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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.
Complex patients requiring operations on the left ventricular outflow tract, aortic valve, or thoracic aorta after previous repair of aortopathy constitute a challenging group, with limited information guiding decision-making. We aimed to use our institutional experience to highlight management challenges and describe surgical pearls to address them.
Forty-one complex patients with surgery on the left ventricular outflow tract, aortic valve, or aorta at Cleveland Clinic Children’s between 2016 and 2021 following previous repair of aortic pathology were retrospectively reviewed. Patients with known connective tissue disease or single ventricle circulation were excluded.
Median age at index procedure was 23 years (range 0.25–48) with median of 2 prior sternotomies. Previous aortic operations included subvalvular (n = 9), valvular (n = 6), supravalvular (n = 13), and multi-level surgeries (n = 13). Four deaths occurred in median follow-up of 2.5 years. Mean left ventricular outflow tract gradients improved significantly for patients with obstruction (34.9 ± 17.5 mmHg versus 12.6 ± 6.0 mmHg; p < 0.001). Technical pearls include the following: 1) liberal use of anterior aortoventriculoplasty with valve replacement; 2) primarily anterior aortoventriculoplasty following the subpulmonary conus in contrast to more vertical incision for post-arterial switch operation patients; 3) pre-operative imaging of mediastinum and peripheral vasculature for cannulation and sternal re-entry; and 4) proactive use of multi-site peripheral cannulation.
Operation to address the left ventricular outflow tract, aortic valve, or aorta following prior congenital aortic repair can be accomplished with excellent outcomes despite high complexity. These procedures commonly include multiple components, including concomitant valve interventions. Cannulation strategies and anterior aortoventriculoplasty in specific patients require modifications.
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.
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.
The essence of so-called heterotaxy is the potential disharmony between the arrangement of the bronchuses, abdominal organs, and the atrial appendages. Accurate description of the heart, however, can only be provided by specific description of these features, all of which are readily discernible in the clinical setting. We argue that, when accurate description of the atrial and visceral arrangement is provided, along with appropriate description of the intracardiac findings, no further accuracy is gained by suggesting that an individual heart is “heterotaxic”.
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.
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.
Despite enormous strides in our field with respect to patient care, there has been surprisingly limited dialogue on how to train and educate the next generation of congenital cardiologists. This paper reviews the current status of training and evolving developments in medical education pertinent to congenital cardiology. The adoption of competency-based medical education has been lauded as a robust framework for contemporary medical education over the last two decades. However, inconsistencies in frameworks across different jurisdictions remain, and bridging gaps between competency frameworks and clinical practice has proved challenging. Entrustable professional activities have been proposed as a solution, but integration of such activities into busy clinical cardiology practices will present its own challenges. Consequently, this pivot towards a more structured approach to medical education necessitates the widespread availability of appropriately trained medical educationalists, a development that will better inform curriculum development, instructional design, and assessment. Differentiation between superficial and deep learning, the vital role of rich formative feedback and coaching, should guide our trainees to become self-regulated learners, capable of critical reasoning yet retaining an awareness of uncertainty and ambiguity. Furthermore, disruptive innovations such as “technology enhanced learning” may be leveraged to improve education, especially for trainees from low- and middle-income countries. Each of these initiatives will require resources, widespread advocacy and raised awareness, and publication of supporting data, and so it is especially gratifying that Cardiology in the Young has fostered a progressive approach, agreeing to publish one or two articles in each journal issue in this domain.
Webinars have recently replaced in-person medical conferences, including paediatric cardiology conferences, given the COVID-19 pandemic.
With increasing environmental concerns, we analysed the differences between the environmental footprint of a paediatric cardiology webinar with a hypothetical conference. Travel data was collected, with assumptions made on the amount of computer use, internet use and accordingly the overall use of electricity for both forms of conference. Life Cycle Assessment methodology was used (OpenLCA and Ecovinvent v 3.7).
We showed that the theoretical environmental impact of a virtual conference is significantly less (4 tons CO2 equivalent) than the traditional international face-to-face conference (192 tons CO2 equivalent). The life cycle assessment methodology showed that resource use for a face-to-face conference lasting 2.5 days for 1374 attendees is equivalent to 400 times what an average person would use in one year, the climate change and photochemical ozone formation approximately 250 times and the eutrophication terrestrial equivalent to 225 times. However, using carbon equivalent emissions to measure environmental harm from flying is an under estimate of the potential damage, when one considers the additional production of airplane contrails. Notwithstanding this, there is a 98% reduction in climate change impact when meetings are held virtually.
While the virtual conference may never completely replace the traditional in-person paediatric cardiology conference, due to networking benefits, the significant theoretical benefits to the environment highlighted in this study, warrants consideration for the virtual conference taking a more common place in sustainable academia.
Dr Rodney Franklin is the focus of our third in a planned series of interviews in Cardiology in the Young entitled, “Global Leadership in Paediatric and Congenital Cardiac Care.” Dr Franklin was born in London, England, spending the early part of his childhood in the United States of America before coming back to England. He then attended University College London Medical School and University College Hospital in London, England, graduating in 1979. Dr Franklin would then go on to complete his general and neonatal paediatrics training in 1983 at Northwick Park Hospital and University College Hospital in London, England, followed by completing his paediatric cardiology training in 1989 at Great Ormond Street Hospital for Children in London, England. During this training, he additionally would hold the position of British Heart Foundation Junior Research Fellow from 1987 to 1989. Dr Franklin would then complete his training in 1990 as a Senior Registrar and subsequent Consultant in Paediatric and Fetal Cardiology at Wilhelmina Sick Children’s Hospital in Utrecht, the Netherlands. He subsequently obtained his research doctorate at University of London in 1997, consisting of a retrospective audit of 428 infants with functionally univentricular hearts.
Dr Franklin has spent his entire career as a Consultant Paediatric Cardiologist at the Royal Brompton & Harefield Hospital NHS Foundation Trust, being appointed in 1991. He additionally holds honorary Consultant Paediatric Cardiology positions at Hillingdon Hospital, Northwick Park Hospital, and Lister Hospital in the United Kingdom, and Honorary Senior Lecturer at Imperial College, London. He has been the Clinical Lead of the National Congenital Heart Disease Audit (2013–2020), which promotes data collection within specialist paediatric centres. Dr Franklin has been a leading figure in the efforts towards creating international, pan European, and national coding systems within the multidisciplinary field of congenital cardiac care. These initiatives include but are not limited to the development and maintenance of The International Paediatric & Congenital Cardiac Code and the related International Classification of Diseases 11th Revision for CHD and related acquired terms and definitions. This article presents our interview with Dr Franklin, an interview that covers his experience in developing these important coding systems and consensus nomenclature to both improve communication and the outcomes of patients. We additionally discuss his experience in the development and implementation of strategies to assess the quality of paediatric and congenital cardiac care and publicly report outcomes.
This study investigated the impact of the Webinar on deep human learning of CHD.
Materials and methods:
This cross-sectional survey design study used an open and closed-ended questionnaire to assess the impact of the Webinar on deep learning of topical areas within the management of the post-operative tetralogy of Fallot patients. This was a quantitative research methodology using descriptive statistical analyses with a sequential explanatory design.
One thousand-three-hundred and seventy-four participants from 100 countries on 6 continents joined the Webinar, 557 (40%) of whom completed the questionnaire. Over 70% of participants reported that they “agreed” or “strongly agreed” that the Webinar format promoted deep learning for each of the topics compared to other standard learning methods (textbook and journal learning). Two-thirds expressed a preference for attending a Webinar rather than an international conference. Over 80% of participants highlighted significant barriers to attending conferences including cost (79%), distance to travel (49%), time commitment (51%), and family commitments (35%). Strengths of the Webinar included expertise, concise high-quality presentations often discussing contentious issues, and the platform quality. The main weakness was a limited time for questions. Just over 53% expressed a concern for the carbon footprint involved in attending conferences and preferred to attend a Webinar.
E-learning Webinars represent a disruptive innovation, which promotes deep learning, greater multidisciplinary participation, and greater attendee satisfaction with fewer barriers to participation. Although Webinars will never fully replace conferences, a hybrid approach may reduce the need for conferencing, reduce carbon footprint. and promote a “sustainable academia”.
Dr. Jennifer Li is the focus of our second in a planned series of interviews in Cardiology in the Young entitled, “Global Leadership in Paediatric and Congenital Cardiac Care”. Dr. Li was born in Boston, Massachusetts, United States of America, and moved to Indianapolis, Indiana where she completed her secondary education. She then attended Stanford University, majoring in Chemistry and English and graduating with distinction in 1983. Dr. Li then attended Duke University School of Medicine, graduating in 1987. She then completed her internship at Children’s Hospital of Philadelphia in 1987–1989, returning to Duke University Medical Center to complete both her residency in general paediatrics in 1989–1990 followed by her fellowship in paediatric cardiology in 1990–1993. She would later complete her Master’s Degree in Health Sciences at Duke University in 2005.
Dr. Li has spent her entire career as a paediatric cardiologist at Duke University Medical Center, where she was appointed a Professor of Pediatrics and Professor of Medicine in 2008 and has held the position as Beverly C. Morgan Endowed Professor of Pediatrics since 2012. She has served as the Chief of Paediatric Cardiology at Duke University Medical Center since 2006. She also was the Director of Paediatric Research at Duke Clinical Research Institute from 2001-2015. Dr. Li has played an instrumental role in evaluating the safety and efficacy of drugs in children, as well as in analysing and linking large multicentric databases to evaluate the outcomes, quality, and cost of paediatric and congenital cardiac care. Dr. Li has received funding from the National Institute of Health of the United States of America, as well as from industry and foundation grants. This article presents our interview with Dr. Li, an interview that covers her experience collaborating with governmental organizations and industry in the pursuit of common interests to design clinical drug trials, link and analyse large, multicentric databases, and improve paediatric health care.
Dr. Jane Newburger is the focus of our first in a planned series of interviews in Cardiology in the Young entitled, “Global Leadership in Paediatric and Congenital Cardiac Care”. Dr. Newburger was born in Manhattan, New York, United States of America. She was raised in the Bronx for her first six years of life, at which point her family moved to Yonkers, New York, where she spent the rest of her childhood. She then attended Bryn Mawr College where she majored in psychology. Dr. Newburger subsequently attended Harvard Medical School, graduating in 1974. She did her internship and residency in paediatrics at Boston Children’s Hospital in 1974–1976, followed by her fellowship at Boston Children’s Hospital in 1976–1979. She received her Masters in Public Health at the Harvard School of Public Health in 1980.
Dr. Newburger has spent her entire career as a paediatric cardiologist at Boston Children’s Hospital and Harvard Medical School, where she was appointed a Professor of Pediatrics in 1999 and has held the position as Commonwealth Professor of Pediatrics since 2008. She has established herself as a leading clinical scientist within the field of paediatric cardiology, with expertise in leadership of multicentre and multidisciplinary research, including the building of collaborative groups. She has been continuously funded by the National Institute of Health since 1982, and amongst other areas has led the field in the areas of neurodevelopmental outcomes in congenital heart disease, improved methods of vital organ support, and management of Kawasaki disease. This article presents our interview with Dr. Newburger, an interview that covers her path towards becoming a clinical scientist, her interests spanning four decades of hard work, and her strategies to design and lead successful multicentre studies.
Online learning has become an increasingly expected and popular component for education of the modern-day adult learner, including the medical provider. In light of the recent coronavirus pandemic, there has never been more urgency to establish opportunities for supplemental online learning. Heart University aims to be “the go-to online resource” for e-learning in CHD and paediatric-acquired heart disease. It is a carefully curated open access library of paedagogical material for all providers of care to children and adults with CHD or children with acquired heart disease, whether a trainee or a practising provider. In this manuscript, we review the aims, development, current offerings and standing, and future goals of Heart University.
We present a case of pulmonary venous baffle obstruction in a child with a history of congenitally corrected transposition status post double switch repair. We highlight two forms of volume rendering three-dimensional reconstructions from computed tomographic data which allowed for detailed pre-surgical planning. These reconstructions emphasise the concept of maximizing previously obtained two-dimensional data in a time-efficient and cost-effective manner. The benefits of these reconstructions are reviewed, highlighting the relatively novel virtual dissection reconstruction technique that appeared identical to what the surgeon encountered in the operating theatre. This technique allowed the surgeon to quickly advance a preconceived detailed surgical repair.
Rapid prototyping is quickly gaining utility in various complex forms of CHD. In properly selected cases, these printed models provide detailed anatomical understanding that help guide potential surgical and cardiac catheterisation interventions. We present a case of a tunnel-like ventricular septal defect referred for surgical repair, where the decision to obtain a three-dimensional printed model helped in better understanding of the anatomy, leading to delaying, and hopefully avoiding altogether, surgical repair.