Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.
Korean Circ J. 2012 Mar;42(3):184-91. doi: 10.4070/kcj.2012.42.3.184. Epub 2012 Mar 26.
In Duchenne and Becker muscular dystrophies, cardiac function deteriorates with time resulting in heart failure which is often fatal. We prospectively evaluated the effect of enalapril and carvedilol on left ventricular (LV) dysfunction in middle childhood and adolescent patients with muscular dystrophy.
Twenty-three patients with LV dysfunction (22 with Duchenne muscular dystrophy, 1 with Becker muscular dystrophy) were enrolled. We prescribed enalapril (13 patients) or carvedilol (10 patients) randomly from July 2008 to August 2010 and followed up the patients until September 2011. The changes in LV function parameters before and after the treatment were evaluated by echocardiography.
The mean age at the start of treatment with enalapril or carvedilol was 12.6±3.7 years (median 13 years), and mean follow-up duration was 20.1±8.9 months. In the enalapril group, LV fractional shortening (FS) increased from 25.8±2.1 to 26.6±3.0 (p=0.241). In the carvedilol group, LV FS increased from 26.4±1.1 to 28.6±4.2 (p=0.110). In all 23 patients, LV FS significantly increased from 26.1±1.7 (before) to 27.6±3.7 (after treatment) (p<0.046). Indexed LV dimension at end diastole and LV end-diastolic volume decreased slightly, but without statistical significance by tri-plane volumetry. LV diastolic functional parameters were maintained during follow-up period.
Enalapril or carvedilol could improve LV systolic function in middle childhood and adolescent patients with muscular dystrophy without significant adverse effects.
在杜氏肌营养不良症和贝克肌营养不良症中,心脏功能随时间恶化,导致心力衰竭,这往往是致命的。我们前瞻性评估了依那普利和卡维地洛对肌肉营养不良症中童年中期和青少年患者左心室(LV)功能障碍的影响。
共纳入 23 例 LV 功能障碍患者(22 例杜氏肌营养不良症,1 例贝克肌营养不良症)。我们于 2008 年 7 月至 2010 年 8 月随机开出处方:依那普利(13 例)或卡维地洛(10 例),并随访患者直至 2011 年 9 月。通过超声心动图评估治疗前后 LV 功能参数的变化。
开始依那普利或卡维地洛治疗时的平均年龄为 12.6±3.7 岁(中位数 13 岁),平均随访时间为 20.1±8.9 个月。依那普利组的 LV 短轴缩短率(FS)从 25.8±2.1 增加到 26.6±3.0(p=0.241)。卡维地洛组的 LV FS 从 26.4±1.1 增加到 28.6±4.2(p=0.110)。在所有 23 例患者中,LV FS 从治疗前的 26.1±1.7 显著增加到治疗后的 27.6±3.7(p<0.046)。三平面容积测量显示,LV 舒张末期内径指数和 LV 舒张末期容积略有下降,但无统计学意义。LV 舒张功能参数在随访期间保持不变。
依那普利或卡维地洛可改善肌肉营养不良症中童年中期和青少年患者的 LV 收缩功能,且无明显不良反应。