孤立性左心室心尖发育不良的影像学特征  

Imaging Features of Patients With Isolated Left Ventricular Apical Hypoplasia

作　　者：蔡瑶瑶 刘倩倩 万琳媛 齐红霞 李慕子 孟红 CAI Yaoyao;LIU Qianqian;WAN Linyuan;QI Hongxia;LI Muzi;MENG Hong(Echocardiography Center,National Center for Cardiovascular Diseases and Fuwai Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100037,China)

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、阜外医院、超声影像中心,北京100037

出　　处：《中国循环杂志》2024年第7期682-687,共6页Chinese Circulation Journal

基　　金：中国医学科学院临床与转化医学研究专项(2022-I2M-C&T-B-051);阜外医院血流向量成像技术评估冠心病患者颈动脉血流流场特点研究专项(2022-GSP-QN-18)。

摘　　要：目的:探讨孤立性左心室心尖发育不良(ILVAH)的临床和影像学特征。方法:连续入选2018年4月至2023年8月我院确诊为ILVAH的患者12例(ILVAH组),所有患者均接受经胸超声心动图(TTE)检查;9例患者行心脏磁共振成像(CMR)检查;1例患者行CT检查。分析所有患者的临床资料和影像学特征。选择同时期年龄、性别相匹配的健康体检者12例作为对照组。结果:TTE显示:与对照组相比,ILVAH组患者的左心室横径[(46.3±5.7)mm vs.(57.8±12.6)mm]、右心室长径[(62.0±4.2)mm vs.(73.6±6.4)mm]更大,左心室长径[(63.0±3.5)mm vs.(53.4±8.2)mm]更小,差异均有统计学意义(P均<0.05);两组间右心室横径的差异无统计学意义(P>0.05);ILVAH组患者室间隔均凸向右心室,心尖部最薄处厚度为(5.2±2.0)mm;乳头肌均附着于扁平的左心室心尖部。进行CMR检查的9例ILVAH患者中7例左心室心尖发现脂肪组织代替,2例可见心内膜纤维化。结论:ILVAH临床表现非特异,但有特异性的影像学表现,可帮助提高该病的认识。Objectives:To explore the imaging features of patients with isolated left ventricular apical hypoplasia(ILVAH).Methods:Twelve patients diagnosed with ILVAH(ILVAH group)in our hospital from April 2018 to August 2023 were enrolled,and all of them received transthoracic echocardiography(TTE)examination;9 patients underwent CMR examination;and 1 patient underwent CT examination.Clinical data and imaging characteristics of the patients were analyzed.At the same time,twelve healthy age and sex matched participants who underwent physical examination in the same period were selected as the control group.Results:TTE showed that:compared with the control group,the left ventricular transverse diameter([46.3±5.7]mm vs.[57.8±12.6]mm)and right ventricular long diameter([62.0±4.2]mm vs.[73.6±6.4]mm)were larger in the ILVAH group(P<0.05),the left ventricular long diameter([63.0±3.5]mm vs.[53.4±8.2]mm)was smaller(P<0.05).There was no significant difference in right ventricular transverse diameter between the two groups(P>0.05).The interventricular septum was convex to the right ventricle,and the thickness at the apex was(5.2±2.0)mm in the ILVAH group.The papillary muscles were all attached to the flat left ventricular apex.Among the 9 patients who underwent CMR examination,adipose tissue replacement was found in the left ventricular apex in 7 out of the 9 patients,and endocardial fibrosis was found in 2 out of the 9 patients.Conclusions:The clinical presentation of ILVAH is non-specific,but there are specific imaging manifestations that may help improve the understanding of the disease.

关 键 词：心尖发育不良 心血管疾病 超声心动描记术 

分 类 号：R54[医药卫生—心血管疾病]