Document Detail


Value of sequential monitoring of left ventricular ejection fraction in the management of thalassemia major.
MedLine Citation:
PMID:  15001468     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Bernard A Davis; Caoimhe O'Sullivan; Peter H Jarritt; John B Porter
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Publication Detail:
Type:  Journal Article     Date:  2004-03-04
Journal Detail:
Title:  Blood     Volume:  104     ISSN:  0006-4971     ISO Abbreviation:  Blood     Publication Date:  2004 Jul 
Date Detail:
Created Date:  2004-06-21     Completed Date:  2004-08-27     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  7603509     Medline TA:  Blood     Country:  United States    
Other Details:
Languages:  eng     Pagination:  263-9     Citation Subset:  AIM; IM    
Affiliation:
Department of Haematology, Royal Free and University College Medical School, and Department of Research and Development, University College London Hospitals, United Kingdom.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Blood Transfusion / adverse effects
Chelation Therapy / adverse effects*
Deferoxamine / therapeutic use*
Female
Humans
Male
Physical Exertion
Prospective Studies
Radionuclide Ventriculography / methods
Survival Analysis
Treatment Outcome
Ventricular Function, Left / drug effects,  physiology*
beta-Thalassemia / drug therapy*,  physiopathology*
Chemical
Reg. No./Substance:
70-51-9/Deferoxamine

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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