Author(s): Peng, ct (Peng, Ching-Tien); Chang, js (Chang, Jeng-Sheng); Wu, kh (Wu, Kang-Hsi); Tsai, ch (Tsai, Chang-Hai); Lin, hs (Lin, Huan-Sheng) Title



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Author(s): Peng, CT (Peng, Ching-Tien); Chang, JS (Chang, Jeng-Sheng); Wu, KH (Wu, Kang-Hsi); Tsai, CH (Tsai, Chang-Hai); Lin, HS (Lin, Huan-Sheng)

Title: Mechanisms of and obstacles to iron cardiomyopathy in thalassemia

Source: FRONTIERS IN BIOSCIENCE, 13: 5975-5987 MAY 2008

Language: English

Document Type: Review

Author Keywords: beta-thalassemia; iron cardiomyopathy; iron cardiotoxicity; cardioprotective chelation therapy; mechanism; review

KeyWords Plus: SICKLE-CELL-DISEASE; HOMOZYGOUS BETA-THALASSEMIA; CONGESTIVE-HEART-FAILURE; LOADED CARDIAC MYOCYTES; TRANSFERRIN-BOUND IRON; MYOCARDIAL IRON; MAGNETIC-RESONANCE; PULMONARY-HYPERTENSION; DIASTOLIC FUNCTION; CHELATING THERAPY

Abstract: 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.

Addresses: [Peng, Ching-Tien] China Med Univ Hosp, Dept Lab Med, Taichung 40414, Taiwan; [Peng, Ching-Tien; Chang, Jeng-Sheng; Wu, Kang-Hsi; Tsai, Chang-Hai] China Med Univ Hosp, Dept Pediat, Taichung 40414, Taiwan; [Peng, Ching-Tien; Tsai, Chang-Hai] Asia Univ, Dept Biotechnol & Bioinformat, Taichung, Taiwan; [Peng, Ching-Tien; Wu, Kang-Hsi; Lin, Huan-Sheng] Taiwan Thalassemia Assoc, Taichung, Taiwan

Reprint Address: Peng, CT, China Med Univ Hosp, Dept Lab Med & Pediat, 2 Yuh Der Rd, Taichung 40414, Taiwan.

E-mail Address: pct@mail.cmuh.org.tw

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Times Cited: 2

Publisher: FRONTIERS IN BIOSCIENCE INC

Publisher Address: C/O NORTH SHORE UNIV HOSPITAL, BIOMEDICAL RESEARCH CENTER, 350 COMMUNITY DR, MANHASSET, NY 11030 USA

ISSN: 1093-9946

DOI: 10.2741/3130

29-char Source Abbrev.: FRONT BIOSCI

ISO Source Abbrev.: Front. Biosci.

Source Item Page Count: 13

Subject Category: Biochemistry & Molecular Biology; Cell Biology

ISI Document Delivery No.: 301GS


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