Abstract：
:Heart disease is the leading cause of mortality and one of the main causes of morbidity in beta-thalassemia. The clinical spectrum of the thalassemia syndrome ranges from the severe, transfusion--dependent thalassemia major and the asymptomatic carrier state. Thalassemia intermedia represents a milder form and is usually transfusion-independent. Two main factors determine cardiac disease in this form. One is the high output state that results from chronic tissue hypoxia and from hypoxia-induced compensatory reactions. The other is the vascular involvement that leads to an increased pulmonary vascular resistance and an increased systemic vascular stiffness. Valvular abnormalities and iron overload also contribute to a less extent. As a result, both right and left ventricles have to maintain a high cardiac output level through a stiff vascular bed. Right heart involvement with age-related pulmonary hypertension followed by congestive heart failure dominates the clinical picture. Although the left heart is also affected, systolic left ventricular function is usually preserved but this may also be decompensated under conditions characterized by excessive cardiac work load.
journal_name
Haematologicajournal_title
Haematologicaauthors
Aessopos A,Kati M,Farmakis Ddoi
10.3324/haematol.10915subject
Has Abstractpub_date
2007-05-01 00:00:00pages
658-65issue
5eissn
0390-6078issn
1592-8721journal_volume
92pub_type
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