Marfan syndrome is associated with recurrent dissection of the dissected aorta

Abstract

Background: Recurrent dissection of a previously dissected aortic segment has been reported as a rare, late complication in single case reports. The infrequency of this event makes informed risk assessment in an individual patient challenging.

Methods: To investigate this issue we examined the database of the Massachusetts General Hospital Thoracic Aortic Center between January 1, 2003 and December 31, 2012. A retrospective review was performed to identify patients with both (1) an acute aortic dissection after a prior aortic dissection and (2) evidence of a new dissection within a previously dissected aortic segment creating a triple lumen aorta. Data were reviewed to identify factors predisposing to dissection of a previously dissected aortic segment.

Results: Over a 10-year period we identified 5 cases of aortic dissection within a previously dissected aortic segment presenting as a new acute aortic syndrome. On average, the recurrent dissection occurred 1 decade after the first aortic dissection (mean = 9.8 ± 1.9 years). Patients identified in this series were significantly younger at first dissection and more likely to carry the diagnosis of Marfan syndrome. Aortic aneurysm diameter was quantified before and after the new dissection event and demonstrated a marked increase in aneurysmal size (mean increase = 1.6 ± 0.3 cm).

Conclusions: We conclude that medial degeneration, as seen in the Marfan aorta, represents a predisposing factor for recurrent dissection of the dissected aorta. Our data indicate that double aortic dissections cause significant arterial destabilization and a low threshold for surgical intervention is appropriate.