新生儿心下型完全性肺静脉异位引流的外科治疗

Surgical treatment of infra-cardiac total anomalous pulmonary venous connection in 13 neonates

目的报告新生儿心下型完全性肺静脉异位引流的矫治方法及效果。方法2009年10月至2015年1月，共纠治新生儿心下型完全性肺静脉异位引流13例。患儿出生6—28d，平均（15．08±7．42）d，体重2．5—4．8kg，平均（3．34±0．67）kg。所有手术均在浅低温高流量灌注下进行，3例患儿经右心房切口，切开房间隔，左心房后壁与肺静脉吻合；10例患儿采用心脏上翻法，左心房斜形切口与肺静脉无张力缝合。结果全组患儿手术顺利，无手术死亡病例。11例患儿术后延迟关胸，3例发生肺高压危象，6例保留房间隔缺损，3例使用临时心脏起搏器。除2例因低心排综合征于术后早期死亡，其余患儿均治愈出院。2例患儿术后反复出现肺部感染，心脏超声发现吻合口流速明显增快，其中1例失随访，1例再次手术解除肺静脉吻合口狭窄，痊愈出院。余9例随访1个月至5年。随访期均行心脏超声、x线胸片、心电图检查。多数患儿心功能良好，心胸比例较术前明显缩小，肺淤血消失，心电图示窦性节律，吻合口血流速度正常。结论新生儿心下型完全性肺静脉异位引流病情危重，早期诊断、早期手术效果良好。

Objective To evaluate the effectiveness of urgent surgical correction for infra-cardiac total anomalous pulmonary venous connection（TAPVC） in neonates. Methods From October 2009 to January 2015,13 patients with infra-cardiac TAPVC received surgical correction. The age of patients ranged from 6 to28 days[ （15.08±7.42）days] ,the body weight ranged from 2.5 to 4.8 kg[ （3.34 ±0.67） kg]. A median sternotomy was performed. Continuous cardiopulmonary bypass using bicaval cannulation with aortic cross-clamping and mild systemic hypothermia were used in all patients. Bi-atrial incision technique making anastomosis between the posterior left atrial wall and the vertical vein were employed in 3 cases. In the other 10 cases,the heart was elevated upward and to the right to expose the anomalous descending vertical vein to facilitate the anastomosis between the posterolateral left atrial wall and vertical vein. Results Emergent or subemergent operations were performed in all patients without surgical death. In the early stage after operation,delayed sternal closure was employed in 11 patients, pulmonary hypertension crisis occurred in 3 patients, small atrial septal defect was kept open for hemodynamic stabilization in 6 patients, temporary cardiac pacemaker for proper heart rate in 3 patients. All patients were uneventfully discharged except 2 patients died of severe low cardiac output syndrome. During the period of follow up, 2 patients presented with recurrent pulmonary infection and signs of pulmonary venous obstruction including the increased flow speed at the site of anastomosis. One of 2 patients was lost of follow up and the other patient was uneventfully discharged after the second operation. The rest 9 patients received postoperative follow up for 1 month to 5 years, echocardiography,X ray chest radiography, and electrocardiogram were performed during this period. As a result, all children had good cardiac function and with sinus rhythm on electrocardiogram and apparently reduced pulmonary congestion on radiography. No obstructive pulmonary venous retum was observed on echocardiography. Conclusion Corrective operation for infra-cardiac TAPVC in neonate on urgent basis may provide favorite outcomes.