介入及手术联合治疗伴有体肺侧枝的法鲁氏四联症和肺动脉闭锁  

Early outcome of surgical repair combined with collateral embolization for tetralogy of fallot or pulmonary atresia with major aorta pulmonary collateral arteries

作　　者：马琼[1] 刘迎龙[1] 闫军[1] 李守军[1] 沈向东[1] 王旭[1] 

机构地区：[1]中国医学科学院阜外心血管病医院外科,100037

出　　处：《中国分子心脏病学杂志》2007年第5期296-299,共4页Molecular Cardiology of China

摘　　要：目的评价介入栓堵侧枝合并手术矫治伴有体肺侧枝的重症法鲁氏四联症和肺动脉闭锁的临床结果,总结临床应用经验。方法回顾1992年至2006年我院介入加外科手术治疗25例伴有体肺侧枝的重症发鲁氏四联症和肺动脉闭锁,其中法四12例,肺动脉闭锁13例,年龄6月～17岁,体重7～55公斤,男性16例,女性9例,造影发现体肺侧枝共65支,平均2.6/例。外科根治手术23例,2例肺动脉闭锁行姑息手术,术前栓堵20例,术后栓堵5例,共栓堵侧枝53支,完全栓堵率86.8%。结果全组病例死亡6例,总死亡率24%,肺动脉闭锁根治术11例,死亡4例,死亡率36.4%,肺动脉闭锁姑息手术2例,死亡1例,法四根治术12例,死亡1例,死亡率8.3%。介入栓堵后根治术共18例,死亡3例,均为肺动脉闭锁,死亡率13%,根治术后介入栓堵5例,死亡2例,死亡率40%,其中法四4例死亡1例,肺动脉闭锁1例死亡1例。死亡原因为充血性心衰1例,低心排1例,肺出血1例,广泛肺渗出1例,肺缺血坏死1例,下腔静脉梗阻致多器官功能衰竭1例。结论介入栓堵体肺侧枝与外科手术联合治疗伴有体肺侧枝的重症法鲁氏四联症和肺动脉闭锁安全有效,可以减轻手术难度,减少术中出血,缩短手术时间,提高手术矫治成功率,减少手术创伤。术前栓堵侧枝对于手术成功尤为重要,术前明确体肺侧枝与固有肺动脉交通情况,对于单独供血的大体肺侧枝不宜拴堵,应于术中进行融合重建。与国外同期治疗结果比较我们的总死亡率较高。Objective To evaluate the clinical outcome of Surgical repair combined with collateral embolization for tetralogy of fallot or pulmonary atresia with major aorta pulmonary collateral arteries. Methotis Review the clinical data of 25 patients with tetralogy of fallot or pulmonary atresia with major aorta pulmonary collateral arteries, which received surgical repair combined with collateral embolization from 1992 to 2006. Among them TOF 12 cases, PAA 13 cases. Age ranged from 6 months to 17 years, weights 7-55Kg, male 16 cases, female 9 cases, 65 MAPCAs were founded （ 2.6 per patient） . Besides 2 PAA received palliative operation, 23 patients were performed surgical correction. 20 patients received MAPCAs embolization before surgical repair, 5 patients were done after surgical performance . Totally 53 MAPCAs were performed embolization, complete occlusion rate is 86.8%. Results All 25 patients received combined therapy of major aorta pulmonary collateral arteries embolization and surgical repair with total mortality of 24% . 11 PAAs were performed completed repair with a mortality of 36.4% （6 died ）. one of 2 PAAs which received palliative operation died for inferior vena cave obstruction. 12 patients of TOF received complete repair with a mortality of 8.3%. 18 patients with TOF or PAA were performed MAPCAs embolization before complete surgical repair, among them 3 with PAA died. 5 patients with TOF or PAA were performed MAPCAs embolization after complete surgical repair, among them 1 TOF and 1 PAA died respectively. Among the six dead patients, 1 died of congestive heart failure, 1 died of low heart pump function, 1 died of lung hemorrhage, 1 died of progressive lunge perfusion, 1 died of ischemic necrosis of lung, 1 died of multiple organ function failure caused by inferior vena cave obstruction. Conclusions In selective group of patients with dual pulmonary blood supply by both MAPCAs and native pulmonary arteries, combined therapy of collateral embolization and surgical repair simplifies complete surgic

关 键 词：法鲁氏四联症 肺动脉闭锁 介入栓堵 

分 类 号：R654.2[医药卫生—外科学] R541.1[医药卫生—临床医学]