改良Carpentier法矫治三尖瓣下移畸形

Correction of Ebstein’s Anomaly Using a Modified Carpentier’s Method

目的探讨改良Carpentier法矫治三尖瓣下移畸形(Ebstein畸形)的手术方法,总结其临床经验。方法回顾性分析2006年6月至2010年8月安徽医科大学第一附属医院13例Ebstein畸形患者手术治疗的临床资料,其中男6例,女7例;年龄(26.8±13.5)岁。所有患者均采用改良Carpentier法矫治,手术方法包括:切除部分房化右心室,折叠环缩三尖瓣瓣环至适当的大小,用自体心包片扩大后瓣叶/隔瓣叶,移位缝合切下的部分前瓣叶(向内旋转后)、后瓣及隔瓣叶缝至正常的瓣环水平,移位相应的乳头肌、腱索,扩大修复瓣叶,加固瓣环,并矫正其他合并的心血管畸形。结果所有患者术后均恢复顺利,无死亡。超声心动图提示:三尖瓣功能良好,三尖瓣瓣叶均在正常位置;三尖瓣有轻度至中度反流3例,无反流或有轻微反流10例。心功能分级(NYHA)I～Ⅱ级。所有患者均得到随访,随访时间3～15个月,平均8个月。术后3个月和1年分别复查超声心动图提示:三尖瓣启闭及右心室功能良好,三尖瓣无明显反流12例,1例出院时三尖瓣有中度反流患者转为轻度反流。所有患者恢复正常的生活或工作。结论改良Carpentier法矫治Ebstein畸形有较好的近期疗效,该手术方法切除无功能的房化右心室后重新缝合塑形,有利于右心室形态重建和功能恢复,通过自体心包片扩大瓣叶、充分的瓣叶移位及后乳头肌、腱索移位重建可达到良好的瓣膜成形效果。

Objective To explore the clinical correction of Ebstein’s anomaly using a modified Carpentier’s method and summarize the clinical experience.Methods We retrospectively analyzed data for 13 consecutive patients（6 males and 7 females,with an age of 26.8±13.5 years）with Ebstein’s anomaly who underwent operation in the First Affiliated Hospital of Anhui Medical University between June 2006 and August 2010.All patients underwent correction using a modified Carpentier’s method.Operative techniques included excising and suturing the right atrialized chamber;puckering and shortening the tricuspid annulus;detaching the septal and posterior leaflet and/or part of the anterior leaflet from the displaced annulus;broadening and enlarging the area of the posterior/septal valve leaflet using autologous pericardium,and reattaching them to the true tricuspid annulus;transecting and reimplanting the papillary muscle and chordae;and simultaneously correcting any other congenital malformations.Results All patients survived and recovered well.The cardiac functional grading ranged from Ⅰ to Ⅱ（New York Heart Association）.All patients were followed up for 3 to 15 months（average 8 months）.Postoperative echocardiograpy showed disappearance of tricuspid incompetence in 10 patients and mild or moderate tricuspid incompetence in 3 patients.The patients’ tricuspid valve leaflets were all at the normal level.At three months and at one year postoperation,rechecked echocardiograpy showed opening and closing of the tricuspid and right ventricular function recovering well,with no obvious incompetence in 12 patients,and moderate tricuspid incompetence lightened to mild in 1 patient.All patients returned to normal work and life.Conclusion Our technique for correcting Ebstein’s anomaly using a modified Carpentier’s method had satisfactory early results.The patients’ right ventricles were effectively reshaped and recovered function through excising and suturing the right atrialized chamber,and favorable tricuspid valvuloplasty effect was achieved by reattaching the enlarged leaflets using autologous pericardium to the true tricuspid annulus,and by transecting and reimplantating the papillary muscle and chordae.