Heart Disease in Thalassemia Intermedia

Abstract

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.

Keywords:

• Thalassemia intermedia
• Pulmonary hypertension
• Hemolysis
• Elastic tissue