双侧体-肺分流治疗左右肺动脉融合部狭窄或闭锁发绀型先天性心脏病  

Bilateral systemic pulmonary shunts for cyanotic congenital heart disease with stenosis or discontinuous pulmonary artery

作　　者：范祥明[1] 李志强[1] 苏俊武[1] 朱耀斌[1] 樊星[1] 刘迎龙[1] 

机构地区：[1]首都医科大学附属北京安贞医院小儿心脏中心,北京100029

出　　处：《中华实用诊断与治疗杂志》2015年第2期155-156,共2页Journal of Chinese Practical Diagnosis and Therapy

基　　金：首都医科大学基础-临床科研合作基金项目(13JL26;11JL50);国家自然科学基金(81371443);科研基地-科技创新平台项目(PXM2011_014226_07_000060);北京市自然科学基金资助项目(7112046);北京市自然科学基金(7122056);北京市自然科学基金(7142049);北京市自然科学基金(7142137)

摘　　要：目的评价双侧体-肺分流对左、右肺动脉融合部狭窄或闭锁的发绀型先天性心脏病的临床疗效。方法 30例复杂先天性心脏病患儿,肺动脉闭锁26例,肺动脉狭窄4例（右心室双出口2例、法洛四联征及功能单心室各1例）;合并体-肺侧支形成4例,内脏异位3例。根据患儿两侧肺动脉发育、肺动脉开口狭窄情况,行双侧改良B-T分流术2例,双侧中心分流术13例,右侧改良B-T＋左侧中心分流术8例,右侧中心分流＋左侧改良B-T分流术7例。结果术后因严重低氧血症死亡1例;并发低心排出量综合征3例,灌注肺2例,严重肺部感染1例;术后左、右肺动脉指数（（101±80）、（97±51）mm2/M2）及动脉血氧饱和度（（83±6）%）均较术前（（38±28）、（45±25）mm2/M2,（62±13）%）提高,血红蛋白水平（（178±30）g/L）较术前（（194±29）g/L）下降,手术前、后比较差异有统计学意义（P〈0.01）;随访18~52个月,8例完成格林手术,3例完成Fontan手术,3例完成根治手术。结论双侧体-肺分流手术可有效促进患儿左、右肺动脉发育,改善发绀,治疗左、右肺动脉开口狭窄或闭锁的发绀型复杂先天性心脏病疗效满意。Objective To explore the surgical outcomes of bilateral systemic pulmonary shunts for cyanotic congenital heart defect with stenosis or discontinuous pulmonary artery.Methods In 30 children with complex congenital heart disease,there were pulmonary atresia in 26 and pulmonary stenosis in 4including double outlet of right ventricular in 2,tetralogy of Fallot in 1and functional single ventricle in 1.Four patients were complicated by aortopulmonary collateral vessels,and 3by heterotopias.According to the development of the pulmonary arteries on both sides and the degree of pulmonary stenosis,2 patients underwent bilateral modified B-T shunts,13 underwent bilateral central shunts,8underwent right modified B-T shunt and left central shunt,and 7underwent right central shunt and left modified B-T shunt.Results One patient died of severe hypoxemia after operation.Three patients were complicated by low cardiac output syndrome,2by perfusion lung and 1by severe pulmonary infection.The left and right pulmonary artery indexes and arterial oxygen saturation were（101±80）mm2/M2,（97±51）mm2/M2 and（83±6）% after operation,higher than those before operation（（38±28）mm2/M2,（45±25）mm2/M2,（62±13）%）,and the hemoglobin level was lower after operation（（178±30）g/L）than that before operation（（194±29）g/L）（P〈0.01）.During the follow-up for 18 to 52months,Glenn,Fontan and radical operation were performed in 8,3and 3patients,respectively.Conclusion Bilateral systemic pulmonary shunts can relieve cyanosis,and promote the growth of left and right pulmonary arteries with a good result for cyanotic congenital heart disease with stenosis or discontinuous pulmonary artery.

关 键 词：先天性心脏病 体-肺分流手术 左右肺动脉融合部狭窄 

分 类 号：R726.5[医药卫生—儿科]