Thoracic endovascular aortic repair in connective tissue disease patients is not a definitive option

Open surgery is the gold standard for patients a connective tissue disorder (CTD). Thoracic endovascular aortic repair (TEVAR) is used in emergencies and patient-specific situations. Limited data on durability of TEVAR in patients with CTD exist. The purpose of this study was to investigate the durability, complications, and outcomes of TEVAR in patients with CTD.

This single-center retrospective study included 40 patients with CTD who underwent TEVAR for thoracoabdominal aortic aneurysm or aortic dissection from February 2014 to April 2021. CTDs included Marfan syndrome, Loey-Dietz syndrome, and nonspecific CTD-related diagnoses. Primary outcomes included aortic-related morbidities, time to and type of postoperative reinterventions, and time to open/hybrid conversion. Time to conversion and reintervention was calculated using Kaplan-Meier estimation. Predictors of reintervention and open/hybrid conversion were evaluated using Cox proportional hazards models.

The median age was 53 years with 52.5% of the patients being female. Marfan syndrome was diagnosed in 57.5%, Loey-Dietz syndrome in 2.5%, and 40% had a diagnosed nonspecific or other CTD. Thirty-two (80%) had prior aortic interventions. Thoracic aneurysm existed in 52.5% and dissection in 82.5%. The average maximum thoracic aortic diameter was 55.2 mm. There were two mortalities within the first month. Of the remaining 38 patients, 71.1% had aneurysm-related morbidities, including 81.5% with aneurysmal degeneration and 33.3% with endoleak. Overall, 62.5% required reintervention. Of those, median time to reintervention was 9.1 months, including redo-TEVAR/extension in 32%, ascending/arch repair in 24%, open thoracoabdominal aortic repair in 56%, and false lumen embolization in 16%. Open conversions and reintervention were most likely to occur within the first year, with freedom of open conversion of 67.2% at 1 year, and 59.7% at 2 and 3 years, and freedom of reintervention of 49.8%, 36.0%, and 30.0% at 1, 2, and 3 years, respectively.

This study suggests that TEVAR for patients with CTD can be performed safely; however, patients are at high risk for aortic-related morbidities and reintervention. Reinterventions and open conversion are common and more likely to occur within 1 year. TEVAR should remain limited in this population until more durable outcomes are possible.