Cardiac Magnetic Resonance Features of Biopsy Proven Endomyocardial Diseases and Hypereosinophilic Syndrome.
Martina Perazzolo Marra, Stefania Rizzo, Manuel De Lazzari, Francesco Corbetti, Sabino Iliceto, Gaetano Thiene, Cristina Basso. , Padua, Italy; University of Padua Medical School, Italy
Background: Hypereosinophilic syndrome (HES) is characterized by marked eosinophilia and eosinophil-mediated organ damage, in the absence of primary causes. Endomyocardial pathology of HES includes eosinophilic myocarditis (EM)-Loeffler endocarditis (LE) and endomyocardial fibrosis (EMF), which are considered different stages of the same disease. Cardiac magnetic resonance (CMR) data on LE/EMF are still missing.
Design: Patients with a clinico-pathologic diagnosis of HES and/or endomyocardial diseases who underwent to CMR with late gadolinium enhancement (LGE) and endomyocardial biopsy (EMB) during the same hospitalization in the time interval 2002-2010 were enrolled. Cases with allergic, infective, autoimmune and vasculitis conditions were excluded. A complete CMR protocol (including T1 and T2 weighted images, post-contrast sequences for LGE evaluation) was performed. EMB was performed from the right ventricle (RV) and processed for histology, immunoistochemistry and polymerase chain reaction.
Results: Eight patients were studied (5 males; 42±11 y, range 25-19), including 5 with EM/LE and 3 with EMF. Molecular pathology investigation ruled out viral genomes, except for 1 case who was positive for PVB19. Among the 5 EM/LE patients, all but one with HES, CMR showed a moderate left ventricular (LV) dysfunction/dilatation in all; RV dysfunction/dilatation in 50%; and LV hypertrophy, myocardial edema and pericardial effusion in all. A LGE was found in LV in all, but never in RV. Endocardial thrombosis was detected in all (4 RV, 1 LV). Among the 3 EMF patients, 1 of whom with documented previous HES, CMR showed RV dilatation/dysfunction with free wall thickening and subendocardial LGE in all, associated with LV LGE in one. One case of chamber obliteration was found. In three EM/LE pts, CMR was repeated during the follow-up showing biventricular dilatation/dysfunction, RV LGE, normalization of LV wall thickness and subendocardial LV LGE in keeping with EMF, as confirmed by a second EMB in two cases.
Conclusions: In endomyocardial diseases with/without HES, CMR is an important diagnostic tool which allows identification of myocardial inflammation, mural thrombi and endocardial fibrosis, besides morpho-functional cardiac evaluation and confirms that EMF and EM/LE are different stages of the same disease process. The non-invasiveness supports its role in the follow-up evaluation.
Wednesday, March 2, 2011 1:00 PM
Poster Session VI # 29, Wednesday Afternoon