%0 Journal Article
%T Cardiac involvement in patients with Becker muscular dystrophy: new diagnostic and pathophysiological insights by a CMR approach
%A Ali Yilmaz
%A Hans-Jščrgen Gdynia
%A Hannibal Baccouche
%A Heiko Mahrholdt
%A Gabriel Meinhardt
%A Cristina Basso
%A Gaetano Thiene
%A Anne-Dorte Sperfeld
%A Albert C Ludolph
%A Udo Sechtem
%J Journal of Cardiovascular Magnetic Resonance
%D 2008
%I BioMed Central
%R 10.1186/1532-429x-10-50
%X 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.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). Myocardial damage as assessed by LGE-imaging was detected in 11 of 15 (73.3%) patients with a median myocardial damage extent of 13.0% (range 0 to 38.0%), an age-related increase and a typical subepicardial distribution pattern in the inferolateral wall. Ten patients (66.7%) were in need of medical heart failure therapy based on CMR results. However, only 4 patients (26.7%) were already taking medication based on clinical criteria (p = 0.009).Cardiac involvement in patients with BMD is underdiagnosed by echocardiographic methods resulting in undertreatment of heart failure. The degree and severity of cardiac involvement in this population is best characterised when state-of-the-art CMR methods are applied. Further studies need to demonstrate whether earlier diagnosis and institution of heart failure therapy will extend the life span of these patients.Muscular dystrophy type Becker-Kiener (BMD) and type Duchenne (DMD) represent X-linked genetic diseases related to mutations in the dystrophin gene which is located on chromosome Xp21.1 [1]. BMD has been described to have a prevalence rate of 2.4 per 100,000 and is less common than DMD [2]. While the protein dystrophin is totally absent or dysfunctional in DMD, BMD is charac
%U http://jcmr-online.com/content/10/1/50