Endovascular Repair in Patients with Marfan Syndrome: Concerns Amid Controversy

Abstract

Endovascular aortic repair is widely used to treat patients with degenerative aneurysms or aortic dissection within the distal aorta. Thoracic endovascular aortic repair (TEVAR) is generally associated with fewer short-term complications than open surgical repair, which is particularly important for older patients with significant comorbid conditions. However, for patients with Marfan syndrome, a heritable thoracic aortic disease associated with aortic dilatation, dissection, and rupture, the utility of endovascular aortic repair remains questionable. Marfan patients have friable aortic tissue and are typically treated at a relatively young age with few comorbidities; they therefore have less operative risk and need a durable solution. Furthermore, those who need distal aortic repair tend to have chronic aortic dissection. Although TEVAR is generally superior to open surgery with regard to short-term complications, it is less durable, and TEVAR reintervention rates are highest in patients with chronic aortic dissection. Thus, Marfan patients seeking definitive aortic repair are often better served by open repair. Nonetheless, TEVAR may be useful in patients with Marfan syndrome as a bridge to open repair or as treatment for late complications of previous open repair.