Prognostic impact of left ventricular noncompaction in patients with Duchenne/Becker muscular dystrophy - Prospective multicenter cohort study
Prognostic impact of left ventricular noncompaction in patients with Duchenne/Becker muscular dystrophy - Prospective multicenter cohort study
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DOI：
10.1016/j.ijcard.2012.12.058
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发表时间：
2013-10-03
影响因子：
3.5
通讯作者：
Komuro, Issei
中科院分区：
文献类型：
--
作者：
Kimura, Koichi;Takenaka, Katsu;Komuro, Issei
背景：左心室致密化不全（LVNC）的报道患病率差异很大，其预后影响仍有争议。我们试图澄清的患病率和预后的影响LVNC患者与Duchenne/Becker肌营养不良症（DMD/BMD）的方法：我们评估了LNVC的存在，DMD/BMD患者年龄4-64岁的研究进入（2007年7月至2008年12月），并前瞻性地随访其随后的课程（n=186）。研究终点是全因死亡，LVNC的存在是盲态的，直到研究结束（中位数随访：46个月;四分位数范围：41-48个月）。结果：LVNC患者（n=35）和对照组无LVNC患者（n=151）之间的基线特征无显着差异，左心室功能除外。与无LVNC的患者相比，LVNC患者的年龄与基线LVEF呈显著负相关（R=-0.7 vs. R=-0.4），且绝对LVEF下降幅度显著更大（-8.6 +/- 4.6 vs. -4.3 +/- 4.5，p
Background: The reported prevalence of left ventricular noncompaction (LVNC) varies widely and its prognostic impact remains controversial. We sought to clarify the prevalence and prognostic impact of LVNC in patients with Duchenne/Becker muscular dystrophy (DMD/BMD).Methods: We evaluated the presence of LNVC in patients with DMD/BMD aged 4-64 years old at the study entry (from July 2007 to December 2008) and prospectively followed-up their subsequent courses (n=186). The study endpoint was all-cause death and the presence of LVNC was blinded until the end of the study (median follow-up: 46 months; interquartile range: 41-48 months).Results: There were no significant differences in baseline characteristics between patients with LVNC (n=35) and control patients without LVNC (n=151), with the exception of LV function. Patients with LVNC showed, in comparison with patients without LVNC, a significant negative correlation between age and LVEF (R=-0.7 vs. R=-0.4) at baseline; and showed a significantly greater decrease in absolute LVEF (-8.6 +/- 4.6 vs. -4.3 +/- 4.5, p