Department of Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Am J Cardiol. 2010 May 15;105(10):1451-5. doi: 10.1016/j.amjcard.2009.12.070. Epub 2010 Mar 30.
The present study evaluated progressive cardiac dysfunction using serial circumferential strain (epsilon(cc)) measurements in patients with Duchenne muscular dystrophy (DMD). DMD is characterized by progressive cardiac dysfunction and myocardial fibrosis late in the disease process. We hypothesized that serial epsilon(cc) changes could be detected in individual patients with DMD during a time when the left ventricular ejection fraction (EF) changes are insignificant. Cardiac magnetic resonance imaging data from patients with DMD were evaluated. The left ventricular EF was calculated from steady-state free precession cine images and the composite epsilon(cc) measurement from tagged cine images. The serial epsilon(cc) and EF values for each patient were analyzed using the Wilcoxon sign rank test. Data from 51 patients with DMD (2 studies per patient, mean age at the initial study 11.8 +/- 3.5 years, range 7.4 to 25.4) were analyzed, with a mean interval between cardiac magnetic resonance studies of 15.6 +/- 6.0 months (range 6.2 to 28.1). In the interval between studies, the epsilon(cc) had decreased in all patients with DMD. The average decrease was 1.8 +/- 1.3 (p <0.001). However, the EF had decreased in 33 of the 51 patients and had increased in 18 of the 51 patients. On average, the EF decreased by 2.9 +/- 8.57% (p = NS). In conclusion, in patients with DMD, epsilon(cc) abnormalities indicate progression within a relatively short period when the EF changes were not significant. Serial epsilon(cc) measurements might provide reliable monitoring of the progression of DMD-associated cardiac dysfunction before overt heart failure develops, because it is more sensitive than the EF.
本研究通过连续测量环状应变（epsilon(cc)）评估杜兴氏肌营养不良症（DMD）患者的进行性心脏功能障碍。DMD 的特征是在疾病过程的后期出现进行性心脏功能障碍和心肌纤维化。我们假设，在左心室射血分数（EF）变化不明显的情况下，个别 DMD 患者的连续 epsilon(cc)变化可以被检测到。评估了 DMD 患者的心脏磁共振成像数据。通过稳态自由进动电影图像计算左心室 EF，并通过标记电影图像计算复合 epsilon(cc)测量值。使用 Wilcoxon 符号秩检验分析每位患者的连续 epsilon(cc)和 EF 值。分析了 51 例 DMD 患者的数据（每位患者 2 项研究，初始研究的平均年龄为 11.8 +/- 3.5 岁，范围为 7.4 至 25.4 岁），两次心脏磁共振检查之间的平均间隔为 15.6 +/- 6.0 个月（范围为 6.2 至 28.1）。在两次研究之间，所有 DMD 患者的 epsilon(cc)均下降。平均下降 1.8 +/- 1.3（p <0.001）。然而，EF 在 51 例患者中有 33 例下降，在 51 例患者中有 18 例增加。平均而言，EF 下降了 2.9 +/- 8.57%（p = NS）。总之，在 DMD 患者中，epsilon(cc)异常表明在 EF 变化不明显的相对较短时间内出现进展。连续的 epsilon(cc)测量可能在明显心力衰竭发生之前提供可靠的监测 DMD 相关心脏功能障碍的进展，因为它比 EF 更敏感。
