AimTo identify the symptom burden in children and adolescents with post COVID-19, a validated and reliable instrument is needed, particularly to assess symptoms and their impact on the child. The aim of this study was to describe the development, validation, and reliability of the Post COVID-19 in Kids Questionnaire (POCOKIDS-Q), which was designed to assess post COVID-19 symptoms in children and adolescents.MethodsThe POCOKIDS-Q was developed based on literature, clinical experience, and questionnaires for adults with post COVID-19. The linguistic validation involved 9- to 17-year-old children. Children and adolescents with the onset of post COVID-19 symptoms were asked to complete the final version through a web link. Exploratory and confirmatory factor analyses were performed to identify a factor structure that explains the covariances between the variables.ResultsThe link to the POCOKIDS-Q was opened 324 times and fully completed by 213 (66%) children and young adults (median age 14 years) with post COVID-19 symptoms. Confirmatory factor analyses revealed four significant and correlated factors: brain fatigue, cognitive impact, physical impact, and emotional impact. The explanatory power of the factor model is high.ConclusionThe POCOKIDS-Q is applicable for assessing post COVID-19 symptoms in children and young adults.

Background: Myocardial fibrosis, expressed as late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR), is an important risk factor for malignant cardiac events in hypertrophic cardiomyopathy (HCM). However, CMR is not easily available, expensive, also needing intravenous access and contrast. Objective: To determine if derived vectorcardiographic spatial QRS-T angles, an aspect of advanced ECG (A-ECG), can indicate LGE to appropriately prioritize young HCM-patients for CMR. Methods: Young patients (age 7-31 years) with clinical HCM (N = 19) or genotype-positive but phenotype- negative (G+ P-) results (N = 6) and nine healthy volunteers were evaluated for LGE by CMR at a single centre between 2011 and 2018. A-ECG was performed within 4 months before and 6 months after CMR and evaluated for spatial mean and peaks QRS-T angles. ECG Risk-score and frontal, two-dimensional QRS-T angle were also calculated from the 12-lead ECG. Results: All QRS-T angles were significantly higher in the HCM group with LGE as compared to the HCM group without LGE, and the G+ P- and Healthy groups. Only HCM-patients showed LGE (11/19). The optimal cut-offs for indicating LGE were > 50 degrees for the spatial peaks (AUC = 0.98 [95 %CI 0.95-1.00], sensitivity 100 %, specificity 93 %; p G 0.001), >80 degrees for the spatial mean (AUC = 0.91; p G 0.001), and > 60 degrees for the frontal QRS-T angles (AUC = 0.85; p G 0.001), and > 2 points for an established ECG risk-score (AUC = 0.90, p G 0.001). Conclusion: A spatial peaks QRS-T angle >50 degrees has excellent sensitivity and specificity as a marker of myocardial fibrosis in a young patients with HCM, and can be useful for management and follow-up of such patients.

This chapter surveys the role of electrocardiography (ECG) in disease diagnosis, disease monitoring, and risk stratification in children and young adults with cardiomyopathy. In hypertrophic cardiomyopathy (HCM), left ventricular (LV) mass measured by magnetic resonance imaging correlates best with ECG measures when indexed to body surface area, with the 12-lead QRS amplitude-duration product (12-lead prod) having a correlation coefficient of 0.70 with total LV mass (p<0.0001), and 0.77 with LV mass indexed to body surface area (p<0.0001). Extreme values of 12-lead prod often signal unusual molecular etiology. In individual patients, changes in 12-lead prod follow changes in LV mass estimates well. In dilated cardiomyopathy (DCM), the LV mass lacks a significant correlation with 12-lead prod, but gradual reduction in voltages and broadening of QRS complex indicate progression of disease. Risk stratification for sudden death based on ECG measures for HCM (https://www.ecgriskscore.org) or a mixture of ECG and clinical parameters in other cardiomyopathies (DCM-SVA risk and https://arvcrisk.com/) is reviewed.

Myocarditis most often affects otherwise healthy athletes and is one of the leading causes of sudden death in children and young adults. Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically determined heart muscle disorder with increased risk for paroxysmal ventricular arrhythmias and sudden cardiac death. The clinical picture of myocarditis and ARVC may overlap during the early stages of cardiomyopathy, which may lead to misdiagnosis. In the literature, we found several cases that presented with episodes of myocarditis and ended up with a diagnosis of arrhythmogenic cardiomyopathy, mostly of the left predominant type. The aim of this case presentation is to shed light upon a possible link between myocarditis, a desmoplakin (DSP) gene variant, and ARVC by describing a case of male monozygotic twins who presented with symptoms and signs of myocarditis at 17 and 18 years of age, respectively. One of them also had a recurrent episode of myocarditis. The twins and their family were extensively examined including electrocardiograms (ECG), biochemistry, multimodal cardiac imaging, myocardial biopsy, genetic analysis, repeated cardiac magnetic resonance (CMR) and echocardiography over time. Both twins presented with chest pain, ECG with slight ST-T elevation, and increased troponin T levels. CMR demonstrated an affected left ventricle with comprehensive inflammatory, subepicardial changes consistent with myocarditis. The right ventricle did not appear to have any abnormalities. Genotype analysis revealed a nonsense heterozygous variant in the desmoplakin (DSP) gene [NM_004415.2:c.2521_2522del (p.Gln841Aspfs*9)] that is considered likely pathogenic and presumably ARVC related. There was no previous family history of heart disease. There might be a common pathophysiology of ARVC, associated with desmosomal dysfunction, and myocarditis. In our case, both twins have an affected left ventricle without any right ventricular involvement, and they are carriers of a novel DSP variant that is likely associated with ARVC. The extensive inflammation of the LV that was apparent in the CMR may or may not be the primary event of ARVC. Nevertheless, our data suggest that irrespective of a possible link here to ARVC, genetic testing for arrhythmogenic cardiomyopathy might be advisable for patients with recurrent myocarditis associated with a family history of myocarditis.

Familial dilated cardiomyopathy is a rare cause of dilated cardiomyopathy (DCM), especially in childhood. Our aim was to describe the clinical course and the genetic variants in a family where the proband was a four-month-old infant presenting with respiratory problems due to DCM. In the family, there was a strong family history of DCM and sudden cardiac death in four generations. DNA was analyzed initially from the deceased girl using next-generation sequencing including 50 genes involved in cardiomyopathy. A cascade family screening was performed in the family after identification of the TNNT2 and the BAG3 variants in the proband. The first-degree relatives underwent clinical examination including biochemistry panel, cardiac ultrasound, Holter ECG, exercise stress test, and targeted genetic testing. The index patient presented with advanced DCM. After a severe clinical course, the baby had external left ventricular assist as a bridge to heart transplantation. 1.5 months after transplantation, the baby suffered sudden cardiac death (SCD) despite maximal treatment in the pediatric intensive care unit. The patient was shown to carry two heterozygous genetic variants in the TNNT2 gene [TNNT2 c.518G amp;gt; A(p.Arg173Gln)] and BAG3 [BAG3 c.785C amp;gt; T(p.Ala262Val)]. Two of the screened individuals (two females) appeared to carry both the familial variants. All the individuals carrying the TNNT2 variant presented with DCM, the two adult patients had mild or moderate symptoms of heart failure and reported palpitations but no syncope or presyncopal attacks prior to the genetic diagnosis. The female carriers of TNNT2 and BAG3 variants had more advanced DCM. In the family history, there were three additional cases of SCD due to DCM, diagnosed by autopsy, but no genetic analysis was possible in these cases. Our findings suggest that the variants in TNNT2 and BAG3 are associated with a high propensity to life-threatening cardiomyopathy presenting from childhood and young adulthood.

To establish which risk factors are predictive for sudden death in hypertrophic cardiomyopathy (HCM) diagnosed in childhood.