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Blood, 1 July 2004, Vol. 104, No. 1, pp. 263-269. Prepublished online as a Blood First Edition Paper on March 4, 2004; DOI 10.1182/blood-2003-08-2841. This Article Full Text Full Text (PDF) All Versions of this Article: 2003-08-2841v1 104/1/263    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Davis, B. A. Articles by Porter, J. B. Search for Related Content PubMed PubMed Citation Articles by Davis, B. A. Articles by Porter, J. B. Related Collections Red Cells Free Research Articles Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  RED CELLS Value of sequential monitoring of left ventricular ejection fraction in the management of thalassemia major Bernard A. Davis, Caoimhe O'Sullivan, Peter H. Jarritt, and John B. Porter From the Department of Haematology, Royal Free and University College Medical School, London; and the Department of Research and Development and the Institute of Nuclear Medicine, University College London Hospitals, United Kingdom. Regular monitoring of left ventricular ejection fraction (LVEF) for thalassemia major is widely practiced, but its informativeness for iron chelation treatment is unclear. Eighty-one patients with thalassemia major but no history of cardiac disease underwent quantitative annual LVEF monitoring by radionuclide ventriculography for a median of 6.0 years (interquartile range, 2-12 years). Intraobserver and interobserver reproducibility for LVEF determination were both less than 3%. LVEF values before and after transfusion did not differ, and exercise stress testing did not reliably expose underlying cardiomyopathy. An absolute LVEF of less than 45% or a decrease of more than 10 percentage units was significantly associated with subsequent development of symptomatic cardiac disease (P < .001) and death (P = .001), with a median interval between the first abnormal LVEF findings and the development of symptomatic heart disease of 3.5 years, allowing time for intervention. In 34 patients in whom LVEF was less than 45% or decreased by more than 10 percentage units, intensified chelation therapy was recommended (21 with subcutaneous and 13 with intravenous deferoxamine). All 27 patients who complied with intensification survived, whereas the 7 who did not comply died (P < .0001). The Kaplan-Meier estimate of survival beyond 40 years of age for all 81 patients is 83%. Sequential quantitative monitoring of LVEF is valuable for assessing cardiac risk and for identifying patients with thalassemia major who require intensified chelation therapy. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: L. J. Noetzli, S. M. Carson, A. S. Nord, T. D. Coates, and J. C. Wood Longitudinal analysis of heart and liver iron in thalassemia major Blood, October 1, 2008; 112(7): 2973 - 2978. [Abstract] [Full Text] [PDF] E. Angelucci, G. Barosi, C. Camaschella, M. D. Cappellini, M. Cazzola, R. Galanello, M. Marchetti, A. Piga, and S. Tura Italian Society of Hematology practice guidelines for the management of iron overload in thalassemia major and related disorders Haematologica, May 1, 2008; 93(5): 741 - 752. [Abstract] [Full Text] [PDF] D. J. Pennell, V. Berdoukas, M. Karagiorga, V. Ladis, A. Piga, A. Aessopos, E. D. Gotsis, M. A. Tanner, G. C. Smith, M. A. Westwood, et al. Randomized controlled trial of deferiprone or deferoxamine in beta-thalassemia major patients with asymptomatic myocardial siderosis Blood, May 1, 2006; 107(9): 3738 - 3744. [Abstract] [Full Text] [PDF] A. R. Cohen New Advances in Iron Chelation Therapy Hematology, January 1, 2006; 2006(1): 42 - 47. [Abstract] [Full Text] [PDF] K. N. Bhalla Epigenetic and Chromatin Modifiers As Targeted Therapy of Hematologic Malignancies J. Clin. Oncol., June 10, 2005; 23(17): 3971 - 3993. [Abstract] [Full Text] [PDF]

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