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Acute Eosinophilic Myocarditis And The Idiopathic Hypereosinophilic Syndrome

L.C. Illig, M.D. University of Wisconsin Medical School, Madison, WI

Case: A 61 year old woman with newly diagnosed hypereosinophilic syndrome (HES) and known skin and lung involvement presented to the emergency department complaining of severe chest pain. She had electrocardiogram changes suggestive of a possible inferior myocardial infarction and troponin-I of 93.4. She had normal coronary arteries on catheterization, but a new global hypokinesis with an ejection fraction of 35% and new mitral regurgitation of echocardiogram. On right ventricular biopsy, the myocardium was infiltrated with eosinophils and lymphocytes and had extensive necrosis. The patient had a difficult clinical course with profound hemodynamic instability and recalcitrant atrial and ventricular tachyarrythmias requiring defibrillation, balloon pump support, antiarrhythmic therapy, pacing and inotropic support. She had a long recovery and was discharged to home.

Discussion: Idiopathic HES has three defining features: first, the patient must have sustained blood eosinophilia of greater than 1,500/µL present for longer than six months; second, other etiologies for eosinophilia must be explored and excluded; lastly, the patient must have evidence of organ involvement. The cardiovascular system is the organ system most frequently involved and is the cause of significant morbidity and mortality. Myocardial damage in HES is generally insidious and progresses through three stages: an acute necrotic stage, a thrombotic stage, and finally a fibrotic stage. Most commonly, the early necrotic stage is unrecognized clinically and patients present in the later thrombotic and fibrotic stages. There have been, however, a number of case reports of patients presenting with acute eosinophilic myocarditis as a feature of HES. This case illustrates that a patient with fulminant eosinophilic myocarditis can survive with a good clinical outcome.

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