室间隔完整型肺动脉闭锁和重度肺动脉瓣狭窄患儿的右心室发育分级及术式选择  

Classification of Right Ventricular Development and Factors Related to Surgery Options in Children With Pulmonary Atresia With Intact Ventricular Septum and Severe Pulmonary Stenosis

作　　者：孟红[1] 李慧[1] 王剑鹏[1] 李慕子 高一鸣[1] 王浩[1] MENG Hong;LI Hui;WANG Jianpeng;LI Muzi;GAO Yiming;WANG Hao(Ultrasound Imaging Center,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超声影像中心,北京市100037

出　　处：《中国循环杂志》2021年第11期1101-1106,共6页Chinese Circulation Journal

基　　金：云南省心血管病临床医学中心项目(FZX2019-06-01-09)。

摘　　要：目的:探讨室间隔完整型肺动脉闭锁和重度肺动脉瓣狭窄患儿的右心室发育分级和术式选择。方法:纳入2010年6月至2019年11月在我院就诊的215例室间隔完整型肺动脉闭锁患儿和重度肺动脉瓣狭窄患儿,根据三尖瓣瓣环直径Z值和右心室与左心室上下径比值,将右心室发育情况分为6级(1~6级依次为右心室增大、右心室大致正常、轻度发育不良、中度发育不良、中重度发育不良、极重度发育不良),总结并对比不同右心室发育分级患儿接受的手术方式。结果:在右心室增大(n=44)和右心室大致正常(n=88)的患儿中,99.2%(131/132)接受双心室矫治;在右心室轻度发育不良的患儿(n=32)中,21.9%(7/32)接受双心室矫治及体肺动脉分流术;在右心室中度(n=13)、中重度(n=13)、极重度(n=25)发育不良患儿中,39.2%(20/51)接受单心室矫治,29.4%(15/51)接受一个半心室矫治。二元Logistic回归分析显示,右心室发育分级(OR=0.639,95%CI:0.491~0.831,P=0.001)、冠状动脉病变(冠状动脉右心室瘘和右心室依赖型冠状动脉循环,OR=0.016,95%CI:0.001~0.204,P=0.001)、男性(OR=0.287,95%CI:0.092~0.897,P=0.032)是影响患儿接受双心室矫治的因素。结论:术前测量并计算三尖瓣瓣环直径Z值和右心室与左心室上下径比值,综合评估右心室发育情况,并按照右心室发育分级选择合理的手术方式,有助于获得较满意的术后疗效。Objectives:To explore the association between preoperative classification of right ventricular(RV)development and choice of related surgical procedures in children with pulmonary atresia with intact ventricular septum(PA/IVS)and severe pulmonary stenosis(PS).Methods:Two hundred and fifteen children diagnosed as PA/IVS and severe PS in our hospital between June 2010 and November 2019 were enrolled.Based on tricuspid valve annular diameter Z value(TV Z value)and the ratio of RV length to left ventricular length(RV/LV ratio),patients were classified into six groups according to RV development level,including enlarged RV,normal RV,mild hypoplastic RV,moderate hypoplastic RV,moderate-to-severe hypoplastic RV,and severe hypoplastic RV.The surgical procedures selected in these patients were analyzed and factors related to surgery option were determined in the children with different levels of RV development.Results:Among the patients with enlarged RV(n=44)or with normal RV(n=88),99.2%(131/132)underwent complete two-ventricular repairs.21.9%(7/32)patients with mild RV hypoplasia underwent two-ventricular repair and systemic pulmonary artery shunts operation.Among the patients with moderate RV hypoplasia(n=13),with moderate-to-severe hypoplastic RV(n=13),and with severe hypoplastic RV(n=25),39.2%(20/51)underwent one-ventricular repair and 29.4%(15/51)underwent one and a half ventricular repairs.Binary Logistic analysis showed that classification of RV development(OR=0.639,95%CI:0.491-0.831,P=0.001),coronary artery disease(coronary artery RV fistula and RV-dependent coronary artery circulation,OR=0.016,95%CI:0.001-0.204,P=0.001),male(OR=0.287,95%CI:0.092-0.897,P=0.032)are factors related to two-ventricular repair.Conclusions:TV Z value and RV/LV ratio are helpful parameters to evaluate RV development before operation.The surgical procedures could be chose based on the classification of RV development,which is helpful to achieve satisfactory outcomes.

关 键 词：室间隔完整型肺动脉闭锁 肺动脉瓣狭窄 右心室 三尖瓣瓣环 预后 

分 类 号：R54[医药卫生—心血管疾病]