Abstract

<h3>Background</h3> Fulminant necrotizing eosinophilic myocarditis (FNEM) is a rare form of eosinophilic myocarditis characterized by new onset heart failure often requiring inotropic and mechanical circulatory support (MCS). Early endomyocardial biopsy (EMB) and prompt immunosuppressive therapy can prevent further decompensation and death, especially in patients presenting with cardiogenic shock. <h3>Case presentation</h3> A 52-year-old female with prior history of mild asthma presented with dyspnea and chest tightness. Vital signs were significant for temperature of 100.9 F, pulse of 140 in sinus rhythm and blood pressure of 89/70. Physical exam was remarkable for crackles at the mid-lower lung fields bilaterally. Baseline EKG showed sinus tachycardia, septal Q waves with ST elevation in V1, V2 and diffuse subtle ST-depressions in the other leads (Figure 1A). Troponin T was elevated to 3.25 ng/ml, pro-BNP was 23,424 pg/ml, CRP was 26.2mg/dl and ESR to 116mm/hour . A 2D echo showed severely reduced left ventricular systolic function with left ventricular ejection fraction (LVEF) of 25-29%, normal left ventricular dimensions, and mildly reduced right ventricular systolic function. Emergent coronary angiography demonstrated no significant coronary artery disease. Right heart catheterization showed a pulmonary artery saturation of 55%, fick cardiac index 1.72, systemic vascular resistance (SVR) of 2128 dynes and elevated bi-ventricular filling pressure (mean right atrial pressure; 23, mean pulmonary capillary wedge pressure; 30mmHg). An endomyocardial biopsy was performed which was consistent with necrotizing eosinophilic myocarditis (Figure 1B). The patient was started on pulse dose steroids. During the first 24 hours she continued to be hemodynamically unstable and required two inotropes to maintain adequate end-organ perfusion. She was placed on VA-ECMO simultaneous with an IABP for left ventricular unloading. Eventually, after being on mechanical support for 7 days, her hemodynamic status improved and her biventricular function completely normalized by day 9. Mechanical and inotropic support was successfully weaned. Patient was discharged on day 23 with a goal to continue a long steroid taper for 6 months. <h3>Conclusion</h3> FNEM is an extremely rare and life threatening pathology that often presents with cardiogenic shock. It is eminently treatable if recognized early during its course. Early EMB should be performed to guide decisions for immunosuppressive therapy. We present a case of FNEM with extremis shock successfully managed with early MCS as bridge-to-recovery.

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