SARS-CoV-2 Associated Pediatric Inflammatory Multisystem Syndrome With a High Prevalence of Myocarditis - A Multicenter Evaluation of Clinical and Laboratory Characteristics, Treatment and Outcome.

Introduction: Pediatric inflammatory multisystem syndrome - temporally associated with SARS-CoV-2 infection (PIMS -TS) comprises a new disease entity having emerged after the COVID-19 outbreak in 2019. Materials and Methods: For this multicenter, retrospective study children between 0 and 18 years with PIMS-TS between March 2020 and May 2021 were included, before availability of vaccination for children. Frequent SARS-CoV-2 variants at that period were the wildtype virus, alpha, beta and delta variants. Inclusion criteria were according to the PIMS-TS criteria, proposed by the Royal College of Pediatrics and WHO. Study aim was to review their clinical, laboratory and echocardiographic data with a focus on cardiac involvement. Results: We report 45 patients, median age 9 years, 64% male. SARS-CoV-2 antibodies were positive in 35/41 (85%). PIMS occurrence followed local COVID-19 peak incidence periods with a time lag. The most common symptoms at presentation were fever (98%), abdominal pain (89%) and rash (80%). Fever history of > 5 days was associated with decreased left ventricular function (p = 0.056). Arterial hypotension and cardiac dysfunction were documented in 72% patients, increased brain natriuretic peptide in 96% and increased cardiac troponin in 64% of the children. Echocardiography revealed mitral valve regurgitation (64%), coronary abnormalities (36%) and pericardial effusions (40%). Increased NT-proBNP was significantly associated with the need of inotropics (p < 0.05), which were necessary in 40% of the patients. Treatment comprised intravenous immunoglobulin (93%), systemic steroids (84%) and acetylsalicylic acid (100%; 26/45 started with high dosages). For insufficient response to this treatment, five (11%) children received the interleukin-1 receptor antagonist anakinra. All patients were discharged with almost resolved cardiac signs. Conclusion: Our analysis of non-vaccinated children with PIMS-TS demonstrates that a considerable number have associated myocarditis requiring intensive care and inotropic support. Most children showed adequate response to intravenous immunoglobulin and steroids and good recovery. Further evaluation of pediatric patients with COVID-19 associated diseases is required to evaluate the impact of new virus variants.

Recommendations for participation in competitive sport in adolescent and adult athletes with Congenital Heart Disease (CHD): position statement of the Sports Cardiology & Exercise Section of the European Association of Preventive Cardiology (EAPC), the European Society of Cardiology (ESC) Working Group on Adult Congenital Heart Disease and the Sports Cardiology, Physical Activity and Prevention Working Group of the Association for European Paediatric and Congenital Cardiology (AEPC).

Improved clinical care has led to an increase in the number of adults with congenital heart disease (CHD) engaging in leisure time and competitive sports activities. Although the benefits of exercise in patients with CHD are well established, there is a low but appreciable risk of exercise-related complications. Published exercise recommendations for individuals with CHD are predominantly centred on anatomic lesions, hampering an individualized approach to exercise advice in this heterogeneous population. This document presents an update of the recommendations for competitive sports participation in athletes with cardiovascular disease published by the Sports Cardiology & Exercise section of the European Association of Preventive Cardiology (EAPC) in 2005. It introduces an approach which is based on the assessment of haemodynamic, electrophysiological and functional parameters, rather than anatomic lesions. The recommendations provide a comprehensive assessment algorithm which allows for patient-specific assessment and risk stratification of athletes with CHD who wish to participate in competitive sports.

Cardiovascular pre-participation screening in young athletes: Recommendations of the Association of European Paediatric Cardiology.

Sudden death in young competitive athletes can be avoided by implementation of pre-participation screening programmes. A screening programme should be performed only by trained physicians and should include the athlete's personal and family history, physical examination results, and the readings from a 12-lead-electrocardiogram. The athlete should undergo this screening programme every second year to detect progressive diseases. In addition, the programme should include detailed instructions to the athletes to pause training during infections in order to prevent sudden death due to myocarditis.

Preventing cardiac diseases in childhood.

OBJECTIVE: The burden of cardiac disease in childhood is unknown. It will be a sum of 1% of living births in the general population, suffering from Congenital Heart Disease (CHD) + approximately 2.5% of the general population suffering from bicuspid aortic valve diseases + an unknown higher prevalence of acquired diseases. Cardiomyopathies, arrhythmias - sudden cardiac death (SCD), rheumatic heard disease, hypertension and accelerating atherosclerosis are among the most frequent. Adding on, genetic syndromes including cardiac defects, endocarditis and myocarditis we can address a large pediatric population worldwide, suffering from heart disease. Diagnosis and treatment of these diseases are not afforded in many countries worldwide due to luck of human and material resources. The aim of this paper is to describe how some of the above mentioned diseases can be either early detected or prevented. The working Group "Cardiovascular Prevention" of the Association of European Pediatric and Congenital Cardiology (AEPC) focused on some forms of them since its formation in 2011. These areas are: 1) some forms of critical CHD, 2) sudden cardiac death linked to sport activities and 3) detecting- preventing cardio vascular diseases CVD in the young. Methods-Populations: Measurements of pre and post ductal saturation of oxygen using pulse oximeters, after the first day from birth, can early and cheaply detect critical Ductal Arteriosus dependent pulmonary or systemic and cyanotic CHD, saving lives and decreasing significantly the cost of medical care. This screening test can be applied to all neonates as late as possible after their birth and before released to their homes. A combination of detailed medical history, physical examination and 12 lead ECG, during a pre-participation in sport activities medical screening test can prevent SCD, related to a variety of nosology. This combined screening test can be applied to all children before they are exposed to school or leisure sport activities. Screening to early detect and treating existent risk factors (RF) for CVD as well as preventing obesity and hypertension, contributes in lowering the burden of CVD. Specific screening tests as laboratory measurements of lipids, fasting glucose or regular measurements of Blood Pressure and waist to hip ratio in children with a family history of CVD or other co-morbidity that provokes accelerating atherosclerosis must be done on a regular basis. RESULTS: Since 2010, four European studies reporting the test accuracy of routine pulse oximetry screening, in over 150.000 babies, have delivered new data. A systematic review and meta-analysis of 230.000 screened babies, reported high specificity, moderate sensitivity and a low false-positive rate. Routine screening for critical CHD using pulse oximetry is being increasingly supported and was added to the recommended uniform screening panel in the USA in 2011. Evaluating children with CHD before their involvement in sport activities, so a clear view in what they can and what they can't to is vital for their safety. For children involved in competitive or leisure sport activities, an initial evaluation and a yearly F/U is vital. In cases of near SCD events an additional thorough investigation and appropriate management is required. Investigating the severity of the existing RF and cooperating with Pediatricians in their treatment (e.g. heredity forms of hyperlipidemias, existing hypertension) of them is essential. Furthermore preventing acceleration of atherosclerosis in patients with: Diabetes Mellitus I, II, chronic renal disease, post Kawasaki disease, post heart transplantation patients, Cardio-Metabolic Syndrome patients, by eradicating RF primordially or by alternating them by opposing a healthy life style or by medicine treatment, has sown in many studies to post pone clinical events in adulthood. DISCUSSION: As many studies have proved the role of preventive measures that can alternate the outcome of cardiac diseases in childhood. AEPC/Preventive Cardiology working group is in the process to publish in the near future guidelines on this topic.

Intima media thickness measurement in children: A statement from the Association for European Paediatric Cardiology (AEPC) Working Group on Cardiovascular Prevention endorsed by the Association for European Paediatric Cardiology.

Atherosclerosis causing cardiovascular disease is the most common cause of death in the developed world. Early precursors of vascular changes - subclinical atherosclerosis - warrant special attention as this process can be stabilized or even reversed if treated in time. Sonographic Intima Media Thickness measurement of the carotid artery (cIMT: carotid Intima-Media-Thickness) is considered a valid surrogate marker for cardiovascular risk allowing assessment of atherosclerotic changes at a very early stage. It is easy to apply due to its non-invasive character. Moreover, cIMT has been proven to provide reliable and reproducible results both in adult and adolescent patients. For the paediatric age group, several characteristics deserve special consideration. The heterogeneity of techniques of scanning, measurement and interpretation impede the comparison and interpretation of IMT values so far. Also, age- and sex-dependent normative data have to be considered for interpretation. Thus, the Association for European Paediatric Cardiology (AEPC) Working Group on Cardiovascular Prevention concludes to refer a statement on cIMT scanning, measurement and interpretation with special focus on paediatric patients. This statement includes an overview on normative data available as well as a practical guideline for the setting, scanning, measurement and interpretation of IMT values. Synchronizing different measurement methods will allow for comparing the results of several research centers. By that, in a large patient number, sufficient information may be given to assess the long-term endpoints of cardiovascular morbidity and mortality.