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首页> 外文期刊>Journal of cardiac surgery. >Surgery of the Ebstein's anomaly: early and late outcomes.
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Surgery of the Ebstein's anomaly: early and late outcomes.

机译：Ebstein异常手术：早期和晚期结果。

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BACKGROUND: Ebstein's anomaly of the tricuspid valve is a complex malformation. Several operations have been undertaken with varying results. The severity of the morphology of the lesion and ventricular function determine the spectrum of surgical techniques that are employed with varying results. METHODS: Between 1980 and 2005, 45 patients with Ebstein's anomaly underwent surgical repair. Age at operation ranged from 3 to 26 years (median 15.5, mean 18.0 years). In 41 patients (91%), tricuspid valvuloplasty was done. The other four patients had valve replacement with bio-prosthesis. Eight patients required ventricular unloading by creating bidirectional Glenn procedure in addition to a valve repair. Seven needed re-operations: one repeat valve repair, two valve replacements and three takedown of Glen shunt, and one Fontan operation. RESULTS: In-hospital deaths occurred in two patients (4.4%) and late mortality in four patients (9%). 95% of 39 survivors were followed for 5 months to 19.5 years (median follow-up, 5.0 years; mean follow-up, 6.9 years). The actuarial survival rate (Kaplan-Meier) was 96.5%+/- 2.4% at 1 year, 84.4%+/- 3.5% at 10 years and 83.3%+/- 5.6% at 19 years. At follow-up 90% were in functional class I or II with substantial improvement compared with their preoperative status. Doppler echocardiographic studies demonstrated good tricuspid valve function in most patients. CONCLUSIONS: Valve repair or replacement tailored to the anatomical substrate of the anomaly yielded good long-term results with substantial improvement in functional status. Bidirectional Glenn anastomosis combined with a valve repair improved ventricular function and improved both the early and late outcomes.
机译：背景：三尖瓣的Ebstein异常是一个复杂的畸形。已经进行了几次操作，结果各不相同。病变形态和心室功能的严重程度决定了所采用的手术技术的范围，其结果各不相同。方法：1980年至2005年间，对45例Ebstein异常患者进行了手术修复。手术年龄为3至26岁（中位数为15.5，平均18.0岁）。 41例（91％）患者进行了三尖瓣成形术。其他四名患者进行了生物假体瓣膜置换术。除瓣膜修复外，八名患者还需要进行双向Glenn手术，以减轻心室负荷。需要进行七次重新操作：一次阀门维修，两次阀门更换和三台Glen shunt拆卸，以及一次Fontan手术。结果：2例患者（4.4％）发生院内死亡，4例患者（9％）发生晚期死亡。 39名幸存者中有95％接受了5个月至19.5年的随访（中位随访时间为5.0年;平均随访时间为6.9年）。精算生存率（Kaplan-Meier）在1年时为96.5％+ /-2.4％，在10年时为84.4％+ /-3.5％，在19年时为83.3％+ /-5.6％。随访时，有90％属于功能性I或II级，与术前状态相比有明显改善。多普勒超声心动图研究表明，大多数患者的三尖瓣功能良好。结论：针对异常的解剖基底进行的瓣膜修复或置换取得了良好的长期效果，功能状态得到了显着改善。双向Glenn吻合结合瓣膜修复改善了心室功能，并改善了早期和晚期结局。

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