小儿左上腔静脉回流异常的外科治疗

Surgical Approach of Left Superior Vena Cava Distal Abnormalities in Infant and Young Children

目的评价左上腔静脉（LSVC）回流异常的外科治疗价值。方法1999年4月～2004年12月我院共收治先天性心脏病伴LSVC回流终点异常患者19例，年龄2．7个月～6．5岁，体重3．1～15．0kg。合并复杂先天性心脏病9例、继发孔型房间隔缺损4例、部分性肺静脉异位引流（PAPVD）伴房间隔缺损（ASD）2例、法洛四联症（TOF）3例，右心室双流出口（DORV）1例。对LSVC的处理方法采用房间隔重建、LSVC移位合并心房间隔重建、LSVC连接到右心房、腔静脉-肺动脉吻合、部分性无顶冠状窦（中间位）的修补。结果全组死亡1例，系LSVC合并DORV和肺动脉高压，主要表现为术后肺动脉高压危象，左侧腔静脉-肺动脉吻合的血流梗阻，严重低心排血量、低氧血症致肾功能不全死亡。术后右心房压9～18mmHg。出院时行双心室修补术者术后脉搏血氧饱和度（SpO2）0．98～1．00，行单心室修补或分期手术者术后SpO2 0．79～0．88。出院前所有患者经彩色多普勒超声心动图检查显示LSVC回流和肺静脉回流均通畅。随访3个月～2年，所有患者活动量增加、心功能改善。结论对LSVC回流终点异常须根据不同的合并畸形采用不同的处理方法，尤其对合并肺动脉高压患者避免使用腔静脉-肺动脉吻合的方法。

Objective To evaluate the surgical approach of left superior vena cava（LSVC） distal abnormalities in infant and young children. Methods From April 1999 to December 2004, 19 cases of LSVC distal abnormalities were corrected by primary repair. There were 10 males and 9 females. Their age ranged from 2.7 months to 6.5 years and body weight from 3. 1 to 15.0 kg. Diseases complicated with LSVC included complex congenital cardiac disease 9 cases, ostium secundum atrial septal defect 4, partial abnormal pulmonary venous drainage （PAPVD） with atrial septal defect（ASI）） 2, tetralogy of Fallot（TOF） 3, and double outlet of right ventricle （DORV） 1 case. The ways for surgical treatment of distal abnormalities of LSVC were reconstruction of atrial septum, translocation of LSVC and reconstruction of atrial septum, right atrium and LSVC anastomosis, cavopulmonary anastomosis and repair of partially unroofed coronary sinus. Results One patient died and the diagnosis for the patient was LSVC with DORV and pulmonary hypertension （PH）. This patient died from crisis of PH , obstruction of blood flow in the left cavopulmonary anastomosis, severe low cardiac output,low arterial oxygen saturation and abnormal function of kidney. The mean pressure of right atrium was 9 to 18 mmHg. The percutaneous oxygen saturation （SpO2） was 0.98 1.00 for biventricular repair and 0.79-0. 88 for single ventricular repair and palliative repair. The echocardiography showed no obstruction of the blood flow in LSVC and pulmonary veins. The results of follow-up were satisfactory, from 3 months to 2 years. Conclusions Key for success of surgical approach of LSVC distal abnormalities is precise evaluation of different kinds of LSVC and different surgical approaches. Cavopulmonary anastomosis can not be used in the case of LSVC with PH.