Mechanisms of and obstacles to iron cardiomyopathy in thalassemia.
Thalassemia is anemia of variable severity, arising from mutations of genes encoding the hemoglobin alpha and beta chains. Severe thalassemia is associated with iron overload, tissue lesions, and high risk for cardiovascular complications, and iron-mediated cardiomyopathy is the main cause of death in this condition. Thalassemia major (TM) patients exhibit cardiovascular abnormalities consistent with chronic anemia; these include enlargement of the ventricular chambers, increased cardiac output, and reduced total vascular resistance. Cardiac iron overload in TM patients due to long-term transfusion can cause further chamber dilation, decreased contractility, and arrhythmia. Paradoxically, many such patients remain asymptomatic until decompensation occurs. For decades, magnetic resonance imaging and echocardiography have been performed to detect advanced cardiac dysfunction; however, reliable evaluation tools for the early detection of cardiac abnormalities are currently in demand. This article reviews the mechanisms underlying the development of heart disease in thalassemia and strategies for therapeutic intervention in TM patients with congestive heart failure.
Peng
Ching-Tien
CT
Department of Laboratory Medicine, China Medical University Hospital, Taichung, Taiwan. pct@mail.cmuh.org.tw
Chang
Jeng-Sheng
JS
Wu
Kang-Hsi
KH
Tsai
Chang-Hai
CH
Lin
Huan-Sheng
HS
D001794:Blood Pressure; D009202:Cardiomyopathies / Q000139:chemically induced / Q000209:etiology*; D003676:Deferoxamine / Q000627:therapeutic use; D004562:Electrocardiography; D006321:Heart / Q000502:physiology* / Q000503:physiopathology; D006333:Heart Failure / Q000139:chemically induced / Q000209:etiology*; D006801:Humans; D007501:Iron / Q000009:adverse effects*; D007502:Iron Chelating Agents / Q000627:therapeutic use; D013789:Thalassemia / Q000150:complications* / Q000503:physiopathology; D017539:Tunica Intima / Q000503:physiopathology; D017540:Tunica Media / Q000503:physiopathology
D007502:Iron Chelating Agents; D007501:Iron; D003676:Deferoxamine