Cardiac involvement in patients with Becker muscular dystrophy: new diagnostic and pathophysiological insights by a CMR
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BioMed Central
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Cardiac involvement in patients with Becker muscular dystrophy: new diagnostic and pathophysiological insights by a CMR approach Ali Yilmaz*1, Hans-Jürgen Gdynia2, Hannibal Baccouche1, Heiko Mahrholdt1, Gabriel Meinhardt1, Cristina Basso3, Gaetano Thiene3, Anne-Dorte Sperfeld2, Albert C Ludolph2 and Udo Sechtem1 Address: 1Division of Cardiology, Robert-Bosch-Krankenhaus, Stuttgart, Germany, 2Department of Neurology, University of Ulm, Ulm, Germany and 3Department of Medico-Diagnostic Sciences, University of Padua Medical School, Padua, Italy Email: Ali Yilmaz* - [email protected]; Hans-Jürgen Gdynia - [email protected]; Hannibal Baccouche - [email protected]; Heiko Mahrholdt - [email protected]; Gabriel Meinhardt - [email protected]; Cristina Basso - [email protected]; Gaetano Thiene - [email protected]; AnneDorte Sperfeld - [email protected]; Albert C Ludolph - [email protected]; Udo Sechtem - [email protected] * Corresponding author
Published: 4 November 2008 Journal of Cardiovascular Magnetic Resonance 2008, 10:50
doi:10.1186/1532-429X-10-50
Received: 4 August 2008 Accepted: 4 November 2008
This article is available from: http://www.jcmr-online.com/content/10/1/50 © 2008 Yilmaz et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract Background : Becker-Kiener muscular dystrophy (BMD) represents an X-linked genetic disease associated with myocardial involvement potentially resulting in dilated cardiomyopathy (DCM). Early diagnosis of cardiac involvement may permit earlier institution of heart failure treatment and extend life span in these patients. Both echocardiography and nuclear imaging methods are capable of detecting later stages of cardiac involvement characterised by wall motion abnormalities. Cardiovascular magnetic resonance (CMR) has the potential to detect cardiac involvement by depicting early scar formation that may appear before onset of wall motion abnormalities. Methods : In a prospective two-center-study, 15 male patients with BMD (median age 37 years; range 11 years to 56 years) underwent comprehensive neurological and cardiac evaluations including physical examination, echocardiography and CMR. A 16-segment model was applied for evaluation of regional wall motion abnormalities (rWMA). The CMR study included late gadolinium enhancement (LGE) imaging with quantification of myocardial damage. Results : Abnormal echocardiographic results were found in eight of 15 (53.3%) patients with all of them demonstrating reduced left ventricular ejection fraction (LVEF) and rWMA. CMR revealed abnormal findings in 12 of 15 (80.0%) patients (p = 0.04) with 10 (66.6%) having reduced LVEF (p = 0.16) and 9 (64.3%) demonstrating rWMA (p = 0.38). Myocar
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