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Unusual endomyocardial fibrosis with apical calcification
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ARTICLE IN PRESS
Diagnostic and Interventional Imaging (2015) xxx, xxx—xxx
LETTER TO THE EDITOR /Cardiovascular Unusual endomyocardial fibrosis with apical calcification Dear Editor, Endomyocardial fibrosis (EMF) is a rare condition in western countries [1]. EMF is characterized by thickening of one or both ventricular endocardia with dense and white fibrous tissue that often extends to the inner third of the myocardium, causing cavity obliteration and restriction of blood ventricular filling. EMF represents the second main cause of restrictive cardiomyopathies. EMF usually occurs in a context of hypereosinophilic syndrome. However, its diagnosis might be difficult in the absence of hyperoesinophilia [2]. The initial stage of the disease is generally characterized by a hypereosinophila and then regression in the later ‘‘fibrotic’’ phase [1]. The etiology of this disease is still unclear and different causes have been suggested: infections (toxoplasmosis, malaria, helminthiasis), chronic inflammatory disease (rheumatoid arthritis), allergies (hypereosinophilia, autoimmunity), malnutrition, and toxic agents such as alcohol [3]. Echocardiography is the first-line imaging technique permitting morphological, functional, and hemodynamic analysis of the heart in daily practice [2]. However, echocardiography does not allow tissue characterization and cardiac biopsy is still needed to confirm EMF [4]. We report herein EMF in a 55-year-old man referred to our hospital for acute heart failure. Patient had history of alcohol abuse and was a current smoker. Clinical examination was consistent with global heart failure associated with mild chest pain at rest. Electrocardiogram revealed isolated, incomplete left bundle branch block. Blood tests were unremarkable except for elevation of heart failure
biomarkers (elevated BNP level). Emergency coronary angiogram showed no remarkable abnormalities. Transthoracic echocardiography showed moderate left ventricular dysfunction without diastolic impairment. Left ventricular volume was reduced and the ventricular filling pattern was restrictive, without atrial dilation, and most importantly a large, calcified apical mass was noted. Cardiac magnetic resonance imaging (CMRI) was performed for better tissue characterization. SSFP-cine revealed a global left ventricular hypokinesia associated with apical hypertrophy. First pass perfusion images during administration of gadoterate meglumine (Dotarem® , Laboratoires Guerbet, Roissy-Charles de Gaulle, France) were acquired as previously described [5]. A marked hyposignal with no enhancement of the apical zone with an unequivocal presence of a mass consistent with calcification was observed (Fig. 1). Ten minutes after contrast administration, late gadolinium-chelate enhanced images revealed a threelayered appearance consisting of an outermost hypointense layer of normal myocardium, a middle high-signal intensity enhancing layer of endocardium fibrosis, and an innermost hypo-intense layer of thrombus or calcifications (Fig. 2). Recent studies showed that CMRI is a robust technique for the diagnosis of EMF [4]. Indeed, a typical 3-layer appearance of the myocardium has been described: an inner layer showing no perfusion due to presence of thrombus, a middle layer on late gadolinium-chelate enhanced MR images reflecting the fibrotic tissue and an outer layer of normal myocardium. An excellent correlation was found between histopathologic examination and CMR findings. Finally, cardiac CT in this particular patient had an additional diagnostic value by distinguishing thrombus from calcification commonly present in EMF, which is hardly discriminated on late gadolinium chelate enhanced MR images (Fig. 2).
Please cite this article in press as: Gzara H, et al. Unusual endomyocardial fibrosis with apical calcification. Diagnostic and Interventional Imaging (2015), http://dx.doi.org/10.1016/j.diii.2015.04.009
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ARTICLE IN PRESS Letter to the editor
Figure 1. Long axis three-chamber view (upper panel) and long axis two chamber view (lower panel) of the left ventricle using SSFP sequence before (left panel) and after gadolinium-chelate injection (right panel). Diffusion of gadolinium-chelate within the myocardium, immediately after injection better show the three-layered appearance of the apex.
Please cite this article in press as: Gzara H, et al. Unusual endomyocardial fibrosis with apical calcification. Diagnostic and Interventional Imaging (2015), http://dx.doi.org/10.1016/j.diii.2015.04.009
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ARTICLE IN PRESS
Letter to the editor
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Figure 2. CMR images performed 10 minutes after gadolinium-chelate injection in long axis three and four chamber view (panel A and B) and a short axis view centered on the apex (panel C). The ‘‘so called’’ late gadolinium-chelate enhanced images better show the typical three-layered appearance of EMF. Panel D displays a four-chamber view computed tomography image with a readily visible calcification of the apex.
Acknowledgments This research has not been funded by any organization.
[5] Dacher JN, Lefebvre V, Dubourg B, Deux JF, Caudron J. Is it possible to do without the study of myocardial perfusion in 2013? Diagn Interv Imaging 2013;94(12):1337—44.
Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. References [1] Mocumbi AO, Yacoub S, Yacoub MH. Neglected tropical cardiomyopathies: II Endomyocardial firbosis: myocardial disease. Heart 2008;94(3):384—90. [2] Hassan WM, Fawzy ME, Al Helaly SA, Hegazy H, Malik S. Pitfalls in diagnosis and clinical, echocardiographic, and hemodynamic findings in endomyocardial fibrosis: a 25-year experience. Chest 2005;128(6):3985—92. [3] Bukhman G, Ziegler J, Parry E. Endomyocardial fibrosis: still a mystery after 60 years. PLoS Negl Trop Dis 2008;2(2):e97. [4] Caudron J, Arous Y, Fares J, Lefebvre V, Dacher JN. Endomyocardial fibrosis in the context of hypereosinophilic syndrome: the contribution of cardiac MRI. Diagn Interv Imaging 2012;93(10):790—2.
H. Gzara a,b , E. Berthelot a,b , P. Soyer a,b , M. Sirol a,b,∗ a
Department of body and interventional imaging, hôpital Lariboisière, 2, rue Ambroise-Paré, 75010 Paris, France b Université Sorbonne Paris-Cité, Diderot Paris 7, 10, avenue de Verdun, 75010 Paris, France ∗ Corresponding
author at: Department of body and interventional imaging, hôpital Lariboisière, Inserm UMRs 942, 2, rue Ambroise-Paré, 75010 Paris, France. E-mail address: [email protected] (M. Sirol) http://dx.doi.org/10.1016/j.diii.2015.04.009 2211-5684/© 2015 Éditions franc ¸aises de radiologie. Publié par Elsevier Masson SAS. Tous droits réservés.
Please cite this article in press as: Gzara H, et al. Unusual endomyocardial fibrosis with apical calcification. Diagnostic and Interventional Imaging (2015), http://dx.doi.org/10.1016/j.diii.2015.04.009
ARTICLE IN PRESS
Diagnostic and Interventional Imaging (2015) xxx, xxx—xxx
LETTER TO THE EDITOR /Cardiovascular Unusual endomyocardial fibrosis with apical calcification Dear Editor, Endomyocardial fibrosis (EMF) is a rare condition in western countries [1]. EMF is characterized by thickening of one or both ventricular endocardia with dense and white fibrous tissue that often extends to the inner third of the myocardium, causing cavity obliteration and restriction of blood ventricular filling. EMF represents the second main cause of restrictive cardiomyopathies. EMF usually occurs in a context of hypereosinophilic syndrome. However, its diagnosis might be difficult in the absence of hyperoesinophilia [2]. The initial stage of the disease is generally characterized by a hypereosinophila and then regression in the later ‘‘fibrotic’’ phase [1]. The etiology of this disease is still unclear and different causes have been suggested: infections (toxoplasmosis, malaria, helminthiasis), chronic inflammatory disease (rheumatoid arthritis), allergies (hypereosinophilia, autoimmunity), malnutrition, and toxic agents such as alcohol [3]. Echocardiography is the first-line imaging technique permitting morphological, functional, and hemodynamic analysis of the heart in daily practice [2]. However, echocardiography does not allow tissue characterization and cardiac biopsy is still needed to confirm EMF [4]. We report herein EMF in a 55-year-old man referred to our hospital for acute heart failure. Patient had history of alcohol abuse and was a current smoker. Clinical examination was consistent with global heart failure associated with mild chest pain at rest. Electrocardiogram revealed isolated, incomplete left bundle branch block. Blood tests were unremarkable except for elevation of heart failure
biomarkers (elevated BNP level). Emergency coronary angiogram showed no remarkable abnormalities. Transthoracic echocardiography showed moderate left ventricular dysfunction without diastolic impairment. Left ventricular volume was reduced and the ventricular filling pattern was restrictive, without atrial dilation, and most importantly a large, calcified apical mass was noted. Cardiac magnetic resonance imaging (CMRI) was performed for better tissue characterization. SSFP-cine revealed a global left ventricular hypokinesia associated with apical hypertrophy. First pass perfusion images during administration of gadoterate meglumine (Dotarem® , Laboratoires Guerbet, Roissy-Charles de Gaulle, France) were acquired as previously described [5]. A marked hyposignal with no enhancement of the apical zone with an unequivocal presence of a mass consistent with calcification was observed (Fig. 1). Ten minutes after contrast administration, late gadolinium-chelate enhanced images revealed a threelayered appearance consisting of an outermost hypointense layer of normal myocardium, a middle high-signal intensity enhancing layer of endocardium fibrosis, and an innermost hypo-intense layer of thrombus or calcifications (Fig. 2). Recent studies showed that CMRI is a robust technique for the diagnosis of EMF [4]. Indeed, a typical 3-layer appearance of the myocardium has been described: an inner layer showing no perfusion due to presence of thrombus, a middle layer on late gadolinium-chelate enhanced MR images reflecting the fibrotic tissue and an outer layer of normal myocardium. An excellent correlation was found between histopathologic examination and CMR findings. Finally, cardiac CT in this particular patient had an additional diagnostic value by distinguishing thrombus from calcification commonly present in EMF, which is hardly discriminated on late gadolinium chelate enhanced MR images (Fig. 2).
Please cite this article in press as: Gzara H, et al. Unusual endomyocardial fibrosis with apical calcification. Diagnostic and Interventional Imaging (2015), http://dx.doi.org/10.1016/j.diii.2015.04.009
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2
ARTICLE IN PRESS Letter to the editor
Figure 1. Long axis three-chamber view (upper panel) and long axis two chamber view (lower panel) of the left ventricle using SSFP sequence before (left panel) and after gadolinium-chelate injection (right panel). Diffusion of gadolinium-chelate within the myocardium, immediately after injection better show the three-layered appearance of the apex.
Please cite this article in press as: Gzara H, et al. Unusual endomyocardial fibrosis with apical calcification. Diagnostic and Interventional Imaging (2015), http://dx.doi.org/10.1016/j.diii.2015.04.009
+Model DIII-621; No. of Pages 3
ARTICLE IN PRESS
Letter to the editor
3
Figure 2. CMR images performed 10 minutes after gadolinium-chelate injection in long axis three and four chamber view (panel A and B) and a short axis view centered on the apex (panel C). The ‘‘so called’’ late gadolinium-chelate enhanced images better show the typical three-layered appearance of EMF. Panel D displays a four-chamber view computed tomography image with a readily visible calcification of the apex.
Acknowledgments This research has not been funded by any organization.
[5] Dacher JN, Lefebvre V, Dubourg B, Deux JF, Caudron J. Is it possible to do without the study of myocardial perfusion in 2013? Diagn Interv Imaging 2013;94(12):1337—44.
Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. References [1] Mocumbi AO, Yacoub S, Yacoub MH. Neglected tropical cardiomyopathies: II Endomyocardial firbosis: myocardial disease. Heart 2008;94(3):384—90. [2] Hassan WM, Fawzy ME, Al Helaly SA, Hegazy H, Malik S. Pitfalls in diagnosis and clinical, echocardiographic, and hemodynamic findings in endomyocardial fibrosis: a 25-year experience. Chest 2005;128(6):3985—92. [3] Bukhman G, Ziegler J, Parry E. Endomyocardial fibrosis: still a mystery after 60 years. PLoS Negl Trop Dis 2008;2(2):e97. [4] Caudron J, Arous Y, Fares J, Lefebvre V, Dacher JN. Endomyocardial fibrosis in the context of hypereosinophilic syndrome: the contribution of cardiac MRI. Diagn Interv Imaging 2012;93(10):790—2.
H. Gzara a,b , E. Berthelot a,b , P. Soyer a,b , M. Sirol a,b,∗ a
Department of body and interventional imaging, hôpital Lariboisière, 2, rue Ambroise-Paré, 75010 Paris, France b Université Sorbonne Paris-Cité, Diderot Paris 7, 10, avenue de Verdun, 75010 Paris, France ∗ Corresponding
author at: Department of body and interventional imaging, hôpital Lariboisière, Inserm UMRs 942, 2, rue Ambroise-Paré, 75010 Paris, France. E-mail address: [email protected] (M. Sirol) http://dx.doi.org/10.1016/j.diii.2015.04.009 2211-5684/© 2015 Éditions franc ¸aises de radiologie. Publié par Elsevier Masson SAS. Tous droits réservés.
Please cite this article in press as: Gzara H, et al. Unusual endomyocardial fibrosis with apical calcification. Diagnostic and Interventional Imaging (2015), http://dx.doi.org/10.1016/j.diii.2015.04.009