Endovascular strategies for the management of aortic connective tissue disorders

During the last several decades, the endovascular management of patients with aortic connective tissue disorders (CTD) has greatly evolved. While open surgery remains the gold standard, endovascular strategies can play an important role in the overall management of arterial lesions. CTD result from mutations in specific genes responsible for maintaining and regulating tissue integrity. While each CTD has a unique phenotype, all patients are at an increased risk for the early onset of aortic aneurysms and dissections, leading to premature death if left untreated. In such patients, the interface between the device and arterial wall remains the primary concern, guiding the selection of appropriate landing zones and the means to ensure a circumferential seal excluding the aneurysm. In patients with aortic dissections, the goal of the endovascular treatment should achieve true lumen perfusion, favorable remodeling of the false lumen, and prevent aortic growth and rupture. While patients with proximal dissections should be treated with open surgery, distal dissections can be managed with a pure endovascular approach by placing the stent-graft proximal and distal to the entry sites of false lumen. Albeit less common than aortic lesions, aneurysms of the subclavian, vertebral, visceral, iliac, and internal iliac arteries may be present, underscoring the need to screen the entire vascular bed. Endovascular strategies may be implemented in each of these anatomical locations but may require a hybrid approach, involving both open and surgical techniques. Last, it must be stressed that both endovascular and open surgical repair represent treatments, not cures for these diseases. Therefore, every intervention must be strategized with the need for future reoperations.