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目的探讨右心室-肺动脉连接在分期治疗肺动脉闭锁/室间隔缺损中的应用。方法选取2013年11月至2015年1月收治的肺动脉闭锁合并室间隔缺损、肺动脉发育不良(Mc Goon指数0.94±0.27)患儿8例,手术年龄10 d~11个月,平均(3.2±4.7)个月。通过建立右心室-肺动脉连接恢复肺动脉前向血流。术后每3个月复查心脏超声。当肺动脉发育满意,Mc Goon指数达到根治标准时实施根治手术。结果无手术后早期死亡。术后随访3~13个月,平均(8.5±3.1)个月,肺动脉Mc Goon指数0.92~1.63,平均1.21±0.35,较术前显著增加(P=0.01)。其中1例术后13个月M c Goon指数为1.63,接受根治手术。1例术后12个月频繁缺氧发作,M c Goon指数1.27,接受根治手术。患儿术后血流动力学稳定,恢复顺利,最后一次随访心功能Ⅰ级。其他6例Mc Goon指数尚未达到根治手术标准,继续随访等待根治手术。结论肺动脉闭锁合并室间隔缺损、肺动脉发育不良患儿早期采用右心室-肺动脉连接恢复肺动脉前向血流,手术死亡率及并发症发生率低,能够明显促进自身肺动脉发育,可在肺血管发育满意时进行根治手术,临床效果良好。
Objective To investigate the application of right ventricular-pulmonary artery connection in the treatment of pulmonary atresia / ventricular septal defect. Methods From November 2013 to January 2015, 8 patients with pulmonary atresia and ventricular septal defect and pulmonary dysplasia (Mc Goon index 0.94 ± 0.27) were enrolled. The mean age was (3.2 ± 4.7) ) Months. Correct pulmonary artery anterior flow by establishing a right ventricular-pulmonary artery connection. Cardiac echocardiography was reviewed every 3 months after surgery. When pulmonary artery development is satisfactory, the Mc Goon index reaches the radical standard when radical surgery is performed. Results no early death after surgery. The patients were followed up for 3 to 13 months with an average of (8.5 ± 3.1) months. The Mc Goon index of the pulmonary artery ranged from 0.92 to 1.63 (mean, 1.21 ± 0.35), significantly higher than that before the operation (P = 0.01). One of the 13 patients had a M c Goon index of 1.63 at 13 months and underwent radical surgery. One case was hypoxemic at 12 months after operation. The M c Goon index was 1.27 and underwent radical surgery. Hemodynamics in children with postoperative stability, recovery smoothly, the last visit of cardiac function Ⅰ level. The other 6 Mc Goon index has not yet reached the standard of radical surgery, continue to wait for radical surgery. Conclusions Early pulmonary artery occlusion combined with ventricular septal defect and pulmonary dysplasia can restore the anterior pulmonary blood flow by using right ventricle - pulmonary artery connection. The mortality and complication rate are low, which can significantly promote the development of pulmonary artery, which is satisfactory in pulmonary vascular development When radical surgery, the clinical effect is good.