Effects of enzyme-replacement therapy in patients with Anderson-Fabry disease: a prospective long-term cardiac magnetic resonance imaging study (Articolo in rivista)

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  • Effects of enzyme-replacement therapy in patients with Anderson-Fabry disease: a prospective long-term cardiac magnetic resonance imaging study (Articolo in rivista) (literal)
Anno
  • 2009-01-01T00:00:00+01:00 (literal)
Alternative label
  • Imbriaco M; Pisani A; Spinelli L; Cuocolo A; Messalli G; Capuano E; Marmo M; Liuzzi R; Visciano B; Cianciaruso B; Salvatore M (2009)
    Effects of enzyme-replacement therapy in patients with Anderson-Fabry disease: a prospective long-term cardiac magnetic resonance imaging study
    in Heart (Lond., 1996)
    (literal)
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  • Imbriaco M; Pisani A; Spinelli L; Cuocolo A; Messalli G; Capuano E; Marmo M; Liuzzi R; Visciano B; Cianciaruso B; Salvatore M (literal)
Pagina inizio
  • 1103 (literal)
Pagina fine
  • 1107 (literal)
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  • 95 (literal)
Rivista
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  • ISI Web of Science (WOS) (literal)
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  • 1. Univ Naples Federico 2, Dept Biomorphol & Funct Sci, Naples, Italy 2. Univ Naples Federico 2, Dept Nephrol, Naples, Italy 3. Univ Naples Federico 2, Dept Clin Med Cardiovasc & Immunol Sci, Naples, Italy 4. Univ Naples Federico 2, Dept Natl Res Council, IBB, CNR, Naples, Italy (literal)
Titolo
  • Effects of enzyme-replacement therapy in patients with Anderson-Fabry disease: a prospective long-term cardiac magnetic resonance imaging study (literal)
Abstract
  • Background: Anderson-Fabry disease is a multisystem X linked disorder of lipid metabolism frequently associated with cardiac symptoms, including left ventricular (LV) hypertrophy gradually impairing cardiac function. Evidence showing that enzyme-replacement therapy (ERT) can be effective in reducing LV hypertrophy and improving myocardial function in the long term is limited. Objective: This study aimed to assess the long-term effects of ERT with recombinant alpha-galactosidase A (agalsidase beta, Fabrazyme) on LV function and myocardial signal intensity in 11 patients with Anderson Fabry disease. Patients: Eleven patients (eight males, three females) with varying stages of genetically confirmed Anderson Fabry disease were examined by means of physical examination and magnetic resonance imaging before ERT with agalsidase beta at 1 mg/kg every other week (study 1) and after a mean treatment duration of 45 months (study 2). Results: At 45 months of treatment, LV mass and LV wall thickness had significantly reduced: 188 (SD 60) g versus 153 (47) g, and 16 (4) mm versus 14 (4) mm, respectively. Furthermore, a significant reduction in myocardial T2 relaxation times was noted in all myocardial regions, that is, interventricular septum 80 (5) ms versus 66 (8) ms, apex 79 (10) ms versus 64 (10) ms, and lateral wall 80 (8) ms versus 65 (16) ms. Changes in LV ejection fraction were not significant. Amelioration of clinical symptoms was observed in all patients. Conclusions: Long-term therapy with agalsidase beta at 1 mg/kg every 2 weeks was effective in significantly reducing LV hypertrophy, improving overall cardiac performance and ameliorating clinical symptoms in patients with Anderson-Fabry disease. (literal)
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