重度右心室肥厚型心肌病的临床特点及预后研究  

Clinical Characteristics and Prognosis of Hypertrophic Cardiomyopathy Patients With Severe Right Ventricular Hypertrophy

作　　者：闫丽荣[1] 段福建[2] 安硕研 杭霏[3] 张宇辉[3] 樊朝美[3] 张健[3] YAN Li-rong;DUAN Fu-jian;AN Shuo-yan;HANG Fei;ZHANG Yu-hui;FAN Chao-mei;ZHANG Jian(Department of Cardiology,National Center for Cardiovascular Diseases and Fuwai Hospital,CAMS and PUMC,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心内科,北京市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医院超声影像中心,北京市100037 [3]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心力衰竭中心,北京市100037

出　　处：《中国循环杂志》2018年第9期879-883,共5页Chinese Circulation Journal

基　　金：中国医学科学院医学与健康科技创新工程项目(2016-I2M-1-006)

摘　　要：目的:研究重度右心室肥厚型心肌病(SRVHCM)患者的临床特点及长期预后。方法:对43例SRVHCM患者的发病率、临床特点、心血管事件发生率和死亡率进行回顾性分析。SRVHCM的诊断标准:舒张末期右心室游离壁、前壁或右心室心尖处室壁厚度≥10 mm,并除外肺原性心脏病、慢性栓塞性肺动脉高压及原发性肺动脉高压等其他可能导致右心室心肌肥厚的疾病。结果:43例SRVHCM患者占同期3 308例HCM患者的1.29%,女性患者(60.5%)多于男性患者(39.5%),诊断时平均年龄(32.0±17.2)岁,其中合并右心室梗阻者13例(30.2%)。平均随访(7.66±6.04)年,SRVHCM患者的心血管死亡率为16.3%,诊断时年龄≤18岁(HR=4.50,95%CI:1.24~20.40,P=0.03)是心血管死亡的独立预测因子。28例(65.1%)患者发生心血管事件,其中发生率最高的心血管事件是进展性心力衰竭,右心室梗阻是心血管事件(HR=1.75,95%CI:1.12~5.76,P=0.04)和进展性心力衰竭(HR=2.84,95%CI:1.30~8.12,P=0.03)的独立预测因子。结论:中国人群中SRVHCM患病率较低,约1/3合并右心室梗阻,预后不良。Objectives： To observe the clinical characteristics and long-term prognosis of patients with severe right ventricular hypertrophic cardiomyopathy（SRVHCM）.Methods： A total of 43 SRVHCM patients treated in our hospital were retrospectively studied for their clinical characteristics, major adverse cardiac events and mortality.Results： There were 43（1.29%） patients suffering from SRVHCM among 3308 patients diagnosed with hypertrophic cardiomyopathy. The prevalence rate was higher in females than in males, and the average diagnostic age was（32.0±17.2） years. Right ventricular outflow tract obstruction was found in thirteen patients（30.2%）. During（7.66±6.04） years of followup, the cardiovascular mortality was 16.3% and an age of diagnosis of SRVHCM ≤18 years（HR=4.50, 95% CI： 1.24-20.40, P=0.03） was the independent predictor for cardiovascular death. Twenty-eight out of 43（65.1%） patients experienced one or more major adverse cardiac events and the most frequent major adverse cardiac event was progressive heart failure. Right ventricular outflow tract obstruction was an independent predictor for major adverse cardiac events（HR=1.75, 95%CI：1.12-5.76, P=0.04） and progressive heart failure（HR=2.84, 95%CI：1.30-8.12, P=0.03）.Conclusions： The prevalence of SRVHCM is rare in Chinese hypertrophic cardiomyopathy patients, right ventricular outflow tract obstruction occurs in about 1/3 SRVHCM patients, and SRVHCM patients usually have unfavorable prognosis.

关 键 词：肥厚型心肌病 右心室肥厚 右心室梗阻 

分 类 号：R54[医药卫生—心血管疾病]