| 摘要: |
| 目的:初步探讨严重主动脉瓣反流并巨大左室的马凡综合征患者合并功能性二尖瓣反流（FMR）的发生率、术后变化规律和手术指征。方法:分析2000年1月至2007年12月19例主动脉瓣重度反流（反流量≥10 ml）并巨大左心室(舒张末直径≥7 cm) 的马凡综合征患者的临床及随访资料。结果:19例患者均采用Bentall手术治疗。无围手术期死亡。术后随访72人年,平均4.5年（3个月～7年）。FMR术后明显下降,改善率87.5％,6个月～1年后接近正常。结论:严重主动脉瓣反流并巨大左室的马凡综合征患者行Bentall手术效果良好。轻到中度的FMR（<10 ml）术后可随着左室的缩小而逐渐恢复消失；重度FMR（≥10 ml）若合并左心收缩功能低下（EF <50%）,FMR下降趋势不良,建议行二尖瓣成形术等积极处理。 |
| 关键词: 马凡综合征 巨大左心室 主动脉瓣重度反流 功能性二尖瓣反流 |
| DOI：10.3724/SP.J.1008.2008.01347 |
| 投稿时间：2008-03-18修订日期：2008-10-06 |
| 基金项目: |
|
| Giant left ventricle complicated with severe aortic regurgitation in Marfan patients with functional mitral regurgitation:a clinical study |
| ZHAO Tie-jun,XU Zhi-yun*,ZOU Liang-jian,WANG Zhi-nong,YANG Li-xin |
| (Department of Cardiothoracic Surgery,Changhai Hospital,Second Military Medical University,Shanghai 200433,China) |
| Abstract: |
| Objective:To analyze the incidence of functional mitral regurgitation (FMR),post-operation characteristics and operation indications of Marfan patients with severe aortic regurgitation and giant left ventricle.Methods: The clinical data and the follow-up data of 19 Marfan patients with severe aortic regurgitation (≥10 ml) and giant left ventricle (LVEDd≥7.0 cm),who were treated from Jan.2000 to Dec.2007,were analyzed retrospectively.Results: All patients received Bentall operation and there was no perioperation death.Follow-up was 72 patient-year (ranging 3 months-7 years).The degree of FMR decreased obviously after operation,with an improvement rate of 87.5%,and the FMR almost disappeared 6 months to 1 year later.Conclusion: Bentall achieves good outcome in Marfan patients with severe aortic regurgitation and giant left ventricle.Mild to moderate FMR(<10 ml) disappears with the decrease of the left ventricle size.For patients with severe FMR (≥10 ml) complicated with dysfunction of left ventricle,managed ventricular pacing is suggested. |
| Key words: marfan syndrome aortic regurgitation giant left ventricle functional mitral regurgitation |
