139例肺动脉闭锁的外科治疗

Surgical treatment of 139 cases with pulmonary atresia

目的 总结 139例肺动脉闭锁 (pulmonary atresia,PA)患者的外科治疗效果 ,探讨手术时机、术式选择和手术危险因素。 方法 1992年 4月至 2 0 0 2年 6月对 139例 PA患者连续行外科治疗 ,其中肺动脉闭锁合并室间隔缺损 (PA- VSD) 12 4例 ,一期根治性手术 75例 ,二期根治手术 10例 ,姑息性手术 39例 ;室间隔完整的肺动脉闭锁(PA- IVS) 15例 ,根治手术 9例 ,Z值平均为 - 0 .4± 0 .8;姑息性手术 6例 ,Z值平均为 - 1.5± 0 .7。双源供血的大主肺侧枝动脉予以结扎或介入栓堵 ,单源供血者需融合连接至固有肺动脉。 结果 围术期死亡 2 3例 ,手术死亡率16 .5 % ,其余患者均经治疗痊愈出院。PA- VSD患者中死亡 2 2例 ,肺动脉指数 (PAI)均 <15 0 mm2 /m2 ;PA- IVS患者中仅 1例中心分流术后因低氧血症死亡。手术危险因素为体外循环时间和患者的体重。 结论 PA患者尽早手术可取得较满意的效果 ;PA- VSD患者可根据 PAI是否 >15 0 mm2 /m2选择实施根治术或姑息手术 ,PA- IVS可根据右心室发育情况和 Z值选择术式。

Objective To sum up surgical treatment results of pulmonary atresia (PA) including 15 PA with intact ventricular septum(PA-IVS) and 124 PA with ventricular septum defects (PA-VSD) and to explore the optimal time, technique and risk factors of operation. Methods From Apr. 1992 to Jun. 2002, 139 patients with PA consecutive underwent surgical treatment. Among the 124 patients PA-VSD, 75 patients underwent one-stage radical operation and 10 patients accepted two-stage ones under hypothermic anesthesia with cardiopulmonary bypass(CPB), 39 patients underwent palliative operation. 15 patients were PA-IVS, 9 patients underwent one-stage radical operation with mean Z value -0.4±0.8, and 6 patients underwent palliative operation with mean Z value -1.5±0.7. Ligation and interventional embolization carried out in the patients supplied with major aortopulmonary collateral arteries(MAPCA) and patent ductus arteriosus(PDA). Unifocalization operation in patients supplied with MAPCA. Results 23 patients died in perioperative period. The operative mortality was 16.5%. Other patients were cured after operation. The pulmonary artery index(PAI) of 22 dead patients PA-VSD were less than 150 mm2/m2. One patient of PA-IVS underwent central palliative procedures died of hypoxemia. The risk factor of operations were time of CPB and body weight. Conclusions An early operation on patients with PA can be accomplished with satisfactory outcomes. It is feasible to evaluate indications of operation and choose radical or palliative procedures according to PAI(>150 mm2/m2) in patients with PA-VSD. The optimal technique of patients with PA-IVS should be dicided by Z value and size of right ventricle.