分流术结合介入治疗分期根治肺动脉发育不良型重症法洛四联症  

Staged aortopulmonary shunt combined with intervention techniques for tetralogy of Fallot with severe hypoplastic pulmonary artery

作　　者：李志强[1,2] 殷劲[1,2] 朱耀斌[1,2] 刘扬[1,2] 范祥明[1,2] 苏俊武[1,2] 刘迎龙[1,2] 

机构地区：[1]首都医科大学附属北京安贞医院 [2]北京市心肺血管疾病研究所小儿心脏中心,北京100029

出　　处：《心肺血管病杂志》2014年第2期178-181,193,共5页Journal of Cardiovascular and Pulmonary Diseases

基　　金：北京市科技计划项目(211100074911001);北京安贞医院种子基金(2002Z02)

摘　　要：目的：总结体肺分流手术结合内科介入,治疗不适于一期根治的肺动脉发育不良型重症法洛四联症（TOF）的临床经验.方法：收集分析2010年3月至2013年8月,我中心行体肺分流术结合介入并最终完成根治手术的14例（男性10例、女性4例）肺动脉发育不良型重症TOF患儿资料.行分流术时,年龄3 ～ 34个月（中位年龄6个月）,体质量5～ 16kg,平均（9.2 ±3.35）kg;行球囊扩张术时年龄18 ～48个月,（中位年龄27个月）,行封堵术及根治术时,年龄12 ～ 72个月（中位年龄25个月,30个月）,体质量9 ～ 24kg,平均（13.88 ±4.77）kg.合并心血管畸形包括：房间隔缺损3例,永存左上腔静脉2例,单冠畸形1例,合并粗大体肺侧枝9例.14例均行姑息性中心分流式体肺分流术,其中2例同期行侧枝融合术（3支）.姑息性外科术后8例行内科介入封堵体肺侧枝（19支）,8例行内科介入肺动脉瓣球囊扩张术,2例同期行内科介入封堵及肺动脉瓣球囊扩张术.结果：全组无死亡病例及严重并发症.14例患儿根治手术前Nakata指数（mm3/mm2）和McGoon比值分别147.2 ～ 214.0,平均（173.2±24.53）和1.49 ～2.24,平均（1.78 ±0.26）相比体肺分流术前79.9 ～ 103.6,平均（93.03 ±7.58）和0.93～1.18,平均（1.00±0.01）明显升高（P＜0.05）.根治术前末梢血氧饱和度75％ ～ 98％,平均（85.36±6.12）％较体肺分流术前60％ ～ 85％,平均（72.86 ±6.32）％显著改善.血红蛋白浓度由体肺分流术前112 ～ 263g/L,平均（171.93 ±38.93） g/L下降至根治术前110 ～ 175g/L,平均（132.93±17.31） g/L.8例体肺分流术结合内科介入封堵体肺侧枝患儿根治术中,术后未见体肺侧枝相关并发症.8例行体肺分流术结合内科介入肺动脉瓣球囊扩张术患儿根治手术前Nakata指数（mm3/mm2）和McGoon比值150.5～210.3,平均（181.1 ±26.22）和1.59 ～2.24,平均（1.83±0.25）较肺动脉瓣球囊扩张术前94.4 ～146.7,Objective：To summarize the clinical experience of staged aortopulmonary shunt combined with intervention techniques for tetralogy of Fallot （TOF） with severe hypoplastic pulmonary artery.Methods：Analyze data of 14 cases（10 male,4 female） TOF with severe hypoplastic pulmonary artery performed with staged aortopulmonary shunt combined with intervention techniques and complete radical repair of tetralogy of Fallot in our center from March 2010 to August 2013.Age with shunt operation was 3-34months （median age 6months）,weighing 5-16kg （9.2 ± 3.35） kg; Age when the ball Dilation was 18-48months （median age 27months）,and when the occlusion and radical surgery was 12-72months （median age 25m,30m）,weighing 9-24kg （13.88 ± 4.77） kg.Associated cardiac anomalies include 3 cases of atrial septal defect,2 cases left superior vena cava,1 case single coronary artery anomoly.Major aortopulmonary collateral arteries were confirmed by angiography in 9 cases.The aortopulmonary shunt was performed in all 14 patients and 3 aortopulmonary collateral arteries were unifocalized in 3 patients simultaneously.Interventional balloon pulmonary valvuloplasty were performed in 8 cases and interventional embolization of aortopulmonary collateral arteries were performed in 8 cases,among which 2 types of interventional techniques were performed synchronously in 2 cases.Results：There was no death and serious complication.Nakata index （mm3/mm2） and McGoon ratios were 147.2-214.0,（173.2 ± 24.53） and 1.49-2.24,（1.18 ± 0.26） compared to aortopulmonary shunt 79.9-103.6,（93.03 ± 7.58） and 0.93-1.18,（1.00 ± 0.01） was significantly higher （P ＜0.05）.Peripheral oxygen saturation before radical operation was 75％-98％,（85.36 ± 6.12） ％,there was significantly improved than before aorotopulmonary shunt 60％-85％ （72.86 ± 6.32） ％ （P ＜ 0.05）.Hemoglobin decreased from before shunt112-263g/L,（171.93 ± 38.93） g/L to radical surgery 110-175g/L,（132.93 ± 17.31） g/L.The

关 键 词：法洛四联症 先天性心脏病 分期手术 介入技术 

分 类 号：R54[医药卫生—心血管疾病]