Myocarditis remains a clinical challenge in pediatrics. Originally, it was recognized at autopsy before the application of endomyocardial biopsy, which led to a histopathology-based diagnosis such as in the Dallas criteria. Given the invasive and low-sensitivity nature of endomyocardial biopsy, its diagnostic focus shifted to a reliance on clinical suspicion. With the advances of cardiac magnetic resonance, an examination of the whole heart in vivo has gained acceptance in the pursuit of a diagnosis of myocarditis. The presentation may vary from minimal symptoms to heart failure, life-threatening arrhythmias, or cardiogenic shock. Outcomes span full resolution to chronic heart failure and the need for heart transplantation with inadequate clues to predict the disease trajectory. The American Heart Association commissioned this writing group to explore the current knowledge and management within the field of pediatric myocarditis. This statement highlights advances in our understanding of the immunopathogenesis, new and shifting dominant pathogeneses, modern laboratory testing, and use of mechanical circulatory support, with a special emphasis on innovations in cardiac magnetic resonance imaging. Despite these strides forward, we struggle without a universally accepted definition of myocarditis, which impedes progress in disease-targeted therapy.

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The objective of this study was to describe a contemporary cohort of pediatric patients hospitalized for clinically suspected myocarditis. A retrospective chart review was performed at seven tertiary pediatric hospitals. Electronic medical records were searched between 2008 and 2012 for patients ≤18 years admitted with an ICD-9 code consistent with myocarditis. Patients were excluded if the admitting or consulting cardiologist did not suspect myocarditis during the admission or an alternative diagnosis was determined. One hundred seventy-one patients were discharged or died with a primary diagnosis of myocarditis. Median age was 13.1 years (IQR 2.1, 15.9), with a bimodal distribution; 24% <2 years and 46% between 13 and 18 years. Patients with moderate or severe systolic dysfunction were younger, had higher BNPs at admission, but had lower troponin. Mortality, heart transplantation, and readmission did not differ between patients who received only IVIG, only steroids, IVIG and steroids, and no immunotherapy. Ninety-four patients (55%) were discharged on heart failure medications, 16 were transplanted, and seven died. The presence at the time of admission of gastrointestinal (GI) symptoms (p = 0.01) and lower echo shortening fraction (SF) (p < 0.01) was associated with death/transplant. Within one year 16% had a readmission, one underwent heart transplant, and 39% received heart failure therapy. Pediatric myocarditis has a bimodal age distribution. The use of IVIG and steroids is not associated with mortality/heart transplantation. The presence of GI symptoms and lower echo SF may identify patients at risk for death and/or transplantation during the admission.

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