Silverman N H, Gerlis L M, Ho S Y, Anderson R H
Department of Pediatrics, University of California, San Francisco 94143-0214.
J Am Coll Cardiol. 1995 Feb;25(2):475-81. doi: 10.1016/0735-1097(94)00379-5.
We examined the nature of ridges within the left ventricular outflow tract associated with ventricular septal defects that might be found by echocardiography.
Echocardiography displays even small ridges well. Surgical removal of such ridges at the time of defect closure is recommended.
We examined 37 heart specimens with ventricular septal defects with a ridge, noting its nature and relation to the defect and adjacent valves. We excluded left ventricular outflow tract obstruction associated with complex lesions.
Defects were perimembranous in 25 specimens, muscular in 8 and part of an atrioventricular septal defect in 5. Some hearts had multiple defects. Many of the original reports had not mentioned ridges. Three distinct ridge patterns were found. The first (n = 18) was a fold of endocardial tissue related to the membranous septum. The second (n = 12) was a defect of a fibrous nature; in 8 this was a discrete, protuberant fibrous ridge, and in 4 the obstruction was diffuse, which we termed keloidal. The third pattern (n = 7) lay circumferentially around the ventricular septal defect, seemingly associated with the defect's attempted spontaneous diminution in size. Endocardial folds were not found in specimens from patients > 5 years old. Fibrous and keloidal lesions, which may represent a continuum of progression, generally were found in specimens from older patients. Histologic studies of 17 specimens confirmed the morphologic findings. The endocardial folds were endothelial tissue, whereas the fibrous and keloidal ridges were of fibrous tissue, as were circumferential lesions. All specimens had mitral-semilunar valvular continuity.
Endocardial fold and circumferential lesions appear to be benign. The endocardial folds arose from the membranous ventricular septum, were not protuberant and usually were found in younger patients. The fibrous ridges, in contrast, were protuberant and were always associated with the underlying muscle of the outlet septum. These pathologic distinctions may facilitate echocardiographic diagnosis and prognosis.
我们研究了左心室流出道内与室间隔缺损相关的嵴的性质，这些嵴可通过超声心动图发现。
超声心动图能很好地显示即使是很小的嵴。建议在闭合缺损时手术切除此类嵴。
我们检查了37个有嵴的室间隔缺损心脏标本，记录其性质以及与缺损和相邻瓣膜的关系。我们排除了与复杂病变相关的左心室流出道梗阻。
25个标本的缺损为膜周部，8个为肌部，5个为房室间隔缺损的一部分。一些心脏有多个缺损。许多原始报告未提及嵴。发现了三种不同的嵴模式。第一种（n = 18）是与膜性间隔相关的心内膜组织褶皱。第二种（n = 12）是纤维性质的缺损；其中8个是离散的、突出的纤维嵴，4个的梗阻是弥漫性的，我们称之为瘢痕疙瘩样。第三种模式（n = 7）围绕室间隔缺损呈环形分布，似乎与缺损试图自发缩小有关。5岁以上患者的标本中未发现心内膜褶皱。纤维性和瘢痕疙瘩样病变可能代表连续的进展过程，通常在老年患者的标本中发现。对17个标本的组织学研究证实了形态学发现。心内膜褶皱是内皮组织，而纤维性和瘢痕疙瘩样嵴是纤维组织，环形病变也是如此。所有标本的二尖瓣-半月瓣连续性均正常。
心内膜褶皱和环形病变似乎是良性的。心内膜褶皱起源于膜性室间隔，不突出，通常见于年轻患者。相比之下，纤维嵴突出，总是与流出道间隔的下层肌肉相关。这些病理差异可能有助于超声心动图诊断和预后判断。
