Showing papers in "Cardiology in The Young in 2020"

Neurodevelopmental evaluation strategies for children with congenital heart disease aged birth through 5 years: recommendations from the cardiac neurodevelopmental outcome collaborative

Abstract: This paper provides specific guidelines for the neurodevelopmental evaluation of children aged birth through 5 years with complex congenital heart disease. There is wide recognition that children with congenital heart disease are at high risk for neurodevelopmental impairments that are first apparent in infancy and often persist as children mature. Impairments among children with complex congenital heart disease cross developmental domains and affect multiple functional abilities. The guidelines provided are derived from the substantial body of research generated over the past 30 years describing the characteristic developmental profiles and the long-term trajectories of children surviving with complex congenital heart conditions. The content and the timing of the guidelines are consistent with the 2012 American Heart Association and the American Academy of Pediatrics scientific statement documenting the need for ongoing developmental monitoring and assessment from infancy through adolescence. The specific guidelines offered in this article were developed by a multidisciplinary clinical research team affiliated with the Cardiac Neurodevelopmental Outcome Collaborative, a not-for-profit organisation established to determine and implement best neurodevelopmental practices for children with congenital heart disease. The guidelines are designed for use in clinical and research applications and offer an abbreviated core protocol and an extended version that expands the scope of the evaluation. The guidelines emphasise the value of early risk identification, use of evidence-based assessment instruments, consideration of family and cultural preferences, and the importance of providing multidimensional community-based services to remediate risk.

Neurodevelopmental evaluation for school-age children with congenital heart disease: recommendations from the cardiac neurodevelopmental outcome collaborative.

Abstract: In 2012, the American Heart Association and the American Academy of Paediatrics released a scientific statement with guidelines for the evaluation and management of the neurodevelopmental needs of children with CHD. Decades of outcome research now highlight a range of cognitive, learning, motor, and psychosocial vulnerabilities affecting individuals with CHD across the lifespan. The number of institutions with Cardiac Neurodevelopmental Follow-Up Programmes and services for CHD is growing worldwide. This manuscript provides an expanded set of neurodevelopmental evaluation strategies and considerations for professionals working with school-age children with CHD. Recommendations begin with the referral process and access to the evaluation, the importance of considering medical risk factors (e.g., genetic disorders, neuroimaging), and the initial clinical interview with the family. The neurodevelopmental evaluation should take into account both family and patient factors, including the child/family's primary language, country of origin, and other cultural factors, as well as critical stages in development that place the child at higher risk. Domains of assessment are reviewed with emphasis on target areas in need of evaluation based on current outcome research with CHD. Finally, current recommendations are made for assessment batteries using a brief core battery and an extended comprehensive clinical battery. Consistent use of a recommended assessment battery will increase opportunities for research collaborations, and ultimately help improve the quality of care for families and children with CHD.

Heart University: a new online educational forum in paediatric and adult congenital cardiac care. The future of virtual learning in a post-pandemic world?

Abstract: 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.

COVID-19 and Kawasaki syndrome: should we really be surprised?

Abstract: A hyperinflammatory response to COVID-19 is being described in children. While this presents, and responds to management, similar to that of Kawasaki Disease it is being coined a new entity. But is it really? We explore how this phenomenon may be Kawasaki Disease with a new trigger.

The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States

Abstract: The Fontan Outcomes Network was created to improve outcomes for children and adults with single ventricle CHD living with Fontan circulation. The network mission is to optimise longevity and quality of life by improving physical health, neurodevelopmental outcomes, resilience, and emotional health for these individuals and their families. This manuscript describes the systematic design of this new learning health network, including the initial steps in development of a national, lifespan registry, and pilot testing of data collection forms at 10 congenital heart centres.

The origins and development of the Cardiac Neurodevelopmental Outcome Collaborative: creating innovative clinical, quality improvement, and research opportunities - Corrigendum.

Abstract: Compared to the general population, individuals with complex congenital heart disease are at increased risk for deficits in cognitive, neurodevelopmental, psychosocial, and physical functioning, resulting in a diminished health-related quality of life. These deficits have been well described over the past 25 years, but significant gaps remain in our understanding of the best practices to improve neurodevelopmental and psychosocial outcomes and health-related quality of life for individuals with paediatric and congenital heart disease. Innovative clinical, quality improvement, and research opportunities with collaboration across multiple disciplines and institutions were needed to address these gaps. The Cardiac Neurodevelopmental Outcome Collaborative was founded in 2016 with a described mission to determine and implement best practices of neurodevelopmental and psychosocial services for individuals and their families with paediatric and congenital heart disease through clinical, quality improvement, and research initiatives. The vision is to be a multi-centre, multi-national, multi-disciplinary group of healthcare professionals committed to working together and partnering with families to optimise neurodevelopmental outcomes for individuals with paediatric and congenital heart disease through clinical, quality, and research initiatives, intending to maximise quality of life for every individual across the lifespan. This manuscript describes the development and organisation of the Cardiac Neurodevelopmental Outcome Collaborative.

New onset severe right ventricular failure associated with COVID-19 in a young infant without previous heart disease.

Abstract: We present our recent experience with a 6-month-old infant with a personal history of short bowel syndrome that presented with fever, cyanosis, and cardiogenic shock secondary to severe pulmonary hypertension and right ventricular failure without pulmonary thromboembolism. He did not present signs of toxin-mediated disease or Kawasaki disease. He was finally diagnosed with SARS-CoV-2 infection. If this presentation is confirmed in future research, the severe cardiovascular impairment in children with COVID-19 could be also attributable to the primary pulmonary infection, not only to a multisystem inflammatory syndrome but also in children without heart disease.

Treatment strategies for protein-losing enteropathy in Fontan-palliated patients.

Abstract: Objective:Protein-losing enteropathy is an infrequent but severe condition occurring after Fontan procedure. The multifactorial pathogenesis remains unclear and no single proposed treatment strategy has proven universally successful. Therefore, we sought to describe different treatment strategies and their effect on clinical outcome and mortality.Material and Methods:We performed a retrospective observational study. From the total cohort of 439 Fontan patients treated in our institution during the study period 1986–2019, 30 patients (6.8%) with protein-losing enteropathy were identified. Perioperative, clinical, echocardiographic, laboratory, and invasive haemodynamic findings and treatment details were analysed.Results:Median follow-up after disease onset was 13.1 years [interquartile range 10.6]. Twenty-five patients received surgical or interventional treatment for haemodynamic restrictions. Medical treatment, predominantly pulmonary vasodilator and/or systemic anti-inflammatory therapy with budesonide, was initiated in 28 patients. In 15 patients, a stable remission could be achieved by medical or surgical procedures (n = 3 each), by combined multimodal therapy (n = 8), or ultimately by cardiac transplantation (n = 1). Phrenic palsy, bradyarrhythmia, Fontan pathway stenosis, and absence of a fenestration were significantly associated with development of protein-losing enteropathy (p = 0.001–0.48). Ten patients (33.3%) died during follow-up; 5-year survival estimate was 96.1%. In unadjusted analysis, medical therapy with budesonide and pulmonary vasodilator therapy in combination was associated with improved survival.Conclusions:Protein-losing enteropathy is a serious condition limiting survival after the Fontan procedure. Comprehensive assessment and individual treatment strategies are mandatory to achieve best possible outcome. Nevertheless, relapse is frequent and long-term mortality substantial. Cardiac transplantation should be considered early as treatment option.

Echocardiographic examination of mitral valve abnormalities in the paediatric population: Current practices

Abstract: We reviewed the recent literature for echocardiographic assessment of mitral valve abnormalities in children. A literature search was performed within the National Library of Medicine using the keywords "mitral regurgitation and/or stenosis, children." The search was refined by adding the keywords "echocardiographic definition, classification, and evaluation." Thirty-one studies were finally included. Significant advances in echocardiographic imaging of mitral valve defects, mainly due to the implementation of three-dimensional technology, contribute to a better understanding of the underlying anatomy. However, heterogeneity between classification systems of mitral valve disease severity is a serious problem. For regurgitant lesions, there is only very limited evidence from small studies that support the adoption of quantitative/semi-quantitative indexes commonly employed in adults. Despite the lack of evidence base, qualitative evaluation of regurgitation severity is often employed. For stenotic lesions, no clear categorisation based on trans-valvular echocardiography-derived "gradients" has been consistently applied to define mild, moderate, or severe obstruction across different paediatric age ranges. Quantitative parameters such as valve area have also been poorly validated in children. Adult recommendations are frequently applied without validation for the paediatric age. In conclusion, significant advances in the anatomical evaluation of mitral valve diseases have been made, thanks to three-dimensional echocardiography; however, limitations remain in the quantitative/semi-quantitative estimation of disease severity, both with respect to valvular regurgitation and stenosis. Because adult echocardiographic recommendations should not be simply translated to the paediatric age, more specific paediatric guidelines and standards for the assessment of mitral valve diseases are needed.

Quantitative cardiac magnetic resonance T2 imaging offers ability to non-invasively predict acute allograft rejection in children.

Abstract: Background Monitoring for acute allograft rejection improves outcomes after cardiac transplantation. Endomyocardial biopsy is the gold standard test defining rejection, but carries risk and has limitations. Cardiac magnetic resonance T2 mapping may be able to predict rejection in adults, but has not been studied in children. Our aim was to evaluate T2 mapping in identifying paediatric cardiac transplant patients with acute rejection. Methods Eleven paediatric transplant patients presenting 18 times were prospectively enrolled for non-contrast cardiac magnetic resonance at 1.5 T followed by endomyocardial biopsy. Imaging included volumetry, flow, and T2 mapping. Regions of interest were manually selected on the T2 maps using the middle-third technique in the left ventricular septal and lateral wall in a short-axis and four-chamber slice. Mean and maximum T2 values were compared with Student's t-tests analysis. Results Five cases of acute rejection were identified in three patients, including two cases of grade 2R on biopsy and three cases of negative biopsy treated for clinical symptoms attributed to rejection (new arrhythmia, decreased exercise capacity). A monotonic trend between increasing T2 values and higher biopsy grades was observed: grade 0R T2 53.4 ± 3 ms, grade 1R T2 54.5 ms ± 3 ms, grade 2R T2 61.3 ± 1 ms. The five rejection cases had significantly higher mean T2 values compared to cases without rejection (58.3 ± 4 ms versus 53 ± 2 ms, p = 0.001). Conclusions Cardiac magnetic resonance with quantitative T2 mapping may offer a non-invasive method for screening paediatric cardiac transplant patients for acute allograft rejection. More data are needed to understand the relationship between T2 and rejection in children.

Cardiac rehabilitation in the paediatric Fontan population: development of a home-based high-intensity interval training programme.

Abstract: Introduction: We evaluated the safety and feasibility of high-intensity interval training via a novel telemedicine ergometer (MedBIKE™) in children with Fontan physiology. Methods: The MedBIKE™ is a custom telemedicine ergometer, incorporating a video game platform and live feed of patient video/audio, electrocardiography, pulse oximetry, and power output, for remote medical supervision and modulation of work. There were three study phases: (I) exercise workload comparison between the MedBIKE™ and a standard cardiopulmonary exercise ergometer in 10 healthy adults. (II) In-hospital safety, feasibility, and user experience (via questionnaire) assessment of a MedBIKE™ high-intensity interval training protocol in children with Fontan physiology. (III) Eight-week home-based high-intensity interval trial programme in two participants with Fontan physiology. Results: There was good agreement in oxygen consumption during graded exercise at matched work rates between the cardiopulmonary exercise ergometer and MedBIKE™ (1.1 ± 0.5 L/minute versus 1.1 ± 0.5 L/minute, p = 0.44). Ten youth with Fontan physiology (11.5 ± 1.8 years old) completed a MedBIKE™ high-intensity interval training session with no adverse events. The participants found the MedBIKE™ to be enjoyable and easy to navigate. In two participants, the 8-week home-based protocol was tolerated well with completion of 23/24 (96%) and 24/24 (100%) of sessions, respectively, and no adverse events across the 47 sessions in total. Conclusion: The MedBIKE™ resulted in similar physiological responses as compared to a cardiopulmonary exercise test ergometer and the high-intensity interval training protocol was safe, feasible, and enjoyable in youth with Fontan physiology. A randomised-controlled trial of a home-based high-intensity interval training exercise intervention using the MedBIKE™ will next be undertaken.

Pre-operative neutrophil-lymphocyte ratio predicts low cardiac output in children after cardiac surgery.

Abstract: Neutrophil-lymphocyte ratio has been associated with clinical outcomes in several groups of cardiac patients, including patients with coronary artery disease, cardiac failure, and cardiac transplant recipients. We hypothesised that pre- and/or post-operative haematological cell counts are associated with clinical outcomes in children undergoing cardiac surgery for CHD. We performed a post hoc analysis of data collected as part of a prospective observational cohort study (n = 83, data available n = 47) of children evaluated for glucocorticoid receptor levels after cardiac surgery (July 2015-January 2016). The association of neutrophil-lymphocyte ratio with low cardiac output syndrome, time to inotrope free, and vasoactive-inotropic score was examined using proportional odds analysis, cox regression, and linear regression models, respectively. A majority (80%) of patients were infants (median/interquartile range 4.1/0.2-7.6 months) with conotruncal (36%) and left-sided obstructed lesions (28%). Two patients required mechanical circulatory support and three died. Higher pre-operative neutrophil-lymphocyte ratio was associated with higher cumulative odds of severe/moderate versus mild low cardiac output on post-operative day 1 (odds ratio 2.86; 95% confidence interval 1.18-6.93; p = 0.02). Pre-operative neutrophil-lymphocyte ratio was not significantly associated with time to inotrope free or vasoactive-inotrope score. Post-operative neutrophil-lymphocyte ratio was also not associated with outcomes. In children after congenital heart surgery, higher pre-operative neutrophil-lymphocyte ratio was associated with a higher chance of low cardiac output in the early post-operative period. Pre-operative neutrophil-lymphocyte ratio maybe a useful prognostic marker in children undergoing congenital heart surgery.

Are there any novel markers in acute rheumatic fever: neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, and monocyte-to-lymphocyte ratio.

Abstract: Objective:The aim of this study was to investigate the relationship between C-reactive protein and erythrocyte sedimentation rate and neutrophil-to-lymphocyte, platelet-to-lymphocyte, and monocyte-to-lymphocyte ratios in acute rheumatic fever in children.Method:In this retrospective study, 182 patients with acute rheumatic fever and 173 controls were included. Complete blood count parameters, and neutrophil-to-lymphocyte, monocyte-to-lymphocyte, and platelet-to-lymphocyte ratios were recorded for all the patients underwent transthoracic echocardiography.Results:Neutrophil-to-lymphocyte, monocyte-to-lymphocyte, and platelet-to-lymphocyte ratios were significantly higher in patients with rheumatic heart disease than patients without cardiac involvement (p < 0.05). C-reactive protein and erythrocyte sedimentation rate levels were found to have a positive correlation with neutrophil-to-lymphocyte (r = 0.228, p = 0.001; r = 0.355, p = 0.001), platelet-to-lymphocyte (r = 0.227, p = 0.01; r = 0.149, p = 0.005), and monocyte-to-lymphocyte ratios (r = 0.117, p = 0.005; r = 0.107, p = 0.044). Cardiac involvement was present in 152 (83.5%) of the patients. Neutrophil-to-lymphocyte, monocyte-to-lymphocyte, and platelet-to-lymphocyte ratios were significantly higher in patients with rheumatic heart disease than patients without cardiac involvement (p < 0.05). Patients with carditis were grouped according to mitral, aortic, or both valve involvement but there was no significant difference between the groups with respect to neutrophil-to-lymphocyte, monocyte-to-lymphocyte, and platelet-to-lymphocyte ratios. In addition, neutrophil-to-lymphocyte and monocyte-to-lymphocyte ratios were significantly higher in patients with Sydenham’s chorea than without chorea (p < 0.05).Conclusion:Neutrophil-to-lymphocyte, platelet-to-lymphocyte, and monocyte-to-lymphocyte ratios may help make the diagnosis of acute rheumatic fever and its prognosis by serial measurements in follow-up but none of them tell us the severity of carditis. Also, this is the first study showing the positive correlation between Sydenham’s chorea and neutrophil-to-lymphocyte and monocyte-to-lymphocyte ratios. Further studies are needed to confirm this hypothesis, as this is the first study in the literature on this topic.

Anxiety reduction after pre-procedure meetings in patients with CHD.

Abstract: Background:Cardiac catheterisations for CHD produce anxiety for patients and families. Current strategies to mitigate anxiety and explain complex anatomy include pre-procedure meetings and educational tools (cardiac diagrams, echocardiograms, imaging, and angiography). More recently, three-dimensionally printed patient-specific models can be added to the armamentarium. The purpose of this study was to evaluate the efficacy of pre-procedure meetings and of different educational tools to reduce patient and parent anxiety before a catheterisation.Methods:Prospective study of patients ≥18 and parents of patients <18 scheduled for clinically indicated catheterisations. Patients completed online surveys before and after meeting with the interventional cardiologist, who was blinded to study participation. Both the pre- and post-meeting surveys measured anxiety using the State-Trait Anxiety Inventory. In addition, the post-meeting survey evaluated the subjective value (from 1 to 4) of individual educational tools: physician discussion, cardiac diagrams, echocardiograms, prior imaging, angiograms and three-dimensionally printed cardiac models. Data were compared using paired t-tests.Results:Twenty-three patients consented to participate, 16 had complete data for evaluation. Mean State-Trait Anxiety Inventory scores were abnormally elevated at baseline and decreased into the normal range after the pre-procedure meeting (39.8 versus 31, p = 0.008). Physician discussion, angiograms, and three-dimensional models were reported to be most effective at increasing understanding and reducing anxiety.Conclusion:In this pilot study, we have found that pre-catheterisation meetings produce a measurable decrease in patient and family anxiety before a procedure. Discussions of the procedure, angiograms, and three-dimensionally printed cardiac models were the most effective educational tools.

Variations in practice in cardiac neurodevelopmental follow-up programs.

Abstract: Over the last two decades, heart centres have developed strategies to meet the neurodevelopmental needs of children with congenital heart disease. Since the publication of guidelines in 2012, cardiac neurodevelopmental follow-up programmes have become more widespread. Local neurodevelopmental programmes, however, have been developed independently in widely varying environments. We sought to characterise variation in structure and personnel in cardiac neurodevelopmental programmes. A 31-item survey was sent to all member institutions of the Cardiac Neurodevelopmental Outcome Collaborative. Multidisciplinary teams at each centre completed the survey. Responses were compiled in a descriptive fashion. Of the 29 invited centres, 23 responded to the survey (79%). Centres reported more anticipated neurodevelopment visits between birth and 5 years of age (median 5, range 2-8) than 5-18 years (median 2, range 0-10) with 53% of centres lacking any standard for routine neurodevelopment evaluations after 5 years of age. Estimated annual neurodevelopment clinic volume ranged from 85 to 428 visits with a median of 16% of visits involving children >5 years of age. Among responding centres, the Bayley Scales of Infant and Toddler Development and Wechsler Preschool and Primary Scale of Intelligence were the most routinely used tests. Neonatal clinical assessment was more common (64%) than routine neonatal brain imaging (23%) during hospitalisation. In response to clinical need and published guidelines, centres have established formal cardiac neurodevelopment follow-up programmes. Centres vary considerably in their approaches to routine screening and objective testing, with many centres currently focussing their resources on evaluating younger patients.

Supporting parenting during infant hospitalisation for CHD.

Abstract: Objective: To characterise the parenting priorities of mothers and fathers of infants hospitalised with CHD and generate recommendations to support parenting during infant hospitalisation. Study design: Through online crowdsourcing, an innovative research methodology to create an online community to serve as a research sample, 79 parents of young children with CHD responded to questions about parenting during hospitalisation via private social networking site. Responses were analysed using qualitative research methods. Results: Three broad themes were identified: (1) establishing a bond with my baby, (2) asserting the parental role, and (3) coping with fear and uncertainty. Parents value provider support in restoring normalcy to the parenting experience during infant hospitalisation. Conclusions: Care teams can support parenting during infant hospitalisation by promoting parents’ roles as primary caretakers and decision-makers and attending to the emotional impact of infant hospitalisation on the family.

Aetiological evaluation of chest pain in childhood and adolescence.

Abstract: BACKGROUND Chest pain, as a common cause of hospital admissions in childhood, necessitates detailed investigations due to a wide range of differential diagnoses. In this study, we aimed to determine the distribution of diseases causing chest pain in children and investigate the clinical characteristics of children with chest pain. METHODS This study included 782 patients aged between 3 and 18 years who presented to a paediatric cardiology outpatient clinic with chest pain between April 2017 and March 2018. Aetiological causes and demographic features of the patients were analysed. RESULTS Most prevalent causes of chest pain were musculoskeletal system (33%) and psychogenic (28.4%) causes. Chest pain due to cardiac reasons was seen in eight patients (1%). Diseases of musculoskeletal and gastrointestinal systems and psychogenic disorders were significantly more common in male and female patients, respectively (p < 0.001 for all). In winter, patients' age and the number of patients with ≥12 years were higher than those in other seasons (p < 0.001). Most of the parents (70.8%) and patients (90.2%) thought that chest pain in their children was caused by cardiac causes. CONCLUSION Most of the diagnoses for chest pain in childhood period are benign and include the musculoskeletal system and psychogenic diseases. Although chest pain due to cardiac diseases is rare, a comprehensive analysis of medical history, detailed physical examination and cardiac imaging with echocardiography is needed to reach more accurate diagnoses.

Perioperative care of the newborns with CHDs in the time of COVID-19.

Abstract: Coronavirus disease 2019 (COVID-19), caused by a novel betacoronavirus (SARS-CoV-2), has led to an unexpected outbreak affecting people of all ages The first data showed that COVID-19 could cause severe pulmonary disease, cardiac injury, and death in adults, especially the elderly and those with concomitant diseases Currently, it was demonstrated that severe COVID-19 may also develop in neonatal age, although rarely Newborns with CHD are known to be at high risk for increased morbidity from viral lower respiratory tract infections because of underlying anatomical cardiac lesions There are limited data on the implications of COVID-19 on patients with cardiovascular disease, especially for those with CHD Herein, we aimed to summarise the COVID-19-specific perioperative management issues for newborns with CHD by combining available data from the perspectives of neonatology and paediatric cardiovascular surgery

Attention-deficit/hyperactivity disorder symptoms in children with surgically corrected Ventricular Septal Defect, Transposition of the Great Arteries, and Tetralogy of Fallot

Abstract: Background Children with complex CHD are at risk for psychopathology such as severe attention-deficit/hyperactivity disorder symptoms after congenital heart surgery. Objective The aim of this study was to investigate if children with Ventricular Septal Defect, Transposition of Great Arteries, or Tetralogy of Fallot have an increased occurrence of attention-deficit/hyperactivity disorder symptoms compared with the background population and to investigate differences between the three CHDs in terms of occurrence and appearance of attention-deficit/hyperactivity disorder symptoms. Method A national register-based survey was conducted, including children aged 10-16 years with surgically corrected CHDs without genetic abnormalities and syndromes. The Attention-Deficit/Hyperactivity Disorder-Rating Scale questionnaires were filled in by parents and school teachers. Results In total, 159 out of 283 questionnaires were completed among children with CHDs and compared with age- and sex-matched controls. Children with CHDs had significantly increased inattention scores (p = 0.009) and total attention-deficit/hyperactivity disorder scores (p = 0.008) compared with controls. Post hoc analyses revealed that children with Tetralogy of Fallot had significantly higher inattention scores compared with children both with Ventricular Septal Defect (p = 0.043) and controls (p = 0.004). Conclusion Attention-deficit/hyperactivity disorder symptoms and inattention symptoms were significantly more frequent among children aged 10-16 years with CHDs, in particular in children with corrected Tetralogy of Fallot.

Effects of metformin on epicardial adipose tissue and atrial electromechanical delay of obese children with insulin resistance.

Abstract: Introduction: Obesity is usually related to insulin resistance and glucose metabolism disorders. The relationship between insulin resistance and epicardial adipose tissue and atrial electromechanical delay has been described in previous studies. Aim: This study aims to demonstrate the effects of metformin on epicardial adipose tissue and electromechanical delay in patients using metformin for insulin resistance. Materials and methods: A total of 30 patients using metformin for insulin resistance were included in the study. Pre-treatment and post-treatment epicardial adipose tissue and electromechanical delay were evaluated. Results: There was a statistically significant decrease in epicardial adipose tissue thickness after 3 months of metformin therapy (6.4 ± 2.1 versus 4.7 ± 2.0; p = 0.008). Furthermore, the inter-atrial and intra-atrial electromechanical delay also significantly decreased after 3 months of metformin monotherapy (23.6 ± 8.2 versus 18.1 ± 5.8; p < 0.001, 9.1 ± 2.9 versus 6.3 ± 3.6; p = 0.003, respectively). Conclusion: In this study, we show that metformin monotherapy significantly decreases epicardial adipose tissue thickness and electromechanical delay in obese children.

Leveraging e-learning for medical education in low- and middle-income countries.

Abstract: Tretter et al. are to be applauded for their commitment to developing Heart University and providing a detailed overview thereof.1 As they proclaim, “Heart University aims to be ‘the go-to online resource’ for e-learning in CHD and paediatric-acquired heart disease”, reaching full speed amidst the COVID-19 pandemic – a time in which medical education is forced to shift to online learning. Heart University has included a notable focus on “Cardiology in Lowand Middle-Income Countries”, and it is exactly there where its impact may be highest – far beyond the era of COVID-19. Disparities in access to comprehensive cardiovascular care for children exist at all levels and is particularly visible in skilled workforce availability.2,3 In lowand middle-income countries, limited training programmes exist to train cardiologists and cardiac surgeons, and especially so with a paediatric focus. This is further complicated by the limited training of anaesthesiologists, nurses, and other healthcare professionals in paediatric cardiac management. Continued medical education is either absent or commonly has to be obtained abroad, traditionally with substantial direct and opportunity costs. As nearly half of the population in Sub-Saharan Africa owns a smartphone and internet connectivity increasingly reaches communities in the last mile, opportunities arise to leverage digital health technologies and online medical education platforms.4 Positive attitudes towards the use of e-learning modalities in lowand middle-income countries have been illustrated prior and may be leveraged in the fields of paediatric cardiology and cardiac surgery.5 Critical components to enable and scale e-learning platforms in lowand middle-income countries include: Open-access information: removing paywalls and user and subscription fees associated with the software; User-friendly operability: easy and gentle learning curve to promote more rapid and widespread adoption; Smartphone compatibility: given the diffuse spread of smartphones, comparably lower device costs, and functionality as a pocket resource, smartphones are preferred over desktop-only platforms; Low-bandwidth needs: although internet connectivity is growing, high-speed internet and large-data mobile plans are associated with higher user fees; Self-paced learning: asynchronous (i.e., not only live or time-restrictive) training modules, able to accessed by healthcare professionals when time is available amidst busy clinical schedules; Discussionand case-based learning: the lack of in-person supervision may be substituted by discussion groups and the utilisation and crowdsourcing of clinical cases to better inform local contexts, navigate resource constraints, and manage atypical pathologies or disease processes; Multiple languages: global health, academia, and international medical education are, traditionally, anglophone-skewed; maximising the impact of e-learning platforms in lowand middle-income countries includes the translation in multiple languages other than English, including, where possible, local languages. Conventional medical education, while having important advantages including real-time discussions, hands-on teaching, and expert oversight, has constraints in terms of rapidly scaling (non-procedural) medical education in large numbers. Developments such as Heart University ought to be leveraged and scaled in order to provide equal opportunities for continued medical education for healthcare professionals across the globe, and, in turn, optimising the quality and scope of care delivery.

A transition clinic intervention to improve follow-up rates in adolescents and young adults with congenital heart disease.

Abstract: Background:Children with congenital heart disease (CHD) require lifelong cardiology follow-up. Many experience gaps in care around the age of transition to adult-oriented care with associated comorbidity. We describe the impact of a clinic-based intervention on follow-up rates in this high-risk population.Methods:Patients ≥11 years seen in a paediatric outpatient CHD Transition Clinic completed self-assessment questionnaires, underwent focused teaching, and were followed on a clinic registry with assessment of care continuation. The cohort “lost to follow-up” rate, defined as absence from care at least 6 months beyond the recommended timeframe, was compared with a control group. Secondary outcomes included questionnaire scores and adult cardiology referral trends.Results:Over 26 months, 53 participants completed an initial Transition Clinic visit; 43% (23/53) underwent a second visit. Median participant age was 18.0 years (interquartile range 16.0, 22.0). The cohort’s “lost to follow-up” rate was 7.3%, which was significantly lower than the control rate (25.9%, p < 0.01). Multivariable regression analyses demonstrated clinic participation as the only factor independently associated with follow-up rates (p = 0.048). Transition readiness was associated with older age (p = 0.01) but not sex, univentricular heart, interventional history, or surgical complexity. One-third of adult participants transferred to adult care.Conclusions:A CHD Transition Clinic intervention can improve follow-up rates in adolescents and young adults. Age is an important factor in transition readiness, and retention of adults in paediatric care appears multi-factorial. We postulate that serial assessments of self-management, focused education, and registry utilisation may improve patient outcomes by reducing lapses in care.

Mixed method study of quality of life for children with trisomy 18 and 13 after cardiac surgery.

Abstract: Background and objectives Cardiac surgical interventions for children with trisomy 18 and trisomy 13 remain controversial, despite growing evidence that definitive cardiac repair prolongs survival. Understanding quality of life for survivors and their families therefore becomes crucial. Study objective was to generate a descriptive summary of parental perspectives on quality of life, family impact, functional status, and hopes for children with trisomy 18 and trisomy 13 who have undergone heart surgery. Methods A concurrent mixed method approach utilising PedsQL™ 4.0 Generic Core Parent Report for Toddlers or the PedsQL™ Infant Scale, PedsQL™ 2.0 Family Impact Module, Functional Status Scale, quality of life visual analogue scale, and narrative responses for 10 children whose families travelled out of state to access cardiac surgery denied to them in their home state due to genetic diagnoses. Results Parents rated their child's quality of life as 80/100, and their own quality of life as 78/100 using validated scales. Functional status was rated 11 by parents and 11.6 by providers (correlation 0.89). On quality of life visual analogue scale, all parents rated their child's quality of life as "high" with mean response 92.7/100. Parental hopes were informed by realistic perspective on prognosis while striving to ensure their children had access to reaching their full potential. Qualitative analysis revealed a profound sense of the child's relationality and valued life meaning. Conclusion Understanding parental motivations and perceptions on the child's quality of life has potential to inform care teams in considering cardiac interventions for children with trisomy 18 and trisomy 13.

The quality, understandability, readability, and popularity of online educational materials for heart murmur.

Abstract: OBJECTIVE Murmurs are abnormal audible heart sounds produced by turbulent blood flow. Therefore, murmurs in a child may be a source of anxiety for family members. Families often use online materials to explore possible reasons for these murmurs, given the accessibility of information on the Internet. In this study, we evaluated the quality, understandability, readability, and popularity of online materials about heart murmur. METHODS An Internet search was performed for "heart murmur" using the Google search engine. The global quality score (on a scale of 1 to 5, corresponding to poor to excellent quality) and Health on the Net code were used to measure the quality of information presented. The understandability of the web pages identified was measured using the Patient Education Materials Assessment Tool (score range from 0 to 100%, scores below 70% reflect poor performance). The readability of each web pages was assessed using four validated indices: the Flesch Reading Ease Score, the Flesch-Kincaid Grade Level, the Gunning Frequency of Gobbledygook, and the Simple Measure of Gobbledygook. The ALEXA traffic tool was used to reference domains' popularity and visibility. RESULTS We identified 230 English-language patient educational materials that discussed heart murmur. After exclusion, a total of 86 web pages were evaluated for this study. The average global quality score was 4.34 (SD = 0.71; range from 3 to 5) indicating that the quality of information of most websites was good. Only 14 (16.3%) websites had Health on the Net certification. The mean understandability score for all Internet-based patient educational materials was 74.6% (SD = 12.8%; range from 31.2 to 93.7%). A score suggesting these Internet-based patient educational materials were "easy to understand". The mean readability levels of all patient educational materials were higher than the recommended sixth-grade reading level, according to all indices applied. This means that the level of readability is difficult. The average grade level for all web pages was 10.4 ± 1.65 (range from 7.53 to 14.13). The Flesch-Kincaid Grade level was 10 ± 1.81, the Gunning Frequency of Gobbledygook level was 12.1 ± 1.85, and the Simple Measure of Gobbledygook level was 9.1 ± 1.38. The average Flesch Reading Ease Score was 55 ± 9.1 (range from 32.4 to 72.9). CONCLUSION We demonstrated that web pages describing heart murmurs were understandable and high quality. However, the readability level of the websites was above the recommended sixth-grade reading level. Readability of written materials from online sources need to be improved. However, care must be taken to ensure that the information of web pages is of a high quality and understandable.

Takotsubo cardiomyopathy in children.

Abstract: Objective: To explore the epidemiology and outcomes of takotsubo cardiomyopathy in children. Methods: A retrospective analysis of the Healthcare Cost and Utilization 2012 and 2016 Kids’ Inpatient Database was performed. Patients admitted with the diagnosis of takotsubo cardiomyopathy in the age group of 1 month–20 years were identified using International Classification of Diseases (ICD)-9 code 429.83 and ICD-10 code I51.81. Results: Among a total of 4,860,859 discharges, there were 153 with the diagnosis of takotsubo cardiomyopathy (3.1 per 100,000 discharges). Among patients with takotsubo cardiomyopathy, 55.0% were male, 62.4% were white, and 16.7% were black. Eighty-nine percent of patients were between 12 and 20 years. Psychiatric diagnosis was documented in 46% and substance use disorder in 36.2%. Sepsis was documented in 22.8% of patients. The median length of stay was 5 days (interquartile range: 2.7–15), and median total charges were $75,080 (interquartile range: 32,176–198,336). The overall mortality for takotsubo cardiomyopathy was 7%. On multivariable regression analysis, mortality was higher in the presence of anoxic injury (odds ratio = 34.42, 95% confidence interval: 4.85–320.11, p = 0.00). Conclusions: Takotsubo cardiomyopathy is uncommon in children and carries a mortality rate of 7%. Most children with takotsubo cardiomyopathy are adolescent males, many of whom have psychiatric disorder or substance use disorder or both. Takotsubo cardiomyopathy should be considered in the differential diagnosis for patients who present with cardiac dysfunction and have underlying psychiatric disorders or drug abuse.

Outcomes of paediatric cardiac patients after 30 minutes of cardiopulmonary resuscitation prior to extracorporeal support

Abstract: OBJECTIVES To characterise the mortality and neurological outcomes of paediatric cardiac patients requiring cardiopulmonary resuscitation for more than 30 minutes prior to extracorporeal membrane oxygenation cannulation and to identify risk factors associated with adverse outcomes in this population. MATERIALS AND METHODS Observational retrospective cohort study in paediatric cardiac patients undergoing cardiopulmonary resuscitation for greater than 30 minutes prior to cannulation in a tertiary children's hospital, from July 2000 to July 2013. RESULTS Seventy-three paediatric cardiac patients requiring cardiopulmonary resuscitation for more than 30 minutes prior to cannulation were included in the study. Survival to hospital discharge was 43.8%, with 75% of survivors having either normal neurologic function or only mild disability. Multivariable logistic regression analysis demonstrated that increased use of calcium during resuscitation (odds ratio 14.5, p 0.01), cardiopulmonary resuscitation duration >50 minutes (odds ratio 4.12, p 0.03), >6 interruptions of chest compressions during cannulation (odds ratio 6.40, p 0.03), the need for continuous renal replacement therapy (odds ratio 11.1, p 0.001), and abnormal pupillary response during extracorporeal membrane oxygenation (odds ratio 33.9, p 0.006) were independent predictors for hospital mortality. CONCLUSION Survival after cardiopulmonary resuscitation for more than 30 minutes prior to extracorporeal membrane oxygenation cannulation in our paediatric cardiac cohort was 43.8%. Factors associated with mortality included calcium use during resuscitation, longer cardiopulmonary resuscitation, increased chest compression pauses during cannulation, the use of continuous renal replacement therapy, and abnormal pupils during extracorporeal membrane oxygenation support. A prospective assessment of these factors in paediatric cardiac patients may be beneficial in improving outcomes.

Everolimus for cardiac rhabdomyomas in children with tuberous sclerosis. The ORACLE study protocol (everOlimus for caRdiac rhAbdomyomas in tuberous sCLErosis): a randomised, multicentre, placebo-controlled, double-blind phase II trial

Abstract: Introduction:Tuberous sclerosis complex is a rare genetic disorder leading to the growth of hamartomas in multiple organs, including cardiac rhabdomyomas. Children with symptomatic cardiac rhabdomyoma require frequent admissions to intensive care units, have major complications, namely, arrhythmias, cardiac outflow tract obstruction and heart failure, affecting the quality of life and taking on high healthcare cost. Currently, there is no standard pharmacological treatment for this condition, and the management includes a conservative approach and supportive care. Everolimus has shown positive effects on subependymal giant cell astrocytomas, renal angiomyolipoma and refractory seizures associated with tuberous sclerosis complex. However, evidence supporting efficacy in symptomatic cardiac rhabdomyoma is limited to case reports. The ORACLE trial is the first randomised clinical trial assessing the efficacy of everolimus as a specific therapy for symptomatic cardiac rhabdomyoma.Methods:ORACLE is a phase II, prospective, randomised, placebo-controlled, double-blind, multicentre protocol trial. A total of 40 children with symptomatic cardiac rhabdomyoma secondary to tuberous sclerosis complex will be randomised to receive oral everolimus or placebo for 3 months. The primary outcome is 50% or more reduction in the tumour size related to baseline. As secondary outcomes we include the presence of arrhythmias, pericardial effusion, intracardiac obstruction, adverse events, progression of tumour reduction and effect on heart failure.Conclusions:ORACLE protocol addresses a relevant unmet need in children with tuberous sclerosis complex and cardiac rhabdomyoma. The results of the trial will potentially support the first evidence-based therapy for this condition.

The global unmet need of congenital cardiac care: a quantitative analysis of the global burden of disease.

Abstract: Background: CHDs are one of the most frequent congenital malformations, affecting one in hundred live births. In total, 70% will require treatment in the first year of life, but over 90% of cases in low- and middle-income countries receive no treatment or suboptimal treatment. As a result, CHDs are responsible for 66% of preventable deaths due to congenital malformations in low- and middle-income countries. This study examines the unmet need of congenital cardiac care around the world based on the global burden of disease. Materials and methods: CHD morbidity and mortality data for 2006, 2011, and 2016 were collected from the Institute for Health Metrics and Evaluation Global Burden of Disease Results Tool and analysed longitudinally to assess trends in excess morbidity and mortality. Results: Between 2006 and 2016, a 20.7% reduction in excess disability-adjusted life years and 20.6% reduction in excess deaths due to CHDs were observed for children under 15. In 2016, excess global morbidity and mortality due to CHDs remained high with 14,788,418.7 disability-adjusted life years and 171,761.8 paediatric deaths, respectively. In total, 90.2% of disability-adjusted life years and 91.2% of deaths were considered excess. Conclusion: This study illustrates the unmet need of congenital cardiac care around the world. Progress has been made to reduce morbidity and mortality due to CHDs but remains high and largely treatable around the world. Limited academic attention for global paediatric cardiac care magnifies the lack of progress in this area.

Association for European Paediatric and Congenital Cardiology recommendations for basic training in paediatric and congenital cardiology 2020

Abstract: The recommendations of the Association for European Paediatric and Congenital Cardiology for basic training in paediatric and congenital cardiology required to be recognised as a paediatric cardiologist by the Association for European Paediatric and Congenital Cardiology are described below. Those wishing to achieve more advanced training in particular areas of paediatric cardiology should consult the training recommendations of the different Association for European Paediatric and Congenital Cardiology Working Groups available on the Association for European Paediatric and Congenital Cardiology website (www.aepc.org) and the respective publications 1-6. The development of training requirements is the responsibility of the Educational Committee and the Association for European Paediatric and Congenital Cardiology Council in collaboration with the Working Groups of the Association for European Paediatric and Congenital Cardiology. Trainees should be exposed to all aspects of general paediatric and congenital cardiology from fetal life to adolescence and adulthood. Centres performing generalised and specialised work in paediatric and congenital cardiology should be committed to deliver postgraduate training. At each training institute, trainers should be appointed to supervise and act as mentors to the trainees. Association for European Paediatric and Congenital Cardiology will provide basic teaching courses to supplement the training process.

The Cardiac Neurodevelopmental Outcome Collaborative: a new community improving outcomes for individuals with congenital heart disease - Corrigendum.

Abstract: Improved survival of patients with paediatric and congenital cardiac disease has led to a heightened awareness of the cognitive, neurodevelopmental, psychosocial, and physical functioning deficits that limit health, academic functioning, adaptive functioning, behavioural and emotional outcomes, health-related quality of life, and well-being for children and adults with congenital heart disease. The Cardiac Neurodevelopmental Outcome Collaborative was founded in 2016; its mission is to determine and implement best practices of neurodevelopmental and psychosocial services for individuals with paediatric and congenital heart disease and their families through clinical, quality improvement, and research initiatives.Cardiology in the Young is devoted to cardiovascular issues affecting the young, and older patients with congenital heart disease, or with other cardiac diseases acquired during childhood. The journal serves the interests of all professionals concerned with these topics. By design, the journal is international and multidisciplinary in its approach, and members of the editorial board take an active role in its mission, helping to make it an indispensable reference for paediatric and congenital cardiac care. All aspects of paediatric and congenital cardiac care are covered in the journal. The content includes original articles, brief reports, editorials, reviews, and papers devoted to continuing professional development. High-quality colour figures are published on a regular basis, and without charge to the authors. Regular supplements are published containing the abstracts of the annual meetings of the Association for European Paediatric and Congenital Cardiology, along with other occasional supplements. These supplements are supplied free to subscribers.The vision of Cardiology in the Young is to use print and electronic media to improve paediatric and congenital cardiac care. The mission of Cardiology in the Young is to be a premier global journal for paediatric and congenital cardiac care - an essential journal that spans the domains of patient care, research, education, and advocacy, and also spans geographical, temporal, and subspecialty boundaries.Cardiology in the Young and the Cardiac Neurodevelopment Outcome Collaborative are pleased to announce that Cardiology in the Young will serve as the official journal of the Cardiac Neurodevelopment Outcome Collaborative. This new partnership between the Cardiac Neurodevelopmental Outcome Collaborative and Cardiology in the Young will facilitate the dissemination of knowledge and recommendations to the scientific, clinical, and patient advocacy communities, ultimately improving paediatric and congenital cardiac care and outcomes for individuals with paediatric and congenital heart disease and their families.