Cardiovascular complications in β-thalassemia patients.

Abstract

Thalassemia syndromes are the most common monogenic disease and a major public health burden in Thailand. The improvement in treatments, especially blood transfusion and iron chelation, results in significantly increased life expectancy of thalassemia patients. However, cardiovascular disorders remain the leading cause of death in both thalassemia major and thalassemia intermedia patients. Notably, clinical manifestations and pathophysiology of cardiovascular complications in both β-thalassemia subgroups are substantially different. The major cardiovascular complication of thalassemia major is left ventricular systolic dysfunction, whereas the major complications of thalassemia intermedia are pulmonary arterial hypertension and right-sided heart failure. In this article, we review the cardiovascular complications focusing on the mechanisms underlying these abnormalities in both thalassemia major and thalassemia intermedia.