目的：总结马凡综合征所致升主动脉瘤的外科治疗经验并探讨手术方法。方法回顾性分析2005年1月~2011年11月16例马凡综合征致升主动脉瘤患者的临床资料。结果16例患者术后早期无死亡。术后早期并发症6例（37.5%），其中心律失常2例，肺部感染2例，肾功能衰竭1例，气胸 1例。随访时间12~63个月，平均（25±9）个月。随访期间死亡2例（12.5%），死亡原因为腹主动脉瘤破裂1例，肾功能衰竭1例。抗凝并发症2例，冠心病1例。结论保留主动脉瓣的主动脉根部置换术与Bentall手术的疗效相近，并且能够避免机械瓣相关并发症，但其适应证相对严格；Bentall手术对于重症患者仍为首选。%Objective To summarize the experience of surgical treatment and to explore the oper-ation method choice of ascending aortic aneurysm caused by Marfan Syndrome. Methods The clinical da-ta of 16 patients from 2005 January to 2011 November were retrospectively analyzed. Results Among all, there was no operative mortality,but 6 cases of early postoperative complications(37. 5%),including 2 ca-ses of arrhythmia,2 cases of pulmonary infection,1 case of renal insufficiency and 1 case of pneumothorax. The follow-up time was 12~63(25 ± 9)months. During follow-up,2 patients died(12. 5%),1 due to rup-tured abdominal aortic aneurysms and 1 due to renal failure. Besides there were 2 cases of anticoagulation complications and 1 case of coronary heart disease. Conclusion Although sparing aortic root replacement of aortic valve has similar curative effect as Bentall operation and avoids the mechanical valve-related com-plications,the indications are relatively strict. Bentall operation is still the first choice for severe patients.
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