Ebstein心脏畸形的外科治疗

Surgical treatment of Ebstein anomaly

目的:总结Ebstein心脏畸形(Ebsteinanomaly)手术治疗的经验,以提高临床治疗水平。方法:1990年5月至1998年10月外科治疗Ebstein心脏畸形27例,其中合并预激综合征6例。年龄4.5～43岁,平均26.5岁。按NYHA推荐标准分级,术前心功能级者2例,级者12例,级者9例,级者4例。手术采用改良Danielson房化心室折叠术22例,三尖瓣置换术5例,其中6例合并预激综合征者行附加旁路切割术。结果:平均随访3年2个月,早期死亡1例,生存26例,无晚期死亡。术后心功能恢复至级者15例,级者9例,级者2例。合并预激综合征患者术后未再发生阵发性室上性心动过速。结论:掌握好手术指征和正确选择三尖瓣矫正方法有助于提高疗效,成形术者后瓣环缩后再加作后瓣环上提术尤为重要;加强术后处理,可明显降低术后早期并发症。

Objective: To introduce the experiences on the surgical treatments of Ebstein anomaly. Methods: Between May 1990 and October 1998, twenty seven patients with Ebstein anomaly were surgically treated, among them 6 patients were associated with preexcitation syndrome. The patient's ages ranged from 4.5 to 43 years with a mean of 26.5 years. The New York Heart Association (NYHA) classes were as follows: ClassⅠ, 2 patient; ClassⅡ, 12 patients; ClassⅢ, 9 patients; and ClassⅣ, 4 patients. Twenty two of these patients underwent modified Danielson procedure for Ebstein anomaly, while 5 of them received tricuspid valve replacement with mechanical prostheses. Division of accessory pathways was performed in 6 patients who had the combination of Ebstein anomaly and preexcitation syndrome. Results: One patient died early postoperation. The rest were followed up for a mean of 3 years and 2 months. There were no late deaths. The postoperative cardiac function of 26 patients had reached to ClassⅠ in 15 patients, ClassⅡin 9 patients and ClassⅢ in 2 patients. During the follow up period, there was no recurrence of tachycardia in the patients undergone the division of accessory pathways. Conclusion: The surgical indication and methods of correcting anomaly should be appropriately selected. During the posterior tricuspid leaflet annuloplasty, it is important to elevate the annulus of this portion . An intensive postoperative management may significantly decrease early postoperative complications.