Role of cardiac MRI in evaluating patients with Anderson-Fabry disease: Assessing cardiac effects of long-term enzyme replacement therapy(580 views) Messalli G, Imbriaco M, Avitabile G, Russo R, Iodice D, Spinelli L, Dellegrottaglie S, Cademartiri F, Salvatore M, Pisani A
Radiol Med (ISSN: 0033-8362, 1826-6983, 1826-6983electronic), 2012 Feb; 117(1): 19-28.
Dipartimento di Scienze Biomorfologiche e Funzionali, Università Degli Studi 'Federico II', Via Posillipo 196, Napoli 80123, Italy
Dipartimento di Nefrologia, Università Degli Studi 'Federico II', Napoli, Italy
Dipartimento di Medicina Clinica e Scienze Cardiovascolari Ed Immunologiche, Università Degli Studi 'Federico II', Napoli, Italy
Istituto di Biostrutture e Bioimmagini, CNR, Napoli, Italy
Azienda Ospedaliera-Universitaria di Parma, Parma, Italy
References: Not available.
Role of cardiac MRI in evaluating patients with Anderson-Fabry disease: Assessing cardiac effects of long-term enzyme replacement therapy
Purpose: Anderson-Fabry disease is a multisystemic disorder of lipid metabolism secondary to X-chromosome alterations and is frequently associated with cardiac manifestations such as left ventricular (LV) hypertrophy, gradually leading to an alteration in cardiac performance. The purpose of this study was to monitor, using magnetic resonance imaging (MRI), any changes produced by enzyme replacement therapy with agalsidase beta at the cardiac level in patients with Anderson-Fabry disease. Materials and methods: Sixteen (ten men, six women) patients with genetically confirmed Anderson-Fabry disease underwent cardiac MRI before starting enzyme replacement therapy (baseline study) and after 48 months of treatment with agalsidase beta at the dose of 1 mg/kg (follow-up study). Results: After 48 months of treatment, a significant reduction in LV mass and wall thickness was observed: 187±59 g vs. 149±44 g, and 16±3 mm vs. 13±3 mm, respectively. A significant reduction in T2 relaxation time was noted at the level of the interventricular septum (81±3 ms vs. 67±7 ms), at the apical level (80±8 ms vs. 63±6 ms) and at the level of the lateral wall (82±8 ms vs. 63±10 ms) (p0.05) (mean bias 1.0); however, an improvement was noted in the New York Heart Association (NYHA) class of the majority of patients (12/16) (p
Role of cardiac MRI in evaluating patients with Anderson-Fabry disease: Assessing cardiac effects of long-term enzyme replacement therapy
No results.
Role of cardiac MRI in evaluating patients with Anderson-Fabry disease: Assessing cardiac effects of long-term enzyme replacement therapy