马方综合征主动脉根部瘤手术治疗84例经验

目的探讨马方综合征合并主动脉根部瘤手术时机、手术方法和疗效总结。方法1995年2月至2004年10月手术治疗马方综合征主动脉根部瘤84例,其中主动脉夹层41例(DebakeyⅠ型32例、DebakeyⅡ型9例),根部瘤合并中～重度主动脉瓣关闭不全52例,根部瘤直径平均68mm±14mm;术前心功能(NYHA)Ⅰ～Ⅱ级43例,Ⅲ级30例,Ⅳ级11例。本组施行Bentall手术68例,Wheat手术6例,Cabrol手术5例,主动脉瓣替换加升主动脉成形术5例。其中急诊手术28例,择期手术56例。同期施行二尖瓣成形术3例,二尖瓣替换术3例。结果全组术后死亡3例,病死率3.57%。5例失访,76例随访3～117个月(平均55个月±31个月)。心功能Ⅰ～Ⅱ级74例,Ⅲ级2例。结论Bentall手术是治疗马方综合征根部瘤的首选方法,远期随访效果良好。

Surgical treatment of the aortic root aneurysm related to Marfan syndrome

OBJECTIVE: To review the experience of surgical treatment of aortic root aneurysm of Marfan syndrome. METHODS: We The clinical data of 84 Marfan syndrome patients, 61 males and 23 females, aged 35 +/- 12 (5 - 62), 41 cases presenting with aortic dissection (Debakey type I in 32 cases and type II in 9), 52 cases with moderate to severe aortic regurgitation, and 9 cases with moderate to severe mitral regurgitation, 43 cases with cardiac function of class I - II, 30 with class III and 11 with class IV according the New York Heart Association (NYHA) standard, who underwent surgical treatment for aortic root aneurysm with a mean diameter of 68 mm +/- 14 mm, were analyzed. Bentall procedure was performed in 68 cases, Wheat procedure in 6, Cabrol procedure in 5, and aortic valve replacement and aortoplasty in 5. Concomitant procedures included mitral value replacement and mitral valvuoplasty in 3 cases respectively. Urgent surgery was conducted in 28 cases, and elective operation in 56 cases. RESULTS: There were 3 in-hospital deaths (3.57%). 76 cases were followed up for a mean duration of 55 +/- 31 months. Three patients underwent reoperation. The cardiac function returned to class I - II except for 2 cases that remained at the class III. CONCLUSION: Bentall procedure should be the first choice of the surgery for aortic root aneurysm of Marfan syndrome with a low mortality and a good late outcome.