Tetralogy of Fallot, Cardiac Hypertrophy, Pulmonary Hypertension and Aomalies of Great Vessels in WKY/NCrj Rats

我们对269只WKY/NCRJ大鼠的心、肺和大血管进行了解剖学研究。90例近月足月胎儿中有15例存在严重的肺动脉瓣增厚，15例有室间隔缺损，2-4d的79例新生儿中分别有10例和9例出现严重的肺动脉瓣增厚。这些异常同时发生在7个近月胎儿和5个新生儿中(法洛四联症)。55%的胎儿和56%的新生儿存在狭窄的肺流出道。19%的胎儿和26%的新生儿的肺动脉支异常增厚，其中大多数没有间隔缺陷。在约80%的胎儿中，室间隔中纬向肌束与左、右游离壁不连续，出生后与两个游离壁不连续。79名Neona…患者中有49%的人心脏异常沉重更多的TES。在大约一半的重心脏中，没有间隔缺陷或肺瓣膜和动脉病变。WKY胎儿和新生儿中4例为双弓，6例为右主动脉弓，47%的胎儿动脉导管异常小，51%的近月胎儿主动脉较大。这种先天性心脏病的起源是遗传的，但在胎儿生命后期被血流动力学改变。我们检查了78只妊娠21天的胎儿(G21)和83名出生后2天的新生儿(A2)，这是WKY大鼠与正常Wistar大鼠(F1)交配的第一代后代。此外，我们还检查了6组孕19天的胎儿：Wistar大鼠65只，WKY大鼠111只，F1大鼠85只，Fl×Fl大鼠100只，Fl×Wistar大鼠92只，Fl×WKY子代97只。这些结果表明，WKY大鼠心血管异常表现为常染色体隐性遗传或不完全常染色体显性遗传，具有不完全外显和变异性，也可能是多基因遗传。对31个WKY胎儿的染色体分析显示，与心血管畸形的发生没有特殊关系。40只正常Wistar、15只自发性高血压大鼠和10只主动脉结扎的大鼠使用高频探头确定了显示心脏的最佳参数。这些老鼠5到30周大，体重在105到705克之间。通过左或右胸骨旁窗分别以俯卧位或右卧位将探头放置在大鼠下方，获得脑室、心房、瓣膜和大动脉的二维和M型图像。超声心动图有助于对大鼠心脏进行解剖学评估和定量测量。较少

We examined anatomically the hearts, lungs, and great vessels of 269 WKY/NCrj rats at three fetal and three neonatal stages. Severe pulmonary valve thickening was present in 16 and ventricular septal defects with overriding of the aorta in 15 of the 90 near-term fetuses and in 10 and nine, respectively, of the 79 neonates at 2-4 d of age. These abnormalities occurred together (tetralogy of Fallot) in seven of the near-term fetuses and in five of the neonates. A narrow pulmonary outflow tract was present in 55% of the fetuses and in 56% of the neonates. The wall of the pulmonary arterial branch was abnormally thick in 19% of the fetuses and in 26% of the neonates, most of which did not have septal defects. In about 80% of the fetuses, the middle latitudinal muscle bundle of the ventricular septum was not-continuous with the left ventricular free wall, but rather with the right ; after birth, it was discontinuous with both free walls. The heart wag abnormally heavy in 49% of the 79 neona … More tes. In about half of the heavy hearts, there were no septal defects or pulmonary valvular and arterial lesions. There were double aortic arches in four and right aortic arches in six of the total WKY fetuses and neonates ; the ductus arteriosus was abnormally small in 47% and the aorta was large in 51% of the nearterm fetuses. This constellation of congenital heart disease is genetic in origin, but altered by hemodynamics late in fetal life.We examined 78 fetuses on d 21 of gestation (G21) and 83 neonates on d 2 after birth (A2), which were first generation offsping of WKY rats mated with normal Wistar rats (Fl). In addition, we examined six groups of fetuses on d 19 of gestation (Gl9) : 65 Wistar rats, 111 WKY rats, 85 Fl, 100 Fl x Fl, 92Fl x Wistar, and 97 Fl x WKY progeny. These results suggest that cardiovascular anomalies of WKY rati are transmitted as autosomal recessive or incomplete autosomal dominant traits with an incomplete penetrance and variable expressivity or as polygenic traits. Chromosomal analysis of 31 WKY fetuses revealed no abberations specifically related to the development of cardiovascular malformations.A high-frequency transducer was used to determine the optimal parameters for visualizing the heart in 40 normal Wistar, 15 SHR, and 10 aorta-banded rats. The rats were 5 to 30 weeks old and weighed between 105 and 705 grams. Two-dimensional and M-mode views of the ventricles, atria, valves, and great arteries were obtained by placing the transducer beneath the rats through the left or right parasternal window in either the prone or the right decubitus positions, respectively. Echocardiography is useful for obtaining anatomical evaluation and quantitative measurements in the hearts of the rats. Less