大型β-thalassemia中的心血管功能和治疗:美国心脏协会的一致声明
Circulation
|June 19, 2013
概括
大型β-thalassemia (TM) 的心力衰竭主要是由心脏铁过载引起的. 早期诊断使用心脏T2*MRI和及时的铁化疗法对于管理心脏功能障碍和预防TM患者致命结局至关重要.
科学领域:
- 心脏病学 心脏病学
- 血液学 血液学 血液学
- 医学共识 医学共识
背景情况:
- 心力衰竭是 β-thalassemia major (TM) 死亡的主要原因,主要是由于心脏铁积累.
- 在TM患者中,心血管适应慢性贫血使心室功能障碍的诊断复杂化.
- 对于心脏铁过载的现有治疗方法缺乏广泛的随机对照试验 (RCT) 数据,特别是急性心力衰竭.
研究的目的:
- 建立一个专家的共识,诊断和管理心脏功能障碍在β-thalassemia大.
- 突出心脏铁量化的重要性及其对心力衰竭发展的影响.
- 总结当前关于铁化剂对心脏铁过载的疗效的知识.
主要方法:
- 基于现有证据和临床经验的专家共识发展.
- 主要通过心脏T2*磁共振成像 (MRI) 评估心脏铁.
- 使用非侵入性技术评估心脏功能,重点是连续测量.
- 对铁化剂有效性的证据的审查 (德菲洛克萨明,德菲利普龙,德菲拉西洛克斯).
主要成果:
- 心脏T2*<10毫秒是TM患者心力衰竭的关键预测指标.
- 血清费里丁和肝脏铁度不足以评估心脏铁负担.
- 随机试验证明了deferiprone的疗效优于deferoxamine,联合疗法优于单独的deferoxamine,以及deferasirox与deferoxamine在减少心脏铁中的相当性.
- 急性不补偿性心力衰竭需要用静脉注射的德费洛克萨和口服的德费里进行紧急治疗.
结论:
- 使用心脏T2*MRI对心脏铁过载的早期和准确的诊断对于预防TM心力衰竭至关重要.
- 铁化疗法,特别是基于德费里的疗法,是控制心脏铁毒性的关键.
- 在TM中急性心力衰竭的管理需要专门的护理,避免从利尿剂或内类药物引起的并发症.
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