保留主动脉瓣的根部重建术治疗马方综合征主动脉根部瘤

Aortic valve preservation and root reconstruction in Marfan syndrome

目的总结保留主动脉瓣的根部重建术治疗马方综合征主动脉根部瘤的经验。方法2003年7月至2007年12月22例马方综合征患者接受保留主动脉瓣的根部重建术。其中男性12例，女性10例；年龄10～57岁；平均（28±10）岁。手术方法包括再植技术9例、成形技术8例、单片法2例，再植手术＋全主动脉置换1例，成形手术＋全弓置换＋支架象鼻手术1例，单片法＋全弓置换＋支架象鼻手术1例。术后随访17～64个月，平均随访（46±16）个月。结果无住院及随访期死亡，1例患者再次开胸止血，随访期无瓣膜相关并发症发生。截至最后一次随访，16例无主动脉瓣反流，4例少量反流，中度及重度反流各1例。中度及重度反流的患者于术后1年进行了再次瓣膜置换手术。结论保留主动脉瓣的根部重建术早、中期效果满意，可用于治疗马方综合征患者的主动脉根部瘤。

Objective To explore the experiences of aortic valve preservation and root reconstruction in patients with Marfan syndrome. Methods From July 2003 to Dec 2007, 22 patients with Marfan syndrome were treated by aortic valve preservation and root reconstruction. There were 12 male and 10 female, the age ranged from 10 to 57 years old with a mean of （28 ± 10） years. The operation procedures included rcimplantation technique in 9 patients, remodeling technique in 8 patients, and patch technique in 2 patients. In addition, reimplantation technique ＋ total aorta replacement in 1 patient, remodeling technique ＋ ＂aortic arch replacement ＋ stent-elephant trunk＂ in 1 patient, patch technique ＋ ＂aortic arch replacement ＋ stent-elephant trunk＂ in 1 patient. The patients were followed-up by 17 to 64 months with a mean of （46 ±16） months. Results No in-hospital and follow-up period death occurred. There was one reexploration for bleeding 1 d postoperative. No valve-related complication oecured during the follow-up. At the end of follow-up, no aortic regurgitation was demonstrate in 16 patients, but mild regurgitation in 4 patients, moderate regurgitation in 1 patient and severe regurgitation in 1 patient. Two patients with moderate and severe aortic regurgitation need reoperation 1 year postoperative. Condusion The early and mid-term results of aortic valve preservation and root reconstruction operations in Marfan syndrome were favorable.