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

Initial palliative repair of pulmonary atresia with ventricular septal defect by rehabilitating right ventricle to pulmonary artery connection

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 assess the efficacy and safety of right ventricle to pulmonary artery connection as an initial procedure in patients with pulmonary atresia and ventricular septal defect. Methods Clinical data of 8 patients （mean age 3.2± 4.7 months, range 10 days-11 months） with pulmonary atresia, ventricular septal defect and pulmonary artery hypoplasia （ McGoon ratio 0.94 ± 0.27 ） treated during Nov. 2013 and Jan. 2015 were retrospectively reviewed. Con- nection of right ventricle to pulmonary artery was constructed to rehabilitate the anterior blood flow. All patients were carefully followed up with echocardiography at 3-month intervals after the procedure. When pulmonary artery achieved satisfactory growth and McGoon index was considered adequate ratio reached the criterion of curative resection, a com- plete repair was performed. Results No early deaths occurred. During the follow-up of 3 to 10 months （ mean 8.5 ± 3.1 months）, significant pulmonary artery growth was obtained （McGoon ratio 1.21 ＋0.35, P =0.01 ） in all patients. One case whose McGoon ratio reached 1.63 after 13 months received complete repair. Another case with severe cyanotic spells 12 months after the initial palliation whose McGoon ratio was 1.27 also underwent anatomic repair. Post- operative recovery was uneventfully with satisfactory haemodynamics. At the last visit, the cardiac function was grade I in New York Heart Association. The other 6 patients were still awaiting complete repair with a satisfactory pulmonary tree growth at last evaluation. Conclusion Early rehabilitating anterior blood flow by connecting right ventricle and pulmonary artery yields a relatively low morbidity and mortality, with good functional results in patients with pulmonary atresia, ventricular septal defect and hypoplasfic native pulmonary arteries. Ultimately, significant pulmonary artery growth can be obtained and this strategy allows complete repair in majority of patients.