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      Early myocardial damage assessment in dystrophinopathies using 99Tc m-MIBI gated myocardial perfusion imaging

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          Abstract

          Background

          Early detection of muscular dystrophy (MD)-associated cardiomyopathy is important because early medical treatment may slow cardiac remodeling and attenuate symptoms of cardiac dysfunction; however, no sensitive and standard diagnostic method for MD at an earlier stage has been well-recognized. Thus, the aim of this study was to test the early diagnostic value of technetium 99m-methoxyisobutylisonitrile ( 99Tc m-MIBI) gated myocardial perfusion imaging (G-MPI) for MD.

          Methods and results

          Ninety-one patients underwent 99Tc m-MIBI G-MPI examinations when they were diagnosed with Duchenne muscular dystrophy (DMD) (n=77) or Becker muscular dystrophy (BMD; n=14). 99Tc m-MIBI G-MPI examinations were repeated in 43 DMD patients who received steroid treatments for 2 years as a follow-up examination. Myocardial defects were observed in nearly every segment of the left ventricular wall in both DMD and BMD patients compared with controls, especially in the inferior walls and the apices by using 99Tc m-MIBI G-MPI. Cardiac wall movement impairment significantly correlated with age in the DMD and BMD groups ( r s=0.534 [ P<0.05] and r s=0.784 [ P<0.05], respectively). Intermittent intravenous doses of glucocorticoids and continuation with oral steroid treatments significantly improved myocardial function in DMD patients ( P<0.05), but not in BMD patients.

          Conclusion

          99Tc m-MIBI G-MPI is a sensitive and safe approach for early evaluation of cardiomyopathy in patients with DMD or BMD, and can serve as a candidate method for the evaluation of progression, prognosis, and assessment of the effect of glucocorticoid treatment in these patients.

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          Most cited references 26

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          The incidence and evolution of cardiomyopathy in Duchenne muscular dystrophy.

          To assess the incidence, nature and evolution of cardiac disease in Duchenne muscular dystrophy, 328 patients were studied between 1976 and 1987 for periods varying from 3 to 11 years. Patients underwent regular clinical examination, electrocardiography, echocardiography and radiological assessment. Pre-clinical cardiac involvement was found in 25% of patients under 6 years old increasing to 59% between the ages of 6 and 10 years and then declining in incidence with age. Clinically apparent cardiomyopathy is first evident after 10 years of age and increases in incidence with age, being present in all patients over 18 years of age. Its clinical impact is discussed.
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            The dystrophin glycoprotein complex: signaling strength and integrity for the sarcolemma.

            The dystrophin glycoprotein complex (DGC) is a specialization of cardiac and skeletal muscle membrane. This large multicomponent complex has both mechanical stabilizing and signaling roles in mediating interactions between the cytoskeleton, membrane, and extracellular matrix. Dystrophin, the protein product of the Duchenne and X-linked dilated cardiomyopathy locus, links cytoskeletal and membrane elements. Mutations in additional DGC genes, the sarcoglycans, also lead to cardiomyopathy and muscular dystrophy. Animal models of DGC mutants have shown that destabilization of the DGC leads to membrane fragility and loss of membrane integrity, resulting in degeneration of skeletal muscle and cardiomyocytes. Vascular reactivity is altered in response to primary degeneration in striated myocytes and arises from a vascular smooth muscle cell-extrinsic mechanism.
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              Cardiac involvement in patients with muscular dystrophies: magnetic resonance imaging phenotype and genotypic considerations.

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                Author and article information

                Journal
                Ther Clin Risk Manag
                Ther Clin Risk Manag
                Therapeutics and Clinical Risk Management
                Therapeutics and Clinical Risk Management
                Dove Medical Press
                1176-6336
                1178-203X
                2015
                10 December 2015
                : 10
                : 1819-1827
                Affiliations
                [1 ]Department of Cardiovascular Disorders
                [2 ]Department of Geriatrics, The Third Hospital of Hebei Medical University
                [3 ]The Public Health Department, Hebei Medical University
                [4 ]Department of Nuclear Medicine
                [5 ]Department of Neuromuscular Disorders, The Third Hospital of Hebei Medical University, Shijiazhuang, People’s Republic of China
                Author notes
                Correspondence: Jing Hu, Department of Neuromuscular Disorders, The Third Hospital of Hebei Medical University, NO 139, Ziqiang Road, 050051 Shijiazhuang, Hebei Province, People’s Republic of China, Tel +86 311 8860 2057, Fax +86 311 8860 2057, Email jinghu201501@ 123456sina.com
                [*]

                Li Zhang and Zhe Liu are first coauthors of this paper

                Article
                tcrm-11-1819
                10.2147/TCRM.S89962
                4677759
                © 2015 Zhang et al. This work is published by Dove Medical Press Limited, and licensed under Creative Commons Attribution – Non Commercial (unported, v3.0) License

                The full terms of the License are available at http://creativecommons.org/licenses/by-nc/3.0/. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed.

                Categories
                Original Research

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