Document Detail

Physiology and pathophysiology of iron cardiomyopathy in thalassemia.
MedLine Citation:
PMID:  16339687     Owner:  NLM     Status:  MEDLINE    
Iron cardiomyopathy remains the leading cause of death in patients with thalassemia major. Magnetic resonance imaging (MRI) is ideally suited for monitoring thalassemia patients because it can detect cardiac and liver iron burdens as well as accurately measure left ventricular dimensions and function. However, patients with thalassemia have unique physiology that alters their normative data. In this article, we review the physiology and pathophysiology of thalassemic heart disease as well as the use of MRI to monitor it. Despite regular transfusions, thalassemia major patients have larger ventricular volumes, higher cardiac outputs, and lower total vascular resistances than published data for healthy control subjects; these hemodynamic findings are consistent with chronic anemia. Cardiac iron overload increases the relative risk of further dilation, arrhythmias, and decreased systolic function. However, many patients are asymptomatic despite heavy cardiac burdens. We explore possible mechanisms behind cardiac iron-function relationships and relate these mechanisms to clinical observations.
John C Wood; Cathleen Enriquez; Nilesh Ghugre; Maya Otto-Duessel; Michelle Aguilar; Marvin D Nelson; Rex Moats; Thomas D Coates
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Annals of the New York Academy of Sciences     Volume:  1054     ISSN:  0077-8923     ISO Abbreviation:  Ann. N. Y. Acad. Sci.     Publication Date:  2005  
Date Detail:
Created Date:  2005-12-12     Completed Date:  2006-07-13     Revised Date:  2014-09-08    
Medline Journal Info:
Nlm Unique ID:  7506858     Medline TA:  Ann N Y Acad Sci     Country:  United States    
Other Details:
Languages:  eng     Pagination:  386-95     Citation Subset:  IM    
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MeSH Terms
Blood Transfusion / adverse effects
Cardiac Output
Cardiomegaly / etiology
Cardiomyopathies / diagnosis,  etiology,  physiopathology*
Chelation Therapy
Combined Modality Therapy
Heart / physiopathology
Iron / metabolism*
Iron Chelating Agents / therapeutic use
Iron Overload / etiology,  physiopathology*
Magnetic Resonance Imaging
Models, Biological
Stroke Volume
Thalassemia / complications*,  drug therapy,  metabolism,  therapy
Vascular Resistance
Grant Support
1 R01 HL 75592-01A1/HL/NHLBI NIH HHS; M01 RR000043/RR/NCRR NIH HHS; M01 RR000043-460925/RR/NCRR NIH HHS; M01 RR000043-460988/RR/NCRR NIH HHS; RR0043-43/RR/NCRR NIH HHS
Reg. No./Substance:
0/Buffers; 0/Iron Chelating Agents; E1UOL152H7/Iron

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