马方综合征主动脉根部瘤的手术时机和治疗对策

Surgical treatment of the aortic root aneurysm related to Mart＇an syndrome

目的总结马方综合征合并主动脉根部瘤的外科治疗经验，探讨手术时机和手术方法，总结疗效。方法1995年2月至2009年8月手术治疗马方综合征主动脉根部瘤113例；其中合并主动脉夹层59例，DeBakeyⅠ型主动脉夹层50例，DeBakeyⅡ型主动脉夹层9例；根部瘤合并中-重度主动脉瓣关闭不全81例，合并中．重度二尖瓣关闭不全9例，根部瘤直径平均（68±15）mm；术前心功能分级Ⅱ级70例，Ⅲ级32例，Ⅳ级11例。本组施行Bentall手术97例，wheat手术6例，Cabrol手术6例，主动脉替换加升主动脉成形术4例。其中急诊手术40例，择期手术73例。同期施行二尖瓣成形术3例，二尖瓣替换术3例，冠状动脉搭桥术2例，全弓置换加术中支架象鼻术3例。结果全组术后死亡3例，死亡率为2．6％。102例随访3—157个月，平均（78±49）个月。晚期死亡5例，随访102例，心功能Ⅰ～Ⅱ级98例，Ⅲ级4例。结论Bentall手术仍是治疗马方综合征主动脉根部瘤的首选方法，远期随访效果良好。对有家族史的患者避免形成主动脉夹层和主动脉破裂，根部直径≥4．5cm推荐手术治疗。

Objective To review the experience of surgical treatment of aortic root aneurysm of Marfan syndrome and to discuss the indications and the methods of surgical treatment. Methods We reviewed 113 cases receiving surgical treatment from February 1995 to August 2009 for aortic root aneurysm of Marfan syndrome. The New York Heart Association （NYHA） cardiac function was class Ⅱ in 70 cases, class Ⅲ in 32 cases and class Ⅳin Ⅱcases. Fifty-nine cases presented with aortic dissection （DeBakey type Ⅰin 50 cases, type Ⅱ in 9 cases） ,moderate to severe aortic regurgitation in 81 cases, moderate to severe mitral regurgitation in 9 cases. The mean diameter of aortic root aneurysm was （68 ±15）mm,Bentall procedure was performed in 97 cases,Wheat procedure in 6 cases, cabrol procedure in 6 cases, Aortic valve replacement and aortoplasty in 4 cases. Concomitant procedure were mitral value replacement and mitral valvuloplasty in 6 cases, coronary artery bypass grafting in 2 cases, total aortic arch replacement with stented elephant trunk procedure in 3 cases,respectively, Emergency surgery was done in 40 cases, elective operation in 73 cases. Results The entire postoperative drainage was 190-1750 （641 ±403）ml; there were 3 cases（2.6% ） in-hospital deaths. One hundred and two patients were followed up for a mean duration of （78 ±49） months. Late death occurred in 5 cases. Seven patients underwent reoperation, thoracic or abdominal aortic aneurysm operation had 3 cases, severe aortic insufficiency of aortic valve replacement had one case, severe mitral regurgitation in mitral valve replacement had 2 cases, perivalvular leak repair had one case. And NYHA class Ⅲwas in 4 cases, out of them was in NYHA class Ⅰ - Ⅱ. One years, 5 years and 8 year survival rates of the patients were 98.0%, 93.1% and 87.3%. Conclusions Bentall procedure should be the first choice of the aortic root aneurysm of Marian syndrome, with a low mortality and good late survival. Prophylactic surgery should be recommended to minimize risk of dissection and rupture if aortic root diameter is greater than 4.5 cm for Chinese.