混合型完全性肺静脉异位引流的外科治疗

目的总结手术治疗混合型完全性肺静脉异位引流(total anomalous pulmonary venous connection,TAPVC)的经验。方法 2006~2018年,我院共完成51例混合型TAVPC患者(排除合并单心室、法洛四联症等患者)的外科治疗,其中男35例、女16例,中位年龄102.0(59.0,181.0)d,中位体重5.0(4.1,6.4)kg。根据解剖形态将患者分为3类:"3+1"型(38例,3支肺静脉回流入同一个部位,而另一支肺静脉回流另一侧部位);"2+2"型(9例,两侧的肺静脉分别回流入不同的位置);怪异型(4例,无法归入以上两类的怪异解剖类型)。结果无患者院内死亡,中位随访时间41.0(18.0,86.5)个月。术后发生肺静脉梗阻10例。Kaplan-Meier生存曲线示3种类型术后肺静脉梗阻率差异无统计学意义(P=0.239)。Cox分析发现术前肺静脉梗阻与术后肺静脉梗阻明显相关(P=0.024)。结论混合型TAPVC解剖形态多变,需要个体化手术方法。

Surgical treatment of mixed total anomalous pulmonary venous connection

Objective To analyze the outcomes of surgical repair for mixed total anomalous pulmonary venous connection(TAPVC).Methods Between 2006 and 2018,a total of 51 patients with mixed TAPVC underwent surgery in our hospital.Patients with such associated anomalies as single ventricle and tetralogy of Fallot were excluded.There were35 males and 16 females with a median age of 102.0(59.0,181.0)days and a median weight of 5.0(4.1,6.4)kg.Patients were divided into three categories based on the anatomy:"3+1"pattern(n=38,three pulmonary veins drained at one site,and the other drained at the opposite site);"2+2"pattern(n=9,the pulmonary veins from each lung joined to form a confluence and drained at separate sites);bizarre pattern(n=4,the anatomy could not be classified into the above two patterns).Results There was no in-hospital death.The median follow-up was 41.0(18.0,86.5)months.Postoperative pulmonary venous obstruction occurred in 10 patients.Kaplan-Meier survival curves showed no statistically significant difference in postoperative pulmonary venous obstruction among the three groups(P=0.239).Cox risk regression showed that preoperative pulmonary venous obstruction was significantly associated with postoperative pulmonary venous obstruction(P=0.024).Conclusion Mixed TAPVC has various anatomic morphologies and requires individualized surgery.