Maron B J, Gottdiener J S, Roberts W C, Henry W L, Savage D D, Epstein S E
Circulation. 1978 Mar;57(3):527-33. doi: 10.1161/01.cir.57.3.527.
Patients with hypertrophic cardiomyopathy (i.e., asymmetric septal hypertrophy) may show obstruction to left ventricular outflow under basal conditions or with provocative maneuvers. The presence of dynamic left ventricular outflow tract obstruction in patients with concentric ventricular wall thickening (but without abnormalities of the aortic valve) has been less well appreciated. Clinical and morphologic features of five patients with nondilated left ventricles and with left ventricular outflow obstruction are presented. In each patient peak systolic pressure gradients between left ventricle and systematic artery were measured at cardiac catheterization and ranged from 60-140 mm Hg under basal conditions or with provocation. Each patient had echocardiographically documented systolic anterior motion of the anterior mitral leaflet, which was apparently responsible for the outflow obstruction, and concentric left ventricular wall thickening (septal-free wall thickness ratio of less than 1.3). Two of the five patients had evidence of genetically transmitted hypertrophic cardiomyopathy, as evidenced by disorganized muscle cells in the ventricular septum or asymmetric septal hypertrophy in first degree relatives. Hence, left ventricular outflow tract obstruction associated with systolic anterior motion of the anterior mitral leaflet may occur in some patients with concentric left ventricular hypertrophy who do not have typical hypertrophic cardiomyopathy.
肥厚型心肌病患者(即不对称性室间隔肥厚)在基础状态下或进行激发动作时可能会出现左心室流出道梗阻。同心性心室壁增厚(但无主动脉瓣异常)的患者出现动态左心室流出道梗阻的情况尚未得到充分认识。本文介绍了5例左心室未扩张且存在左心室流出道梗阻患者的临床和形态学特征。在每例患者中,心脏导管检查时测量了左心室与体循环动脉之间的收缩期峰值压力梯度,基础状态下或激发状态下其范围为60 - 140 mmHg。每例患者经超声心动图证实二尖瓣前叶有收缩期向前运动,这显然是流出道梗阻的原因,且存在同心性左心室壁增厚(室间隔与游离壁厚度比小于1.3)。5例患者中有2例有基因传递性肥厚型心肌病的证据,表现为室间隔肌细胞排列紊乱或一级亲属中有不对称性室间隔肥厚。因此,二尖瓣前叶收缩期向前运动相关的左心室流出道梗阻可能发生在一些没有典型肥厚型心肌病的同心性左心室肥厚患者中。