Abstract 14357: Straight for the Jugular: Cardiomyopathy After Recent EBV Infection and Lemierre Syndrome

Abstract

A 21-year-old man presented to an outside hospital with 10 days of fever, fatigue, and malaise. On exam, temperature was 106F, blood pressure 85/50 mm Hg, and heart rate 121 bpm. He was cold and clammy, with elevated jugular venous pressure, normal heart sounds, clear lungs, and cool extremities without edema. Laboratory results were notable for elevated white blood cell count (predominantly neutrophils), serum triglycerides, ferritin, C-reactive protein, coagulation parameters, and positive Monospot test. Following blood cultures, he was initiated on broad spectrum antibiotics, fluid resuscitated, and started on norepinephrine, epinephrine, and phenylepehrine. Shortly after transfer to our institution, although asymptomatic, an electrocardiogram demonstrated diffuse 5 mm ST-elevations with elevated cardiac biomarkers. A transthoracic echocardiogram demonstrated biventricular contractile dysfunction with ejection fraction 20% and apical akinesis. The patient was diagnosed with mixed cardiogenic and distributive shock. Cardiogenic shock was attributed to myocarditis with possible vasospasm or cytokine-induced myocardial suppression. Coronary angiogram was deferred given low suspicion for thrombotic acute coronary syndrome. Cardiac MRI showed subepicardial early and late gadolinium enhancement consistent with myocarditis, but negative viral serologies. Distributive shock was thought to be due to sepsis or hemophagocytic lymphohistiocytosis (HLH). The patient received empiric dexamethasone for HLH; and the diagnosis was confirmed on bone marrow biopsy. Blood cultures grew Fusobacterium necrophorum and neck imaging revealed thrombosis of the left internal jugular vein. Lemierre syndrome, thought to be bacterial invasion in the setting of tonsillitis was diagnosed. He was treated with antibiotics and dexamethasone with rapid clinical improvement and recovery of biventricular function. We suspect EBV infection lead to Lemierre syndrome and HLH with secondary myocardial suppression from the resultant cytokine storm. This case describes the first case of HLH-related myocarditis and highlights clinical decision-making surrounding the workup and management of mixed etiologies of shock with suspected myocarditis.