Treatment of thoracoabdominal aortic disease in patients with connective tissue disorders

Objective: Aneurysmal degeneration of the entire aorta is common in patients with connective tissue disorders (CTDs). Potential treatment options of these aneurysms include open repair and endovascular repair. Our objective herein was to review available evidence for different approaches to treatment of thoracoabdominal aortic disease in patients with CTDs. Methods: We performed a systematic literature review using PubMed and referenced manuscripts on open and endovascular treatment of thoracoabdominal aortic aneurysms and dissections in patients with CTDs. Results: A total of 28 studies were identified for inclusion in this review, 8 reporting on outcomes after open thoracoabdominal aortic aneurysm repair in patients with CTD, 8 on open branched graft use, and 12 on endovascular aortic repair in this population of patients. Reported outcomes were characterized by low perioperative morbidity and mortality, good branch patency, and low rate of reintervention for open repair and significant rates of endograft-related complications and substantial need for secondary endovascular interventions and open conversions for endovascular repair. Conclusions: There is a lack of high-quality evidence to support any particular approach to aortic repair in patients with CTD and a dearth of comparative data between open repair and endovascular repair. There are distinct differences in the published lengths of follow-up between the two repair approaches as well as in the prevalence of their use in an acute vs elective setting. It is evident that endovascular interventions for aortic disease in patients with CTDs are associated with many device- and aorta-related complications both in the short term and in the long term. Despite the lack of level 1 evidence, open repair currently remains the standard approach to treatment of aortic disease due to CTDs. Open branched graft repair in particular is the preferred technique. Endovascular interventions may be cautiously used in patients with CTDs in selective circumstances.