MRI characterization of myocardial tissue in patients with Fabry's disease

AJR Am J Roentgenol. 2007 Mar;188(3):850-3. doi: 10.2214/AJR.05.0442.

Abstract

Objective: Fabry's disease is a multisystem X-linked disorder of lysosomal metabolism frequently associated with left ventricular (LV) hypertrophy. In this study, we aimed to assess whether myocardial T2 relaxation time determined by a black blood multiecho multishot MRI sequence could be used to evaluate cardiac involvement in patients with Fabry's disease.

Conclusion: Myocardial T2 relaxation time is prolonged in patients with Fabry's disease compared with that of hypertrophic patients and healthy control subjects. MRI may be useful for the characterization of myocardial tissue in patients with Fabry's disease.

MeSH terms

  • Adolescent
  • Adult
  • Cardiomyopathies / complications*
  • Cardiomyopathies / pathology*
  • Fabry Disease / complications*
  • Fabry Disease / pathology*
  • Feasibility Studies
  • Female
  • Humans
  • Magnetic Resonance Imaging / methods*
  • Male
  • Middle Aged