The fate of nonaortic arterial segments in Marfan patients

Abstract

Objectives: The aim of this study was to investigate the fate of nonaortic arterial segments in patients with Marfan syndrome (MFS).

Methods: This was a retrospective analysis of 100 consecutive patients with MFS fulfilling Ghent criteria who underwent 192 interventions on any segment of the arterial tree and were followed over the past 20 years. A review of the available imaging regarding 9 defined regions of interest of the carotid, innominate, subclavian, iliac, and femoral arteries was performed.

Results: Mean follow-up interval was 11.6 ± 7.7 years. Of 600 measurements that were performed, 414 (69%) arterial segments showed dilatation above the upper range of normal. There were no significant sex differences. In 100 patients, 66 dissections in nonaortic arterial segments in 33 patients were identified. Nineteen patients with or without previous dissection underwent 34 interventions. Most interventions were performed on the iliac arteries (56%), followed by the subclavian arteries (21%), the intercostal arteries (9%), the carotid arteries (6%), the visceral arteries (6%), and the innominate artery (3%). Most iliac artery interventions (88%) were caused by dilatations due to previous dissections, whereas this was only the case in 17% of interventions on the subclavian arteries.

Conclusions: Most patients with MFS presented with at least 2 dilated nonaortic arterial segments. The current data suggest that 20% of MFS patients will need some form of intervention on nonaortic arterial segments 5 to 6 years after their first aortic intervention, referring to the first aortic dissection of the patient if the patient had a history of dissection. Routine long-term follow-up imaging should include the iliac arteries as well as the supra-aortic branches.

Keywords: Marfan syndrome; aortic surgery; connective tissue disease.