三尖瓣置换治疗重症儿童Ebstein心脏畸形  

Treatment of Ebstein's Anomaly through Tricuspid Valve Replacement in Children

作　　者：甘辉立[1] 张健群[1] 葛永强 穆军升[1] 周其文[1] 孔睛宇[1] 郑斯宏[1] 伯平[1] 

机构地区：[1]首都医科大学附属北京安贞医院北京心肺血管疾病研究所心外科,北京100029 [2]西平县人民医院胸心外科,河南西平463900

出　　处：《实用儿科临床杂志》2008年第3期229-231,共3页Journal of Applied Clinical Pediatrics

摘　　要：目的评价三尖瓣置换术(TVR)治疗儿童重症Ebstein心脏畸形的效果,探讨提高其疗效的方法。方法回顾性分析1993年5月-2007年7月北京安贞医院收治的15例行16次TVREbstein心脏畸形患儿的资料。根据手术技巧不同,将14例术后存活患儿分别按3种方法分组,并行统计学比较,即房化心室折叠组7例,房化心室不折叠组7例;自体三尖瓣保留组8例,自体三尖瓣不保留组6例;人工瓣膜高位置入组8例,人工瓣膜原位置入组6例。应用Kaplan-Meier生存曲线计算5、10a生存率和事件发生率。结果1.死亡1例(6.6%),14例随访(91±84)个月,晚期死亡1例(7.1%),术后5、10aKaplan-Meier生存曲线生存率为(92.8±4.6)%、(86.5±5.81)%。发生瓣膜毁损而再次行TVR1例。2.全组心胸比率术前0.72±0.16,术后0.5a降低到0.61±0.17(P<0.05)。心功能纽约心脏协会(NYHA)分级Ⅰ级11例,Ⅱ级2例。3.在真瓣环原位置入人工瓣膜组出现心律紊乱比率显著高于高位置入组(P<0.05)。术后远期超声检查发现,未折叠房化心室组在出现心室矛盾运动或运动减弱及左心室功能受损比率显著高于折叠房化心室的患儿(P<0.05)。结论TVR治疗重症儿童Ebstein心脏畸形的围术期及中长期效果良好;根据病理解剖特点个体化选择术式可提高手术效果,TVR加房化心室折叠术能更有效地改善Ebstein心脏畸形左右心室功能。Objective To evaluate the effect of treatment of Epstein＇s anomaly through tricuspid valve replacement（TVR）in children, and analyze the method to improve the effect of TVR. Methods Data of 16 times TVR in 15 children with Ebstein＇s anomaly from May. 1993 to Jul. 2007 in Anzhen hospital were reviewed retrospectively. Results were analyzed and Kaplan - Meier curves were constructed to estimate patients＇ survival probability of remaining free of reoperation. Dividing the operated surviving patients （14 cases） according to the different procedure of techniques into groups with （7 cases） and without （7 cases） plication of the atrialized right ventricle,or groups with （8 cases） and without （6 cases） preserving autologous tricuspid valve, or groups implanting prosthesis in the true annulus （6 cases） or above the coronary sinus （8 cases） ,statistical comparison was made between these groups. Results 1. One case was early death（ 6.6% ）. With follow - up of 14 children who survived（91 ± 84） months. Another case died later（7.1% ） ,and the 5 and 10 - year survival rates were（92.8 24.6） %, （86.5 ± 5.81 ）% respectively in Kaplan -Meier curve, and 1 case had second-set TVR due to valve damage. 2. Eleven cases were in New York Heart Association （NYHA） class Ⅰ ,and 2 cases in NYHA class Ⅱ. The cardio thoracic ratio decreased after TVR in all 14 patients [ from a mean of （0.72 ± 0.16 ） to （0.61 ± 0.17 ） ] （P 〈 0.05 ）. 3. The group implanting prosthesis in the true annulus had more abnormal heart rhythm than the group implanting prosthesis above the coronary sinus （P 〈 0.05 ）, and the late echocardiography findings had revealed that group without plication of the atrialized right ventricle had more hypo kinetic or paradoxical motion of the atrialized ventricle, and more hypo function of the left as well as the right heart than the group with placation of atrialized right ventricle （ P 〈 0.05 ）. Conclusions TVR for children Epstein＇s an

关 键 词：EBSTEIN心脏畸形 三尖瓣置换 人工瓣膜 儿童 

分 类 号：R726.5[医药卫生—儿科]