title: Right ventricular involvement in patients with Fabry's disease and the effect of enzyme replacement therapy.
abstract: OBJECTIVE
  According to echocardiography reports, Fabry cardiomyopathy not only affects the left ventricle (LV) but also the right ventricle (RV). Until now no MRI studies about the effect of enzyme replacement therapy (ERT) on the RV are available. We evaluated the effect of ERT on the RV.
  
  
  METHODS
  In this prospective trial 14 patients with genetically proven Fabry's disease were examined using a 1.5 T MR scanner before ERT and after 13 ± 1 months of ERT. All patients underwent cardiac MR imaging and the RV/LV cardiac morphology and function were analyzed.
  
  
  RESULTS
  At baseline examination the values were as follows: RV mass 31 ± 6 g/m (2), end-diastolic volume (EDV) 88 ± 13 ml/m (2), end-systolic volume (ESV) 39 ± 9 ml/m (2), stroke volume (SV) 49 ± 7 ml/m (2) and ejection fraction (EF) 56 ± 5 %. The RV mass and EDV decreased significantly after 13 ± 1 months on ERT (mass 27 ± 7 g/m (2), p < 0.05, EDV 76 ± 24 ml/m (2), p < 0.05), with no significant change of ESV (33 ± 13 ml/m (2)), SV (43 ± 12 ml/m (2)) and EF (57 ± 7 %). The LV mass (102 ± 26 g/m (2) vs. 94 ± 27 g/m (2), p < 0.05), EDV (76 ± 13 ml/m (2) vs. 66 ± 22 ml/m (2), p < 0.05) and ESV (29 ± 9 ml/m (2) vs. 23 ± 9 ml/m (2), p < 0.05) decreased significantly while the EF (64 ± 7 % vs. 66 ± 5 %; p < 0.05) increased significantly.
  
  
  CONCLUSIONS
  Besides the known beneficial effect on the LV, ERT improves RV mass and EDV.
authors.affiliation: Radiologisches Institut, Universitätsklinikum Erlangen.
authors.forename: W
authors.initials: W
authors.lastname: Wuest
authors.forename: W
authors.initials: W
authors.lastname: Machann
authors.forename: F
authors.initials: F
authors.lastname: Breunig
authors.forename: F
authors.initials: F
authors.lastname: Weidemann
authors.forename: H
authors.initials: H
authors.lastname: Koestler
authors.forename: D
authors.initials: D
authors.lastname: Hahn
authors.forename: C
authors.initials: C
authors.lastname: Wanner
authors.forename: M
authors.initials: M
authors.lastname: Beer
mesh_terms: D000328:Adult; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000188:drug therapy* / Q000503:physiopathology; D003971:Diastole / Q000502:physiology; D004452:Echocardiography; D000795:Fabry Disease / Q000175:diagnosis* / Q000188:drug therapy* / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D017380:Hypertrophy, Right Ventricular / Q000175:diagnosis* / Q000188:drug therapy* / Q000503:physiopathology; D007091:Image Processing, Computer-Assisted / Q000379:methods*; D021621:Imaging, Three-Dimensional / Q000379:methods*; D007527:Isoenzymes / Q000008:administration & dosage*; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D008875:Middle Aged; D013318:Stroke Volume / Q000502:physiology; D013599:Systole / Q000502:physiology; D018497:Ventricular Dysfunction, Right / Q000175:diagnosis* / Q000188:drug therapy* / Q000503:physiopathology; D000519:alpha-Galactosidase / Q000008:administration & dosage*
chemical_list: D007527:Isoenzymes; D000519:alpha-Galactosidase; C459420:agalsidase beta