复合姑息手术治疗肺动脉发育不良型先天性心脏病

Combined palliative procedures for cyanotic congenital heart defects with intractable hypoplasia of pulmonary arteries

目的 探讨复合姑息手术治疗合并难治性肺动脉发育不良的肺血减少型复杂先天性心脏病（先心病）临床经验.方法 对自2001年8月至2009年9月我院外科治疗的31例合并难治性肺动脉发育不良的肺血减少型复杂先心病患儿进行回顾性分析,其中合并室间隔缺损的肺动脉闭锁26例,其他合并肺动脉狭窄的复杂先心病5例.根据两侧肺动脉发育情况选择2种或2种以上的姑息手术：一侧肺动脉发育良好者行格林（Glenn）术;肺动脉发育较差者常规行改良Blalock-Taussig分流或改良Waterston分流术;肺动脉极度发育不良者采用改良Melbourne分流.对合并的粗大体肺侧支血管者行肺动脉融合或侧支血管结扎及封堵术.两侧均行体肺分流23例;一侧行体肺分流,另一侧行Glenn者8例.结果 术后早期1例死于心跳骤停,早期死亡率为3.2%,术后并发症包括低心排综合征5例,灌注肺3例,肺部感染3例.3例因分流管道堵塞而再次行分流手术.平均随访（25±16）（6～72）个月,与术前比较,左肺动脉指数（14.9±6.2比8.1±3.7）、右肺动脉指数（17.7±7.8比12.7±8.1）和肺动脉指数（32.6±11.7比20.9±9.4）均显著提高,差异均有统计学意义（均P＜0.001）,与术前比较,Blalock-Taussig分流术（7.0±2.0比5.5±1.0）、改良Waterston分流术（9.2±3.6比5.7±4.0）和Melbourne分流术（7.4±2.5比2.2±0.4）术后肺动脉直径均增加,差异均有统计学意义（均P＜0.05）,两侧肺动脉均明显发育,血红蛋白浓度和末梢血氧饱和度分布由术前的（194±27）g/L和（65±11）%改善至（174±24）g/L（P＜0.05）和（84±6）%（P＜0.001）.4例肺动脉发育良好,已行二期矫治手术,另1例术后2年完成了二期Glenn手术.结论 对合并难治性肺动脉发育不良的肺血减少型复杂先心病,外科治疗应遵循个性化原则,不管是作为根治手术前的过渡治疗,还是最终治疗,复合姑息手术都是值得推荐的一种术式选择.

Objective To explore the clinical experiences of combined palliative procedures for cyanotic congenital heart defects with intractable hypoplasia of pulmonary arteries. Methods From August 2001 to September 2009, 31 patients with cyanotic congenital heart defects and intractable hypoplasia of pulmonary arteries underwent surgical procedures. Among them, 26 patients were pulmonary atresia with ventricular septal defect and the other 5 cases complicated congenital heart defects with pulmonary stenosis. Different kinds of palliative procedures were performed according to the morphology of right and left pulmonary arteries in every patient. If the pulmonary artery was well developed, Glenn procedure was performed. Modified Blalock-Taussig or Waterston shunt was performed if pulmonary arteries had hypoplasia. If the pulmonary arteries were of severe hypoplasia, Melbourne shunt was performed. Systemic-pulmonary artery shunts were performed bilaterally in 23 cases. Systemic-pulmonary shunt was performed in one side and Glenn procedure contralaterally in 8 cases. Results There was one early death because of cardiac arrest. The number of patients suffered from low cardiac output syndrome, perfusion lung and pulmonary infection postoperatively was 5, 3 and 2, respectively. Systemic-pulmonary shunts were reperformed after theoriginal operation in 3 cases because of occlusion of conduits. The mean follow-up time was 25 ± 16 months （6-72 months）. I〉eft pulmonary index（8. 1 ±3.7 vs 14. 9 ±6. 2）, right pulmonary index （12. 7 ±8. 1 vs 17.7 ±7. 8）and pulmonary index（20. 9 ±9.4 vs 32. 6 ± 11. 7） increased significantly （all P〈0. 001）. The pulmonary diameter increased significantly after modified Blalock-Taussig shunt （5. 5 ± 1. 0 vs 7. 0 ±2. 0） , modified Waterston shunt （5. 7 ± 4. 0 vs 9. 2 ± 3. 6） and melbourne shunt （2. 2 ± 0. 4 vs 7.4 ± 2. 5） （all P 〈 0.05） . Bilateral pulmonary arteries developed well compared with that of preoperative condition. Hemoglobin decreased from （194±27） g/L to （174±24） g/L （P〈0.05） and peripheral oxygen saturation increased from （65 ±11）% to （84 ±6）% （ P 〈 0. 001 ） . During the follow-up ultimate complete repair were performed in 3 cases and one patients underwent Glenn procedure. Conclusions The procedures should be considered for hypoplasia of pulmonary arteries in cyanotic congenital heart defects. Combined palliative operation is an adequate therapy.