婴儿完全性肺静脉异位连接外科治疗

Experience with Operation for Total Anomalous Pulmonary Venous Connection in Infancy

目的总结23例婴儿完全性肺静脉异位连接(totalanomalous pulmonary venous connection,TAPVC)的外科治疗经验。方法全组均在中度低温体外循环下进行手术,其中急诊手术6例。患儿平均年龄(5.7±3.6)个月,平均体重(5.8±1.5)kg。心上型11例采用双房径路矫治并结扎垂直静脉,用可吸收缝线吻合肺静脉共干与左心房后壁切口;心内型12例,仔细辨认合并三房心畸形,避免损伤传导系统。均采用心包补片修补扩大的房间隔缺损。结果手术早期死亡2例(死亡率8.7%)。18例随访2个月～3年,患儿心功能明显改善,生长发育正常。结论TAPVC可由超声心动图确诊,应尽早手术矫治。对于新生儿或婴儿期反复出现肺炎、低氧血症、心衰等患儿,在临床症状相对改善的情况下应急诊手术;同时加强体外循环和监护室的密切协作,完善术后处理方法。

Objective To report our experience of surgical treatment for total anomalous pulmonary venous connection （TAPVC） in infancy. Methods Twenty-three patients aged from 25 days to 12 months （mean 5.7 months） with TAPVC underwent surgical repair in this institute between April 2005 and June 2008. The type of deformities included supracardiac （11 cases） and intracardiac （12 cases）. The correction was performed under cardiopulmonary bypass and 6 received urgent operation. The technique for repair of supracardiac TAPVC was to perform a hi-atrial transverse incision followed by adequate anastomosis between the pulmonary venous confluence and left atrium, as well as ligation of vertical vena proximally. With intracardiac TAPVC, the repair was performed by completely un-roofing the coronary sinus. Enlarged atrial septal defect was then closed with a patch of pericardium with caution taken to avoid the area of the conduction tissue. Results The operative mortality was 8.7%. The postoperative follow-up was available in 18 patients ranging from 2 months to 3 years. All patients recovered well and were in NYHA functional class Ⅰ.Conclusion The diagnosis of TAPVC can be confirmed by eehocardiography, and surgical repair of TAPVC should be performed as soon as possible. Preoperatively, in patients receiving medical therapy for respiratory tract infection or heart failure, especially requiring mechanical ventilation, urgent operation should be considered.