一例43岁的女性马凡综合征患者，主诉进行性呼吸困难和胸痛2小时来诊，她于5年前接受了升主动脉夹层修复术。生命体征正常，无心血管、神经或肾功能异常。CT检查确诊为始于主动脉根部的非常复杂的主动脉夹层(A图)，下至两侧髂动脉(E图)，上至两侧颈动脉(F图)，一个小的闭塞真腔至肾动脉和髂动脉(图C和E)。经胸超声心动图检查显示，主动脉根部扩张，夹层组织下垂至升主动脉、正常主动脉瓣和几乎被扩张的降主动脉和巨大血栓闭塞的左心耳(图G和H)。

患者在专科诊疗中心接受了紧急外科手术，行主动脉复杂修复术和外周动脉的修复术，无任何神经或终末器官损害，恢复良好。

原文：

CT and TTE Imaging Reveal a Complex Aortic Dissection in a Patient With Marfan Syndrome | Clinical Images

Apr 21, 2015 | Kusum Lata, MD

A 43-year-old woman with Marfan syndrome who underwent ascending aortic dissection repair five years ago presents with progressive dyspnea and chest pain for two hours. Her vital signs were normal, and there was no cardiovascular, neurological, or renal dysfunction. Computed tomographic examination of the body confirmed a very complex aortic dissection starting from the aortic root (Figure A), below to bilateral iliac arteries (Figure E) and upwards to both carotid arteries (Figure F). A small obliterated true lumen feeding the renal and iliac arteries is displayed in Figure C and E. Transthoracic echocardiogram revealed enlarged aortic root with dissection flap in ascending aorta, normal aortic valve, and almost obliterated left atrium by dilated descending aorta and large thrombus burden (Figures G, H). The patient underwent urgent surgery at specialized center. Complex repair of the aorta and peripheral arteries was performed, and she is recuperating well without any neurological or end organ deficit.