梗阻型完全性肺静脉异位引流的外科治疗

The surgical treatment of obstructed total anomalous pulmonary venous connection

目的分析梗阻型完全性肺静脉异位引流(TAPVC)的外科治疗效果。方法收集2012年12月至2018年1月TAPVC患儿资料。入选标准:进行手术治疗的梗阻型TAPVC患儿。排除标准:(1)合并复杂畸形,包括单心室、内脏反位;(2)肺静脉最大流速小于1.2 m/s;(3)未接受过外科手术。共57例患儿入选,其中男33例,女24例。年龄(3.2±1.7)个月,体质量(5.0±0.9)kg。依据Darling分型分为心上型(30例)、心内型(16例)、心下型(5例)和混合型(6例)。行TAPVC矫治术,术后进行随访。结果术后随访(52±21)个月。全组术后死亡8例(14.0%,8/57)。术后出现肺静脉再狭窄7例(12.3%,7/57),死亡4例(57.1%,4/7);术后无肺静脉再狭窄50例,死亡4例(8.0%,4/50)。术后出现肺静脉再狭窄患者的死亡比例明显高于术后无肺静脉再狭窄者。结论梗阻型TAPVC患者的手术效果良好,但仍需关注术后肺静脉再狭窄的发生。

Objective To analyze the results of surgical treatment in patients with obstructed total anomalous pulmonary venous connection.Methods Inclusion criteria:Patients with obstructed total anomalous pulmonary venous connection,who underwent surgical treatment in our hospital between December 2012 to January 2018.Exclusion criteria:(1)With complicated malformations,such as single ventricle and visceral inversion;(2)The maximum flow velocity of pulmonary vein was less than 1.2 m/s;(3)Had not underwent surgery.57 patients[33 males and 24 females,age:(3.2±1.7)months,weight:(5.0±0.9)kg]were enrolled.There were four anatomical subtypes:supracardiac type(n=30),cardiac type(n=16),infracardiac type(n=5),and mixed type(n=6).Patients were followed up after operation.Results During the follow-up of(52±21)months,8 cases(14.0%,8/57)died postoperatively.Postoperative pulmonary vein stenosis was present in 7 patients(12.3%,7/57).The mortality of patients with postoperative pulmonary vein stenosis(57.1%,4/7)was significantly higher than that of patients without postoperative pulmonary vein stenosis(8.0%,4/50).Conclusion The results of surgical treatment in patients with obstructed total anomalous pulmonary venous connection were encouraging,however,recurrent pulmonary venous stenosis still should be taken care.