Kieffer R W, Hutchins G M, Moore G W, Bulkley B H
Am J Med. 1979 May;66(5):831-5. doi: 10.1016/0002-9343(79)91124-0.
In the normal heart the interventricular septum is curved concave to the left and functions as a component of the left ventricular wall. We have studied the hearts of five patients at autopsy, after postmortem angiography and fixation in distention, in which the septum was concave to the right ventricle. These patients ranged in age from eight months to 25 years (average, 16 years); three had primary pulmonary hypertension, and two had coarctation of the aorta and congenital aortic stenosis. Catheterization studies in the latter two patients had demonstrated subaortic stenosis. At autopsy there was no evidence of asymmetric septal hypertrophy or of abnormal septal myocardial fiber disarray. The right ventricles were markedly hypertrophied. In each, the septal curvature was reversed leading to protrusion of septal muscle into the left ventricular outflow tract with marked narrowing of the outflow tract. Since the septal configuration is determined early in life, this abnormality of ventricular topography may develop primarilary or it may develop in hearts subject to early as well as long-standing right ventricular overload.
在正常心脏中，室间隔向左呈凹形弯曲，并作为左心室壁的一个组成部分发挥作用。我们对5例患者的心脏进行了尸检研究，这些患者在死后血管造影并在扩张状态下固定，其室间隔向右心室呈凹形。这些患者年龄从8个月至25岁不等（平均16岁）；3例患有原发性肺动脉高压，2例患有主动脉缩窄和先天性主动脉瓣狭窄。后2例患者的导管检查显示有主动脉瓣下狭窄。尸检时未发现不对称性室间隔肥厚或室间隔心肌纤维排列异常的证据。右心室明显肥厚。在每例中，室间隔弯曲方向相反，导致间隔肌突入左心室流出道，使流出道明显变窄。由于室间隔的形态在生命早期就已确定，这种心室形态异常可能是原发性的，也可能发生在早期以及长期存在右心室负荷过重的心脏中。
