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目的:本研究旨在探究左心室中部肥厚型梗阻性心肌病(MVOHCM)患者的临床特点及长期预后。方法:对66例MVOHCM患者的发病率、临床特征、心血管事件发生率和死亡率进行了回顾性分析。根据KaplanMeier法计算累积生存率。使用单因素和多因素Cox风险模型计算心原性死亡和心血管事件的危险因素及95%可信区间。结果:66例MVOHCM患者在2 413例肥厚型心肌病(HCM)患者中约占2.74%。平均诊断年龄为(40.16±14.64)岁。经过平均(7.30±6.25)年的随访,MVOHCM患者的心血管死亡率为13.64%,不明原因晕厥[风险比(HR):13.37,95%可信区间(CI):1.65~114.46,P=0.015]是心血管死亡的独立预测因子。30例(45.45%)患者至少发生了一次心血管事件,其中最常见的是非持续性室性心动过速(NSVT)。13例(19.70%)患者合并心尖室壁瘤,此类患者更易发生NSVT。结论:MVOHCM预后不良。心血管事件发生率高,部分患者合并心尖室壁瘤形成。早期诊断对MVOHCM患者的合理治疗十分必要。
Objective: This study was designed to investigate the clinical features and long-term prognosis of patients with hypertrophic obstructive cardiomyopathy (MVOHCM) in the middle of the left ventricle. Methods: The incidence, clinical features, incidence of cardiovascular events and mortality in 66 patients with MVOHCM were analyzed retrospectively. The cumulative survival rate was calculated according to the KaplanMeier method. Univariate and multivariate Cox risk models were used to calculate risk factors for cardiac death and cardiovascular events with a 95% confidence interval. Results: 66% of MVOHCM patients accounted for 2.74% of the 2 413 hypertrophic cardiomyopathy (HCM) patients. The average diagnosis age was (40.16 ± 14.64) years old. After a mean of 7.30 ± 6.25 years of follow-up, the cardiovascular mortality was 13.64% in MVOHCM patients and unexplained syncope [hazard ratio (HR): 13.37; 95% CI: 1.65-114.46; P = 0.015 ] Is an independent predictor of cardiovascular death. Thirty patients (45.45%) had at least one cardiovascular event, the most common of which was non-sustained ventricular tachycardia (NSVT). Thirteen (19.70%) patients had apical aneurysm, which is more likely to develop NSVT. Conclusions: MVOHCM has a poor prognosis. The incidence of cardiovascular events is high, some patients with apical aneurysm formation. Early diagnosis of MVOHCM patients with reasonable treatment is necessary.