法乐四联症合并单侧肺动脉缺如的外科治疗  

Efficacy of Various Surgical Treatment Strategies for Tetralogy of Fallot Patients Complicating With Unilateral Absence of Pulmonary Artery

作　　者：杨腾蛟 孙境[1] 王强[1] 李守军[1] 闫军[1] 张旌[1] YANG Tengjiao;SUN Jing;WANG Qiang;LI Shoujun;YAN Jun;ZHANG Jing(Center of Pediatric Cardiac Surgery,National Center for Cardiovascular Diseases and Fuwai Hospital,CAMS and PUMC,Beijing (100037),China)

机构地区：[1]中国医学科学院北京协和医学院国家心血管病中心阜外医院小儿心脏外科中心,北京市100037

出　　处：《中国循环杂志》2019年第3期276-280,共5页Chinese Circulation Journal

基　　金：国家重点研发计划资助(2017YFC1308100)

摘　　要：目的:总结法乐四联症合并单侧肺动脉缺如患者的临床资料,探讨治疗策略的合理选择。方法:回顾性分析我院2009年3月至2017年11月期间收治的法乐四联症合并单侧肺动脉缺如病例共10例,患者中位年龄3岁(6个月～39岁),所有患者均为左肺动脉缺如。术前根据计算机断层摄影术(CT)或心血管造影检查计算右侧肺动脉Nakata指数、Nakata指数Z值及McGoon比值,根据患者的肺动脉发育程度,选择根治手术或姑息手术,根治手术为全麻低温体外循环下行单肺矫治的法乐四联症根治术。姑息手术包括行右心室流出道重建术或改良Blalock-Taussig分流术。结果:5例患者行一期根治手术。另5例患者行姑息手术,后者随访中有2例完成根治手术,根治手术及姑息手术无院内死亡及并发症。5例一期根治患者的右侧肺动脉Nakata指数平均(805.7±857.0) mm^2/m^2(223.3～2 433.0 mm^2/m^2),Nakata指数Z值平均-0.8±3.6(-4.5～5.3)。5例姑息手术患者的肺动脉Nakata指数平均(89.2±33.6) mm^2/m(2 53.1～149.0mm^2/m^2),Nakata指数Z值平均-6.9±1.4(-8.2～-4.5)。一期根治患者平均随访(53.4±36.2)个月(11～98个月),姑息手术患者平均随访(40.8±32.7)个月(5～94个月),随访结果良好。结论:针对法乐四联症合并单侧肺动脉缺如患者,可以用肺动脉Nakata指数及Nakata指数Z值评估对侧肺动脉发育,根据肺动脉发育情况选择根治或姑息治疗,能够取得良好结果。Objectives: Tetralogy of Fallot(TOF) complicating with unilateral absence of pulmonary artery(UAPA) is a rare congenital heart disease, suitable surgical treatment strategy is of importance for prognosis. This research aims to explore the impact of surgical strategy on prognosis in these patients by summarizing the clinical information of TOF patients complicating with UAPA.Methods: A total of 10 cases of TOF complicating with UAPA hospitalized in our hospital from the March 2009 to the November 2017 were included in this study. The median age of the patients was 3 years(6 months to 39 years). The Nakata Index, Nakata Index Z score and McGoon ratio were calculated according to the data derived from CT and cardioangiography. Procedures, which included complete repair and palliative procedure, were performed according to the size of right pulmonary artery. The complete repair refers to TOF repair with hypothermia, extracorporeal circulation with intervening the pulmonary artery. Palliative procedure refers to right ventricular outlet reconstruction and modified right-sided Blalock-Taussig shunts.Results: Five patients were treated with a one-stage complete repair. Another 5 patients underwent palliative surgery for the first time, and 2 out of the 5 patients received complete repair during follow-up period. There was no hospital mortality and complication after complete repair and palliative operation in this patient cohort. The average Nakata index of pulmonary arteries in one stage repair patients was(805.7±857.0) mm2/m2(223.3 to 2 433.0 mm2/m2) and average Nakata Index Z score was-0.8±3.6(-4.5 to 5.3). The average Nakata Index of pulmonary arteries in palliative surgery patients was(89.2±33.6) mm2/m2(53.1 to 149.0 mm2/m2), and average Nakata Index Z score was-6.9±1.4(-8.2 to-4.5). The average follow-up time of one-stage repair patients was(53.4±36.2) months(11 months to 98 months), and the average follow-up time of palliative patients was(40.8±32.7) months(5 months to 94 months), the follow-up results of b

关 键 词：法乐四联症 单侧肺动脉缺如 Nakata指数 Nakata指数Z值 

分 类 号：R541[医药卫生—心血管疾病]