摘要目的 分析左心室中部肥厚型梗阻性心肌病患者的临床及心血管造影特征.方法 5例(男:女=3:2)临床诊断为左心室中部肥厚型梗阻性心肌病的患者,年龄16～73(44±22)岁.所有患者均接受左心导管及心血管造影检查,并记录左心室心尖部-左心室基底部-左心室流出道-升主动脉连续压力.结果 5例患者中胸闷气短者4例,其中2例合并晕厥.所有患者心前区均可闻及收缩期杂音,且均存在左心室壁肥厚,室间隔厚度为19～31(23.8±5.4)mm,左心室舒张末期横径为35～55(43.4±7.4)mm,左心室射血分数为53%～70%、平均为(64.2±6.9)%.5例患者均存在左心室高电压伴异常Q波,其中1例患者合并阵发性室性心动过速,另有1例患者合并完全性左束支传导阻滞.左心导管检查证实收缩期左心室中部均存在梗阻,其中1例患者同时合并左心室流出道梗阻.左心室中部收缩期压力阶差为45～102(68.6±24.1)mm Hg(1 mm Hg=0.133 kPa).心血管造影检查提示前降支中段肌桥1例,冠状动脉粥样硬化性心脏病1例.另外,2例患者合并左心室心尖部室壁瘤.结论 左心室中部肥厚型梗阻性心肌病的临床特征不同于其他类型的肥厚型心肌病,完善的左心导管检查及造影有利于诊断并指导临床治疗.

abstractsObjective To analyze the clinical and angiographic characteristics of mid-ventricular hypertrophic obstructive cardiomyopathy (MV-HOCM). Methods MV-HOCM was diagnosed in 5 patients [3 males, mean age: 16 - 73 (44 ± 22) years]. Left ventricular catheterization and angiography were performed in all patients, and the pressures were recorded in the left ventricular apical chamber, basal chamber, outflow tract and ascending aorta. Results Of five patients with MV-HOCM, chest discomfort occurred in four patients and syncope in two patients. All patients presented systolic murmur and asymmetric left ventricular hypertrophy. The thickness of ventricular septum was 19 - 31 ( 23. 8 ± 5.4 ) mm, the dimension of left ventricle was 35 - 55 (43.4 ± 7.4 ) mm and the LVEF was 53% - 70% (64. 2% ±6. 9% ). Electrocardiogram showed left ventricular hypertrophy with Q waves in all patients, ventricular tachycardia in 1 patient and complete left bundle branch block in 1 patient. Mid-ventricular obstruction was found in all patients and the pressure gradient in mid-ventricle was45 - 102(68.6 ±24. 1 )mm Hg( 1 mm Hg =0. 133 kPa). Coronary angiogram documented muscular bridge presented in 1 patient and coronary artery disease in 1 patient. Left ventricular apical aneurysm was seen in 2 patients. Conclusion MV-HOCM was a distinguished subtype of hypertrophic obstructive cardiomyopathy, and left cardiac catheterization and angiography examinations are necessary for confirming diagnosis and guiding related therapy.