小儿三尖瓣下移畸形圆锥重建手术的应用体会

Application of conical reconstruction with three apical petal deformity in children

目的:分享一组以小儿为主病例三尖瓣下移畸形圆锥重建手术的应用体会。方法:选取13例三尖瓣下移畸形患者接受圆锥重建手术,其中男4例,女9例;1例成人,余12例均为小儿,年龄2.9~11.0岁,平均6.7岁,体重16.3~32.5 kg,平均23.0 kg。根据Carpentier分型,A型1例、B型8例、C型4例。合并房间隔缺损9例,动脉导管未闭2例,肺动脉瓣狭窄1例,二尖瓣关闭不全1例。术前三尖瓣反流程度中度3例,重度10例,合并紫绀4例。NYHA心功能分级Ⅱ级4例,Ⅲ级7例,Ⅳ级2例。结果:全组患者无手术死亡,12例成功行圆锥重建,1例因重建失败行三尖瓣置换术。全组13例患者中,围手术期再手术患者1例,2例接受圆锥远端开窗,3例应用自体心包瓣叶扩大,2例应用人工腱索,2例加双向Glenn手术。随访时间(24.4±17.0)个月,期间无死亡和再次手术病例。成功行圆锥重建的12例患者中,三尖瓣反流程度无反流和微量反流各2例,少量反流3例,少-中量反流3例,中量反流2例;心功能Ⅰ级10例,Ⅱ级2例。三尖瓣反流程度(P=0.002)及心功能(P<0.001)均较手术前显著改善。结论:圆锥重建是针对三尖瓣下移畸形的一种安全有效的手术方法,根据解剖结构的差别有针对性的应用个体化的技术是手术成功的关键,可以获得满意的早期疗效。手术的中远期效果尚需进一步的随访结果明确。

Objective To analyze the application of cone reconstruction in Ebstein＇ s anomaly,mainly in pediatrics.Method We retrospectively analyzed the clinical data of 13 consecutive patients underwent cone reconstruction in our hospital 4 males and 9 females were included in this cohort,among which 1 was adult,the other 12 were pediatrics.The age was 2.9 -11.0 years old,with an median age of 6.7 years,the weight was 16.3-32.5 kg,with a medium of 23.0 kg.According to Carpentier＇s classification,1、8 and 4 patients were classified as A、B and C type respectively.Combined deformities included：9 atrial septal defect,2 patent ductus arteriosus,1 pulmonary stenosis and 1 mitral regurgitation.3 patients＇ tricuspid regurgitations were moderate and 10 were severe.4 were cyanotic.According to NYHA classification,4、7 and 2 patients were classified as Ⅱ、Ⅲ and Ⅳdegree respectively.Results No death occurred in this cohort,among which,12 received successful cone reconstruction and 1 underwent tricuspid valve replacement due to failure of valvuloplasty.There were 1 reoperation,2 received distal fenestrations of the cone,3 valvular augmentations by autologous pericardium.Artificial chordae tendineaes and bidirectional Glenn shunts were applied in 2 patients respectively.During a（24.4 ± 17.0） months＇ follow-up,no death or reoperation occurred.Among the 12 patients who successfully underwent cone reconstruction,tricuspid regurgitations were significantly decreased（no regurgitation 2,trivial 2,mild 3,mild-moderate 3,moderate 2）（P=0.002） as well as improved NYHA classification（DegreeⅠand Ⅱ,10 and 2 patients respectively）（P〈0.001） compared with those preoperatively.Conclusion Cone reconstruction is a safe and effective procedure for the correction of Ebstein＇ s anomaly.The proper application of targeting surgical techniques based on individualized characteristics is the keypoint of the operation.Short-time results are satisfactory,while longer follow-ups are needed to verify the duration of the new valves.