大动脉调转手术的临床应用

Arterial switch operation in older infants with severe pulmonary hypertension

目的 观察大动脉调转手术治疗合并心内畸形较多 ,重度肺动脉高压患儿完全性大动脉转位 (TGA)或Tausing Bing畸形根治性手术后疗效。方法 对 30例患儿实施了大动脉调转手术。男 2 0例 ,女 10例。年龄 3天～ 6岁 ,平均 9 4± 15月。新生儿 9例 ,1岁以上儿童 6例。体重 2 7～16kg,平均 6 1kg± 2 7kg。室间隔完整的TGA 7例 ,TGA合并室间隔缺损 (VSD) 19例 ,Tausing Bing畸形 3例 ,矫正性大动脉转位 1例。合并房间隔缺损 (ASD) 12例 ,多发VSD 3例 ,动脉导管未闭 (PDA) 18例 ,左室流出道狭窄 2例 ,右侧并列心耳 1例 ,重度肺动脉高压 2 3例。冠状动脉开口位置正常 2 6例 ,冠状动脉开口位置或走行异常 4例 ,其中 1例左前降支走行于主动脉壁内。术前 3例应用前列腺素E1治疗 ,1例行肺动脉束窄术 +体肺分流手术 ,1例行房间隔球囊扩张术。手术在全麻、低温、低流量体外循环下完成。经右心房切口 ,修补VSD。在两大动脉瓣上方 ,横断两大动脉 ,将左右冠状动脉开口移植至肺动脉近端。经肺动脉分叉下方 ,吻合肺动脉近端和升主动脉远端。用自体心包修复主动脉近端缺失部分 ,并吻合主动脉近端和肺动脉远端切口。结果 院内死亡 2例 ,死亡率为 6 7%。 2 2例术前肺动脉高压患儿 ,术后肺动脉压力均有明显下降。其余 2

Objective To investigate the clinical efficay of arterial swith operation on transposition of great artery (TGA) and Tausing-Bing anomaly. Methods Between June 2000 and December 2002, 30 consecutive patients, aged 3 days to 6 years (mean, 9.4±15 months) with the mean body weight was 6.1 kg±2.7 kg, underwent arterial switch operation. Among the 30 patients 7 suffered from TGA with intact ventricular septum, 19 from TGA with ventricular septal defect (VSD), 3 from Taussing-Bing anomaly, and 1 from corrected TGA; 12 were complicated by atrial septal defect (ASD) and 18 complicated by patent ductus arteriosus (PDA); 23 had severe pulmonary hypertension; 2 had left ventricular outlet stenosis. Coronary type A distribution was recognized in 26 cases, type D in 4, and one of them having the origin of the left descending artery tunneled in the aortic wall. The operation was performed under general anesthesia and extracorporeal circulation with low temperature and low volume blood flow. Prostaglandin 1 was administered pre-operatively in 3 patients, one of which underwent balloon atrial septostomy and one underwent pulmonary banding and systemic to pulmonary shunt pre-operatively. The aorta and pulmonary artery were transected above the valvular commisures, the coronary ostia with all the adjacent sinus of Valsalva were excised and re-implanted to the proximal neo-aorta, and then aortic anastomosis was completed. The proximal neo-pulmonary trunk was reconstructed with a large autologous native pericardiun as a posterior patch. The pulmonary anastomosis was completed after the aortic cross-clamp was released. The VSD was repaired through the atrium or proximal aorta with dacron patches. Results Two patients died with a hospital mortality rate of 6.7%. No death was directly related to any coronary artery problem. One 5 day-old neonate with TGA and an intact septum having refractory hypotension, hypoxemia, and acidosis pre-operatively underwent a smooth emergency operation. The patient had a refractory low cardiac output syndrome post-operatively and died 20 hours after the operation. Another patient with chylothorax died of allergy from iodophor 22 days postoperatively. The pulmonary pressure decreases significantly in 22 patients who had severe pulmonary hypertension preoperatively with the mean pressure 46.7 mm Hg preoperatively and 31.3 mm Hg postoperatively. 28 patients were discharged from hospital uneventfully. Follow-up of 1 to 31 months showed survival with no late complications and death. Conclusion The arterial switch procedure has a satisfying effect on TGA for patients older than 1 month with severe pulmonary hypertension.