马凡综合征主动脉根部瘤的外科治疗  

Surgical treatment of the asending aortic root aneurysm related to Marfan syndrome

作　　者：梁志强[1] 王平凡[1] 何发明[1] 王涛[1] 张力[1] 

机构地区：[1]河南省胸科医院心血管外科,郑州市450008

出　　处：《中国心血管病研究》2007年第5期325-327,共3页Chinese Journal of Cardiovascular Research

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。Objective To summarize the experience of surgical treatment of aortic root aneurysm related to Marfan syndrome. Methods 34 eases 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 eases presenting with acute type Ⅰ aortic disseetion （DeBakey type） and acute type Ⅱ aortic dissection in 2, chronic type Ⅱ aortic dissection in 2 eases. Before operation, 17 eases with eardie function of class Ⅱ （NYHA）, 15 eases with class Ⅲ and 2 with class IV .Bentall procedure was performed in 27 eases, Bentall procedure and right hemi-areh replacement in 1 eases,6 eases 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 eases 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 proeedure and total aortic arch replacement combined with stented elephant trunk implantation.

关 键 词：马凡综合征 主动脉瘤 心脏外科手术 

分 类 号：R654.2[医药卫生—外科学]