DOI: 10.1111/j.1540-8175.2011.01570.x C  2011, Wiley Periodicals, Inc. Characterization of L¨ oeffler Eosinophilic Myocarditis by Means of Real Time Three-Dimensional Contrast-Enhanced Echocardiography Maria Riccarda Del Bene, M.D., Ph.D., ∗ Francesco Cappelli, M.D., ∗ Luigi Rega, M.D.,† Francesco Venditti, M.D.,‡ and Giuseppe Barletta ∗ , M.D., F.E.S.C. ∗ ∗ Non Invasive Cardiology, †Imaging Diagnostics, and ‡Department of Heart and Vessels, Azienda Ospedaliero-Universitaria Careggi, Firenze, Italy L¨ oeffler endocarditis is a rare myocardial disease often due to eosinophil leukemia or idiopathic hy- pereosinophilic syndrome. Degranulation of eosinophils within the eosinophil infiltrated myocardium is associated with myocardial necrosis due to the release of toxic cationic proteins, and with mu- ral thrombi formation, which can occur anywhere in the ventricles. Thrombus formed on denuded myocardium is replaced by fibrosis as the final pathological stage of the disease, eventually leading to restrictive cardiomyopathy. We describe a multimodality imaging approach to the diagnosis and follow- up evaluation of L¨ oeffler disease complicated by thrombus formation and neoangiogenesis of LV apex. (Echocardiography 2012;29:E62-E66) Key words: idiopathic hypereosinophilic syndrome, L¨ oeffler endomyocarditis, real time three- dimensional echocardiography, late gadolinium enhancement A 63-year-old caucasian woman was admitted with worsening asthenia, anorexia, and persistent fever. She had been suffering from a myeloprolif- erative disorder with idiopathic hypereosinophilic syndrome (HES) without FIP1L1/PDGFRA rear- rangement and T cell clonality since 2 years. Recently, a hard and painful swelling had ap- peared on her right upper limb when she was given an unsuccessful 2-week treatment with imatinib. ECG showed sinus rhythm, with ST depression and T-wave inversion (Fig. 1). Car- diac troponin I was elevated (2.81 ng/mL); complete blood cell count revealed leukocy- tosis (11,500/mm 3 ) with 27.3% eosinophils (3,140/ mm 3 ); D-dimer and fibrinogen were high (668 ng/mL and 491 mg/dL, respectively). Lo- cal thrombotic phenomena of the right upper limb were excluded by ultrasonography, whereas magnetic resonance (MR) suggested muscular tissue infiltration and a biopsy showed signs of acute eosinophil-based inflammation. Transtho- racic two-dimensional echocardiography showed the presence of endocardial thickening and a large hypoechogenic mass occupying the apex of the left ventricle (LV—movie clip 1), highly suggestive of a thrombus in that it lacked fea- tures of a tumor (i.e., nonintramural, absence of a base of attachment to the endocardial sur- Disclosures: None. Address for correspondence and reprint requests: Francesco Cappelli, M.D., Viale Morgagni, 85-50134 Firenze, Italy. Fax +39 55 7947578; E-mail: cappellifrancesco@inwind.it face, absence of pericardial effusion) and api- cal hypertrophic cardiomyopathy (Ace of Spades configuration). Both ventricles had normal dimensions and preserved systolic function in the absence of regional wall motion abnormalities; there were a pseudo-normalization LV filling pat- tern with high E/E ′ ratio, mild to moderate mitral regurgitation, and mild tricuspid regurgitation; estimated systolic pulmonary artery pressure was 21 mmHg. AT cardiac MR, DP-weighted morpho- logical images showed mild hypertrophy of the distal right ventricular apical wall (Fig. 2A) and overall LV thickening (Fig. 2B), especially at the apex. On T 2 -weighted serial images (Fig. 2C), it was possible to identify three different layers at the apex of the LV: an external slightly hy- pertrophic and hypokinetic zone, with the same signal as normal myocardium, an intermediate T 2 hyperintense layer, and a third hypointense inner area. Gadolinium administration demon- strated normal early enhancement of the exter- nal myocardial layer, intense linear delayed en- hancement (LGE) of endocardium and a large nonenhancing apical thrombus (Fig. 2D). Diag- nosis of L¨ oeffler endomyocarditis 1 with left api- cal thrombus in HES was therefore confirmed. 2 Sonovue-enhanced left ventricular opacification echocardiography (Philips iE33 X3-1 probe, An- dover, MA, USA; Fig. 2E) confirmed the large apical filling defect of the LV, and hypertrophy of right ventricular apex and distal free wall. Low-mechanical-index contrast real time three- dimensional (RT3D) full-volume imaging cen- tered on the LV revealed neoangiogenesis within E62