15例肺动脉发育不良的肺动脉闭锁合并室间隔缺损的临床预后分析

Surgical staged treatment for hypoplastic pulmonary atresia with ventricular septal defect in fifteen cases

目的:探讨肺动脉发育不良的肺动脉闭锁合并室间隔缺损(PA-VSD)的手术策略,手术方法以及影响早期预后的因素。方法:回顾性分析15例肺动脉发育不良的肺动脉闭锁合并室间隔缺损患者资料,根据肺血主要来源分为两组,导管依赖组6例:男性2例,女性4例,中位年龄18(4~252)个月。非导管依赖组9例:男性4例,女性5例,中位年龄33(16~96)个月。全组行Sano分流13例,BT分流1例,中心分流1例。结果:全组15例患者全部接受分期手术,Sa O2由术前(62±16)%升至术后(89±10)%,术后患者均合并不同程度的肺部渗出,右心功能不全,早期出现呼吸机相关感染3例,经治疗后痊愈,死亡2例。随访11例,随访率85%(11/13)。全组随访期间无死亡。9例接受二期手术根治术,4例等待手术。结论:肺动脉发育不良的肺动脉闭锁合并室间隔缺损选择不同的手术方式,个体化治疗,可以取得较好的疗效。其中导管依赖型的围术期风险远低于非导管依赖型。

Objective： To investigate the surgical strategies of hypoplastic pulmonary atresia with ventricular septal defect（ PA-VSD） and the outcomes of postoperative prognosis in early stage. Methods： We retrospectively analyzed the clinical data of fifteen hypoplastic PA-VSD patients. Among them 6 patients with a median age of 18 months（ range,4-252 months） as ductal-dependent group. And 9 patients with a median age of 33 months（ range,2-96 months） as nonductal-dependent group. There are three surgical procedures in palliative surgery such as sano shunt（ n = 13）,Blalock-Taussig shunt（ n = 1）,central aorto-pulmonary shunt（ n= 1）. Results： Postoperative pulse oximetryoxy hemoglobin saturation Sp O2（ 89 ± 10） % was significantly higher than preoperative Sp O2（ 62 ± 16） %（ P 0. 05）. ALL the 15 patients underwent palliative surgery. Postoperative patients all complicated with pulmonary effusion,right cardiac insufficiency in varying degrees. 9 patients accepted two-stage operation after palliative surgery. 12 patients（ 12/14,85%） were followed up for 3-60 months. One case occurred bovine pericardial shunt blocking after sano shunt 1 year later. Another patient occurred Left pulmonary artery stenosis and left ventricular outflow tract stenosis. There was no late death during follow-up. Conclusion： hypoplastic pulmonary atresia with ventricular septal defect can be accomplished with satisfactory outcomesby examining the different procedures. The perioperative risk of ductal-dependent group is much lower than nonductal-dependent group.