Abstract

Objective: To investigate the clinical features and the diagnostic and follow-up value of acute fulminant myocarditis (AFM) in children.Methods: A total of 20 children diagnosed with AFM admitted to our department were reviewed, and the clinical manifestations; pathogenic examination results; myocardial injury biomarkers; and electrocardiography, echocardiogram, and cardiovascular magnetic resonance (CMR) results were analyzed.Results: Twenty children with AFM, including 12 males and 8 females, aged 3–16 years, were analyzed. The initial symptoms were abdominal pain, vomiting, fatigue, syncope, and convulsions. All children had significantly increased hs-cTnT and NT-pro BNP. In addition to nonspecific ST-T changes, there were 10 cases of complete atrioventricular block, 2 cases of advanced atrioventricular block, and 1 case of ventricular tachycardia. Echocardiography showed an increase in the cardiac chamber sizes in 15 patients and a decrease in left ventricular ejection fraction (LVEF) in 17 patients. There were 16 patients with abnormal CMR findings, including 13 cases of high T2-weighted image (T2WI) signal and 14 cases of late gadolinium enhancement (LGE). In the patients who underwent CMR within 14 days of onset, the sensitivity of T2WI and LGE and the positive diagnosis rate were higher than in those who underwent CMR after 14 days, but the difference was not statistically significant. CMR was followed up in 10 patients: 7 patients returned to normal, 2 patients still had mild LGE, and 1 patient developed inflammatory dilated cardiomyopathy. All patients were treated with high-dose immunoglobulin, 11 of whom received high-dose immunoglobulin combined with glucocorticoids. Eight patients received temporary pacemakers, and 1 patient received ECMO. None of the patients died. The peak of hs-cTnT was significantly higher in the glucocorticoid group than in the unused glucocorticoid group (2853.4 ± 2217.2 and 1124.7 ± 527.3 pg/ml, respectively).Conclusion: Children with AFM have unique clinical features. Early identification and effective treatment can reduce the mortality rate and improve the prognosis. CMR is highly sensitive in the diagnosis of ARM, especially within 14 days of onset, and is a useful noninvasive imaging technique for the early identification of AFM in children. The dynamic observation and follow-up of children with AFM through CMR can guide clinical decision-making and prognosis assessment.

Highlights

  • Acute fulminant myocarditis (AFM) in children has a rapid onset and progresses rapidly

  • late gadolinium enhancement (LGE) was found in 9 patients (75%), including in the ventricular septum in 2 patients, in the left ventricle in 5 patients, and in both the ventricular septum and left ventricle in 2 patients

  • The gold standard for AFM diagnosis is still endomyocardial biopsy (EMB), but because of its invasiveness and low sensitivity, it is not used as a firstline examination, especially for children [4, 6]

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Summary

Introduction

Acute fulminant myocarditis (AFM) in children has a rapid onset and progresses rapidly. AFM is a clinically critical pediatric illness with a high mortality rate. A recent national survey in Japan found that the survival rate of children with AFM was only 48.6% [1]. The key to affecting the mortality rate is related to early identification and effective treatment. Cardiovascular magnetic resonance (CMR) has received increasing attention in the diagnosis of children with AFM. It has been reported in the literature that the positive predictive value of CMR for acute myocarditis is over 90%. Our early studies have shown that CMR is more sensitive than AFM in the diagnosis of conventional myocarditis. This study retrospectively analyzed the clinical data and CMR findings of 20 children with AFM and explored the clinical features of AFM in children and the value of CMR in diagnosis, treatment, and prognosis to provide valuable and important information for the diagnosis and treatment of this disease

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