Article
Subepicardial dysfunction leads to global left ventricular systolic impairment in patients with limb girdle muscular dystrophy 2I.
European journal of heart failure - 1 Sept 2013
Hollingsworth Kieren G, Willis Tracey A, Bates Matthew G D, Dixon Ben J, Lochmüller Hanns, Bushby Kate, Bourke John, MacGowan Guy A, Straub Volker
Abstract excerpt
AIM: The mechanisms of cardiac dysfunction in limb girdle muscular dystrophy 2I (LGMD2I) are unclear. This study assessed deficits in cardiac morphology, function, and metabolism quantitatively in patients with a confirmed genetic diagnosis of the homozygous c.826C > A FKRP (fukutin-related protein) mutation, using a comprehensive magnetic resonance (MR) examination. METHODS AND RESULTS: Ten patients (7 male and...
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