Pennell Dudley J
CMR Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK.
Ann N Y Acad Sci. 2005;1054:373-8. doi: 10.1196/annals.1345.045.
Magnetic resonance T2* values of the myocardium are directly related to tissue iron levels. Minor effects from myocardial oxygenation and fibrosis are overwhelmed by the highly dominant iron effect in clinically relevant levels of myocardial iron overload. Myocardial T2* values less than 20 ms indicate iron overload, and this is considered severe when T2* is less than 10 ms. Decreasing myocardial T2* levels are associated with systolic and diastolic ventricular dysfunction. Most recorded cases of heart failure in thalassemia to date have occurred in patients with very low T2* values (in the severe range). Exceptions to this have occurred in patients with other causes of heart failure such as concomitant congenital heart disease. In patients presenting with heart failure who undergo aggressive chelation with continuous intravenous deferoxamine, longitudinal studies show that myocardial T2* increases, and this is accompanied by increases in ejection fraction and relief of heart failure. In cross-sectional studies, the myocardial T2* and ejection fraction of patients on deferiprone was superior to that of patients on deferoxamine. Randomized controlled prospective trials comparing these two drugs for their action in clearing myocardial iron, as measured by myocardial T2*, are under way and should report in 2005/2006. These trials will clarify the role of different chelators in the management of myocardial iron overload and may be valuable in reducing the toll of death in thalassemia from heart failure.
心肌的磁共振T2值与组织铁水平直接相关。在临床相关的心肌铁过载水平下,心肌氧合和纤维化的微小影响被高度占主导的铁效应所掩盖。心肌T2值小于20毫秒表明存在铁过载,当T2小于10毫秒时则被认为是严重铁过载。心肌T2水平降低与心室收缩和舒张功能障碍相关。迄今为止,大多数记录在案的地中海贫血心力衰竭病例发生在T2值极低(处于严重范围)的患者中。也有例外情况,即患有其他心力衰竭病因(如合并先天性心脏病)的患者。对于出现心力衰竭并接受持续静脉注射去铁胺积极螯合治疗的患者,纵向研究表明心肌T2会升高,同时射血分数增加,心力衰竭症状缓解。在横断面研究中,则显示接受地拉罗司治疗患者的心肌T2和射血分数优于接受去铁胺治疗的患者。目前正在进行比较这两种药物清除心肌铁作用(通过心肌T2测量)的随机对照前瞻性试验,预计将于2005/2006年公布结果。这些试验将阐明不同螯合剂在治疗心肌铁过载中的作用,可能对降低地中海贫血心力衰竭的死亡率具有重要意义。