Westwood Mark A, Anderson Lisa J, Maceira Alicia M, Shah Farrukh T, Prescott Emma, Porter John B, Wonke Beatrix, Walker J Malcolm, Pennell Dudley J
Cardiovascular MR Unit, Royal Brompton Hospital and Imperial College, London, United Kingdom.
J Magn Reson Imaging. 2007 Jun;25(6):1147-51. doi: 10.1002/jmri.20915.
To determine the reference range in thalassemia major (TM) for left ventricular (LV) function.
We used cardiovascular magnetic resonance (CMR) to measure heart volumes and function in 81 TM patients with normal myocardial T2* measurements (T2* > 20 msec) and by inference without excess myocardial iron. Forty age- and gender-matched healthy controls were also studied.
Resting LV volumes and function normalized to body surface area differed significantly between TM patients and controls. The lower limit and the mean for ejection fraction (EF) were higher in TM patients (males 59 vs. 55%, mean 71% vs. 65%; females 63 vs. 59%, mean 71% vs. 67%; both P < 0.001). The upper limit and mean for end-diastolic volume index were higher in TM patients (males 152 vs. 105 mL/m(2), mean 97 vs. 84 mL/m(2); females 121 vs. 99 mL/m(2), mean 87 vs. 79 mL/m(2); both P < 0.05). In TM patients the cardiac index (P < 0.001) was increased.
At rest, TM patients with a normal myocardial T2* have different "normal" values for LV volume and function parameters compared to controls, and this has the potential to lead to a misdiagnosis of cardiomyopathy. We present new reference "normal" ranges in TM to alleviate this problem.
确定重型地中海贫血（TM）患者左心室（LV）功能的参考范围。
我们采用心血管磁共振（CMR）对81例心肌T2测量值正常（T2 > 20毫秒）且推断无过多心肌铁的TM患者进行心脏容积和功能测量。还研究了40名年龄和性别匹配的健康对照者。
TM患者与对照组相比，静息时左心室容积和功能经体表面积校正后存在显著差异。TM患者的射血分数（EF）下限和平均值更高（男性分别为59%对55%，平均值71%对65%；女性分别为63%对59%，平均值71%对67%；P均<0.001）。TM患者的舒张末期容积指数上限和平均值更高（男性分别为152对105 mL/m²，平均值97对84 mL/m²；女性分别为121对99 mL/m²，平均值87对79 mL/m²；P均<0.05）。TM患者的心指数增加（P < 0.001）。
静息时，心肌T2*正常的TM患者与对照组相比，左心室容积和功能参数的“正常”值不同，这有可能导致心肌病的误诊。我们提出了TM新的参考“正常”范围以缓解这一问题。
