Progression of cardiac involvement in patients with limb-girdle type 2 and Becker muscular dystrophies: A 9-year follow-up study
Progression of cardiac involvement in patients with limb-girdle type 2 and Becker muscular dystrophies: A 9-year follow-up study
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DOI：
10.1016/j.ijcard.2014.12.090
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发表时间：
2015-03-01
影响因子：
3.5
通讯作者：
Vissing, John
中科院分区：
文献类型：
--
作者：
Petri, Helle;Sveen, Marie-Louise;Vissing, John
目的：探讨带状肌病合并Becker型肌营养不良患者心脏受累的程度和进展。方法：对10 0例肢带型2型肌营养不良患者(A~L型)和30例骨质疏松症患者进行心电图和超声心动图检查，并进行动态心电图监测。结果：中位死亡年龄8.9年(0.4~13.7岁)，其中肢带型2例(n=10，平均61+/11岁)，BMD(n=2，年龄43岁和45岁)。在其余118例患者中，89例完成了随访：(n=48+/-13岁)和骨密度(n=25，年龄40+/-13岁)。在骨密度方面，左心室射血分数从60%(10-62)下降到50%(10-)，p=0.02，相当于每年一个百分点的下降。在LGMD2患者中，LGMD 2I型患者(n=28)的LVEF从59%(15-72)下降到55%(20-61)，p=0.03，即每年下降0.4个百分点，
Aim: To assess the degree and progression of cardiac involvement in patients with limb-girdle type 2 (LGMD2) and Becker muscular dystrophies (BMD).Methods: A follow-up study of 100 LGMD2 (types A-L) and 30 BMD patients assessed by electrocardiogram (ECG) and echocardiography, supplemented by Holter-monitoring at follow-up.Results: After a median of 8.9 years (range 0.4-13.7), twelve patients had died: LGMD2 (n=10, mean age 61 +/- 11 years), BMD (n =2, age 43 and 45 years). Of the remaining 118 patients, 89 completed follow-up: LGMD2 (n=64, age 48 +/- 13 years) and BMD (n=25, age 40 +/- 13 years).In BMD, LVEF decreased from 60% (10-62) to 50% (10-64), p=0.02 corresponding to a one percentage drop annually. Among patients with LGMD2, LVEF decreased significantly in patients with LGMD type 2I (n=28) from 59% (15-72) to 55% (20-61), p=0.03, i.e. a 0.4 percentage drop annually, and LVEF