新生儿梗阻型完全性肺静脉异位引流的治疗

The emergency operation of total obstructive anomalous pulmonary venous connection in newborn

目的评估新生儿完全性肺静脉异位引流（TAPVC）不同类型矫治手术方法和预后。方法1999年至2011年，共收治68例新生儿梗阻型TAPVC急诊手术治疗，平均年龄16天，其中心上型21例，心内型8例，心下型36例和混合型3例。心上型和心下型TAPVC是将肺静脉共汇与左心房后壁作侧侧吻合，心内型TAPVC在心房内将扩大的冠状窦去顶将异位的肺静脉隔入左心房。结果术后早期死亡2例，占2．9％。随访6个月至3年，经超声心动图随访，甘市静脉吻合口均无明显狭窄，血液流速1．10～1．42m／s。结论早期的梗阻型TAPVC的纠治中左心房后壁与肺静脉共汇的侧侧吻合远期效果良好，肺静脉的梗阻情况需要远期进一步随访。

Objective Evaluate the outcome of total anomalous pulmonary venous connection （TAPVC） repair in newborn, controlling for anatomic subtypes and surgical technique. Methods Between 1999 and 2011,68 patients （median age 16 days） underwent repair for supracardiac （21 ） , cardiac （ 8 ） , infracardiac （ 36 ） or mixed （ 3 ） TAPVC. All patients were emergencies, due to obstructed drainage. Supracardiae and infracardlac TAPVC repair included the side-to-side anastomosis be- tween the pulmonary venous （PV） confluence and the left atrium. Coronary sinus unroofing was preferred for cardiac TAPVC repair. Results Early mortality was 2.9% （2/68）. The echo showed no obstruction in the puhnonary vein anastomosis and flow rate was 1.1 m/s - 1.42 m/s in the follow-up of 3 years. Side-to-side anastomosis provides excellent results for TAPVC repair while left atrial enlargement procedures appear to be associated with higher risk of late a^Thythmias. Although early and aggressive reintervention for recurrent PV obstruction is mandatory, intrinsic PV stenosis remains a predictor of adverse out- come. The incidence of pulmonary vena is gradually reduced to 6% - 11%. This often occurred in the infraeardiac or mixed TAPVC. Conclusion The nicety of preoperative diagnose, the improvement of protection of heart function, using of pulmonary vena tissue for anastomose and avoiding of distortion of pulmonary venues and delayed closure of stemum can reduce the mortality. The preoperative degree of puhnonary veno obstruction and the time of emergency operation and the infracardiac or mixed TAPVC can affect prognosis. Along with the surgical technique, the mortality of TAPVC is gradually reduced and the result is amazing, but it is important to attach importance to the patient with re-stenosis of pulmonary veno, the time and method for reoperation.