2岁以上合并重度肺动脉高压先天性心脏病行单心室修复的初步研究

Preliminary research on single-ventricle repair procedure in congenital heart disease with severe pulmonary artery hypertension more than two years old

目的:探讨2岁以上合并重度肺动脉高压先天性心脏病(先心病)行单心室修复的手术指征及效果。方法:回顾性分析年龄>2岁合并重度肺动脉高压仅能行单心室修复的先心病患儿15例,男性9例,女性6例。年龄2.0～10岁,平均(7.8±3.8)岁。体质量11～24kg,平均(15.32±4.24)kg,术前经皮血氧饱和度(SPO2)88%～95%,平均(91.75±2.29)%。肺动脉压(mPAP)52～91mmHg((1 mm-Hg=0.133 kPa)),平均(54.61±16.8)mmHg。心脏畸形:单心室7例,三尖瓣闭锁3例,室缺远离两大动脉伴左心室发育不良型右心室双出口2例,心室不均衡型完全型房室通道2例,十字交叉心1例。均在静吸复合麻醉下先期行肺动脉环缩术,后期逐步完成双向格林或全腔肺动脉吻合术。结果:全组无住院死亡;PAB术前后mPAP、SPO2明显下降;PAB术后3～6年内完成双向格林(Glenn)4例,全腔肺动脉吻合术(TCPC)2例。结论:PAB术能有效降低肺动脉压力,≥2岁PAB术后可进一步完成Glenn或TCPC术。

Objective： study the clinical experience, operation effect and indication of Single-ventricle procedure in congenital heart disease with severe pulmonary artery hypertension more than twoyears old. Meth- ods： retrospective analysis 15 cases, can only do single ventricle repair procedure with severe pulmonary artery hypertension, male 9 and female 6 patients. Age, 2. 0 - 10 （7. 8 ±3. 8 ） years old, weight 11 - 24 （ 15. 32 ± 4. 24 ） kg, percutaneous blood oxygen saturation （SPO2 ） 88% - 95% （91.75 ± 2. 29）%, preoperative pulmonary aver- age pressure （mPAP） 52 - 91 （ 54. 61 - 16. 8 ） mmHg （ 1 mmHg = 0. 133 kpa）. Cardiac malformations ： single ventricle in 7 cases, tricuspid atresia 3 cases, Double outlet of fight ventricular with left ventricular dysplasia type in 2 eases, complete atrioventricular channel ventricular imbalance type 2 cases, cross heart 1 case, all cases had done the pulmonary artery banding （PAB） under Vein-inhalative anesthesia, and then complete Glenn or TCPC procedure further more. Results： No in-hospital death;The postoperative mPAP, SPO2 declined obviously, and 5 cases completed Glenn and 2 eases completed Fontan procedure in 3 -6 years after PAB. Conclusion： The pa- tients more than two years old may have taken Glenn and TCPC procedure after PAB.