摘要
目的探讨适当的手术方式,评价手术矫治室间隔完整的肺动脉闭锁的临床疗效。方法1992年1月至2004年8月手术治疗17例室间隔完整的肺动脉闭锁病儿,男9例,女8例;年龄15d^12岁,平均(25.5±7.9)个月;体重3.5~28.0kg,平均(7.8±5.4)kg。其中根治手术10例,三尖瓣Z值-2.3~1.2,平均(-0.78±0.34),肺动脉发育指数149.53~297.89mm2/m2,平均(206.35±82.15)mm2/m2,其中2例病儿因严重低氧血症术后次日加行体肺分流手术;生理矫治手术6例,Z值-6.1~0.2,平均(-2.7±0.92),肺动脉发育指数39.88~218.29mm2/m2,平均(131.85±72.93)mm2/m2,包括双向格林手术2例,体肺动脉分流手术1例,右室流出道重建合并体肺动脉分流术3例;一个半心室矫治术1例,首先行双向格林手术,2年后行房间隔缺损修补术,动脉导管结扎术和右室流出道重建术。结果围术期死亡3例(16.67%),2例为先行根治术次日加行体肺分流手术者,1例为右室流出道重建合并体肺动脉分流术者。术后并发症低心排综合征3例,低氧血症3例,乳糜胸1例,右心衰3例,余者均顺利康复。结论合并室间隔完整的肺动脉闭锁是一种少见的先天性心脏病,依照右心室大小、三尖瓣环大小、肺动脉发育情况及冠状动脉解剖等采用适当的手术方式可取得满意的治疗效果。
Objective Pulmonary artery atresia （PAA） with intact ventricular septum （WS） is an anatomically heterogeneous entity. A variety of surgical strategies is possible. We sought to evaluate the clinical results of various surgical corrections of PAA with IVS. Methods A retrospective review of our surgical database revealed 17 patients with PAA and IVS operation between January 1992 to August 2004. There were 9 males and 8 females. The age ranged from 15 days to 12 years [ （25.5 ± 7.9） months]. The body weight was 3.5 to 28.0 kg [ （7.8± 5.4） kg]. Radical operation was performed in 10 cases with the Z score - 2.3 ～ 1.2 （ -0.78 ± 0.34）, the pulmonary artery index （PAl） 149.53 ～ 297.89 mm^2/m^2 （206.35 ±82.15 mm^2/m^2 ）. Two infants received BT shunt operation for the severe hypoxia at first postoperative day. Palliative operation was performed in 6 cases with the Z score -6.1 ～ 0.2 （ - 2.7± 0.92）, the PAl 39.88 - 218.29 mm^2/m^2 （ 131.85 ± 72.93 mm^2/m^2 ）, including bi-direetional Glenn bypass （2 cases）, systemic-to-puhnousry arterial （BT） shunt （1 case）, right ventricular outflow tract （RVOT） reconstruction and BT shunt （3 cases）. One patient accepted one and a half ventricular repair, first underwent bi-directienal Glenn bypass operation, two years later ,underwent ASD oceulsian,PDA occlusion and RVOT reconstruction. Results 3 patients（16.7%） died at perioperative time [two patients who had the radical operation, but next day, had the BT shunt operation, one patient had the fight ventricular outflow tract （RVOT） reconstruction and BT shunt]. The rest recovered smoothly. The main complications included low cardiac output in 3 patients, hypoxemia in 3 patients, hydrothorax in 1 patients and tight heart failure in 3 patients. Conclusion Surgical outcome for patients with the PAA with IVS maybe satisfactory, strategries are to be chosen according to the anatomic subtypes such as the tricuspid valve diameter, fight ventricular size, pulmonary artery index and coronary anatomy.
出处
《中华胸心血管外科杂志》
CSCD
北大核心
2006年第2期76-78,共3页
Chinese Journal of Thoracic and Cardiovascular Surgery
