title: Early myocardial damage assessment in dystrophinopathies using (99)Tc(m)-MIBI gated myocardial perfusion imaging.
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 ((99)Tc(m)-MIBI) gated myocardial perfusion imaging (G-MPI) for MD.
  
  
  RESULTS
  Ninety-one patients underwent (99)Tc(m)-MIBI G-MPI examinations when they were diagnosed with Duchenne muscular dystrophy (DMD) (n=77) or Becker muscular dystrophy (BMD; n=14). (99)Tc(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 (99)Tc(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.
  
  
  CONCLUSIONS
  (99)Tc(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.
authors.affiliation: Department of Cardiovascular Disorders.
authors.forename: Li
authors.initials: L
authors.lastname: Zhang
authors.affiliation: Department of Geriatrics, The Third Hospital of Hebei Medical University.
authors.forename: Zhe
authors.initials: Z
authors.lastname: Liu
authors.affiliation: The Public Health Department, Hebei Medical University.
authors.forename: Ke-You
authors.initials: KY
authors.lastname: Hu
authors.affiliation: The Public Health Department, Hebei Medical University.
authors.forename: Qing-Bao
authors.initials: QB
authors.lastname: Tian
authors.affiliation: Department of Nuclear Medicine.
authors.forename: Ling-Ge
authors.initials: LG
authors.lastname: Wei
authors.affiliation: Department of Neuromuscular Disorders, The Third Hospital of Hebei Medical University, Shijiazhuang, People's Republic of China.
authors.forename: Zhe
authors.initials: Z
authors.lastname: Zhao
authors.affiliation: Department of Neuromuscular Disorders, The Third Hospital of Hebei Medical University, Shijiazhuang, People's Republic of China.
authors.forename: Hong-Rui
authors.initials: HR
authors.lastname: Shen
authors.affiliation: Department of Neuromuscular Disorders, The Third Hospital of Hebei Medical University, Shijiazhuang, People's Republic of China.
authors.forename: Jing
authors.initials: J
authors.lastname: Hu
keywords: 99Tcm-MIBI; Becker muscular dystrophy; Duchenne muscular dystrophy; cardiomyopathy; dystrophinopathy