CAJ | 학술논문

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,DeBakey Ⅰ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≥4.5 cm推荐手术治疗.
목적 총결마방종합정합병주동맥근부류적외과치료경험,탐토수술시궤화수술방법,총결료효.방법 1995년2월지2009년8월수술치료마방종합정주동맥근부류113례；기중합병주동맥협층59례,DeBakey Ⅰ형주동맥협층50례,DeBakeyⅡ형주동맥협층9례；근부류합병중-중도주동맥판관폐불전81례,합병중-중도이첨판관폐불전9례,근부류직경평균(68±15) mm;술전심공능분급Ⅱ급70례,Ⅲ급32례,Ⅳ급11례.본조시행Bentall수술97례,Wheat수술6례,Cabrol수술6례,주동맥체환가승주동맥성형술4례.기중급진수술40례,택기수술73례.동기시행이첨판성형술3례,이첨판체환술3례,관상동맥탑교술2례,전궁치환가술중지가상비술3례.결과 전조술후사망3례,사망솔위2.6％. 102례수방3～157개월,평균(78±49)개월.만기사망5례,수방102례,심공능Ⅰ～Ⅱ급98례,Ⅲ급4례.결론 Bentall수술잉시치료마방종합정주동맥근부류적수선방법,원기수방효과량호.대유가족사적환자피면형성주동맥협층화주동맥파렬,근부직경≥4.5 cm추천수술치료.
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 ⅣV in 11cases.Fifty-nine cases presented with aortic dissection ( DeBakey type Ⅰ in 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 Ⅲ was 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 Marfan 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.