TITLE

Systemic disease and the heart: Effects of enzyme-replacement therapy in patients with Anderson-Fabry disease: a prospective long-term cardiac magnetic resonance imaging study

AUTHOR(S)
Imbriaco, M.; Pisani, A.; Spinelli, L.; Cuocolo, A.; Messalli, G.; Capuano, E.; Marmo, M.; Liuzzi, R.; Visciano, B.; Cianciaruso, B.; Salvatore, M.
PUB. DATE
July 2009
SOURCE
Heart;Jul2009, Vol. 95 Issue 13, p26
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
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 α-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.
ACCESSION #
43671861

 

Related Articles

  • Utility of gadolinium enhanced cardiovascular MRI to differentiate Fabry's disease from other causes of hypertrophic cardiomyopathy. Hoey, Edward T. D.; Neil-Gallagher, Ed // Postgraduate Medical Journal;Dec2012, Vol. 88 Issue 1046, p731 

    The article describes the case of a 65-year-old man who was admitted to a cardiology clinic for investigation of exertional dyspnoea and atypical chest pain. It discusses the results of a gadolinium-enhanced cardiovascular magnetic resonance imaging (CMRI) conducted on the patient to...

  • Rapid and accurate measurement of LV mass by biplane real-time 3D echocardiography in patients with concentric LV hypertrophy: comparison to CMR. Sing-Chien Yap; Robert-Jan M. van Geuns; Attila Nemes; Folkert J. Meijboom; Jackie S. McGhie; Marcel L. Geleijnse; Maarten L. Simoons; Jolien W. Roos-Hesselink // European Journal of Echocardiography;Mar2008, Vol. 9 Issue 2, p255 

    Aims To evaluate the accuracy of real-time three-dimensional echocardiography (RT3DE) using a biplane and multiplane method in determining left ventricular (LV) mass compared to cardiac magnetic resonance imaging (CMR). Methods and results LV mass was measured in 18 adult patients with...

  • Steep left ventricle to aortic root angle and hypertrophic obstructive cardiomyopathy: study of a novel association using three-dimensional multimodality imaging. Kwon, D. H.; Smedira, N. G.; Popovic, Z. B.; Lytle, B. W.; Setser, R. M.; Thamilarasan, M.; Schoenhagen, P.; Flamm, S. D.; Lever, H. M.; Desai, M. Y. // Heart;Nov2009, Vol. 92 Issue 21, p10 

    Background: Patients with hypertrophic cardiomyopathy (HCM) exhibit a difference in left ventricular outflow tract (LVOT) obstruction, independently of basal septal thickness (BST). Some patients with HCM have a steeper left ventricle to aortic root angle than controls. Objective: To test the...

  • Apical Hypertrophic Cardiomyopathy. Ates, Mehmet; Kwong, Raymond Y.; Lipton, Martin J.; Tatli, Servet; Stainback, Raymond F. // Texas Heart Institute Journal;2006, Vol. 33 Issue 3, p408 

    The article reports the case of a 75-year-old white man with abnormal electrocardiographic findings. The patient has a family history of heart disease and faces the risk of having hyperlipidemia. An electrocardiogram examination showed sinus bradycardia, left ventricular hypertrophy and negative...

  • Atlantis Massif. Shah, Rajnil; Kirsch, Jacobo // International Journal of Cardiovascular Imaging;Aug2012, Vol. 28 Issue 6, p1279 

    No abstract available.

  • SCMR president's page. Nagel, Eike // Journal of Cardiovascular Magnetic Resonance (BioMed Central);2010, Vol. 12, p23 

    News from the Society for Cardiovascular Magnetic Resonance

  • Comprehensive phenotyping of salt-induced hypertensive heart disease in living mice using cardiac magnetic resonance. Cochet, Hubert; Lefrançois, William; Montaudon, Michel; Laurent, François; Pourtau, Line; Miraux, Sylvain; Parzy, Elodie; Franconi, Jean-Michel; Thiaudière, Eric // European Radiology;Feb2013, Vol. 23 Issue 2, p332 

    Objectives: To characterise the effects of high-salt diet (HSD) on left ventricular (LV) mass, systolic function and coronary reserve in living mice using cardiac magnetic resonance imaging (MRI). Methods: Thirty C57BL/6 1-month-old female mice were fed either a control ( n = 15) or an HSD ( n =...

  • Familial Hypertrophic Cardiomyopathy with Atypical Location. Guler, Ahmet; Tigen, Kursat M.; Aung, Soe M.; Karabay, Can Yucel; Karaahmet, Tansu; Kirma, Cevat // Texas Heart Institute Journal;2012, Vol. 39 Issue 1, p138 

    The article describes the clinical case of a 25-year-old woman who was diagnosed with familial hypertrophic cardiomyopathy (HCM). The patient's electrocardiogram (ECG) revealed ST-segment depressions and T-wave, while cardiovascular magnetic resonance (CMR) showed hypertrophied inferior and...

  • Cardiovascular Magnetic Resonance Imaging of Non-ischaemic Heart Disease: Established and Emerging Applications Bohl, Steffen; Schulz-Menger, Jeanette // Heart, Lung & Circulation;Mar2010, Vol. 19 Issue 3, p117 

    This review aims to summarise state-of-the-art CMR-methodology, established applications, future directions and potentials in the assessment of patients with NonIschaemic-Heart Disease. CMR offers the unique possibility to differentiate myocardial tissue changes. One of the most challenging...

Share

Read the Article

Courtesy of VIRGINIA BEACH PUBLIC LIBRARY AND SYSTEM

Sign out of this library

Other Topics