Neonatal fulminant myocarditis mimicking inborn errors of metabolism

Abstract

Background and aims: Neonatal fulminant myocarditis is a rare but often fatal disease. Since the clinical presentation of myocarditis in neonate is often non-specific, it is diffICUlt to diagnose in a timely manner. We report a case of 10-day-old female with suspected inborn errors of metabolism that was ultimately diagnosed with enterovirous-associated fulminant myocarditis by autopsy. Aims: α Methods: case report. Results: This was a previously healthy 10-day-old female who presented to the Emergency Room in the outside hospital with a sudden onset of lethargy and agonal breathing. She was immediately intubated and given inotropic support. Echocardiography showed severely depressed cardiac function without anatomical abnormalities. Laboratory results showed severe metabolic acidosis, hyperammonemia, hypoglycemia and coagulopathy. She was transported to our pediatric intensive care unit for further management. Our initial differential diagnoses were inborn errors of metabolism, mitochondria disease or neonatal acute hepatic failure. Continuous veno-venous hemofiltration (CVVH) was commenced. Although severe metabolic acidosis was corrected, her cardiac function was never improved. Within 36 hrs, she developed multi-organ failure, resulting in her death. Autopsy revealed lymphocyte infiltration and extensive fibrosis in the myocardium. Enterovirus was recovered from the myocardium subsequently. Conclusions: Neonatal fulminant myocarditis can be quite diffICUlt to diagnose as its presentation is often nonspecific and mimics diseases such as ductal shock, septic shock or inborn errors of metabolism. It has a very high mortality without prompt recognition and interventions. ECMO should be considered early in the stage in neonates with rapid hemodynamic deterioration that is refractory to inotropic supports, regardless of the etiology.