单纯先天性二尖瓣关闭不全的临床解剖特点分析及意义

摘要

目的 分析单纯先天性二尖瓣关闭不全(PCMI)的病理解剖特点及临床意义.方法 选取2010年8月至2014年12月首都医科大学附属北京安贞医院收治的PCMI患儿25例(PCMI组),随机选取同期收治的室间隔缺损合并二尖瓣关闭不全25例作为对照组,对比2组病理解剖特点.术前超声心动图检查评估二尖瓣反流程度、瓣叶增厚情况及二尖瓣瓣环径值;结合术中探查,观察二尖瓣瓣环径值、瓣下结构、瓣裂程度和部位及瓣叶增厚情况.结果 2组二尖瓣瓣环均有不同程度的扩大,PCMI组瓣环径值为(25.1 ±4.4)mm,对照组为(23.5±2.6)mm,2组差异无统计学意义(P＞0.05).PCMI组中度反流14例,重度反流11例;21例存在瓣裂,其中前瓣瓣裂13例(A2区8例,A3区4例,A1区1例),后瓣瓣裂4例(P2区2例,P1和P3区各1例),前后瓣均存在瓣裂4例(A2区+P2区1例,A2区+P3区1例,A3区+P2区1例,A3区+P3区1例);I度瓣裂6例,Ⅱ度瓣裂12例,Ⅲ度瓣裂3例;无瓣裂的4例中1例为瓣叶增厚瓣膜发育短小并脱垂致对合不良,1例存在副瓣致关闭不全,另2例A2区无腱索附着致前瓣脱垂.对照组瓣裂4例、瓣叶增厚僵硬6例、瓣叶增厚僵硬病例合并二尖瓣瓣裂2例,PCMI组瓣裂、瓣叶增厚僵硬比例、瓣叶增厚僵硬病例合并二尖瓣瓣裂的比例均明显高于对照组,差异有统计学意义[84.0％ (21/25)比16.0％ (4/25)、88.0％(22/25)比24.0％(6/25)、86.4％(19/22)比33.3％(2/6),均P＜0.05].结论 PCMI多存在二尖瓣瓣裂及瓣膜的异常,更易引起瓣膜的继发性损害,临床处理应持积极态度.%Objective To summarize the anatomic and pathological characteristics of pure congenital mitral insufficiency.Methods Totally 25 patients with pure congenital mitral insufficiency (PCMI group) and 25 patients with mitral valve insufficiency complicated with ventricular septal defect (control group) from August 2010 to October 2014 were retrospectively studied.Preoperative echocardiography was performed to evaluate the mitral insufficiency,the thickenings of the mitral valve and the diameter of the mitral valve ring.The intraoperative findings,such as mitral valve diameter,abnormality of subvalvular apparatus,mitral clefts and the thickenings of valve,were recorded.Results The mitral ring was enlarged in both groups but there were no significant differences between PCMI group and control group [(25.1 ± 4.4) mm vs (23.5 ± 2.6) mm].In PCMI group,there were 14 moderate regurgitation and 11 severe regurgitation;21 cases had mitral clefts including 13 anterior leaflet cleft (A2 8 cases,A3 4 cases and A1 1 case),4 posterior leaflet cleft (P2 2 cases,P1 1 case,and P3 1 case) and 4 both anterior leaflets and the posterior leaflets cleft (A2 + P2 1 case,A2 + P3 1 case,A3 + P2 1 case and A3 +P3 1 case);6 cases were I degree,12 cases were Ⅱ degree and 3 cases were Ⅲ degree leaflet cleft.Among 4 cases without mitral clefts,1 had extremely small and prolapsus mitral valve leaflets;1 had accessory valve leading to mitral regurgitation;2 cases had no chordae attached to A2 area leading to anterior leaflet prolapse.The incidence of mitral clefts,thickenings of mitral valve,thickenings of mitral valve associated with clefts in PCMI group were significantly higher than those in control group [84.0％ (21/25) vs 16.0％ (4/25),88.0％ (22/25) vs24.0％ (6/24),86.4％ (19/22)vs33.3％ (2/6),P＜0.05].Conclusion Mitralvalve malformations are common in the patients with PCMI,which may cause the secondary damages to the mitral valve.