Abstract

Hypereosinophilic syndrome (HES) is a rare disorder characterized by a marked and persistent peripheral blood eosinophilia combined with organ-system dysfunction caused by eosinophil-associated tissue damage [ 1 Chusid M.J. Dale D.C. West B.C. Wolff S.M. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore). 1975; 54: 1-27 Crossref PubMed Scopus (1183) Google Scholar , 2 Klion A.D. Bochner B.S. Gleich G.J. et al. The Hypereosinophilic Syndromes Working G. Approaches to the treatment of hypereosinophilic syndromes: a workshop summary report. J Allergy Clin Immunol. 2006; 117 (Epub 2006 May 3.): 1292-1302 Abstract Full Text Full Text PDF PubMed Scopus (303) Google Scholar ]. The clinical manifestations of HES are variable and depend on the target-organ affection by eosinophils. Cardiac involvement, originally described by Loeffler [ [3] Löffler W. Endocarditis parietalis fibroplastica mit Bluteosinophilie. Ein eigenartiges Krankheitsbild. Schweiz Med Wochenschr. 1936; 66: 817-820 Google Scholar ], is frequent and cardiac dysfunction is the major cause of morbidity and mortality in HES-patients [ [4] Weller P.F. Bubley G.J. The idiopathic hypereosinophilic syndrome. Blood. 1994; 83: 2759-2779 PubMed Google Scholar ]. The cardiac pathology of HES has been temporally divided into three stages: early acute necrotic, then thrombotic, and finally a late fibrotic stage [ [4] Weller P.F. Bubley G.J. The idiopathic hypereosinophilic syndrome. Blood. 1994; 83: 2759-2779 PubMed Google Scholar ]. The diagnosis of eosinophilic cardiac disease is based on the co-existence of endomyocardial eosinophils on myocardial biopsy and cardiovascular abnormalities for which other possible causes have been excluded. However, biopsy is invasive and most standard non-invasive diagnostic tools such as echocardiography are often unsatisfactory in detecting cardiac involvement. Contrast-enhanced cardiovascular magnetic resonance imaging (CE-CMR) can characterize different myocardial tissue abnormalities by typical patterns of late gadolinium enhancement (LGE), including necrosis, inflammation, and fibrosis [ [5] Pennell D.J. Cardiovascular magnetic resonance. Circulation. 2010; 121: 692-705 Crossref PubMed Scopus (211) Google Scholar ]. Thus, CE-CMR is an attractive technique to directly visualize the various cardiac pathologies that occur during the different stages of Loeffler endocarditis. So far, the usage of CMR in diagnosing HES associated heart disease has been described only in single case reports [ 6 Puvaneswary M. Joshua F. Ratnarajah S. Idiopathic hypereosinophilic syndrome: magnetic resonance imaging findings in endomyocardial fibrosis. Australas Radiol. 2001; 45: 524-527 Crossref PubMed Scopus (25) Google Scholar , 7 Syed I.S. Martinez M.W. Feng D.L. Glockner J.F. Cardiac magnetic resonance imaging of eosinophilic endomyocardial disease. Int J Cardiol. 2008; 126 (Epub 2007): e50-e52 Abstract Full Text Full Text PDF PubMed Scopus (63) Google Scholar , 8 Cheung S.C. Chan C.W. Insights of prognostication of Davies disease: what could we learn from serial magnetic resonance imaging studies?. Int J Cardiol. 2009; 12: 12 Google Scholar , 9 Chang S.A. Kim H.K. Park E.A. Kim Y.J. Sohn D.W. Images in cardiovascular medicine. Loeffler endocarditis mimicking apical hypertrophic cardiomyopathy. Circulation. 2009; 120: 82-85 Crossref PubMed Scopus (13) Google Scholar , 10 Deb K. Djavidani B. Buchner S. et al. Time course of eosinophilic myocarditis visualized by CMR. J Cardiovasc Magn Reson. 2008; 10: 21 Crossref PubMed Scopus (55) Google Scholar ] but no systematic evaluation has been reported. The purpose of this study was to evaluate the presence of cardiac involvement in patients with HES using CMR.

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