Endomyocardial fibrosis complicating primary hypereosinophilic syndrome

Excerpt

A 65-year-old woman was admitted for refractory acute pulmonary oedema requiring mechanical ventilation. Biological analysis showed hypereosinophilia (2300/mm³), which was detected more than 6 months previously. Transthoracic echocardiography showed obliteration of the left ventricular cavity with material whose echodensity was distinct from that of myocardium (Fig. 1a, video 1). Left ventricular systolic function was preserved but diastolic function was impaired. Bedside contrast echocardiography confirmed material with contrast enhancement that was distinct from that of the myocardium. Absence of contrast in this material revealed areas of suspected thrombi (Fig. 1b, video 2). This technique was able to distinguish this material with hypertrophic cardiomyopathy whose contrast is not distinct from that of the myocardium. All these findings were suggestive of endomyocardial fibrosis (EMF) caused by hypereosinophilia. Cardiovascular magnetic resonance confirmed the presence of EMF associated with thrombi (Fig. 1c). Urgent surgical decortication was performed (Fig. 1d, video 3). The patient was weaned from mechanical ventilation and discharged from hospital. The pathology confirmed the presence of fibrosis with mural thrombi (Fig. 1e).

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