Cardiac disease occurs in more than 50% of patients with idiopathic hypereosinophilic syndrome; it is the major cause of morbidity and mortality in patients with this syndrome. The overall mortality is high if untreated. We aim to rapport recent experience in these cardiac diseases, especiall echographic features and the contribution of multimodalities imaging.

This retrospective, descriptive and multicenter study reports the findings of patients in cardiac centers of Marseille, Montpellier and Rennes between September 2008 and March 2014. Echographic, MRI cardiac, coronarographic, PET CT, biology and histologic findings are reported. Seven patients have been reported. Echocardiography is the reference diagnostic method. Classical findings are progressive endomyocardial thickening, apical obliteration of one or both ventricles by echogenic material suggestive of fibrosis or thrombus formation, posterior mitral leaflet involvement and papillary dysfunction resulting in severe mitral regurgitation. These features are involved in embolic events or heart failure. MRI helps refine the diagnosis. The characteristic irreversible stage shows a circumferential thickened fibrotic sub endocardium with wall thrombi. The CT identifies non ischemic thrombus excluding coronary artery disease by coronary imaging. Coronary angiography may reveal fibrosis by the smoothed appearance of the ventricular walls and apical filling. PET CT shows general signs of myocarditis, such as myocyte necrosis, in the first stage of the disease.

Symptomatic treatment can lead to cardiac surgery (mitral valve replacement, resection of endomyocardial fibrosis). Each imaging technique plays a role in each of the three stages of the disease and the typical features of myocarditis, thrombus or fibrosis must be identified. Only seven cases of this rare disease have been identified in three french cardiac centers since 2008; this too small sample does not allow us to draw statistical conclusions.