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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.
Publicly available health information is increasingly important for patients and their families. While the average US citizen reads at an 8th-grade level, electronic educational materials for patients and families are often advanced. We assessed the quality and readability of publicly available resources regarding hypoplastic left heart syndrome (HLHS).
We queried four search engines for “hypoplastic left heart syndrome”, “HLHS”, and “hypoplastic left ventricle”. The top 30 websites from searches on Google, Yahoo!, Bing, and Dogpile were combined into a single list. Duplicates, commercial websites, physician-oriented resources, disability websites, and broken links were removed. Websites were graded for accountability, content, interactivity, and structure using a two-reviewer system. Nonparametric analysis of variance was performed.
Fifty-two websites were analysed. Inter-rater agreement was high (Kappa = 0.874). Website types included 35 hospital/healthcare organisation (67.3%), 12 open access (23.1%), 4 governmental agency (7.7%), and 1 professional medical society (1.9%). Median total score was 19 of 39 (interquartile range = 15.8–25.3): accountability 5.5 of 17 (interquartile range = 2.0–9.3), content 8 of 12 (interquartile range = 6.4–10.0), interactivity 2 of 6 (interquartile range = 2.0–3.0), and structure 3 of 4 (interquartile range = 2.8–4.0). Accountability was low with 32.7% (n = 17) of sites disclosing authorship and 26.9% (n = 14) citing sources. Forty-two percent (n = 22) of websites were available in Spanish. Total score varied by website type (p = 0.03), with open access sites scoring highest (median = 26.5; interquartile range = 20.5–28.6) and hospital/healthcare organisation websites scoring lowest (median = 17.5; interquartile range = 13.5–21.5). Score differences were driven by differences in accountability (p = 0.001) – content scores were similar between groups (p = 0.25). Overall readability was low, with median Flesch–Kincaid Grade Level of 11th grade (interquartile range = 10th–12th grade).
Our evaluation of popular websites about HLHS identifies multiple opportunities for improvement, including increasing accountability by disclosing authorship and citing sources, enhancing readability by providing material that is understandable to readers with the full spectrum of educational background, and providing information in languages besides English, all of which would enhance health equity.
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.
Diversification of the medical and cardiothoracic surgical workforce represents an ongoing need. A congenital cardiac surgery shadowing programme for undergraduate students was implemented at the University of Florida Congenital Heart Center.
Students shadowing in the Congenital Heart Center from 17 December 2020 through 20 July 2021 were sent a survey through Qualtrics to evaluate the impact of their shadowing experience. The main objectives of the survey were to determine the personal relationship(s) of the students to physicians prior to shadowing, how the presence or absence of physicians in the family of a given student related to the exposure of the student to a medical setting prior to shadowing, and the interest of the students in medicine and cardiothoracic surgery prior to and after the shadowing experience. Survey responses included “Yes/No” questions, scaled responses using a Likert scale, selection lists, and free text responses. When applicable, t-tests were utilised to assess differences between student groups.
Of the 37 students who shadowed during the study period, 26 (70%) responded. Most students were female (58%, n = 15), and the mean age was 20.9 ± 2.4 years. Students spent a mean duration of 95 ± 138 hours shadowing providers as part of the shadowing programme. Likert scale ratings of interest in the professions of medicine, surgery, and cardiothoracic surgery all increased after the shadowing experience (p < 0.01). Students with a family member in medicine had more clinical exposure prior to the shadowing programme (p < 0.01).
A surgical shadowing programme at a Congenital Heart Center may have an important formative impact on the views of undergraduate students regarding potential careers in surgery and medicine. Additionally, students without family members in medicine tend to have less prior exposure to medicine and could likely benefit more from this type of shadowing programme.
Transposition of great arteries with intact ventricular septum and left ventricular outflow tract obstruction (TGA + IVS + LVOTO) is uncommon. We reviewed operations performed in patients with TGA + IVS + LVOTO in the European Congenital Heart Surgeons Association Congenital Database (ECHSA-CD).
All 109 patients with a diagnosis of TGA + IVS + LVOTO in ECHSA-CD who underwent cardiac surgery during a 21-year period (01/2000-02/2021, inclusive) were included. Preoperative variables, operative data, and postoperative outcomes were collected.
These 109 patients underwent 176 operations, including 37 (21.0%) arterial switch operations (ASO), 26 (14.2%) modified Blalock-Taussig-Thomas shunts (MBTTS), 11 (6.2%) Rastelli operations, and 13 (7.3%) other palliative operations (8 superior cavopulmonary anastomosis[es], 4 Fontan, and 1 other palliative procedure). Of 37 patients undergoing ASO, 22 had a concomitant procedure.
There were 68 (38.6%) reoperations, including 11 pacemaker procedures and 8 conduit operations. After a systemic-to-pulmonary artery shunt, reoperations included shunt reoperation (n = 4), Rastelli (n = 4), and superior cavopulmonary anastomosis (n = 3).
Overall Operative Mortality was 8.2% (9 deaths), including three following ASO, two following “Nikaidoh, Kawashima, or LV-PA conduit” procedures, and two following Rastelli. Postoperative complications occurred after 36 operations (20.4%). The most common complications were delayed sternal closure (n = 11), postoperative respiratory insufficiency requiring mechanical ventilation >7 days (n = 9), and renal failure requiring temporary dialysis (n = 8).
TGA + IVS + LVOTO is rare (109 patients in ECHSA-CD over 21 years). ASO, MBTTS, and Rastelli are the most common operations performed for TGA + IVS + LVOTO. Larger international studies with long-term follow-up are needed to better define the anatomy of the LVOTO and to determine the optimal surgical strategy.
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.
The primary objective of this study was to determine whether Altmetric score, number of reads, and citations for paediatric cardiology manuscripts correlate with one another. A secondary objective was to determine the extent to which factors mediated citation number for paediatric cardiology manuscripts.
Data for this study came from manuscripts published in Cardiology in the Young (2010–2021). Data were extracted by using data shared on the journal website. Spearman’s correlation analyses were conducted between manuscript reads, citations, and Altmetric score. Regression analyses were conducted with number of citations as the dependent variable and year of publication, publication type, number of reads, and Altmetric score as independent variables.
A total of 2642 manuscripts were included in the final analyses. Reads and citations had poor correlation (r-value 0.32); reads and Altmetric score had negligible correlation (r-value 0.26); and Altmetric score and citations had negligible correlation (r-value 0.07). Year of publication was independently associated with number of citations (β –0.95, p-value <0.01). Manuscript type was independently associated with number of citations (β 1.04, p-value <0.01). Number of reads was independently associated with citations (β 0.01, p-value <0.01). Altmetric score was independently associated with number of citations (β 0.05, p-value <0.01).
This study describes the correlation of reads, citations, and Altmetric score in manuscripts published in Cardiology in the Young, demonstrating poor correlation, at best, between these metrics. Each bibliometric index seems to represent a different phenomenon of manuscript consumption. No single bibliometric index in isolation offers ample representation of manuscript consumption.
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.