Selection of the best of 2022 in congenital heart disease

This paper shows a selection of the most relevant articles in congenital heart diseases in the last year. About intervencional procedures, we comment the latest guidelines in 2021, as well as some interesting papers on the implementation of materials and techniques in the field of percutaneous treatment of congenital heart diseases. In cardiac imaging, we focus on articles related to the revolutionary advance of 4D-NMR and 3D echo in the study of these congenital diseases. Respect pediatrics, there are interesting studies about multisystem inflammatory syndrome linked to SARS-CoV-2 (MIS-C) and also in the field of hypertrophic cardiomyopathy. Related to surgery, we comment the hybrid technique for treating hypoplastic left ventricle. Finally, regarding arrhythmias in congenital heart disease, we focus our attention on atrial fibrillation, due to the peculiarities that exist in this type of patients. Copyright © 2022 Sociedad Espanola de Cardiologia

Effects of COVID-19 on paediatric cardiac centres in low-income and middle-income countries: a mixed-methods study.

OBJECTIVES: The aim of this study was to understand the effects of the COVID-19 pandemic on paediatric cardiac services in critical access centres in low-income and middle-income countries. DESIGN: A mixed-methods approach was used. SETTING: Critical access sites that participate in the International Quality Improvement Collaborative (IQIC) for congenital heart disease (CHD) were identified. PARTICIPANTS: Eight IQIC sites in low-income and middle-income countries agreed to participate. OUTCOME MEASURES: Differences in volume and casemix before and during the pandemic were identified, and semistructured interviews were conducted with programme representatives and analysed by two individuals using NVivo software. The qualitative component of this study contributed to a better understanding of the centres' experiences and to identify themes that were common across centres. RESULTS: In aggregate, among the seven critical access sites that reported data in both 2019 and 2020, there was a 20% reduction in case volume, though the reduction varied among programmes. Qualitative analysis identified a universal impact for all programmes related to Access to Care/Clinical Services, Financial Stability and Professional/Personal Issues for healthcare providers. CONCLUSIONS: Our study identified and quantified a significant impact of the COVID-19 pandemic on critical access to CHD surgery in low-income and middle-income countries, as well as a significant adverse impact on both the skilled workforce needed to treat CHD and on the institutions in which care is delivered. These findings suggest that the COVID-19 pandemic has been a major threat to access to care for children with CHD in resource-constrained environments and that this effect may be long-lasting beyond the global emergency. Efforts are needed to preserve vulnerable CHD programmes even during unprecedented pandemic situations.

Selección de lo mejor del año 2022 en cardiopatías congénitas

Resumen En este artículo se muestran las publicaciones que se han considerado más relevantes sobre cardiopatías congénitas en el último año. En el campo del intervencionismo se comentarán las principales novedades publicadas en las últimas guías de 2021, así como algunos artículos interesantes sobre la implementación de materiales y técnicas en el tratamiento percutáneo de lesiones cardiacas congénitas. En cuanto a la imagen se centrará en los artículos relacionados con el revolucionario avance de la 4D-RMN y el eco 3D en el estudio de las cardiopatías congénitas. Existen estudios interesantes en cuanto al síndrome infamatorio multisistémico pediátrico vinculado al SARS-CoV-2 (MIS-C) y también en el ámbito de la miocardiopatía hipertrófica en edad infantil. En cuanto a la cirugía en esta franja de edad se ha considerado interesante comentar un estudio en relación con la técnica híbrida de tratamiento del ventrículo izquierdo hipoplásico. Por último, en esta ocasión en lo referente a las arritmias en las cardiopatías congénitas, la atención se centra en la fibrilación auricular, por las peculiaridades existentes en este tipo de pacientes. This paper shows a selection of the most relevant articles in congenital heart diseases in the last year. About intervencional procedures, we comment the latest guidelines in 2021, as well as some interesting papers on the implementation of materials and techniques in the field of percutaneous treatment of congenital heart diseases. In cardiac imaging, we focus on articles related to the revolutionary advance of 4D-NMR and 3D echo in the study of these congenital diseases. Respect pediatrics, there are interesting studies about multisystem inflammatory syndrome linked to SARS-CoV-2 (MIS-C) and also in the field of hypertrophic cardiomyopathy. Related to surgery, we comment the hybrid technique for treating hypoplastic left ventricle. Finally, regarding arrhythmias in congenital heart disease, we focus our attention on atrial fibrillation, due to the peculiarities that exist in this type of patients.

Cardiac cycle: an observational/interventional study protocol to characterise cardiopulmonary function and evaluate a home-based cycling program in children and adolescents born extremely preterm.

INTRODUCTION: Extremely preterm (EP)/extremely low birthweight (ELBW) individuals may have an increased risk for adverse cardiovascular outcomes. Compared with term-born controls, these individuals have poorer lung function and reduced exercise capacity. Exercise interventions play an important role in reducing cardiopulmonary risk, however their use in EP/ELBW cohorts is unknown. This study, cardiac cycle, aims to characterise the cardiopulmonary system of children and adolescents who were born EP compared with those born at term, following acute and chronic exercise bouts. METHODS AND ANALYSIS: The single-centre study comprises a home-based exercise intervention, with physiological characterisation at baseline and after completion of the intervention. Fifty-eight children and adolescents aged 10-18 years who were born EP and/or with ELBW will be recruited. Cardiopulmonary function assessed via measures of blood pressure, arterial stiffness, capillary density, peak oxygen consumption, lung clearance indexes and ventricular structure/function, will be compared with 58 age-matched and sex-matched term-born controls at baseline and post intervention. The intervention will consist of a 10-week stationary cycling programme, utilising Zwift technology. ETHICS AND DISSEMINATION: The study is approved by the Ethics Committee of the Royal Children's Hospital Melbourne under HREC2019.053. Results will be disseminated via peer-reviewed journal regardless of outcome. TRIAL REGISTRATION NUMBER: 12619000539134, ANZCTR.

Health-related quality of life in postcardiac interventional catheterization patients with congenital heart disease: a mixed-methods study protocol from Pakistan

IntroductionImprovement in health-related quality of life (HRQoL) has been reported in patients with congenital heart disease treated with interventional cardiac catheterization;however, there is a significant dearth of literature from low/middle-income countries (LMICs) about this aspect. Multiple factors like sociodemographic and cultural differences, variable procedural outcomes due to lack of technical expertise and limited resources and inconsistent postprocedure follow-up may affect HRQoL in LMICs. This protocol paper aims to describe the study methodology to determine the HRQoL and its predictors in patients who have undergone interventional cardiac catheterization. Conclusions from this protocol study will help prepare a holistic approach to delivering care to patients in low-resource settings.Methods and analysisA mixed-methods study design will be used. The quantitative arm will compare the HRQoL of these postcardiac interventional catheterization patients with their age-matched healthy siblings to identify the HRQoL predictors, whereas the qualitative arm will further explore the experiences of these patients and parents. A minimum number of 108 patients of age 2 years and above, at least 6 months postinterventional catheterization follow-up and ability to understand Urdu/English will be enrolled. PedsQL 4.0 Generic Core Scales, PedsQL Cognitive Functioning Scale and PedsQL 3.0 Cardiac Module will be used. The Student’s t-test will analyse the difference in the means of HRQoL between patients and siblings. Multiple regression will identify HRQoL predictors. A subsample of enrolled patients and parents will be interviewed and analysed using directed content analysis (a qualitative component of the study).Ethics and disseminationEthics approval has been obtained from Ethics Review Committee of The Aga Khan University, Pakistan (ERC #2020-3456-11808). Study findings will be published in peer-reviewed journals and presented at conferences.

Assessing the utility of screening electrocardiograms in paediatric patients following COVID-19.

OBJECTIVE: To determine the utility of screening electrocardiograms after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection among children in detecting myocarditis related to coronavirus disease 2019 (COVID-19). STUDY DESIGN: A retrospective chart review was performed at a large paediatric academic institution to identify patients with prior SARS-CoV-2 infection who received a screening electrocardiogram by their primary care providers and were subsequently referred for outpatient cardiology consultation due to an abnormal electrocardiogram. The outcomes were the results from their cardiology evaluations, including testing and final diagnoses. RESULTS: Among 46 patients, during their preceding COVID-19 illness, the majority had mild symptoms, 4 were asymptomatic, and 1 had moderate symptoms. The median length of time from positive SARS-CoV-2 test to screening electrocardiogram was 22 days, and many electrocardiogram findings that prompted cardiology consultation were normal variants in asymptomatic adolescent athletes. Patients underwent frequent additional testing at their cardiology appointments: repeat electrocardiogram (72%), echocardiogram (59%), Holter monitor (11%), exercise stress test (7%), and cardiac MRI (2%). Five patients were incidentally diagnosed with CHD or structural cardiac abnormalities, and three patients had conduction abnormalities (pre-mature atrial contractions, pre-mature ventricular contractions, borderline prolonged QTc), although potentially incidental to COVID-19. No patients were diagnosed with myocarditis or ventricular dysfunction. CONCLUSION: In a small cohort of children with prior COVID-19, who were primarily either asymptomatic or mildly symptomatic, subsequent screening electrocardiograms identified various potential abnormalities prompting cardiology consultation, but no patient was diagnosed with myocarditis. Larger multi-centre studies are necessary to confirm these results and to evaluate those with more severe disease.

Cardiac echocardiogram findings of severe acute respiratory syndrome coronavirus-2-associated multi-system inflammatory syndrome in children.

BACKGROUND: A novel paediatric disease, multi-system inflammatory syndrome in children, has emerged during the 2019 coronavirus disease pandemic. OBJECTIVES: To describe the short-term evolution of cardiac complications and associated risk factors in patients with multi-system inflammatory syndrome in children. METHODS: Retrospective single-centre study of confirmed multi-system inflammatory syndrome in children treated from 29 March, 2020 to 1 September, 2020. Cardiac complications during the acute phase were defined as decreased systolic function, coronary artery abnormalities, pericardial effusion, or mitral and/or tricuspid valve regurgitation. Patients with or without cardiac complications were compared with chi-square, Fisher's exact, and Wilcoxon rank sum. RESULTS: Thirty-nine children with median (interquartile range) age 7.8 (3.6-12.7) years were included. Nineteen (49%) patients developed cardiac complications including systolic dysfunction (33%), valvular regurgitation (31%), coronary artery abnormalities (18%), and pericardial effusion (5%). At the time of the most recent follow-up, at a median (interquartile range) of 49 (26-61) days, cardiac complications resolved in 16/19 (84%) patients. Two patients had persistent mild systolic dysfunction and one patient had persistent coronary artery abnormality. Children with cardiac complications were more likely to have higher N-terminal B-type natriuretic peptide (p = 0.01), higher white blood cell count (p = 0.01), higher neutrophil count (p = 0.02), severe lymphopenia (p = 0.05), use of milrinone (p = 0.03), and intensive care requirement (p = 0.04). CONCLUSION: Patients with multi-system inflammatory syndrome in children had a high rate of cardiac complications in the acute phase, with associated inflammatory markers. Although cardiac complications resolved in 84% of patients, further long-term studies are needed to assess if the cardiac abnormalities (transient or persistent) are associated with major cardiac events.

Didactic education in paediatric cardiology during the COVID-19 pandemic: a national fellow survey.

The coronavirus disease 2019 (COVID-19) pandemic has had a profound impact on medical educational curricula. We aimed to examine the impact of these unprecedented changes on the formal education of paediatric cardiology fellows through a nationwide survey. A REDCap™-based voluntary anonymous survey was sent to all current paediatric cardiology fellows in the United States of America in May, 2020. Of 143 respondents, 121 were categorical fellows, representing over one-fourth of all categorical paediatric cardiology fellows in the United States of America. Nearly all (140/143, 97.9%) respondents utilised online learning during the pandemic, with 134 (93.7%) reporting an increase in use compared to pre-pandemic. The percentage of respondents reporting curriculum supplementation with outside lectures increased from 11.9 to 88.8% during the pandemic. Respondents considered online learning to be "equally or more effective" than in-person lectures in convenience (133/142, 93.7%), improving fellow attendance (132/142, 93.0%), improving non-fellow attendance (126/143, 88.1%), and meeting individual learning needs (101/143, 70.6%). The pandemic positively affected the lecture curriculum of 83 respondents (58.0%), with 35 (24.5%) reporting no change and 25 (17.5%) reporting a negative effect. A positive effect was most noted by those whose programmes utilised supplemental outside lectures (62.2 versus 25.0%, p = 0.004) and those whose lecture frequency did not decrease (65.1 versus 5.9%, p < 0.001). Restrictions imposed by the COVID-19 pandemic have greatly increased utilisation of online learning platforms by medical training programmes. This survey reveals that an online lecture curriculum, despite inherent obstacles, offers advantages that may mitigate some negative consequences of the pandemic on fellowship education.

Left ventricular longitudinal strain alterations in asymptomatic or mildly symptomatic paediatric patients with SARS-CoV-2 infection.

AIMS: Compared with adult patients, clinical manifestations of children's coronavirus disease-2019 (COVID-19) are generally perceived as less severe. The objective of this study was to evaluate cardiac involvement in previously healthy children with asymptomatic or mildly symptomatic severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection. METHODS AND RESULTS: We analysed a cohort of 53 paediatric patients (29 males, 55%), mean age 7.5 ± 4.7 years, who had a confirmed diagnosis of SARS-CoV-2 infection and were asymptomatic or only mildly symptomatic for COVID-19. Patients underwent standard transthoracic echocardiogram and speckle tracking echocardiographic study at least 3 months after diagnosis. Thirty-two age, sex, and body surface area comparable healthy subjects were used as control group. Left ventricular ejection fraction was within normal limits but significantly lower in the cases group compared to controls (62.4 ± 4.1% vs. 65.2 ± 5.5%; P = 0.012). Tricuspid annular plane systolic excursion (20.1 ± 3 mm vs. 19.8 ± 3.4 mm; P = 0.822) and left ventricular (LV) global longitudinal strain (-21.9 ± 2.4% vs. -22.6 ± 2.5%; P = 0.208) were comparable between the two groups. Regional LV strain analysis showed a significant reduction of the LV mid-wall segments strain among cases compared to controls. Furthermore, in the cases group, there were 14 subjects (26%) with a regional peak systolic strain below -16% (-2.5 Z score in our healthy cohort) in at least two segments. These subjects did not show any difference regarding symptoms or serological findings. CONCLUSION: SARS-CoV-2 infection may affect left ventricular deformation in 26% of children despite an asymptomatic or only mildly symptomatic acute illness. A follow-up is needed to verify the reversibility of these alterations and their impact on long-term outcomes.

Medical education and training within congenital cardiology: current global status and future directions in a post COVID-19 world.

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 reduce the environmental footprint of pediatric cardiology conferences.

BACKGROUND: Webinars have recently replaced in-person medical conferences, including paediatric cardiology conferences, given the COVID-19 pandemic. METHODS: 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). RESULTS: 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. CONCLUSIONS: 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.

Maintaining paediatric cardiac services during the COVID-19 pandemic in a developing country in sub-Saharan Africa: guidelines for a "scale up" in the face of a global "scale down".

The COVID-19 pandemic is currently ravaging the globe and the African continent is not left out. While the direct effects of the pandemic in regard to morbidity and mortality appear to be more significant in the developed world, the indirect harmful effects on already insufficient healthcare infrastructure on the African continent would in the long term be more detrimental to the populace. Women and children form a significant vulnerable population in underserved areas such as the sub-Saharan region, and expectedly will experience the disadvantages of limited healthcare coverage which is a major fall out of the pandemic. Paediatric cardiac services that are already sparse in various sub-Saharan countries are not left out of this downsizing. Restrictions on international travel for patients out of the continent to seek medical care and for international experts into the continent for regular mission programmes leave few options for children with cardiac defects to get the much-needed care.There is a need for a region-adapted guideline to scale-up services to cater for more children with congenital heart disease (CHD) while providing a safe environment for healthcare workers, patients, and their caregivers. This article outlines measures adapted to maintain paediatric cardiac care in a sub-Saharan tertiary centre in Nigeria during the COVID-19 pandemic and will serve as a guide for other institutions in the region who will inadvertently need to provide these services as the demand increases.

Paediatric/congenital cardiology physician scientists-An endangered species.

Producing excellent physician scientists starts with the active discovery of talent and dedication, supported by the strong belief that physician involvement in biomedical research is essential to make fundamental discoveries that improve human health. The revolution of surgical and interventional therapy of structural heart disease has had 'profoundly positive effects on survival and quality of life over the decades. (…) Small increments in clinical improvement will still be possible in the future, but for the most part, the potential for major advancement using these techniques has been exhausted' (Frank Hanley, MD; Stanford). Personalized medicine, rapid genetic diagnostics, RNA and extracellular vesicle biology, epigenetics, gene editing, gene and stem cell-derived therapy are exemplary areas where specialized training for paediatric/congenital cardiology physician scientists will be increasingly needed to further advance the field. About a decade ago, a series in Circulation discussed academic career models and highlighted the major challenges facing the cardiovascular 'clinician scientist' (syn. physician scientist), which have not abated since. To develop the skills and expertise in both clinical congenital cardiology and basic research, the training of fellows must be focused and integrated. The current pandemic COVID-19 puts additional pressure and hurdles on fellows-in-training (FIT) and early career investigators (ECI) who aim to establish, consolidate or expand their own research group. Here, we discuss the major challenges, opportunities and necessary changes for academic institutions to sustain and recruit physician scientists in paediatric/congenital cardiology in the years to come.

Learning from the past in the COVID-19 era: rediscovery of quarantine, previous pandemics, origin of hospitals and national healthcare systems, and ethics in medicine.

After the dramatic coronavirus outbreak at the end of 2019 in Wuhan, Hubei province, China, on 11 March 2020, a pandemic was declared by the WHO. Most countries worldwide imposed a quarantine or lockdown to their citizens, in an attempt to prevent uncontrolled infection from spreading. Historically, quarantine is the 40-day period of forced isolation to prevent the spread of an infectious disease. In this educational paper, a historical overview from the sacred temples of ancient Greece-the cradle of medicine-to modern hospitals, along with the conceive of healthcare systems, is provided. A few foods for thought as to the conflict between ethics in medicine and shortage of personnel and financial resources in the coronavirus disease 2019 era are offered as well.