β受体阻滞剂治疗马方综合征主动脉根部扩张的临床观察  

Effect of long-term beta-blockade on aortic root dilation in children and adolescents patients with Marfan syndrome

作　　者：谢进生[1] 裴金凤[2] 孙衍庆[1] 徐保卫[1] 乔志钰[1] 

机构地区：[1]首都医科大学附属北京安贞医院心外科 [2]首都医科大学附属北京安贞医院心超声科

出　　处：《中国心血管病研究》2006年第5期326-328,共3页Chinese Journal of Cardiovascular Research

基　　金：北京市科技新星计划资助项目(编号:954813700)

摘　　要：目的观察长效β受体阻滞剂氨酰心安对儿童和青少年马方综合征(MFS)主动脉根部扩张的临床治疗效果。方法15例临床诊断明确的MFS患者,药物治疗组7例,年龄3.5～2(011.5±5.7)岁,另外8例未用药物作为对照组。应用UCG测定用药前、后主动脉根部最大直径,记录用药前后身高、体重,按回归方程计算出体表面积,进而得出主动脉根部增长速度、增长指数和年增长指数,以及应用氨酰心安的剂量。结果两组在性别、年龄、随访时间、体表面积和家族史差异无统计学意义。用药组根据个体化服用氨酰心安(0.81±0.32)mg·kg-·1d-1,随访时间1.4～3.(32.47±0.68)年,服药后安静心率由(93±9)次/min降为(74±8)次/min,其主动脉根部直径年增长速度[(2.33±0.82)mmvs(.5.30±1.89)mm)]和年增长指数[(1.14±1.09)mmv(s4.85±3.07)mm)明显慢于对照组(P<0.05)。随访期间有2例治疗无效,表现为主动脉窦进行性扩张并反流而行改良带瓣管道置换术。结论β受体阻滞剂氨酰心安对大多数儿童和青少年马方综合征的主动脉根部具有明显延缓扩张的效果,但对主动脉窦部已中-重度扩张者无效。Objective This study was undertaken to observe the effect of long-term beta-blocker on the aortic root dilation in children and adolescents patients with the Marfan syndrome. Methods Aortic root diameter was measured with eehocardiography before and after an average of 26 months of atenolol administration. Results 7 asymptomatic patients were studied [4 male and 3 female, aged （11.5±2.7） years] compared with 8 patients untreated as control group. The follow-up was （2.47± 0.68） years. The mean dose of atenolol was （0.81±0.32）mg·kg^-1·d^-1, Heart rate decreased from （93±9）beats/rain to （74±8） beats/min （P〈0.05）, aortic growth rate and index per year were significantly slower than that of untreated patients [（2.33±0.82） mm vs （5.30±1.89）mm/yr, P=0.002; （1.14±1.09）mm·m^-2·yr^-1 vs （4.85±3.07）mm·m^-2·yr^-1, P=0.010]. Two patients （29%） in the treatment group with aortic root diameter 〉40 mm at the beginning of therapy did not respond to the treatment. Conclusion Beta-blocker therapy could retard aortic growth rate significantly in the majority of children and adolescents patients with Marfan syndrome. There was also no response patients when their aortic roots were dilated medially or severely.

关 键 词：马方综合征 主动脉根部扩张 Β受体阻滞剂 

分 类 号：R593.2[医药卫生—内科学]