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患有埃布斯坦畸形和腔肺分流术的成人的中期结果。

Mid-term outcomes in adults with ebstein anomaly and cavopulmonary shunts.

作者信息

Al-Najashi Khalid S, Balint Olga H, Oechslin Erwin, Williams William G, Silversides Candice K

机构信息

Toronto Congenital Cardiac Centre for Adults, University Health Network, University of Toronto, Toronto, Canada.

出版信息

Ann Thorac Surg. 2009 Jul;88(1):131-6. doi: 10.1016/j.athoracsur.2009.03.062.

DOI:10.1016/j.athoracsur.2009.03.062
PMID:19559211
Abstract

BACKGROUND

In patients with Ebstein anomaly and poorly functioning right ventricles, a cavopulmonary shunt (CPS) can be created to reduce the preload on the right ventricle. The purpose of this study was to examine the early and mid-term outcomes in adults with Ebstein anomaly who have undergone tricuspid valve repair or replacement with or without a concomitant CPS.

METHODS

We examined the outcomes of 40 consecutive patients seen at our center with Ebstein anomaly who had undergone tricuspid valve repair or replacement with (n = 23) or without (n = 17) concomitant CPS. Follow-up data were obtained by either chart review or contacting the referring cardiologist. Mid-term survival was examined using Kaplan-Meier curves.

RESULTS

The mean age at surgery was similar in patients with and without CPS (42 +/- 12 versus 39 +/- 19 years; p = 0.63). There were 2 early postoperative deaths owing to refractory right-sided heart failure. Mid-term follow-up data were available in 95% of patients. The mean follow-up time was 6.7 +/- 4.8 years. Patients who received a CPS more commonly had preoperative heart failure or cyanosis (p = 0.04) and had worse preoperative functional status (p = 0.09). In both groups, arrhythmias were the most common late complication. There were 5 late deaths, 3 of which occurred in patients with CPS. Five-year survival with or without CPS was comparable (83% +/- 9% versus 86% +/- 10%; p = 0.85).

CONCLUSIONS

Adolescent and adult patients with Ebstein anomaly undergoing tricuspid valve replacement or repair and concomitant CPS are at risk for early and mid-term complications. However, Ebstein surgery along with CPS appears to be a reasonable surgical strategy in patients not thought to be suitable for tricuspid valve surgery alone.

摘要

背景

对于患有埃布斯坦畸形且右心室功能不佳的患者,可建立腔肺分流术(CPS)以减轻右心室的前负荷。本研究的目的是检查接受三尖瓣修复或置换术(伴或不伴CPS)的成年埃布斯坦畸形患者的早期和中期结局。

方法

我们检查了本中心连续40例患有埃布斯坦畸形且接受三尖瓣修复或置换术(伴CPS者23例,不伴CPS者17例)患者的结局。通过查阅病历或联系转诊的心脏病专家获取随访数据。使用Kaplan-Meier曲线检查中期生存率。

结果

接受CPS和未接受CPS的患者手术时的平均年龄相似(42±12岁对39±19岁;p = 0.63)。有2例患者术后早期因难治性右心衰竭死亡。95%的患者有中期随访数据。平均随访时间为6.7±4.8年。接受CPS的患者术前更常出现心力衰竭或发绀(p = 0.04),术前功能状态更差(p = 0.09)。两组中,心律失常是最常见的晚期并发症。有5例晚期死亡,其中3例发生在接受CPS的患者中。接受或未接受CPS的患者5年生存率相当(83%±9%对86%±10%;p = 0.85)。

结论

接受三尖瓣置换或修复术及CPS的青少年和成年埃布斯坦畸形患者有早期和中期并发症的风险。然而,对于那些被认为不适合单独进行三尖瓣手术的患者,埃布斯坦手术联合CPS似乎是一种合理的手术策略。

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