肥厚型心肌病合并左心室心尖部室壁瘤患者的临床特征

Clinical Features in Patients With Hypertrophic Cardiomyopathy Combining Left Ventricular Apical Aneurysm

目的:分析总结肥厚型心肌病合并左心室心尖部室壁瘤患者的临床特征。方法:2007-01至2013-01共1 194例肥厚型心肌病患者在北京同仁医院接受检查,其中23例(1.94%)合并左心室心尖部室壁瘤,男19例,女4例。所有患者均除外冠心病,并接受超声心动图和心血管造影检查。结果:23例患者中,左心导管检查证实其中21例患者左心室中部存在梗阻,其中7例患者同时合并左心室流出道梗阻。21例左心室梗阻患者收缩期压差为(56.8±12.9)mm Hg(1 mm Hg=0.133 k Pa);另外2例患者为心尖肥厚型心肌病。患者的左心室室壁最大厚度为(21.8±6.3)mm,左心室横径(39.4±5.2)mm。心电图检查提示,3例患者合并阵发性室性心动过速。心血管造影检查发现,6例患者合并冠状动脉肌桥,并且均位于冠状动脉前降支。(2.7±1.3)年随访过程中,5例患者发生心血管不良事件,其中2例患者充血性心力衰竭加重,3例患者发生室性心动过速。结论:肥厚型心肌病伴发左心室心尖部室壁瘤最常见于左心室中部肥厚型梗阻性心肌病患者,其中部分患者同时合并左心室流出道梗阻。这些患者心血管不良事件发生率高,早期准确诊断对指导临床治疗至关重要。

Objective： To analyze the clinical features in patients with hypertrophic cardiomyopathy（HCM） combining left ventricular apical aneurysm（LVAA）.Methods： A total of 1194 HCM patients treated in our hospital from 2007-01 to 2013-01 were studied. There were 23（1.93%） patients combining with LVAA including 19 male and 4 female; coronary artery disease（CAD） was excluded and the patients received echocardiography and coronary angiography（CAG） examinations.Results： There were 21/23 LVAA patients having left mid-ventricular obstruction and 7 of them combining simultaneous left ventricular outflow obstruction.The average pressure gradient in those 21 patients was（56.8 ± 12.9） mmH g and the rest 2 patients suffered from apical hypertrophic cardiomyopathy.The mean maximum thickness of left ventricular wall was（21.8 ± 6.3） mm and the dimension of left ventricle was（39.4 ± 5.2） mm. Electrocardiography showed that 3 patients had paroxysmal ventricular tachycardia; CAG indicated that 6 patients combined with coronary artery muscular bridge at left anterior descending（LAD） artery. The patients were followed-up for（2.7 ± 1.3） years and adverse cardiovascular events occurred in 5 patients during that period. Conclusion： HCM combining LVAA was most frequently happened in patients with left mid-ventricular hypertrophic cardiomyopathy, some of them combining simultaneous left ventricular outflow obstruction and had the higher occurrence rate of adverse cardiovascular events. Early and accurate diagnosis is very important for guiding clinical treatment.