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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 (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 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 Social Bookmarking                   What's this? Submitted August 18, 2003 Accepted January 27, 2004 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* Haematology, Royal Free and University College Medical School, London, United Kingdom Research and Development, University College London Hospitals, London, United Kingdom Nuclear Medicine, University College London Hospitals, London, United Kingdom * Corresponding author; email: j.porter{at}ucl.ac.uk. Regular monitoring of left ventricular ejection fraction (LVEF) for thalassemia major (TM) is widely practiced, but its value in informing iron chelation treatment is unclear. 81 TM patients without previous cardiac history received quantitative yearly monitoring of LVEF by radionuclide ventriculography for a median of 6.0 years (interquartile range: 2-12 years). Intra-and inter-observer reproducibility for LVEF determination were both <3%. LVEF values pre- and post-transfusion did not differ and exercise stressing did not reliably expose underlying cardiomyopathy. An absolute LVEF of <45% or fall of >10 percentage units was significantly associated with subsequent development of symptomatic cardiac disease (p<0.001) and death (p=0.001) with a median interval between the first abnormal LVEF and development of symptomatic heart disease of 3.5 years, allowing time for intervention. In 34 patients where the LVEF was <45% or fell by >10 percentage units, intensification of chelation was recommended (21 with subcutaneous and 13 with intravenous deferoxamine). All 27 patients who complied with intensification survive, while the 7 who failed to comply died (p<0.0001). The Kaplan-Meier estimate of survival beyond 40 years for all 81 patients is 83%. Sequential quantitative monitoring of LVEF is valuable for assessing cardiac risk and for identifying TM patients requiring chelation intensification. 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