Royal Brompton Hospital London SW3 6NP, UK.
J R Soc Med. 2010 Sep;103(9):370-5. doi: 10.1258/jrsm.2010.100070.
Fatal aortic dissection occurs at young age in Marfan syndrome. Prevention relies on elective replacement of the aortic root. The placement of an external aortic root support, tailored to the anatomy of the individual patient has been proposed as a feasible alternative. DESIGN, SETTING AND MAIN OUTCOME MEASURES: External aortic root support was offered to patients with Marfan syndrome with aortic root diameter of 40-55 mm and without aortic regurgitation. By computer-aided design, a model of the individual patient's aorta was created from cardiac magnetic resonance images and a bespoke external aortic support was manufactured. Comparative measurements were made of the ascending aorta at the level of closure of the aortic valve cusps from magnetic resonance imaging studies taken preoperatively, at first follow-up, and at most recent follow-up. For patients having aortic root surgery at the same institution, in the same time frame as the first 10 patients, clinical data were retrieved on Marfan and other patients having aortic root replacement to serve as a reference data.
Twenty patients were operated upon from May 2004 to October 2009, 13 men and 7 women, median age 33 years. All 20 patients are alive and well at the time of last follow-up. Preoperative aortic diameters were 40-54 mm. All postoperative images were satisfactory with an overall reduction in aortic root dimensions. The surgery took half the time of other aortic root surgery. Cardiopulmonary bypass was used only in the first patient, myocardial ischemia was not required in any patient, and no postoperative anticoagulation is mandated.
The primary objective of this surgery was fully achieved in 19 of the 20 patients, reinforcing the ascending aorta while leaving the native aortic valve intact and conserving the blood/endothelium interface.
马凡综合征患者的主动脉夹层在年轻时就会致命。预防依赖于主动脉根部的选择性置换。已经提出了一种可行的替代方法，即放置针对个体患者解剖结构定制的外部主动脉根部支撑物。
设计、设置和主要结果测量：为主动脉根部直径为 40-55 毫米且无主动脉瓣反流的马凡综合征患者提供外部主动脉根部支撑。通过计算机辅助设计，从心脏磁共振图像创建了个体患者主动脉的模型，并制造了定制的外部主动脉支撑物。对术前、第一次随访和最近随访时心脏磁共振成像研究中主动脉瓣瓣叶关闭水平的升主动脉进行了比较测量。对于在同一机构、同一时间框架内接受主动脉根部手术的患者，检索了马凡综合征和其他接受主动脉根部置换术的患者的临床数据，作为参考数据。
2004 年 5 月至 2009 年 10 月期间对 20 名患者进行了手术，其中男性 13 名，女性 7 名，中位年龄为 33 岁。所有 20 名患者在最后一次随访时均存活且健康。术前主动脉直径为 40-54 毫米。所有术后图像均令人满意，主动脉根部尺寸总体减小。手术时间为其他主动脉根部手术的一半。仅在第一例患者中使用体外循环，任何患者均无需心肌缺血，并且不需要术后抗凝。
该手术的主要目标在 20 名患者中的 19 名中得到了完全实现，在保留原生主动脉瓣完整的同时加强了升主动脉，并保留了血液/内皮界面。
