永存左上腔静脉为一少见的先天性心血管畸形,我们近期遇到3例,且均伴有心内其它畸形,现报告如下。例1,7岁、女孩。患儿自生后3～4个月发现口唇紫绀,活动时耐力差,行走喜蹲踞,有缺氧发作史。查体:发育差,面颊、口唇及指趾端重度紫绀、杵状指。胸廓对称,胸骨左沿3—4肋问可闻及Ⅱ/Ⅵ级收缩期杂音,肺动脉第2音呈单一音。胸片:肺血少,主动脉宽,左上纵隔影增宽,肺动脉凹陷,心影呈“靴形”。心电图:电轴右偏,右房右室大。二维超声提示“法四”。临床诊断:法鲁氏四联症。 Permanent left superior vena cava is a rare congenital cardiovascular malformations, we recently encountered three cases, and are associated with other cardiac deformities, are as follows. Example 1,7-year-old girl. Children born 3 to 4 months after found cyanosis lips, activity, poor endurance, walking like squats, a history of hypoxia. Examination: poor development, cheeks, lips and toe severe cyanosis, clubbing refers to. Thoracic symmetry, sternal left ribs along the ask 3-4 and Ⅱ / Ⅵ systolic murmur, pulmonary sound was a single second tone. Chest radiograph: less pulmonary blood, aortic width, left upper mediastinum widening, pulmonary embolism, heart shadow was “shoe-shaped.” ECG: right axis deviation, right atrium right ventricular large. Two-dimensional ultrasound prompts “France four.” Clinical diagnosis: Faruq’s quadruple disease.