Document Detail

Beta-thalassemia cardiomyopathy: history, present considerations, and future perspectives.
MedLine Citation:
PMID:  20484195     Owner:  NLM     Status:  MEDLINE    
Beta-thalassemia is an inherited hemoglobin disorder resulting in chronic hemolytic anemia that typically requires life-long transfusion therapy. Although traditionally prevalent in the Mediterranean basin, Middle East, North India, and Southeast Asia, immigration of those populations to North America and Western Europe has rendered beta-thalassemia a global health problem. Cardiac complications represent the primary cause of mortality and one of the major causes of morbidity in those patients. Heart disease is mainly expressed by a particular cardiomyopathy that progressively leads to heart failure and death. The beta-thalassemia cardiomyopathy is mainly characterized by 2 distinct phenotypes, a dilated phenotype, with left ventricular dilatation and impaired contractility and a restrictive phenotype, with restrictive left ventricular filling, pulmonary hypertension, and right heart failure. The pathophysiology of the disorder is multifactorial, with a central role of myocardial iron overload and the significant contribution of immunoinflammatory mechanisms. Patients' management is demanding and requires a multidisciplinary approach, preferably in specialized centers.
Dimitrios T Kremastinos; Dimitrios Farmakis; Athanasios Aessopos; George Hahalis; Eftychia Hamodraka; Dimitrios Tsiapras; Andre Keren
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Circulation. Heart failure     Volume:  3     ISSN:  1941-3297     ISO Abbreviation:  Circ Heart Fail     Publication Date:  2010 May 
Date Detail:
Created Date:  2010-05-20     Completed Date:  2010-07-15     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101479941     Medline TA:  Circ Heart Fail     Country:  United States    
Other Details:
Languages:  eng     Pagination:  451-8     Citation Subset:  IM    
Second Department of Cardiology, Athens University Medical School, Attikon University Hospital, 1 Rimini St., Athens, Greece.
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MeSH Terms
Cardiomyopathies / epidemiology*,  physiopathology,  therapy
Heart Failure / epidemiology*,  physiopathology,  therapy
beta-Thalassemia / complications*,  physiopathology,  therapy

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