Beckmann Erik, Martens Andreas, Krueger Heike, Korte Wilhelm, Kaufeld Tim, Arar Morsi, Shrestha Malakh
Minneapolis Heart Institute, Minneapolis, MN, USA.
Oldenburg University Hospital, Oldenburg, Germany.
Ann Cardiothorac Surg. 2025 Jul 31;14(4):303-308. doi: 10.21037/acs-2025-evet-0091. Epub 2025 Jul 29.
Marfan syndrome (MFS) is a connective tissue disease which can lead to aortic aneurysm and dissection. The performance outcomes of total aortic arch replacement with frozen elephant trunk (FET) are not well known in these patients. This study summarizes our experience with FET in MFS.
Between August 2001 and December 2021, 435 patients underwent FET at Hannover Medical School. Of these, 34 patients had MFS. The mean age was 43.3±11.9 years and 27 (79%) were male. The indication for surgery was aortic aneurysm in 1 (3%), acute aortic dissection in 12 (35%), and chronic aortic dissection in 21 (62%) patients.
All patients underwent total aortic arch replacement with FET. In addition, the following procedures were performed: conventional aortic root replacement (Bentall operation, n=8), valve-sparing aortic root replacement (David procedure, n=8), coronary artery bypass grafting (n=3), mitral valve surgery (n=2), and tricuspid valve surgery (n=1). Cardiopulmonary bypass (CPB) and aortic cross clamp times were 270±87 and 139±69 minutes, respectively. Postoperatively, there were 2 (6%) disabling strokes, and 0 (0%) patients required permanent dialysis or suffered from permanent paraplegia, respectively. In-hospital mortality was 12% (n=4). The mean follow-up time was 8.4±5.9 years. The 1-, 5-, 10, and 15-year survival rates were 82%, 70%, 70% and 65, respectively. There were 18 (53%) re-interventions on the distal aorta. Mean time to re-intervention was 2.7±3.1 years. The majority of patients underwent open surgical repair (n=14, 77%), while only 4 (22%) had endovascular therapy. The freedom from distal aortic re-intervention at 1-, 5-, 10- and 15 years was 86%, 61%, 55% and 44%, respectively.
The main indication for FET surgery in MFS is acute or chronic aortic dissection. Despite multiple concomitant procedures, early mortality was relatively low, suggesting that FET is feasible and effective to treat complex aortic pathology in MFS. However, our study showed a high incidence of distal aortic re-interventions, underscoring the progressive nature of the disease and the need for tailored long-term management strategies.
马方综合征（MFS）是一种可导致主动脉瘤和主动脉夹层的结缔组织疾病。在这些患者中，采用带冷冻象鼻支架（FET）的全主动脉弓置换术的手术效果尚不清楚。本研究总结了我们在MFS患者中应用FET的经验。
2001年8月至2021年12月期间，435例患者在汉诺威医学院接受了FET手术。其中，34例患有MFS。平均年龄为43.3±11.9岁，男性27例（79%）。手术适应证为1例（3%）主动脉瘤、12例（35%）急性主动脉夹层和21例（62%）慢性主动脉夹层。
所有患者均接受了FET全主动脉弓置换术。此外，还进行了以下手术：传统主动脉根部置换术（Bentall手术，n = 8）、保留瓣膜主动脉根部置换术（David手术，n = 8）、冠状动脉搭桥术（n = 3）、二尖瓣手术（n = 2）和三尖瓣手术（n = 1）。体外循环（CPB）和主动脉阻断时间分别为270±87分钟和139±69分钟。术后，有2例（6%）发生致残性卒中，0例（0%）患者需要永久性透析或发生永久性截瘫。住院死亡率为12%（n = 4）。平均随访时间为8.4±5.9年。1年、5年、10年和15年生存率分别为82%、70%、70%和65%。远端主动脉有18例（53%）再次干预。再次干预的平均时间为2.7±3.1年。大多数患者接受了开放手术修复（n = 14，77%），而只有4例（22%）接受了血管内治疗。1年、5年、10年和15年免于远端主动脉再次干预的比例分别为86%、61%、55%和44%。
MFS患者FET手术的主要适应证是急性或慢性主动脉夹层。尽管同时进行了多项手术，但早期死亡率相对较低，这表明FET治疗MFS复杂主动脉病变是可行且有效的。然而，我们的研究显示远端主动脉再次干预的发生率较高，突出了该疾病的进展性以及制定个性化长期管理策略的必要性。
