Mori K, Ando M, Satomi G, Nakazawa M, Momma K, Takao A
Department of Pediatric Cardiology, Heart Institute of Japan, Tokyo Women's Medical College.
Pediatr Cardiol. 1992 Jan;13(1):24-9. doi: 10.1007/BF00788225.
Nine cases of imperforate tricuspid valve associated with dysplasia of the right ventricular myocardium, pulmonary valve, and right coronary artery are described. The mean frontal QRS axis of the electrocardiograms did not indicate left axis deviation in seven of the nine. Two-dimensional echocardiograms showed an imperforate tricuspid valve, normally aligned atrioventricular septum, and an irregular-shaped right ventricular cavity with a thin wall. In three cases with absence of the pulmonary valve, pulsed Doppler echocardiograms of the right ventricular outflow tract revealed antegrade flow during systole and retrograde flow during diastole, and selective angiography through a persistent ductus arteriosus showed retrograde filling of the blind-ended right ventricular cavity via the main pulmonary artery. Necropsy examinations showed an imperforate tricuspid valve with a thin-walled aneurysmal right ventricle in all six cases examined. Pulmonary atresia was observed in two cases, and the absence of the pulmonary valve in four. The right coronary artery was hypoplastic in four cases and absent in two. A shunt operation should be carried out in early infancy to prevent hypoxia after closure of the ductus arteriosus.
本文描述了9例三尖瓣闭锁合并右心室心肌、肺动脉瓣及右冠状动脉发育异常的病例。9例患者中，7例心电图平均额面QRS电轴未提示电轴左偏。二维超声心动图显示三尖瓣闭锁，房室间隔正常排列，右心室腔形状不规则且壁薄。在3例肺动脉瓣缺如的病例中，右心室流出道的脉冲多普勒超声心动图显示收缩期为前向血流，舒张期为逆向血流，经动脉导管未闭进行的选择性血管造影显示，盲端右心室腔通过主肺动脉逆向充盈。尸检显示，在所有6例接受检查的病例中，均存在三尖瓣闭锁且右心室呈薄壁动脉瘤样改变。观察到2例肺动脉闭锁，4例肺动脉瓣缺如。4例右冠状动脉发育不全，2例缺如。应在婴儿早期进行分流手术，以防止动脉导管闭合后出现缺氧。
