中文摘要：

目的探讨马凡综合征与先天二叶式主动脉瓣畸形发生急性StanfordA型主动脉夹层的临床特征和手术早期预后。方法回顾性分析2008年4月至2012年4月复旦大学附属中山医院心外科接受手术治疗的急性StanfordA型主动脉夹层患者，其中马凡综合征39例、先天二叶式主动脉瓣畸形28例，比较两组患者的临床表现、手术方式和术后30d预后。结果马凡综合征和先天二叶式主动脉瓣畸形发生急性夹层时均以胸背痛为主要症状（P〉0．05），马凡综合征发病更早[（35±8）岁比（47±13）岁，P〈0．01]，主动脉窦部较先天二叶式主动脉瓣畸形扩张更明显[（55．4±9．8）mm比（42．6±8．6）mm，P〈0．01]，且主动脉瓣病变以反流较多见（69．2％比32．1％，P=0．003），先天二叶式主动脉瓣畸形则有25．0％表现为主动脉瓣狭窄。两组患者手术方式相似（P〉0．05），但先天二叶式主动脉瓣畸形的术后30d病死率明显高于马凡综合征（25．0％比5．1％，P=0．020）。结论马凡综合征和先天二叶式主动脉瓣畸形发生急性StanfordA型主动脉夹层有各自独特的临床特征，先天二叶式主动脉瓣畸形患者手术早期预后较差。

英文摘要：

Objective To compare the clinical features of type A aortic dissection （AAD） in patients with Marfan syndrome （MFS） and bicuspid aortic valves （BAV）. Methods Data from patients undergoing surgery for acute AAD between April 2008 and April 2012 at our institute were retrospectively collected. Patients were categorized into MFS group （ n = 39 ） and BAV group （ n = 28 ） to investigate their clinical and prognostic features. Results Patients in MFS and BAV groups always experienced the sudden onset of chest pain. MFS patients tended to have younger age [ （ 35 ± 8 ） y vs （47 ± 13 ） y, P 〈 0. 001 ], wider aortic sinus [ （55.4 ± 9.8 ） mm vs （42. 6 ± 8.6） ram, P 〈 0. 01 ] and higher rate of moderate-to-severe aortic regurgitation （69. 2% vs 32. 1%, P =0. 003）. Patients in BAV group were featured with higher rate of moderate-to-severe aortic stenosis. Though the operation procedures were similar in both groups, the 30- day postoperative mortality was significantly higher among BAV patients （25.0% vs 5.1% , P = 0. 020）. Conclusions MFS and BAV represent unique subgroups of acute type A aortic dissection. BAV-associated dissection demonstrated strikingly higher postoperative mortality in our study population.