PULMONARY ARTERY PRESSURE IN β - THALASSEMIA MAJOR-EFFECT OF CARNITINE

Abstract

The thalassemais are hereditary hemolytic anemias caused by mutations affecting synthesis of hemoglobin (Colah, 1998). Cardiac complications are the most common causes of death in patients with Cooley's anemia. Pulmonary hypertension may be another important factor contributing to the cardiac death in patients with thalassemia major (Du et al., 1997). Pulmonary hypertension is a multifactorial disorder in which different factors may prevail in every single patient. It is due to an elevated pressure in the pulmonary vascular bed caused by increased PVR and CO (Assospos et al., 1995). Increased PVR is due to hypoxemia, restriction of pulmonary vascular bed by chronic pulmonary hemosiderosis, and the prescence of a restrictive pulmonary disease in thalassemics. Increased CO is due to anemia, low PO., altered 0 delivery to the tissues and low 2,3 DPG in transfused blood ( Aessospos et al., 1995).