肺动脉闭锁合并室间隔缺损患儿的肺循环血供和治疗策略  

Classification of pulmonary arterial supply in pulmonary atresia with ventricular septal defect and treatment strategies

作　　者：叶赞凯[1] 范祥明[1] 李志强[1] 杨学勇[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科北京市心肺血管疾病研究所, 100029

出　　处：《中国医药》2014年第5期636-640,共5页China Medicine

基　　金：北京市卫生系统高层次卫生技术人才培养计划（2011-14）;北京市科技计划项目（Z111100074911001）

摘　　要：目的 探讨肺动脉闭锁合并室间隔缺损患儿的肺循环血供和相应的治疗对策.方法 2012年4月至2013年10月在北京安贞医院接受手术治疗的48例肺动脉闭锁合并室间隔缺损患儿,患儿术前均接受多排螺旋CT检查及经胸超声心动图检查.根据多排螺旋CT检查结果确定侧支血管起源及走形路径,计算McGoon比值及Nakata指数.结果 48例患儿中,肺循环血供主要来自于动脉导管16例（33.3％）,粗大的体肺侧支动脉14例（29.1％）,动脉导管与体肺侧支共存18例（37.5％）;McGoon比值为（1.2±0.4）,Nakata指数为（130±88） mm2/m2.术式包括侧支汇聚术2例,体肺分流术29例,双向格林术13例,体肺分流加腔肺分流术4例.术后早期死亡2例.结论 肺动脉闭锁合并室间隔缺损患儿的肺动脉血供及解剖形态复杂多样化,多排螺旋CT结果结合经胸超声心动图结果综合评价肺血管情况及心内畸形可制定相应的治疗方案.Objective To investigate the classifications of pulmonary arterial supply in pulmonary atresia with ventricular septal defect and treatment strategies.Methods From April 2012 to October 2013,a total of 48 patients with pulmonary atresia with ventricular septal defect were enrolled.All the patients were scanned with multidetector-row computed tomography（MDCT） and transthoracic echocardiography（TTE）.All patients were evaluated in terms of the origins and the traces of major systemic-pulmonary collateral arteries （MAPCAs） as well as the McGoon ratio and Nakata Index.Results In the 48 cases,the pulmonary arterial supply was from the arterial duct（PDA） in 16 cases（33.3%）,MAPCAs in 14 cases （29.1%）,PDA coexist with MAPCAs in 18 cases （37.5%） ; the McGoon ratio was （1.21 ± 0.41） and the Nakata index was （130 ± 88） mm2/m2 ; During the perioperative stage,only two patients were dead.Procedures included unifocalization in 2 cases,Modified Blalock-Taussing shunt in 29 cases and bidirectional cavopulmonary anastomosis in 13 cases.Modified Blalock-Taussing shunt with bidirectional cavopulmonary anastomosis occurred in 4 cases.Conclusions Variations are found in the site of pulmonary atresia and the morphology of the pulmonary arterial supply in congenital anomaly of pulmonary atresia with ventricular septal defect.Generally,combined with MDCT and TTE,surgeons can obtain full-scale information of the specific cardiovascular abnormality and make surgical decisions to manage pulmonary atresia with ventricular septal defect.

关 键 词：肺动脉闭锁 室间隔缺损 肺动脉血供 

分 类 号：R542.54[医药卫生—心血管疾病]