Attenhofer Jost Christine H, Connolly Heidi M, Scott Christopher G, Ammash Naser M, Bowen Juan M, Schaff Hartzell V
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA.
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA. Electronic correspondence:
J Heart Valve Dis. 2017 Jan;26(1):45-53.
The study aim was to analyze the authors' experience with aortic root surgery in Marfan syndrome (MFS), and to expand the surgical outcome data of patients meeting the Ghent criteria (Marfan registry).
Analyses were performed of data acquired from MFS patients (who met the Ghent criteria), including an aortic root surgery and Kaplan-Meier survival.
Between April 2004 and February 2012, a total of 59 MFS patients (mean age at surgery 36 ± 13 years) underwent 67 operations for aortic root aneurysm (n = 52), aortic valve (AV) regurgitation (n = 15), acute aortic dissection (n = 2), and/or mitral valve (MV) regurgitation resulting from MV prolapse (n = 7). Of 59 initial operations, 21 (36%) involved AV-replacing root surgery, 38 (64%) AV-sparing root surgery, seven (12%) aortic arch or hemi-arch repair, and five (8%) simultaneous MV surgery. There were no early mortalities. The mean follow up was 6.8 ± 1.2 years, with five deaths (8%) and a relatively low reoperation rate (10 reoperations in nine patients; 14%). Seven reoperations involved AV or aortic root surgery (including four for AV regurgitation following failed AV-sparing surgery), two MV repair/replacements, and one coronary artery bypass graft. Eight patients (21%) with AV-sparing surgery had moderate/severe AV regurgitation at the last follow up before re-intervention. The mean five-year freedom from postoperative death was 91.2 ± 8.8%, from cardiac reoperation 86.3 ± 4.5%, and more-than-moderate AV regurgitation 90.3 ± 4.8%.
Prophylactic aortic surgery in MFS patients with AV-replacing root or AV-sparing root surgery carries a low risk of operative morbidity and death when performed at an experienced center. AV-sparing root surgery increases the risk of AV regurgitation and, possibly, of re-intervention. Regular clinical follow up is important after any aortic root surgery in MFS patients, with a delineation of risk factors for AV regurgitation after AV rootsparing surgery.
本研究旨在分析作者在马方综合征（MFS）患者中进行主动脉根部手术的经验，并扩充符合根特标准患者（马方综合征注册研究）的手术结果数据。
对从符合根特标准的MFS患者获取的数据进行分析，包括主动脉根部手术及Kaplan-Meier生存分析。
2004年4月至2012年2月期间，共有59例MFS患者（手术时平均年龄36±13岁）接受了67次手术，手术原因包括主动脉根部瘤（n = 52）、主动脉瓣（AV）反流（n = 15）、急性主动脉夹层（n = 2）和/或因二尖瓣（MV）脱垂导致的二尖瓣反流（n = 7）。在59例初次手术中，21例（36%）涉及置换主动脉瓣的根部手术，38例（64%）为保留主动脉瓣的根部手术，7例（12%）为主动脉弓或半弓修复，5例（8%）为同期二尖瓣手术。无早期死亡病例。平均随访时间为6.8±1.2年，5例死亡（8%），再手术率相对较低（9例患者10次再手术；14%）。7次再手术涉及主动脉瓣或主动脉根部手术（包括4例保留主动脉瓣手术失败后因主动脉瓣反流进行的手术），2例二尖瓣修复/置换，1例冠状动脉旁路移植术。8例（21%）接受保留主动脉瓣手术的患者在再次干预前的最后一次随访时出现中/重度主动脉瓣反流。术后5年无死亡的平均自由度为91.2±8.8%，无心脏再手术的平均自由度为86.3±4.5%，无中重度以上主动脉瓣反流的平均自由度为90.3±4.8%。
在经验丰富的中心，对MFS患者进行置换主动脉瓣或保留主动脉瓣的预防性主动脉手术，手术并发症和死亡风险较低。保留主动脉瓣的根部手术会增加主动脉瓣反流以及可能再次干预的风险。MFS患者进行任何主动脉根部手术后，定期临床随访很重要，同时要明确保留主动脉瓣手术后主动脉瓣反流的危险因素。
