【摘要】 目的总结小儿三尖瓣下移畸形圆锥重建(Cone重建)的应用。方法 12例14岁以下三尖瓣下移畸形患儿接受Cone重建手术,其中男4例,女8例,年龄(6.8±4.2)岁,体质量(24.1±11.8)kg。根据Carpentier分型,A型1例、B型7例、C型4例。合并房间隔缺损8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。术前三尖瓣反流中度3例,重度9例。紫绀4例。NYHA心功能分级Ⅱ级4例,Ⅲ级7例,Ⅳ级1例。结果全组患者无手术死亡,11例成功行Cone重建,1例因重建失败行三尖瓣置换术。2例接受圆锥远端开窗,2例应用自体心包瓣叶扩大,2例应用人工腱索,2例加用双向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Cone重建的11例患者中,三尖瓣无反流和微量反流各2例,轻度反流3例,轻-中度反流3例,中度反流1例;心功能Ⅰ级10例,Ⅱ级1例。三尖瓣反流程度及心功能均较手术前显著改善(P<0.05)。结论 Cone重建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。更多还原

【Abstract】 Objective To summarize the cone reconstruction of Ebstein’s anomaly in children. Methods The clinical data of 12 consecutive children(under 14 years old) underwent cone reconstruction in our hospital from August 2013 to October 2017 were retrospectively analyzed. There were 4 males and 8 females, aged(6.8 ±4.2) years, with body mass(24.1 ± 11.8) kg, were included in this cohort. According to Carpentier’s classification, 1,7 and 4 patients were classified as A, B and C types, respectively. The combined deformities included 8 atrial septal defect, 2 patent ductus arteriosus, 1 pulmonary stenosis and 1 mitral regurgitation. There were 3 patients with moderate tricuspid regurgitations and 9 patients with severe tricuspid regurgitations. There were 4 patients with cyanosis. According to NYHA classification, 4, 7 and 1 patients were classified as Ⅱ,Ⅲ and Ⅳ degree respectively. Results No death occurred in this cohort. Among these patients, 11 received successful cone reconstruction and 1 underwent tricuspid valve replacement due to failure of valvuloplasty. There were 2 patients received distal fenestrations of the cone, 2 received valvular augmentations by autologous pericardium, 2 received artificial chordae tendineaes and 2 received bidirectional Glenn shunts respectively.During(24.4± 17.0)-month-follow up,no death or reoperation occurred. Among the 11 patients who successfully underwent cone reconstruction, there were 2 cases of tricuspid regurgitation(no regurgitation) and 2 cases of micro-regurgitation, 3 cases of mild-moderate regurgitation, 1 case of moderate regurgitation. And there were 10 cases of grade I and 1 case of grade II cardiac function. The tricuspid regurgitation and cardiac function were significantly improved after operation compared with those before operation(P < 0.05). Conclusion The proper application of targeting surgical techniques based on individualized characteristics is the key point of Cone reconstruction.更多还原