Nagai T
First Department of Internal Medicine, Niigata University, School of Medicine, Japan.
Jpn Circ J. 1989 May;53(5):406-15. doi: 10.1253/jcj.53.406.
The relation between heart function and the prognosis of patients with Duchenne muscular dystrophy (DMD) was analyzed in 27 non-survivors and 40 survivors by fractional shortening (FS) of the left ventricle from M-mode echocardiogram, and by the PEP/ET ratio from the systolic time interval. The patients were divided into 5 groups; (1) patients who died of congestive heart failure (group 1), (2) those who died of congestive heart failure and respiratory failure (group 2), (3) those who died of respiratory failure (group 3), (4) those who died suddenly of undefined etiology (group 4), and (5) survivors (group 5). Data from non-invasive cardiac function tests were analyzed retrospectively for 2 years and compared at 2 years, 1 year and about 3 months before their deaths in cases of group 1, 2, 3 and 4, and the data obtained at the same period were also compared with those of survivors (group 5). The age at death in group 1 (17.3 +/- 4.9 years) was significantly younger than that in group 2 (25.3 +/- 4.2 years), however, it did not statistically differ from group 3 (19.8 +/- 3.3 years old). The values of FS in group 1 were significantly lower than those in group 3, 4 and 5 at three examinations, whereas no difference in PEP/ET was observed among them. Cardiothoracic ratio (CTR) on chest X-ray in group 1 was not significantly different from other groups in each period, but the left ventricle dilated rapidly in the terminal stage of congestive heart failure which could be more precisely detected by the M-mode echocardiogram. These results indicate that in DMD, early development of congestive heart failure was associated with a poor prognosis. FS was a most sensitive non-invasive tool in predicting the prognosis. A significant reduction in FS was detectable 2 years before death. Progression in abnormality of left ventricular dimension as well as in FS may be another useful index for evaluating the prognosis of patients dying from congestive heart failure.
通过M型超声心动图测量左心室缩短分数（FS）以及通过收缩期时间间期测量PEP/ET比值，对27例杜氏肌营养不良症（DMD）非存活患者和40例存活患者的心功能与预后的关系进行了分析。患者被分为5组：（1）死于充血性心力衰竭的患者（第1组），（2）死于充血性心力衰竭和呼吸衰竭的患者（第2组），（3）死于呼吸衰竭的患者（第3组），（4）病因不明突然死亡的患者（第4组），以及（5）存活患者（第5组）。对非侵入性心功能测试数据进行了为期2年的回顾性分析，并在第1、2、3和4组患者死亡前2年、1年和大约3个月时进行比较，同时将同期获得的数据与存活患者（第5组）的数据进行比较。第1组患者的死亡年龄（17.3±4.9岁）显著低于第2组（25.3±4.2岁），但与第3组（19.8±3.3岁）无统计学差异。在三次检查中，第1组的FS值显著低于第3、4和5组，而它们之间的PEP/ET无差异。第1组胸部X线的心胸比（CTR）在各时期与其他组无显著差异，但在充血性心力衰竭末期左心室迅速扩张，这通过M型超声心动图能更精确地检测到。这些结果表明，在DMD中，充血性心力衰竭的早期发生与预后不良相关。FS是预测预后最敏感的非侵入性工具。在死亡前2年可检测到FS显著降低。左心室大小异常以及FS的进展可能是评估死于充血性心力衰竭患者预后的另一个有用指标。
