体肺动脉分流术后分期矫治法洛四联症临床研究

Staged repair in pediatric tetralogy of Fallot after pulmonary artery shunt

目的分析体肺动脉分流术后行分期矫治术的小儿法洛四联症(TOF)患者的临床资料,探讨分期矫治的早期及中期疗效。方法选取自2009年1月至2015年12月沈阳军区总医院收治的完成5岁以下TOF分期矫治手术的46例患儿为研究对象,Ⅰ期姑息手术包括主-肺动脉中央分流27例,右侧改良B-T分流16例,右心室流出道补片加宽3例。姑息手术与矫治手术间隔12 d至23个月,平均间隔6.7个月。结果本组患儿术后因严重低心排综合征病死1例。45例患儿平均于姑息手术后6.7个月完成矫治手术;1例分流术后3个月再次行主-肺动脉中央分流,二次分流术后12个月完成矫治手术。矫治手术前患儿肺动脉Mc Goon比值为(1.45±0.34),明显较Ⅰ期姑息手术时的(0.90±0.23)增加;矫治手术前患儿肺动脉指数(PAI)为(170.1±44.4)mm^2/m^2,明显较Ⅰ期姑息手术时的(103.4±33.5)mm^2/m^2增加,差异均有统计学意义(P<0.05)。本组患儿平均呼吸机辅助时间(36.4±10.5)h,ICU滞留时间(5.2±3.6)d。术后主要并发症包括开胸止血1例,严重低心排综合征3例,呼吸功能低下(氧合指数<100 mm Hg)者5例,分别给予床旁腹膜透析和延长呼吸机辅助时间。45例患儿随访1~5年,1年随访率为100%,3年随访率为53%,5年随访率为24%。随访结果显示,所有患儿心功能Ⅰ级,左心室射血分数基本正常(50%~65%),右心室不同程度扩大,肺动脉瓣中-重度返流比例为71%。结论分期矫治小儿TOF效果满意,对于肺血管发育不良患者,分期矫治手术可降低病死率及术后严重并发症发生率,但也影响Ⅱ期矫治手术方式及术后肺动脉反流。

Objective To investigate the short and middle term effect of staged repair in pediatric tetralogy of Fallot（ TOF） after pulmonary artery shunt. Methods A retrospective study was performed on 46 cases of children younger than 5 years old with TOF undergoing staged repair received in the General Hospital of Shenyang Military Command from January 2009 to December 2015. Stage Ⅰpalliative shunt including the main central pulmonary artery shunt in 27 cases,the right side of the improved B-T shunt in 16 cases and right ventricular outflow tract patch widened in 3 cases. The interval between palliative shunt and repair was from 12 days to23 months,with an average interval of 6. 7 months. Results There was one early postoperative death,and the cause of death was severe low cadiac output syndrome. There were 45 cases performed repair in an average time of 6. 7 months after palliative shunt; the other one was performed second main pulmonary artery shunt 3 months after first palliative shunt,then was performed repair 12 months after second shunt. Before repair,the pulmonary Mc Goon ratio was（ 1. 45 ± 0. 34）,which was significantly increased than（ 0. 90 ±0. 23） in Stage I palliative shunt; PAI was（ 170. 1 ± 44. 4） mm2/m2 before repair,which was significantly increased than（ 103. 4 ±33. 5） mm2/m2 in Stage I palliative shunt,all the differences were statistically significant（ P 0. 05）. The average ventilator assisted time was（ 36. 4 ± 10. 5） hours and the retention time of ICU was（ 5. 2 ± 3. 6） days. Main postoperative complications included chest bleeding in 1 case,3 cases of severe low cardiac syndrome,low respiratory function（ oxygenation index 100 mm Hg） in 5 cases,patients were given bed peritoneal dialysis and prolonged breathing machine auxiliary. In the 45 cases,the follow-up was 1-5 years months,the follow-up rate of 1-year was 100%,3-year was 53% and 5-year was 24%. Follow-up results showed that all children were in heart function gradeⅠ with normal LVEF（ 50%-65%）,while the severity of pulmonary regurgitation in patients was 71% as the right ventricular enlargement in different levels. Conclusion Pediatric TOF stage repair effect is satisfied,for patients with pulmonary vascular dysplasia,staging and correcting surgery can reduce the mortality and the incidence of serious complications,while also affect Stage Ⅱ repair procedure and postoperative pulmonary regurgitation.