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Midwall myocardial fibrosis in Becker-Kiener muscular dystrophy.
Süselbeck, T; Haghi, D; Neff, W; Borggrefe, M; Papavassiliu, T.
Afiliação
  • Süselbeck T; 1st Department of Medicine, University Hospital of Mannheim, Faculty of Clinical Medicine of the University of Heidelberg, Mannheim, Germany.
Z Kardiol ; 94(7): 465-8, 2005 Jul.
Article em En | MEDLINE | ID: mdl-15997348
We report on a 38- year-old man with Becker-Kiener muscular dystrophy (BMD) and dilated cardiomyopathy without clinical symptoms of congestive heart failure who was referred for risk evaluation of sudden cardiac death. The degree of cardiac involvement in BMD varies greatly from no or hardly any cardiac abnormality to severe arrhythmias, dilatative cardiomyopathy and heart failure to heart transplantation or sudden cardiac death. These cardiac abnormalities have been related to replacement of the cardiomyocytes by connecting tissue or fat. In the clinical setting, cardiovascular magnetic resonance (CMR) has been proved to be a valid non-invasive method for obtaining anatomical and structural information of the heart. Furthermore, gadolinium-enhanced CMR can also characterize areas of myocardial fibrosis. Demonstration of extensive areas of fibrosis in an early stage of the disease might be a surrogate marker for an impaired clinical outcome. Therefore, serial CMR examinations starting upon diagnosis of the disease should be considered, as this may lead to an earlier recognition of cardiac involvement and may affect further management of the patient.
Assuntos
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Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Imageamento por Ressonância Magnética / Cardiomiopatia Dilatada / Distrofia Muscular de Duchenne / Cardiomiopatias Tipo de estudo: Diagnostic_studies / Etiology_studies Limite: Adult / Humans / Male Idioma: En Ano de publicação: 2005 Tipo de documento: Article
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Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Imageamento por Ressonância Magnética / Cardiomiopatia Dilatada / Distrofia Muscular de Duchenne / Cardiomiopatias Tipo de estudo: Diagnostic_studies / Etiology_studies Limite: Adult / Humans / Male Idioma: En Ano de publicação: 2005 Tipo de documento: Article