Fisher D J, Snider A R, Silverman N H, Stanger P
Pediatr Cardiol. 1982;2(4):265-9. doi: 10.1007/BF02426971.
Subaortic stenosis is rarely mentioned as a lesion that may be associated with a ventricular septal defect. We have encountered 4 patients with discrete subaortic stenosis adjacent to a ventricular septal defect, all of whom posed significant problems in diagnosis. In all 4 patients the subaortic stenosis was silent clinically and in 3 cases the obstruction was also not detected at the initial cardiac catheterization and angiocardiography. In the latter 3 cases, after surgical closure of the ventricular septal defect, there was a loud systolic murmur initially thought to be due to a small residual ventricular septal defect. In time, the clinical findings became more typical of isolated subaortic stenosis. In each of these cases the obstruction was verified at cardiac catheterization with peak systolic pressure gradients of 145, 45, and 70 mmHg. During reoperation, a discrete subaortic shelf was found opposite the patch used to close the ventricular septal defect. In the 4th case, the subaortic stenosis was unsuspected by clinical evaluation but was diagnosed by echocardiography as well as at cardiac catheterization, and both the ventricular septal defect and subaortic stenosis were corrected at the initial operation. A discrete subaortic shelf situated adjacent to a ventricular septal defect may be "silent," producing minimal, if any, pressure gradient and may pose diagnostic difficulties. Failure to recognize such a shelf and to remove it at the time of surgical closure of the ventricular septal defect, may result in the creation of a severe subaortic obstruction.
主动脉瓣下狭窄很少被提及为可能与室间隔缺损相关的病变。我们遇到了4例与室间隔缺损相邻的局限性主动脉瓣下狭窄患者，所有这些患者在诊断上都存在重大问题。在所有4例患者中，主动脉瓣下狭窄在临床上没有症状，并且在3例中，在初次心导管检查和心血管造影时也未检测到梗阻。在这3例患者中，在室间隔缺损手术闭合后，出现了响亮的收缩期杂音，最初认为是由于小的残余室间隔缺损所致。随着时间的推移，临床表现变得更符合孤立性主动脉瓣下狭窄的典型特征。在这些病例中的每一例中，通过心导管检查证实了梗阻，收缩期峰值压力阶差分别为145、45和70 mmHg。再次手术时，在用于闭合室间隔缺损的补片对面发现了一个局限性主动脉瓣下嵴。在第4例中，临床评估未怀疑有主动脉瓣下狭窄，但经超声心动图以及心导管检查确诊，并且在初次手术时同时纠正了室间隔缺损和主动脉瓣下狭窄。位于室间隔缺损附近的局限性主动脉瓣下嵴可能是“无症状的”，即使有压力阶差也很小，可能会造成诊断困难。在室间隔缺损手术闭合时未能识别并切除这样的嵴，可能会导致严重的主动脉瓣下梗阻。
