Open surgery is the gold standard for connective tissue disorder (CTD) patients. Thoracic endovascular aortic repair (TEVAR) is utilized in emergencies and patient specific situations. Limited data on durability of TEVAR in CTD patients exists. The purpose of this study is to investigate durability, complications, and outcomes of TEVAR in CTD patients. This single center retrospective study included 40 CTD patients who underwent TEVAR for thoracoabdominal aortic aneurysm or aortic dissection from 2/2014 - 4/2021. CTDs included Marfan Syndrome (MFS), Loey-Dietz Syndrome (LDS), and non-specific CTD related diagnoses. Primary outcomes included aortic-related morbidities, time to and type of post-operative 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. Median age was 53yrs with 52.5% being female. MFS was diagnosed in 57.5%, LDS in 2.5%, and 40% had diagnosed non-specific or other CTD. Thirty-two (80%) had prior aortic interventions. Thoracic aneurysm existed in 52.5% and dissection in 82.5%. Average maximum thoracic aortic diameter was 55.2mm. There were 2 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 9.1mths, 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 at 67.2% at 1 year, and 59.7% at 2 and 3 years, and freedom of reintervention at 49.8%, 36%, and 30% at 1, 2, and 3 years, respectively. This study suggests that TEVAR for CTD patients 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 one year. TEVAR should remain limited in this population until more durable outcomes are possible.
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