肺动脉发育不良型法洛四联症外科分期治疗的临床体会

Surgical strategies for tetralogy of Fallot with severe hypoplastic pulmonary artery

目的：总结肺动脉发育不良重症法洛四联症的外科治疗经验。方法：自2002年12月至2013年12月,共矫治例肺动脉发育不良型法洛四联症33例,其中男性22例,女性11例,根治手术时年龄12~282个月,平均（40±47）个月,体质量7~34kg,平均（14±5）kg。在根治手术前进行了一次或一次以上的姑息手术。合并心血管畸形包括：房间隔缺损8例,动脉导管未闭4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3例,合并粗大体肺侧枝血管12例。第一次姑息手术至根治手术时间间隔7~40个月,平均19个月。根治手术前行一次姑息手术者20例,2次者8例,3次者5例。姑息手术术式包括改良Blalock-Taussig分流术、改良Waterston分流术、右心室流出道重建术、肺动脉瓣球囊扩张术、肺动脉环缩以及侧枝血管结扎融合或介入封堵。结果：全组死亡1例,为根治手术后严重感染死亡,1例在首次姑息手术时因人工血管堵塞在术后第一天再次行体肺分流术,患儿根治手术前Nakata指数和Mc Goon比值分别为[（230±90）和（1.91±0.45）mm2/M2,均较姑息手术前（103±46）和（1.20±0.32）mm2/M2,有明显增加（P〈0.001）],末梢血氧饱和度[（71±11）%vs.（85±5）%]和血红蛋白浓度[（165±48）vs.（147±20）g/L]均显著改善（P〈0.05）。所有33例患儿均完成了最终的根治手术。体外循环时间82~240分钟,平均（139±39）分钟,主动脉阻断时间42~180分钟（77±28）分钟,气管插管时间5~875小时,平均59小时,ICU滞留时间1~37天,平均5天。结论：根据肺动脉发育以及体肺侧枝情况设计个性化的治疗策略,能有效改善肺动脉发育,完成肺血的单元化供血,提高肺动脉发育不良型法洛四联症根治手术疗效。

Objective：To evaluate the experiences of surgical repair for tetralogy of Fallot with severe hypoplastic pulmonary artery. Methods： From 2002 to 2009, definitive repair of tetralogy of Fallot with severe hypoplastic pulmonary artery were performed in 33 patients （22 male and 11 female） after （19 ± 10） month of palliative operations. Age and weight were （40 ±47 ） month and （14 ±5 ）kg at the time of definitive repair. Associated cardiac anomalies include 8 cases of atrial septal defect, 4 cases of patent ductus arteriosus, 2 cases of absence of left pulmonary artery, 1 case of, 3 case of left superior vena tara. Major aortopulmonary collateral arteries were confirmed by angiography in 12 patients. Once palliative operations were performed in 20 cases, twice in 8 cases and thrice in 5 patient before complete repair. Palliative operations include modified Blalock- Taussig shunt, modified Waterston shunt, right ventricle outflow tract reconstruction and balloon pulmonary valvuloplasty. Major aortopulmonary collateral arteries were ligated, unifoeajized or interventional embolized before complete repair. Results： There was 1 early death because of severe infection. Reoperation was performed be cause of the obstruction of vascular prosthesis in the next day of shunt operation. Nakata index （ 103 ±46） vs. （230 ± 90） mmZ/Ma and McGoon ratio （ 1.20 ± 0. 32） vs. （ 1.91 ± 0.45 ） were increased before definitive repair （ P 〈 0. 001 ）. Hemoglobin decreased from （ 165 ± 48） g/L to （ 147 ±20） g/L （ P 〈 0.05 ） and peripheral oxygen saturation increased from （71 ±11 ） % to （ 85 ±5 ） % （ P 〈 0. 001 ）. Complete repair were performed in all 33 patients eventually. CPB time was 82 -240 （139± 39）min and ACC time was 42 - 180 （77 ± 28 ）rain. Condusion： Individualized surgical strategies on the basis of the development of pulmonary artery and major aortopulmonary collateral arteries can ameliorate the growth of pulmonary artery effectually and unifocalize the pulmonary blood, eventually improve the result of definitive repair of tetralogy of Fallot with severe hypoplastic pulmonary artery.