斯基米塔综合征:欧洲先天性心脏外科医生协会 (ECHSA) 的多中心研究
Vladimiro L Vida1, Massimo A Padalino, Giovanna Boccuzzo
1Pediatric and Congenital Cardiac Surgery Unit, University of Padua, Via Giustiniani 2, Padua, Italy.
Circulation
|September 9, 2010
概括
修复手术是安全有效的,大多数患者在手术后无症状. 然而,剩余的干狭窄仍然是一个问题,在这两种手术技术中都发生了类似的情况.
科学领域:
- 心脏病学 心脏病学
- 生产性心脏病 手术 手术 生产性心脏病
背景情况:
- 斯基米塔综合征是一种罕见的先天性心脏异常.
- 欧洲先天性心脏外科医生协会 (ECHSA) 进行了一项多中心研究,以评估手术结果.
研究的目的:
- 为了评估手术干预的安全性和有效性,用于scimitar综合征.
- 为了比较外科手术技术和确定长期的结果.
主要方法:
- 分析了从1997年至2007年期间接受了68名因西米塔综合征手术的患者的数据.
- 手术方法包括心脏内隔膜,状静脉再植入和肺切除.
- 医院死亡率和随访疗效是主要结局指标.
主要成果:
- 医院死亡率为5.9%,肺切除组 (33%) 的死亡率更高.
- 随访时间中位数为4.5年,有2例因肺动脉高血压而晚期死亡.
- 在13年内,在两个初级手术组中,无干管狭窄症超过83%;狭窄症在4.8-6%的患者中需要干预.
结论:
- 术治疗斯基米塔综合征是安全有效的,导致大多数患者无症状状态.
- 观察到一个显著的残留干管狭窄的发病率.
- 剩余静脉狭窄的发生率在心室内混和尖角静脉再植入技术之间是可比的.
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