【摘要】 目的总结马凡综合征主动脉根部瘤的外科治疗经验。方法2002年1月至2006年12月,手术治疗马凡综合征主动脉根部瘤34例,年龄16～54(29.5±4.1)岁。其中合并急性I型主动脉夹层(DeBakey分型)7例,急性Ⅱ型主动脉夹层2例,慢性Ⅱ型主动脉夹层2例。术前心功能分级(NYHA)Ⅱ级17例,Ⅲ级15例,Ⅳ级2例。共行Bentall手术27例,Bentall及右半弓置换1例,Bentall及全弓置换加“象鼻”手术6例,同期行二尖瓣成形术6例,胸骨翻转1例。结果全组术后死亡1例,死亡率2.9%。33例随访1个月至5年,2例接受二次手术,其余恢复良好,心功能I～Ⅱ级,无晚期死亡。结论Bentall手术是治疗马凡综合征主动脉根部瘤的首选方法,合并I型主动脉夹层的病例应行Bentall及全弓置换加支架“象鼻”手术。更多还原

【Abstract】 Objective To summarize the experience of surgical treatment of aortic root aneurysm related to Marfan syndrome. Methods 34 cases with asending aortic root aneurysm related to Marfan syndrome underwent surgical treatment from January 2002 to December 2006,aged 16-54(29.5±4.1)years.7 cases presenting with acute type I aortic dissection (DeBakey type) and acute type Ⅱ aortic dissection in 2, chronic type Ⅱ aortic dissection in 2 cases. Before operation, 17 cases with cardic function of class Ⅱ(NYHA),15 cases with class Ⅲ and 2 with class Ⅳ.Bentall procedure was performed in 27 cases, Bentall procedure and right hemi-arch replacement in 1 cases,6 cases underwent Bentall procedure and total aortic arch replacement combined with elephant trunk implantation. Results The in-hospital mortality was 2.9%(1/34),one patient died of multiorgan failure,33 cases were followed up ranged from 1 month to 5 years.2 cases underwent reoperation. There was no late death. The cardiac function returned to class I-Ⅱ. Conclusion Bentall procedure should be the first choice for ascending aortic root aneurysm of Marfan syndrome. Cases presenting with type I aortic dissection should be underwent Bentall procedure and total aortic arch replacement combined with stented elephant trunk implantation.更多还原