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目的 总结重症马方综合征患者的外科治疗经验及策略,评价其治疗效果和预后.方法 回顾性分析 2012年 1 月至 2016年 10 月南方医科大学珠江医院及广东省人民医院入院收治的 15 例重症马方综合征患者 [左心室射血分数(LVEF)≤40％ 或左心室舒张末期内径(LVEDD)≥75 mm]的临床资料,其中男 11 例、女 4 例,年龄 19～55(32.9±8.7)岁.5 例马方综合征患者合并主动脉夹层均行 Bentall+主动脉弓置换+支架象鼻术.10 例非夹层患者主动脉根部处理采用 Bentall 术 7 例,Cabrol 术 1 例,Bentall 术+右半弓置换 2 例,所有患者同期均予二尖瓣修复或置换处理,其中二尖瓣成形术 12 例,二尖瓣置换术 3 例.另同期行三尖瓣成形术 12 例.结果 该组患者中因心律失常出现心搏骤停死亡 1 例.主动脉内球囊反搏(IABP)辅助治疗 11 例,占 73.3％.术后 3 个月 LVEDD 较术前明显缩短[(80.5±7.4)mm vs.(58.3±6.0)mm,P<0.05];LVEF 较术前增加(37.3％±5.2％ vs. 46.3％±4.4％,P<0.05).术后 1年 LVEDD 较术前明显缩短[(80.5±7.4)mm vs.(53.7±3.6)mm,P<0.05];LVEF 较术前明显增加(37.3％±5.2％ vs. 57.7％±4.2％,P<0.05).随访期间无死亡及再手术病例.结论 必要的术前治疗、术中同期彻底矫治所有瓣膜及血管病变问题以及术后较长时间的 ICU 监护治疗是成功治疗重症马方综合征患者的关键.“,”Objective To retrospectively reviewed our experience of the surgical and perioperative treatment of patients suffering from critical Marfan syndrome with severe left ventricular dysfunction and to evaluate its therapeutic effect and prognosis. Methods Between January 2012 and October 2016, 15 patients diagnosed with Marfan syndrome combined with severe left ventricular dysfunction (left ventricular ejection fraction≤40％ or left ventricular end diastolic diameter≥75 mm) underwent operations for aortic root aneurysm in Zhujiang Hospital and Guangdong General Hospital. Among them, 11 were males and 4 were females with a mean age of 32.9±8.7 years ranging from 19 to 55 years. Five patients with aortic dissection underwent Bentall procedure and total arch reconstruction with stent graft implantation. Two patients underwent Bentall procedure and hemi-arch replacement, seven patients underwent Bentall procedure and one patient underwent Cabrol procedure. Concomitant procedures included mitral valve repair in 12 patients, mitral valve replacement in 3 patients and tricuspid valve repair in 12 patients. Results There were 11patients (73.3％) receiving intra-aortic balloon pumping implantation. One (6.7％) in-hospital death occurred. The left ventricular end diastolic diameter decreased from 80.5±7.4 mm to 58.3±6.0 mm (P＜0.05) and the left ventricular ejection fraction improved from 37.3％±5.2％ to 46.3％±4.4％ 3 months postoperatively (P＜0.05). The left ventricular end diastolic diameter decreased from 80.5±7.4 mm to 53.7±3.6 mm (P＜0.05) and the left ventricular ejection fraction improved from 37.3％±5.2％ to 57.7％±4.2％ after one year (P＜0.05). No death and reoperation occurred in the follow- up. Conclusion Although the patients with Marfan syndrome and severe left ventricular dysfunction usually have a high surgical mortality, the key to satisfactory outcomes of severe Marfan syndrome is adequate preoperative preparation, complete correction of all vascular lesions during the operation, application of circulatory auxiliary device and perioperative strict and long-term ICU monitoring.