肺动脉发育不良型法洛四联症根治术前姑息手术和介入技术的应用

目的 总结姑息手术和介入技术治疗合并肺动脉发育不良的重症法洛四联症的临床经验.方法 2002年12月至2009年12月,1586例患儿行法洛四联症根治术中18例(男12例、女6例)在根治手术前进行了姑息手术和介入技术相结合的复合治疗.合并心血管畸形包括:房间隔缺损3例,左肺动脉缺如2例,左肺动脉起自主动脉1例,永存左上腔静脉1例,合并粗大体肺侧支血管7例.根治手术前行一次姑息手术者13例,两次者4例,三次者1例.手术术式包括改良Blalock-Taussig分流术14例次,Waterston分流术4例次,右室流出道重建术3例次,肺动脉瓣球囊扩张术3例次,肺动脉环缩1例,行侧支血管结扎6例(16支),侧支血管融合1例(2支),侧支血管介入封堵2例(6支).结果 全组无死亡,1例因人工血管堵塞在术后第1天再次行体肺分流术,患儿根治手术前Nakata指数和McGoon比值[(200±81)和(1.77±0.51)]均较姑息手术前[(84±40)和(1.14±0.33)]有明显增加(P＜0.001),末梢血氧饱和度和血红蛋白浓度[(0.71±0.09)和(175±46) g/L]均显著改善[(0.86±0.05)和(149±15) g/L,P＜0.05].所有18例患儿均完成了最终的根治手术.结论 采用姑息手术和介入技术相结合的复合治疗措施能有效改善肺动脉发育,为合并肺动脉发育不良的重症法洛四联症根治手术创造条件.

Pallative procedures combined with intervention techniques for tetralogy of Fallot with severe hypoplastic pulmonary artery

Objective To evaluate the results of palliative procedures combined with intervention techniques for tetralogy of Fallot with severe hypoplastic pulmonary artery.Methods From December 2002 to Decembre 2009,complete repair of tetralogy of Fallot were performed in 1586 patients including 18 cases ( 12 male and 6 female) with severe hypoplastic pulmonary artery after 8 - 40 (20 + 11 ) month of palliative operations.The patients'age and body weight were 12 - 72 months and 8.0 - 16.5 kg at the time of complete repair.Associated cardiac anomalies include 3 cases of atrial septal defect,2 cases of absence of left pulmonary artery,1 case of the left pulmonary artery arising from the aorta,1 case of left superior vena cava.Major aortopulmonary collateral arteries were confirmed by angiography in 7 patients.Palliative operation was performed once in 13cases,twice in 4 cases and thrird in 1 patient before complete repair.Palliative operations included 14 procedures of Blalock-Taussig shunt,6 of Waterston shunt,3 of right ventricle outflow tract reconstruction and 3 of balloon pulmonary valvuloplasty.Major aortopulmonary collateral arteries were ligated in 4 cases,unifocalized in 1 and interventional embolized in 2 before complete repair.Results There was no early death.One reoperation was performed because of the obstruction of vascular prosthesis in the next day of shunt operation.Nakata index [ (84 ±40) versus (200 ±81 )] and McGoon ratio were increased after complete repair as compared with before operation [ ( 1.14 + 0.33 ) versus ( 1.77 + 0.51 ),P ＜ 0.001 ].Hemoglobin decreased from ( 175 ±46) g/L to ( 149 ± 15 ) g/L (P ＜0.05) and peripheral oxygen saturation increased from (71 ±9)％ to ( 86 ± 5 ) ％ ( P ＜ 0.05 ).Complete repair were performed in all 18 patients eventually.Conclusion Palliative procedures combined with intervention techniques may ameliorate the growth of pulmonary artery effectively and improve the results of complete repair of tetralogy of Fallot with severe hypoplastic pulmonarv arterv.