目的　采用彩色多普勒超声心动图分析先天性纠正性大动脉换位的病理分型和合并畸形，研究该病病理特点。  方法 　2000年1月-2012年6月，40例经心血管造影或CT或手术证实为先天性纠正性大动脉换位(CCTGA)的患儿纳入研究，首次诊断年龄2天~4岁7月(平均年龄1.6±1.3岁),采用节段诊断法进行诊断。  结果　S.L.L（心房正位，心室左袢，主动脉左位）28例，均为左位心，I.D.D（心房反位，心室右袢，主动脉右位）12例，4例为左位心，8例为镜像右位心；合并畸形中最常见的是三尖瓣畸形导致的关闭不全（27例，67.5%），其次是室间隔缺损（15例，37.5%）和肺动脉瓣狭窄和/或肺动脉瓣下狭窄导致的左室流出道狭窄（14例，35%）；室间隔完整25例的患儿全部合并三尖瓣畸形导致的关闭不全，无1例合并左室流出道狭窄；室间隔缺损的15例患儿14例（93.3%）合并肺动脉瓣狭窄和/或肺动脉瓣下狭窄导致的左室流出道狭窄，仅2例合并三尖瓣畸形导致的关闭不全（13.3%）。  结论　S.L.L是先天性纠正性大动脉换位最常见的分型、三尖瓣畸形导致的关闭不全，室间隔缺损和肺动脉瓣狭窄和/或肺动脉瓣下狭窄导致的左室流出道狭窄是最常见的合并畸形，室间隔完整的CCTGA最常合并三尖瓣畸形导致的关闭不全，伴有室间隔缺损的CCTGA最常合并肺动脉瓣狭窄和/或肺动脉瓣下狭窄导致的左室流出道狭窄。

ition of great arteries (CCTGA) by color doppler echocardiography (CDE).　Methods　Forty patients with CCTGA established by surgery or CT or angiocardiography were analyzed retrospectively.  Results　28 cases were S.L.L, all of these were levocardia，12 cases were I.D.D，8 cases were mirror image dextrocardia and the other were levocardia. Tricuspid valve anomaly was the most common accompanied malformations, ventricular septal defect(VSD) was the second and the left ventricular outflow tract obstruction (LVOTO) was third. 25 cases with intact ventricular septum had tricuspid valve anomaly, none of these had LVOTO, most of 15 cases with VSD had  LVOTO, an only 2 cases had tricuspid valve anomaly.　Conclusion S.L.L was the most common type in CCTGA, tricuspid valve anomaly was the most common accompanied malformations, VSD was the second and LVOTO was the third. Tricuspid valve anomaly is always accompanied by CCTGA with intact ventricular septum, left ventricular outflow tract obstruction was always accompanied by CCTGA with ventricular septal defect.