lifespan is critical as well, with some areas being more impactful at some ages than others. Finally, the field needs to account for the impact of race/ethnicity, socio-economic status, cultural and linguistic diversity on our measurement, interpretation of data, and approach to intervention and how to improve generalisability to the larger worldwide population of patients and families living with CHD.

Isolated leftward prolapse or deviation of the primary atrial septum is a rare CHD that can mimic abnormal pulmonary venous return at first sight. We present a case of a newborn infant, referred for surgical correction of totally anomalous pulmonary venous return into the right atrium, with the peri-operative finding of a leftward deviation of the superior margin of the primary atrial septum. The distinction with a dividing atrial shelf could not be confirmed with certainty. Fifty-three similar cases from the literature are incorporated. A detailed review of the current account on atrial septation is studied. The embryological and clinical features of a dividing partition of the left atrium are discussed.

The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present.