Current status of thoracoabdominal aortic aneurysm repair in Marfan syndrome

Patients with Marfan syndrome frequently develop aneurysms or dissections involving multiple segments of the aorta; graft replacement of multiple aortic segments prolongs survival. Indications for graft replacement of the thoracoabdominal aortic segment include impending rupture, ischemic complications from dissection, or an aortic diameter measuring 5.5 cm or more. We retrospectively reviewed our experience with 50 consecutive thoracoabdominal aortic aneurysm repairs in Marfan patients to evaluate the results of surgery using contemporary techniques.

From January 1986 through July 1996, 800 patients underwent graft replacement of thoracoabdominal aortic aneurysms. Fifty patients (6.3%) had Marfan syndrome. Aneurysms without dissection were present in nine (18%) of the Marfan patients. The remaining 41 patients (82%) had chronic dissection. Both chronic dissection and Crawford extent II aneurysms were more prevalent in patients with Marfan syndrome than in non-Marfan patients. Extensive thoracoabdominal aortic aneurysm repairs (extents I and II) were performed in 72% of Marfan patients; atriodistal bypass was used in 22 patients (44%).

The 30-day survival rate was 96%. Renal failure requiring hemodialysis occurred in three patients (6%). Two patients (4%) had postoperative lower extremity neurological deficits. No patient receiving atriodistal bypass developed paraplegia or paraparesis, versus two patients (7.1%) among the 28 who did not.

Patients with Marfan syndrome have a high prevalence of extent II aneurysms and associated dissection. Using contemporary surgical techniques, Marfan patients can undergo thoracoabdominal aortic aneurysm repair with low mortality and morbidity, despite the extensive nature of their disease.