Abstract
Background: Severe myocarditis leading to heart failure is a specific entity complicating the acute phase of Kawasaki disease (KD) in children. Clinical course and response to therapy appear to differ in those children with myocarditis requiring inotropic treatment. Objective: To characterize specific clinical, laboratory and imaging findings in patients with severe myocarditis and delineate their response to therapeutic intervention. Methods: A 3 year retrospective chart review of all patients seen at the HSC (1998-2001) with the diagnosis of KD plus severe myocarditis was performed. The demographic features, clinical, lab and imaging findings and response to treatment were analyzed. Results: 5 patients (3female/ 2male) with a mean age at onset of 3.4years (range 1.5 to 4.4yrs) met diagnostic criteria for KD. In addition all children had significant fatigue, severe tachycardia (mean HR161/min) and hypotension (5/5 <10.perc.). Lab testing revealed elevated ESR (mean 105mm), anemia (mean HGB 112g/l), hypocalcemia (mean ion.Ca 0.99mmol/l) and hypoalbuminemia (mean 26g/l). All children received 2 doses of IVIG (2g/kg/dose). In addition to non-responsiveness to IVIG clinical signs of heart failure were aggravated (tachycardia: mean HR 195/min) supported by diagnostic findings of hemodilution and reduced heart function (anemia: mean HGB 82g/l; ECHO: mean EF 41%, range 18-59%; x-ray bilateral lung effusion). Coronary arteries showed only minor involvement. All patients required positive inotropic support (range 2-5 days). 4 out of 5 patients were treated with high dose methylprednisolone IV-therapy for at least 2 days resulting in rapid clinical improvement. All were maintained on an oral steroid regimen, which was tapered according to the clinical response. Conclusion: The development of severe myocarditis in KD patients leads to specific findings on clinical examination, lab testing and imaging. Multiple interventions with IVIG did not result in the resolution of the clinical features and in fact led to clinical aggravation. These observations suggest a volume-restricted strategy and a consideration of early intervention with high dose corticosteroid.
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Benseler, S., Levy, D., Tse, S. et al. Severe Myocarditis in Children with Kawasaki Disease. Pediatr Res 53, 177 (2003). https://doi.org/10.1203/00006450-200301000-00141
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DOI: https://doi.org/10.1203/00006450-200301000-00141