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This article discusses a study conducted on pediatric patients with myocarditis, a condition that ranges from asymptomatic to severe heart failure. The study aimed to evaluate the occurrence and severity of arrhythmias in these patients. The results showed that arrhythmias were common in pediatric myocarditis patients, regardless of age and sex. Patients with arrhythmias had a lower left ventricular ejection fraction and were more likely to experience major cardiac events. The study emphasizes the importance of rhythm monitoring in patients with myocarditis. [Extracted from the article]

Myocarditis is a major cause of severe heart failure in pediatric patients. In some patients, this may lead to the need of mechanical circulatory support (MCS) or heart transplantation. MYKKE is a multicenter prospective registry of pediatric patients with suspected myocarditis. We aimed to determine characteristics and outcome of patients with severe heart failure requiring MCS and/or heart transplantation within the MYKKE registry in order to get a better understanding of this critically ill subgroup. Between September 2013 and October 2022, patients within the MYKKE registry were analyzed. Requirement of MCS including Extracorporeal Membrane Oxygenation (ECMO) and Ventricular Assist Device (VAD), heart transplantation and/or occurrence of death defined a sub-cohort of special interest. A total amount of 649 patients was enrolled by 26 medical centers. Median age was 14.3 years, 67% were male. Thirteen percent within this population (n=84/649) received MCS (3 % ECMO; 6 % VAD; 4 % both, ECMO and VAD). Thirty-nine patients (6 %) needed a heart transplant and 33 patients (5 %) died. The subgroup of MCS, heart transplant and dead patients (17 %, n=108) presented with a young median age of 2 years, 55 % were male. Left ventricular dysfunction at initial admission was represented by a median LVEF of 21 %. Out of 63 patients requiring VAD, 17 could be successfully weaned (27 %) after a median time of 97 days. Thirty-one patients were MCS-bridged to transplantation (37 %, n=31/84). Within our subgroup 79 % of patients (n = 85/108) underwent endomyocardial biopsy and in 64 cases diagnosis of myocarditis was confirmed (28 % acute myocarditis, 64 % chronic/healing myocarditis, 8 % healed), further diagnoses included dilated cardiomyopathy (n=9) or others (n=12). Myocarditis represents a life-threatening condition in pediatric patients. Especially young patients are affected by a severe clinical course, which requires MCS and transplantation due to end-stage heart failure. Different underlying pathomechanisms may contribute in a possibly age-dependent manner as genetic predispositions and/or immunological factors. [ABSTRACT FROM AUTHOR]

COVID-19 is a heterogenous infection-asymptomatic to fatal. While the course of pediatric COVID-19 infections is usually mild or even asymptomatic, individuals after adult heart transplantation are at high risk of a severe infection. We conducted a retrospective, multicenter survey of 16 pediatric heart transplant centers in Germany, Austria and Switzerland to evaluate the risk of a severe COVID-19 infection after pediatric heart transplantation between 02/2020 and 06/2021. Twenty-six subjects (11 male) with a median age of 9.77 years at time of transplantation and a median of 4.65 years after transplantation suffered from COVID-19 infection. The median age at time of COVID-10 infection was 17.20 years. Fourteen subjects had an asymptomatic COVID-19 infection. The most frequent symptoms were myalgia/fatigue (n = 6), cough (n = 5), rhinitis (n = 5), and loss of taste (n = 5). Only one subject showed dyspnea. Eleven individuals needed therapy in an outpatient setting, four subjects were hospitalized. One person needed oxygen supply, none of the subjects needed non-invasive or invasive mechanical ventilation. No specific signs for graft dysfunction were found by non-invasive testing. In pediatric heart transplant subjects, COVID-19 infection was mostly asymptomatic or mild. There were no SARS-CoV-2 associated myocardial dysfunction in heart transplant individuals., (© 2024 The Authors. Clinical Transplantation published by John Wiley & Sons Ltd.)

Background: Since the onset of widespread COVID-19 vaccination, increased incidence of COVID-19 vaccine-associated myocarditis (VA-myocarditis) has been noted, particularly in male adolescents., Methods: Patients <18 years with suspected myocarditis following COVID-19 vaccination within 21 days were enrolled in the PedMYCVAC cohort, a substudy within the prospective multicenter registry for pediatric myocarditis "MYKKE." Clinical data at initial admission, 3- and 9-months follow-up were monitored and compared to pediatric patients with confirmed non-vaccine-associated myocarditis (NVA-myocarditis) adjusting for various baseline characteristics., Results: From July 2021 to December 2022, 56 patients with VA-myocarditis across 15 centers were enrolled (median age 16.3 years, 91% male). Initially, 11 patients (20%) had mildly reduced left ventricular ejection fraction (LVEF; 45%-54%). No incidents of severe heart failure, transplantation or death were observed. Of 49 patients at 3-months follow-up (median (IQR) 94 (63-118) days), residual symptoms were registered in 14 patients (29%), most commonly atypical intermittent chest pain and fatigue. Diagnostic abnormalities remained in 23 patients (47%). Of 21 patients at 9-months follow-up (259 (218-319) days), all were free of symptoms and diagnostic abnormalities remained in 9 patients (43%). These residuals were mostly residual late gadolinium enhancement in magnetic resonance imaging. Patients with NVA-myocarditis (n=108) more often had symptoms of heart failure (P = .003), arrhythmias (P = .031), left ventricular dilatation (P = .045), lower LVEF (P < .001) and major cardiac adverse events (P = .102)., Conclusions: Course of COVID-19 vaccine-associated myocarditis in pediatric patients seems to be mild and differs from non-vaccine-associated myocarditis. Due to a considerable number of residual symptoms and diagnostic abnormalities at follow-up, further studies are needed to define its long-term implications., Competing Interests: Disclosures None reported., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Primary cardiac tumors in children are very rare and may be associated with severe arrhythmias and sudden infant death syndrome. These cardiac arrhythmias vary depending on the location and size of the tumor. Sixty-four percent of children with cardiac fibroma, the second most common benign cardiac tumor in children, have ventricular arrhythmias, affecting therapeutic management and risk profile of these children. We report on two siblings with cardiac fibromas whose clinical presentations differed depending on their locations and size of the tumors. The first child, a three-year-old girl, was diagnosed with a cardiac fibroma in the left ventricle at the age of 8 months after surviving resuscitation due to ventricular fibrillation. Secondary prophylactic implantation of an ICD was performed. On propranolol, no further malignant arrhythmias have occurred to date. The seven-month-old brother was diagnosed postnatally with a cardiac tumor adjacent to the right ventricle. A few weeks after birth, the boy had refractory supraventricular tachycardia and ventricular arrhythmia that only resolved with amiodarone. In genetic testing, Gorlin-Goltz syndrome was diagnosed in both children. Conservative pharmacological therapy is a therapeutic strategy for asymptomatic patients with cardiac fibromas. The anti-arrhythmic medication depends on the location of the tumor. Implantation of an ICD should be performed in cases of malignant arrhythmias. In rare cases, there is an association between cardiac tumors and genetic syndromes, such as Gorlin-Goltz syndrome. These should always be considered when such a tumor is diagnosed., (© 2023. The Author(s).)

Background: Heart failure (HF) due to myocarditis might not respond in the same way to standard therapy as HF due to other aetiologies. The aim of this study was to investigate the value of endomyocardial biopsies (EMB) for clinical decision-making and its relation to the outcome of paediatric patients with myocarditis., Methods: Clinical and EMB data of children with myocarditis collected for the MYKKE-registry between 2013 and 2020 from 23 centres were analysed. EMB studies included histology, immunohistology, and molecular pathology. The occurrence of major adverse cardiac events (MACE) including mechanical circulatory support (MCS), heart transplantation, and/or death was defined as a combined endpoint., Results: Myocarditis was diagnosed in 209/260 patients: 64% healing/chronic lymphocytic myocarditis, 23% acute lymphocytic myocarditis (AM), 14% healed myocarditis, no giant cell myocarditis. The median age was 12.8 (1.4-15.9) years. Time from symptom-onset to EMB was 11.0 (4.0-29.0) days. Children with AM and high amounts of mononuclear cell infiltrates were significantly younger with signs of HF compared to those with healing/chronic or healed myocarditis. Myocardial viral DNA/RNA detection had no significant effect on outcome. The worst event-free survival was seen in patients with healing/chronic myocarditis (24%), followed by acute (31%) and healed myocarditis (58%, p = 0.294). A weaning rate of 64% from MCS was found in AM., Conclusions: EMB provides important information on the type and stage of myocardial inflammation and supports further decision-making. Children with fulminant clinical presentation, high amounts of mononuclear cell infiltrates or healing/chronic inflammation and young age have the highest risk for MACE., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Partial detachment of the septal and anterior leaflets of the tricuspid valve (TV) is a technique to visualize a perimembranous ventricular septal defect (VSD) for surgical closure in cases where the VSD is obscured by TV tissue. However, TV incision bears the risk of causing relevant postoperative TV regurgitation and higher degree atrioventricular (AV) block. A total of 40 patients were identified retrospectively in our institution who underwent isolated VSD closure between January 2013 and August 2015. Visualization of the VSD was achieved in 20 patients without and in 20 patients with additional partial detachment of the TV. The mean age of patients with partial tricuspid valve detachment (TVD) was 0.7 ± 0.1 years compared with 1 ± 0.3 years ( p  = 0.22) of patients without TVD. There was no difference in cardiopulmonary bypass time between patients of both groups (123 ± 11 vs. 103 ± 5 minutes, p  = 0.1). Cross-clamp time was longer if the TV was detached (69 ± 5 vs. 54 ± 4 minutes, p  = 0.023). There was no perioperative mortality. Echocardiography at discharge and after 2.5 years (2 months-6 years) of follow-up showed neither a postoperative increase of tricuspid regurgitation nor any relevant residual shunt. Postoperative electrocardiograms were normal without any sign of higher degree AV block. TVD offers enhanced exposure and safe treatment of VSDs. It did not result in higher rates of TV regurgitation or relevant AV block compared with the control group., Competing Interests: None declared., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

A 16-year-old girl with history of treated congenital mitral valve disease and signs of respiratory infection was admitted to our paediatric cardiology department. She was tested positive for severe acute respiratory syndrome coronavirus 2. Despite her severe pre-existing cardiac conditions with pulmonary hypertension, atrial arrhythmias and mitral valve stenosis, the infection did not lead to any cardiac or pulmonary deterioration. In adults, cardiac co-morbidities are known risk factors for a severe course of coronavirus disease 2019 infections. This case illustrates that in children even severe cardiac disease is not necessarily associated with a severe course of coronavirus disease 2019.