Stent graft repair of descending aortic dissection in patients with Marfan syndrome: an effective alternative to open reoperation?

Objective: Aneurysms or dissections can involve multiple aortic segments in patients with Marfan syndrome, requiring staged replacement of the entire aorta. The optimal treatment of descending aortic dissection in these patients is a major challenge. We investigated the feasibility and outcomes of endovascular repair of the descending aorta in patients already submitted to open aortic root/arch surgery.

Methods: From March 1998 to July 2008, 12 patients (10 male and 2 female patients; mean age, 37.8 +/- 11.6 years) affected by Marfan syndrome underwent endovascular treatment for dissection of the descending aorta after previous open aortic root/arch surgery. Stent graft procedures were performed urgently in 5 patients and electively in 7 patients.

Results: Neither in-hospital deaths nor perioperative paraplegia or stroke occurred. Follow-up (median, 31 months; range, 3-57 months) was 100% complete. One patient needed surgical conversion for persistent type I endoleak, leading to false lumen expansion 3 months after endovascular repair. Extension of the dissection occurred in 2 patients 1 month and 2 years after the procedure, respectively. No late death or aortic rupture was observed.

Conclusions: Endovascular repair of the dissected descending thoracic aorta can be performed in patients with Marfan syndrome with a low risk of death or major complications. In case of staged procedures, stent graft treatment can be considered a possible alternative to open reoperation. Long-term durability remains to be determined.