European Heart Journal - Cardiovascular Imaging Advance Access published October 9, 2014

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doi:10.1093/ehjci/jeu196

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Cardiac involvement in hypereosinophilic syndrome: role of multimodality imaging Blandine Simonnet1, Alexis Jacquier2, Erwan Salaun1, Sandrine Hubert1, and Gilbert Habib1* 1 Department of Cardiology, La Timone Hospital Marseille, Boulevard Jean Moulin, 13005 Marseille, France; and 2Department of Radiology, Aix Marseille Universite´, Hoˆpital Universitaire La Timone, Marseille, France

* Corresponding author. Tel: +33 4 91 38 75 88, Email: [email protected]

Supplementary data are available at European Heart Journal – Cardiovascular Imaging online. Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2014. For permissions please email: [email protected].

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Cardiac involvement is a rare but severe complication of idiopathic hypereosinophilic syndrome. This case report illustrates the value of multimodality imaging in this disease. A 38-year-old man without previous medical history was referred to our cardiology department for suspected infective endocarditis, because of asthenia, fever, and new murmur. Echocardiography showed no typical vegetation, but a severe restriction of the posterior mitral leaflet associated with involvement of the subvalvular apparatus and severe mitral regurgitation (Panels A and B, and see Supplementary data online, Videos S1 and S2). Laboratory findings showed a severe hypereosinophilia (eosinophils: 25 × 109/L). Cardiac magnetic resonance confirmed the severe endocardial involvement and mitral regurgitation (Panel C, and see Supplementary data online, Video S3). A chronic eosinophilic leukaemia was diagnosed by bone marrow biopsy and a specific therapy was initiated, including corticotherapy and chemotherapy. Mitral valve replacement was needed because of persisting heart failure, with an uneventful postoperative course. Fifteen months later, recurrence of hypereosinophilia occurred, associated with severe worsening of cardiac involvement, including massive infiltration of the left ventricle endocardium (Panel D, and see Supplementary data online, Video S4) extending into the bioprosthesis [see Supplementary data online, Videos S5 (transthoracic echocardiography) and S6 (transoesophageal echocardiography)]. A repeat biological prosthetic mitral valve replacement was ultimately necessary with a good outcome. Finally, bone marrow transplantation was performed, along with continuous chemotherapy, with a good outcome and no relapse after a 2-year follow-up. This case illustrates the role of imaging in the evaluation of cardiac involvement in hypereosinophila, with progressive and recurrent endomyocardial thickening. Thickening of the posterobasal wall was associated with impairment of posterior mitral leaflet function, resulting in severe mitral regurgitation.

Cardiac involvement in hypereosinophilic syndrome: role of multimodality imaging.

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