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目的 探讨改良锥形重建法三尖瓣下移畸形矫治术近中期临床效果.方法 回顾性分析2008年5月至2015年8月我院收治的18例三尖瓣下移畸形患者的临床资料,其中男8例、女10例,平均年龄20.3 (5～41)岁.根据纽约心脏病协会(NYHA)心功能分级,Ⅱ级12例,Ⅲ级6例,合并继发性房间隔缺损反常栓塞致左下肢急性动脉栓塞、截肢1例,术后因三尖瓣关闭不佳,联合双孔技术1例.采用改良锥形重建法矫治三尖瓣下移畸形,形成瓣叶间完全对合及中心性血流.对于前瓣叶发育差及较小者,用自体心包加宽瓣叶.所有患者均用自体心包行瓣环加固.结果 2例发生心律失常,经药物治疗恢复正常,余患者术后均恢复顺利,无死亡发生.复查超声心动图:1例三尖瓣中度反流,余17例患者瓣叶对合均较良好.术后随访9～38个月,心功能I级14例,Ⅱ级4例.结论 改良锥形重建法三尖瓣下移畸形矫治术近期临床效果肯定,可形成瓣叶的完全对合,抗反流能力强,减少二次手术及瓣膜置换的发生,降低术后死亡率.“,”Objective To investigate the early and mid-term clinical outcomes of the modified cone reconstruction in the treatment of Ebstein’s anomaly (EA).Methods Clinical data of 18 consecutive patients with EA in our hospital between May 2008 and August 2015 were retrospectively analyzed.All patients were diagnosed by echocardiography.There were 8 males and 10 females with an average age of 20.3 years ranging from 5 to 41 years.According to New York Heart Association classification,12 patients were classified into grade Ⅱ and 6 grade Ⅲ.One patient had acute arterial embolism and amputation of left lower extremity caused by paradoxical embolism of combined secundum atrial septal defect,and another one was combined with double-orifice technique due to postoperative poor closure of tricuspid valve.The modified cone reconstruction was used to correct the EA,to make leaflets coapted well and form central blood flow.For those patients whose anterior leaflet developed poor and smaller,valve leaflet was widened by using autologous pericardial.For all patients,tricuspid annulus was reinforced by autologous pericardial.Results Two patients suffered arrhythmia,and returned to normal after medication.The rest patients recovered well without death.Echocardiography found 1 patient with moderate regurgitation and the rest of patients’ leaflets coapted well and had no tricuspid stenosis.They were followed up 9 to 38 months postoperatively,and cardiac function of grade Ⅰ in 14 patients and grade Ⅱ in 4 patients.Conclusion The early and mid-term clinical outcomes of the modified cone reconstruction in the treatment of EA are affirmative which can make leaflets coapt completely and have a strong anti-regurgitation ability,reducing the incidence of re-operation,valve replacement and postoperative mortality.