LaCorte M A, Cooper R S, Kauffman S L, Schiller M S, Golinko R J, Griepp R B
Pediatr Cardiol. 1982;2(4):289-95. doi: 10.1007/BF02426975.
A rare form of endocardial cushion defect is an atrioventricular (AV) canal-type ventricular septal defect with cleft mitral valve in the absence of a primum atrial septal defect. In two infants with Down's syndrome, preoperative delineation of this entity was accomplished. M-mode echocardiographic features included presence of two distinct AV valves, attachment of the anterior portion of the anterior leaflet of the mitral valve to the interventricular septum, and normal septal motion. Two-dimensional echocardiography demonstrated the intact atrial septum, ventricular septal defect, presence of two distinct AV valves, and the cleft mitral valve. Angled (axial) cineangiography was employed to delineate the AV valve morphological characteristics, position of ventricular septal defect, and absence of a primum atrial septal defect. Since the surgical approach to this lesion is different from other forms of endocardial cushion defects and specific problems related to AV valve morphology may be encountered, the preoperative diagnosis of this entity is important.
一种罕见的心内膜垫缺损形式是房室（AV）管型室间隔缺损合并二尖瓣裂，而不存在原发孔房间隔缺损。在两名患有唐氏综合征的婴儿中，完成了该病症的术前诊断。M型超声心动图特征包括存在两个不同的房室瓣、二尖瓣前叶前部附着于室间隔以及正常的间隔运动。二维超声心动图显示房间隔完整、室间隔缺损、存在两个不同的房室瓣以及二尖瓣裂。采用成角（轴向）电影血管造影来描绘房室瓣形态特征、室间隔缺损位置以及不存在原发孔房间隔缺损。由于针对该病变的手术方法与其他形式的心内膜垫缺损不同，并且可能会遇到与房室瓣形态相关的特定问题，因此该病症的术前诊断很重要。
