Total aortic arch replacement with frozen elephant trunk in patients with Marfan syndrome.

Background: 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.

Methods: 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.

Results: 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.

Conclusions: 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.