本文报告1例先天性紫绀型心脏病伴多脾综合症婴儿在胎儿期和出生后超声心动图检查诊断为左位异构合并双侧上腔静脉、共同心房、心室双入口伴共同房室瓣、完全性大血管右转位、肺动脉瓣下狭窄、下腔静脉断离并与半奇静脉连续、动脉导管未闭。患儿体格良好,血氧饱和度83％。在出生二个月,肺动脉流出道收缩期压力阶差从出生时3.3kPa增至8.5kPa,紫绀加重,血氧饱和度50％。为避兔主肺分流术的并发症,在非 This article reports 1 case of congenital cyanotic heart disease with multiple spleen syndrome in infants and postnatal echocardiography diagnosis of left heterozygous with bilateral superior vena cava, common atrium, ventricular dual inlet with common atrioventricular valve , Complete right ventricle of large vessels, pulmonary valve stenosis, inferior vena cava disconnection and continuous with the semi-azygos vein, patent ductus arteriosus. Children with good physical fitness, oxygen saturation of 83%. At birth two months, pulmonary artery outflow tract systolic pressure gradient from 3.3kPa at birth to 8.5kPa, cyanosis increased, oxygen saturation 50%. To avoid the complications of the main pulmonary shunt in Africa