Coselli Joseph S, Green Susan Y, Price Matt D, Hash Jonathan A, Ouyang Yafei, Volguina Irina V, Preventza Ourania, de la Cruz Kim I, LeMaire Scott A
Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Cardiovascular Research Institute, Baylor College of Medicine, Houston, Texas; Department of Cardiovascular Surgery, Texas Heart Institute, Houston, Texas.
Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Department of Cardiovascular Surgery, Texas Heart Institute, Houston, Texas.
Ann Thorac Surg. 2016 Jun;101(6):2193-201. doi: 10.1016/j.athoracsur.2015.11.008. Epub 2016 Feb 12.
In patients with Marfan syndrome (MFS), distal aortic dissection can necessitate thoracoabdominal aortic aneurysm (TAAA) repair in survivors of acute DeBakey type I dissection and those with DeBakey type III dissection. We examined outcomes of surgical repair of TAAA in patients with MFS with distal aortic dissection.
Data were analyzed for 127 consecutive TAAA repairs performed between January 2004 and June 2014 in patients with MFS and distal aortic dissection-DeBakey types I (n = 73) and III (n = 54). The median time from dissection onset to TAAA repair was 5.2 years (interquartile range [IQR]: 2.1 to 9.8 years) for the overall group and was longer in patients with DeBakey I (6.5 years, IQR: 3.5 to 13.9 years) than patients with DeBakey III (2.9 years, IQR: 0.6 to 6.0 years, p < 0.001). Eleven patients (9%) had acute or subacute dissection at the time of repair. Sixty-six patients (52%) underwent Crawford extent II TAAA repair. A composite end point, adverse event, was defined as operative death or permanent stroke, renal failure, paraplegia, or paraparesis.
Eight patients had adverse events (6%), including 5 operative deaths (4%). There was no permanent stroke and 1 case each of permanent paraplegia and paraparesis. At discharge, 2 early survivors (2%) had renal failure. Extent II repairs did not have substantially different outcomes from other repairs.
In these patients with MFS with aortic dissection, open TAAA repair incurred reasonable operative risk, but improvements are needed to reduce rates of renal failure. Extent II TAAA repair does not appear to increase operative risk in patients with MFS.
在马凡综合征（MFS）患者中，对于急性Ⅰ型德巴基（DeBakey）夹层分离的幸存者以及Ⅲ型德巴基夹层分离患者，远端主动脉夹层可能需要进行胸腹主动脉瘤（TAAA）修复。我们研究了MFS合并远端主动脉夹层患者TAAA手术修复的结果。
分析了2004年1月至2014年6月期间连续127例MFS合并远端主动脉夹层（Ⅰ型德巴基夹层分离73例，Ⅲ型德巴基夹层分离54例）患者的TAAA修复数据。整个队列从夹层分离开始至TAAA修复的中位时间为5.2年（四分位间距[IQR]：2.1至9.8年），Ⅰ型德巴基夹层分离患者（6.5年，IQR：3.5至13.9年）的时间长于Ⅲ型德巴基夹层分离患者（2.9年，IQR：0.6至6.0年，p<0.001）。11例患者（9%）在修复时存在急性或亚急性夹层分离。66例患者（52%）接受了克劳福德（Crawford）Ⅱ型TAAA修复。复合终点不良事件定义为手术死亡或永久性卒中、肾衰竭、截瘫或轻截瘫。
8例患者发生不良事件（6%），包括5例手术死亡（4%）。无永久性卒中，永久性截瘫和轻截瘫各1例。出院时，2例早期幸存者（2%）出现肾衰竭。Ⅱ型修复与其他修复的结果无显著差异。
在这些MFS合并主动脉夹层的患者中，开放性TAAA修复的手术风险合理，但需要改进以降低肾衰竭发生率。Ⅱ型TAAA修复似乎不会增加MFS患者的手术风险。
