Endovascular treatment for type B dissection in Marfan syndrome : Is it worthwhile?

Marfan syndrome is the most frequently inherited disorder of connective tissue and is strongly associated with aortic dilatation, dissection, and rupture; in these patients, type B dissection occurs substantially. It is not known whether stent grafting, which is now frequently used in type B aortic dissection and descending thoracic aneurysms in non-Marfan patients, is a valuable option in Marfan patients, and reports from the literature are sparse and sporadic. We performed a systematic review of studies reporting the early and late results of endovascular stent grafting in Marfan patients with type B dissection in the attempt to quantify possible benefits or potential drawbacks of this approach in these usually very sick patients. Although associated with a low operative risk (1.9%), endovascular stent grafting in patients with Marfan syndrome carries a substantial risk of early and late complications, mainly endoleaks and surgical conversions, and of death at midterm follow-up. Because these complications are relatively more frequent in patients undergoing endovascular stent grafting for chronic dissections, these data suggest caution against the routine use of endovascular stent grafting in Marfan patients.