Matsuda M, Akatsuka N, Yamaguchi T, Saito T, Takahashi H
Jpn Heart J. 1977 Sep;18(5):638-51. doi: 10.1536/ihj.18.638.
Systolic time intervals (STIs) were measured in 57 patients with progressive muscular dystrophy (PMD) of the Duchenne type, and were correlated with the stages of physical disability. The total electromechanical systole (Q-A2), pre-ejection period (PEP), and left ventricular ejection time (LVET) were corrected for heart rate using the linear regression equations calculated from 91 normal subjects. Each systolic time interval index (STII), i.e. corrected STI, was analyzed. In 15 ambulatory patients (Group P-1), the STIIs did not differ significantly from those in normal controls. In 37 patients who were unable to walk (Group P-2) and 5 patients who were confined to bed (Group P-3), there was a significant increase (p less than 0.001) in the PEP index (PEPI), a decrease (p less than 0.001) in the LVET index (LVETI), and an increase (p less than 0.001) in the PEP/LVET compared with those in controls. The PEPI (p less than 0.001), LVETI (p less than 0.025), and PEP/LVET (p less than 0.001) in Group P-2 differed significantly from those in Group P-1. In Group P-3, the PEPI (p less than 0.005), LVET (p less than 0.001), and PEP/LVET (p less than 0.001) were significantly changed in comparison with those in Group P-2. Remarkable myocardial histopathologic changes suggestive of dystrophy were revealed in autopsies of 3 patients who had been in the severely disabled stage and had shown impaired STIs. It is suggested that cardiac function in patients with moderate to advanced PMD of the Duchenne type is deteriorated in proportion to the disability stages, i.e. the severity of changes in the skeletal muscles.
对57例杜氏型进行性肌营养不良（PMD）患者测量了收缩期时间间期（STIs），并将其与身体残疾阶段相关联。使用从91名正常受试者计算出的线性回归方程对总电机械收缩期（Q - A2）、射血前期（PEP）和左心室射血时间（LVET）进行心率校正。分析了每个收缩期时间间期指数（STII），即校正后的STI。在15名能走动的患者（P - 1组）中，STIIs与正常对照组无显著差异。在37名不能行走的患者（P - 2组）和5名卧床的患者（P - 3组）中，与对照组相比，PEP指数（PEPI）显著升高（p < 0.001），LVET指数（LVETI）降低（p < 0.001），PEP/LVET升高（p < 0.001）。P - 2组的PEPI（p < 0.001）、LVETI（p < 0.025）和PEP/LVET（p < 0.001）与P - 1组有显著差异。在P - 3组中，与P - 2组相比，PEPI（p < 0.005）、LVET（p < 0.001）和PEP/LVET（p < 0.001）有显著变化。在3名处于严重残疾阶段且STIs受损的患者尸检中发现了提示营养不良的显著心肌组织病理学变化。提示杜氏型中度至重度PMD患者的心脏功能与残疾阶段成比例恶化，即与骨骼肌变化的严重程度相关。
