Heart disease in thalassemia intermedia: a review of the underlying pathophysiology

摘要: Heart disease is the leading cause of mortality and one main causes morbidity in beta-thalassemia. The clinical spectrum thalassemia syndrome ranges from severe, transfusion--dependent major asymptomatic carrier state. Thalassemia intermedia represents a milder form usually transfusion-independent. Two factors determine cardiac this form. One high output state that results chronic tissue hypoxia hypoxia-induced compensatory reactions. other vascular involvement leads to an increased pulmonary resistance systemic stiffness. Valvular abnormalities iron overload also contribute less extent. As result, both right left ventricles have maintain level through stiff bed. Right heart with age-related hypertension followed by congestive failure dominates picture. Although affected, systolic ventricular function preserved but may be decompensated under conditions characterized excessive work load.