Heart Disease in Thalassemia Intermedia

Thalassemia intermedia (TI) presents with a clinical phenotype ranging between the severe, transfusion-dependent TM and the asymptomatic carrier state. Cardiovascular complications represent the primary cause of mortality both in TM and in TI. Several factors have been reported to interfere in the p...

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Bibliographic Details
Published inHemoglobin Vol. 33; no. S1; pp. S170 - S176
Main Authors Aessopos, A., Tsironi, M., Andreopoulos, A., Farmakis, D.
Format Journal Article
LanguageEnglish
Published England Informa UK Ltd 2009
Taylor & Francis
Subjects
beta-Thalassemia - complications
Elastic tissue
Heart Diseases - etiology
Heart Diseases - pathology
Heart Failure
Hemolysis
Humans
Phenotype
Pulmonary hypertension
Thalassemia intermedia
Ventricular Remodeling
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Summary:Thalassemia intermedia (TI) presents with a clinical phenotype ranging between the severe, transfusion-dependent TM and the asymptomatic carrier state. Cardiovascular complications represent the primary cause of mortality both in TM and in TI. Several factors have been reported to interfere in the pathophysiology of cardiovascular abnormalities in TI, including high output state, vascular abnormalities, endothelial dysfunction, elastic tissue disorders, hypercoagulability, valvular abnormalities and, to a less extent, iron overload. These mechanisms end up affecting both left and right heart, hence leading to ventricular remodeling and finally heart failure.
Bibliography:ObjectType-Article-2
SourceType-Scholarly Journals-1
ObjectType-Feature-3
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ObjectType-Review-1
ISSN:0363-0269
1532-432X
DOI:10.3109/03630260903351676