Wells D G, Podolakin W
Can J Anaesth. 1987 May;34(3 ( Pt 1)):311-4. doi: 10.1007/BF03015173.
An 18-year-old male with Marfan's syndrome underwent surgery for repair of an ascending aortic dissection. The clinical features of this congenital syndrome and the acute management of its complications are described. As elective surgery is frequently required for ocular, musculo-skeletal or cardiovascular problems in people with Marfan's syndrome, patients must be carefully assessed preoperatively for specific complications. Significant pulmonary problems include restriction of lung function due to pectus excavatum or kyphoscoliosis and intrinsic pulmonary involvement with emphysema, bronchogenic cysts and "honeycomb lung." This leads to a significant incidence of spontaneous pneumothorax and the danger of tension pneumothorax with positive pressure ventilation. The commonest causes of sudden death are cardiovascular complications, in particular rupture and dissection of the ascending aorta. Preoperative assessment should include echocardiography to determine the size of the aortic root. The anaesthetic technique chosen should both decrease myocardial contractility and avoid sudden increases in contractility, in order to minimise the risk of aortic dissection or rupture.
一名患有马凡氏综合征的18岁男性接受了升主动脉夹层修复手术。本文描述了这种先天性综合征的临床特征及其并发症的急性处理方法。由于马凡氏综合征患者的眼部、肌肉骨骼或心血管问题经常需要进行择期手术，因此术前必须对患者进行仔细评估，以确定是否存在特定并发症。严重的肺部问题包括漏斗胸或脊柱侧凸导致的肺功能受限，以及肺气肿、支气管源性囊肿和“蜂窝肺”等肺部内在病变。这导致自发性气胸的发生率显著增加，以及正压通气时发生张力性气胸的危险。猝死的最常见原因是心血管并发症，尤其是升主动脉破裂和夹层。术前评估应包括超声心动图，以确定主动脉根部的大小。所选择的麻醉技术应既能降低心肌收缩力，又能避免收缩力突然增加，以尽量降低主动脉夹层或破裂的风险。
