Document Detail

Pulmonary hypertension in beta-thalassemia.
MedLine Citation:
PMID:  16339682     Owner:  NLM     Status:  MEDLINE    
Cardiac involvement represents the leading cause of mortality in both forms of beta-thalassemia, namely, thalassemia major (TM) and thalassemia intermedia (TI), and pulmonary hypertension (PHT) is part of the cardiopulmonary complications of the disease. PHT was initially documented in a small group of TI patients with right heart failure. In a subsequent study of a large 110-patient series, aged 32.5 +/- 11.4 years, age-related PHT was encountered in nearly 60% of cases, having caused right heart failure in six of them; interestingly, all patients had preserved left ventricular systolic function. Conflicted evidence, however, existed with respect to the development of PHT in heterogeneously treated and young TM populations. To resolve this discrepancy, a recent study compared cardiac disease between two large aged-matched groups of TM (n = 131) and TI (n = 74) patients, both treated uniformly in the currently accepted manner (regular transfusion and chelation therapy in TM, absence of any particular treatment in TI); well-treated TM patients, in contrast to TI patients, did not develop PHT, while systolic left ventricular dysfunction was present only in TM cases. PHT in beta-thalassemia results from a rather complex pathophysiology, in which chronic tissue hypoxia seems to hold a key role. Although both forms of the disease share a common molecular background, the diverse severity of the genetic defect and of the resulting clinical phenotype require a different therapeutic approach. Regular lifelong therapy in TM patients eliminates chronic hypoxia, thereby preventing PHT, whereas the absence of systematic treatment in TI leads to a cascade of reactions that compensate for chronic anemia, but at the same time allow the development of PHT.
Athanasios Aessopos; Dimitrios Farmakis
Publication Detail:
Type:  Journal Article; 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:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  7506858     Medline TA:  Ann N Y Acad Sci     Country:  United States    
Other Details:
Languages:  eng     Pagination:  342-9     Citation Subset:  IM    
First Department of Internal Medicine, University of Athens Medical School, Laiko General Hospital, 17 Aghiou Thoma St., Athens 115 27, Greece.
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MeSH Terms
Age Factors
Anoxia / etiology,  physiopathology
Blood Transfusion
Cardiac Output
Chelation Therapy
Heart Failure / etiology,  physiopathology
Hypertension, Pulmonary / etiology*,  physiopathology
Iron Overload / etiology,  physiopathology
Middle Aged
Thromboembolism / etiology,  physiopathology
Vascular Resistance
Ventricular Dysfunction, Left / etiology,  physiopathology
beta-Thalassemia / complications*,  drug therapy,  physiopathology,  therapy

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