肥厚型心肌病伴限制性表型患者的临床及MRI特征  

Clinical and MRI characteristics of hypertrophic cardiomyopathy with restrictive phenotype

作　　者：武柏林[1,3] 陆敏杰[1] 赵世华[1] 凌坚[1] 张岩[1] 尹刚[1] 杨新令[1] 郭午玲 王好玲 王浩[2] 

机构地区：[1]中国医学科学院北京协和医学院国家心血管病中心阜外心血管病医院核磁影像科,100037 [2]中国医学科学院北京协和医学院国家心血管病中心阜外心血管病医院超声科,100037 [3]河北医科大学第二医院影像科,石家庄050000

出　　处：《中华放射学杂志》2015年第11期818-822,共5页Chinese Journal of Radiology

基　　金：国家自然科学基金（81370036,81000604）;协和青年创新基金（2012D04）;中央高校基本科研业务费专项资金（3332013105）;阜外心血管病医院科研新星项目（2012-FWXX01）

摘　　要：目的探讨肥厚型心肌病（HcM）伴限制性表型的临床和MRI特征。方法回顾性搜集19例伴显著限制性特征的HCM患者（HcM伴限制性表型组）及年龄、性别、病程相匹配的19例典型非梗阻性肥厚型心肌病患者（典型HCM组），行黑血序列及亮血电影序列的四腔心、两腔心和短轴扫描，注射对比剂后10～15min行延迟强化扫描。回顾性比较两组患者临床特征、室壁厚度及延迟强化节段数、MR功能参数的差异，计量资料采用配对t检验、计数资料采用Fisher确切概率法进行统计分析。结果HCM伴限制性表型组中出现持续房颤和心包积液的例数均为11例，典型HCM组分别为0、1例，HCM伴限制性表型组发生率显著高于典型HCM组（P值均〈0．01）。其心功能分级明显低于典型HCM组（P〈0．01）。HCM伴限制性表型组左、右心房前后径分别为（55．8±5．3）和（61．3±11．0）mm，均明显大于对照组[左、右心房前后径分别为（37．0±7．8）和（39．3±5．3）mm]，差异具有统计学意义（t值分别为8．82和25．55，P值均〈0．01）。HCM伴限制性表型组患者表现出明显的右心室长轴短缩，类似限制型心肌病改变。其延迟强化的范围为（7．7±3．0）个节段，明显多于典型HCM组的（5．1±2．8）个节段，差异具有统计学意义（F2．80，P〈0．01）。心功能参数提示HCM伴限制性表型组左心室舒张末期容积指数、心脏指数和左心射血分数均明显小于典型HCM组，差异具有统计学意义。结论HCM伴限制性表型是HCM的一种亚型，临床症状重，MRI在该病的诊断及全面评价上具有重要的应用价值。Objective To investigate the clinical and MR characteristics of hypertrophic cardiomyopathy（HCM） with restrictive phenotype. Methods Nineteen patients of HCM with obviously restrictive characteristics （HCM with restrictive phenotype） and 19 typical patients with non obstructive HCM, matched with age, gender and suffering time, were retrospectively collected. A short-axis stack, one 4-chamber and 2-chamber views were acquired using black blood sequence and bright blood cine sequence. Ten to15 minutes after injection of contrast medium late gadolinium enhancement images were acquired. The clinical features, the wall thickness and segments of delayed enhancement, and the MR function parameters of the two groups were compared retrospectively. The paired sample t test and Fisher exact probability method were used for the analysis of quantitative data and categorical data respectively.Results Eleven of patients of HCM with restrictive phenotype suffered sustained atrial fibrillation and pericardialeffusion, whereas only 0 and 1 cases was found had the same two symptoms in HCM（all P〈0.01）. The heart function classification was worse compared with controls （P〈0.01）; The left and right atrium diameter were （55.8±5.3） mm and （61.3± 11.0） mm in case group, which were significantly greater than the controls[（37.0± 7.8） and （39.3±5.3）mm respectively]（t=8.82 and 25.55, P〈0.01）; HCM with restrictive phenotype showed obvious right ventricular long axis shortening, similar to restrictive cardiomyopathy. The segments with late gadolinium enhancement were 7.7±3.0 in patients with restrictivephenotype, which were significantly greater than controls （5.1 ±2.8, t=2.80, P〈0.01）. The left ventricular end-diastolic volume index, the cardiac index, and the left heart ejection fraction of patients with restrictive phenotype were all significantly less than those of the controls. Conclusions HCM with restrictive phenotype is a subtype of HCM. The patients often have severe clinical sympt

关 键 词：心肌病 肥厚型 磁共振成像 

分 类 号：R542.2[医药卫生—心血管疾病]