Mitochondrial trifunctional protein deficiency is a long-chain fatty acid disorder that may include manifestations of severe cardiomyopathy and arrhythmias. The pathophysiology for the severe presentation is unclear but is an indicator for worse outcomes. Triheptanoin, a synthetic medium chain triglyceride, has been reported to reverse cardiomyopathy in some individuals, but there is limited literature in severe cases. We describe a neonatal onset of severe disease whose clinical course was not improved despite mechanical support and triheptanoin.

Several metrics exist for matching the size of donor to recipient in paediatric cardiac transplantation. Different centres employ different metrics for size-matching to determine the viability of donor hearts. Inconsistent evidence exists, with limited consensus as to the metric for size-matching that is most predictive of outcomes after cardiac transplantation. Furthermore, any metric must function within the tight timeline available for the assessment of the suitability of the donor. At the time of the writing of this paper, the most commonly used metric for size-matching in paediatric cardiac transplantation is the donor-to-recipient body weight ratio. In this article, we review published literature evaluating commonly used metrics for size-matching in paediatric cardiac transplantation, including weight, height, body surface area, and imaging parameters.

Heart University [https://www.heartuniversity.org/] is a free educational website providing structured training curricula with knowledge-based testing and access to webinars and conference recordings for practicing and in-training providers of paediatric and congenital cardiac care. To date, there are over 15,000 registered website users from over 140 countries on Heart University, with over 2,000 training modules and/or recorded educational videos. Heart University has developed an “asynchronous” educational lecture series entitled “Pediatric and Congenital Cardiac Care in Resource-Limited Settings.” This recorded lecture series is specifically focused on topics relevant to practicing paediatric and/or congenital cardiac care in low-resource settings.

A relatively new initiative, “Cardiology Across Continents,” supplements the existing educational resources for providers of paediatric and/or congenital cardiac care in low-income countries and lower-middle-income countries by providing an additional live, interactive, case-based forum. Sessions occur every 1–2 months and focus on challenging cases from diagnostic or management perspective with a view to promote collaboration between partnered institutions. “Cardiology Across Continents” is an expanding initiative that facilitates learning and collaboration between clinicians across varied practice settings via interactive case discussions. We welcome trainees and providers of paediatric and congenital cardiac care to join the sessions and invite any insight that can enhance learning for clinicians around the world. This manuscript describes “Cardiology Across Continents” and discusses the development, history, current status, and future plans of Heart University.

The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries. For information about the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery, please visit the following website: [www.WCPCCS2023.org]. The purpose of this manuscript is to review the activities related to global health and advocacy that will occur at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.

Acknowledging the need for urgent change, we wanted to take the opportunity to bring a common voice to the global community and issue the Washington DC WCPCCS Call to Action on Addressing the Global Burden of Pediatric and Congenital Heart Diseases. A copy of this Washington DC WCPCCS Call to Action is provided in the Appendix of this manuscript. This Washington DC WCPCCS Call to Action is an initiative aimed at increasing awareness of the global burden, promoting the development of sustainable care systems, and improving access to high quality and equitable healthcare for children with heart disease as well as adults with congenital heart disease worldwide.

The European Congenital Heart Surgeons Association (ECHSA) Congenital Database (CD) is the second largest clinical pediatric and congenital cardiac surgical database in the world and the largest in Europe, where various smaller national or regional databases exist. Despite the dramatic increase in interventional cardiology procedures over recent years, only scattered national or regional databases of such procedures exist in Europe. Most importantly, no congenital cardiac database exists in the world that seamlessly combines both surgical and interventional cardiology data on an international level; therefore, the outcomes of surgical and interventional procedures performed on the same or similar patients cannot easily be tracked, assessed, and analyzed. In order to fill this important gap in our capability to gather and analyze information on our common patients, ECHSA and The Association for European Paediatric and Congenital Cardiology (AEPC) have embarked on a collaborative effort to expand the ECHSA-CD with a new module designed to capture data about interventional cardiology procedures. The purpose of this manuscript is to describe the concept, the structure, and the function of the new AEPC Interventional Cardiology Part of the ECHSA-CD, as well as the potentially valuable synergies provided by the shared interventional and surgical analyses of outcomes of patients. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow centers to have access to robust surgical and transcatheter outcome data from their own center, as well as robust national and international aggregate outcome data for benchmarking. Each contributing center or department will have access to their own data, as well as aggregate data from the AEPC Interventional Cardiology Part of the ECHSA-CD. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow cardiology centers to have access to aggregate cardiology data, just as surgical centers already have access to aggregate surgical data. Comparison of surgical and catheter interventional outcomes could potentially strengthen decision processes. A study of the wealth of information collected in the database could potentially also contribute toward improved early and late survival, as well as enhanced quality of life of patients with pediatric and/or congenital heart disease treated with surgery and interventional cardiac catheterization across Europe and the world.

Dr Krishna Kumar is the focus of our sixth in a series of interviews in Cardiology in the Young entitled, “Global Leadership in Paediatric and Congenital Cardiac Care.” Dr Kumar was born in Raurkela, India. He attended medical school at Maulana Azad Medical College in New Delhi, graduating in 1984. Dr Kumar then went on to complete internal medicine, emergency medicine, and adult cardiology training at All India Institute of Medical Sciences in 1988, 1989, and 1990, respectively. He then pursued paediatric cardiology training at Harvard Medical School in Boston, MA, USA. Dr Kumar began his clinical position as a paediatric cardiologist at Amrita Institute of Medical Sciences in Kochi, Kerala, India.

During his impressive career, Dr Kumar has made significant contributions to educational advancement, research and innovation, public health advocacy, and clinical care. Dr Kumar is credited for distinguishing paediatric cardiology as a distinct subspecialty in India. He was a founding member of the Pediatric Cardiology Society of India and the original editor of the society’s academic journal. Recognising the deficit of paediatric cardiology-trained physicians in low- and middle-income countries, Dr Kumar helped establish formal structured training programmes for paediatric cardiology in India. More recently, he established the Children’s HeartLink Fellowships in paediatric cardiac sciences at Amrita Institute of Medical Sciences in Kochi and Institut Jantung Negara in Malaysia. Through educational programmes, Dr Kumar has taught countless caregivers and paediatricians, in India and neighbouring countries, the early identification and management of children with CHD. Dr Kumar has established a premier paediatric heart programme at Amrita Institute of Medical Sciences. As department Chief, he emphasises the importance of teamwork, advocacy, and continuous quality improvement. He has developed numerous low-cost strategies for the management of CHD. He has established large community-based studies on rheumatic heart disease and CHD in South India. Dr Kumar’s focus on advocacy and policy change in India has made a substantial impact on early identification and treatment of CHD in the subcontinent. He has made a global impact on the care of paediatric cardiology patients through his educational programmes, research and innovation, large-scale research registries, and advocacy for public health policy changes. He is an incredibly humble and generous leader, and his patients and community are the source of his unending motivation.

Over the past 2 decades, several categorizations have been proposed for the abnormalities of the aortic root. These schemes have mostly been devoid of input from specialists of congenital cardiac disease. The aim of this review is to provide a classification, from the perspective of these specialists, based on an understanding of normal and abnormal morphogenesis and anatomy, with emphasis placed on the features of clinical and surgical relevance. We contend that the description of the congenitally malformed aortic root is simplified when approached in a fashion that recognizes the normal root to be made up of 3 leaflets, supported by their own sinuses, with the sinuses themselves separated by the interleaflet triangles. The malformed root, usually found in the setting of 3 sinuses, can also be found with 2 sinuses, and very rarely with 4 sinuses. This permits description of trisinuate, bisinuate, and quadrisinuate variants, respectively. This feature then provides the basis for classification of the anatomical and functional number of leaflets present. By offering standardized terms and definitions, we submit that our classification will be suitable for those working in all cardiac specialties, whether pediatric or adult. It is of equal value in the settings of acquired or congenital cardiac disease. Our recommendations will serve to amend and/or add to the existing International Paediatric and Congenital Cardiac Code, along with the Eleventh iteration of the International Classification of Diseases provided by the World Health Organization.

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.