Cardiac failure in β-thalassemia: diagnosis, prevention and management

  • A. Aessopos | paola.granata@pagepress.org First Dept. of Internal Medicine, University of Athens Medical School, Laiko Hospital, Athens, Greece.
  • D. Farmakis First Dept. of Internal Medicine, University of Athens Medical School, Laiko Hospital, Athens, Greece.
  • V. Berdoukas Division of Haematology/Oncology, Children's Hospital, Los Angeles, CA, United States.

Abstract

Heart failure always represented and still remains the leading cause of mortality in β (β)-thalassemia, despite the therapeutic advances and the considerable amelioration of prognosis accomplished over the last decades. High cardiac output due to chronic anemia and myocardial iron overload due to repetitive blood transfusions are the two main pathogenetic mechanisms causing heart failure in β-thalassemia. In regularly treated thalassemia major patients, left ventricular dysfunction, resulting mainly from myocardial siderosis, is considered to be the primary cause of heart failure and thus the prevention, early recognition and effective management of iron overload is of key importance. However, the spectrum of cardiovascular complications that may ultimately lead to heart is wide and should be individually investigated in each one of the patients. Echocardiography is the main modality used for the regular follow-up and screening of asymptomatic patients and for the evaluation of patients with cardiac symptoms, while the T2* relaxation time provided by magnetic resonance imaging allows the accurate identification and quantification of myocardial iron burden and thus the proper guidance of iron chelation therapy.

 

近几十年来,尽管治疗方法取得进步和预断方法得到显著改进,但是心脏衰竭仍是引起β地中海贫血症患者死亡的主要原因。 慢性贫血导致的高心输出量和反复输血导致的心脏铁过载,是导致β地中海贫血患者心脏衰竭的两大发病机制。 在常规治疗的重型地中海贫血患者中,心脏铁质沉着病引起的可逆性左心室功能障碍,被认为是心脏衰竭的主要原因。因此,预防、早期确诊和有效控制铁过载至关重要。 然而,最终导致心脏衰竭的心血管并发症的症状繁多,应对每个患者单独进行检查。 超声心动图仪是用于无症状患者定期随访、筛查和诊断有心脏病症状患者的主要仪器,磁共振成象显示的T2*松弛时间可更准确地识别和量化心脏的铁负荷,有助于正确引导铁螯合疗法。

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Published
2011-12-30
Keywords:
thalassemia major, thalassemia intermedia, heart failure, iron overload, iron chelation, cardiovascular magnetic resonance, T2*.
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How to Cite
Aessopos, A., Farmakis, D., & Berdoukas, V. (2011). Cardiac failure in β-thalassemia: diagnosis, prevention and management. Thalassemia Reports, 1(1), e16. https://doi.org/10.4081/thal.2011.s2.e16