Contemporary Outcomes of Thoracic Endovascular Aortic Repair in Patients with Syndromic Genetic Aortopathy: A Multi-Centre National Study.

BACKGROUND: Thoracic aortic aneurysms and dissection in individuals with syndromic genetic aortopathy (SGA) due to Marfan syndrome, Loeys-Dietz syndrome, and vascular Ehlers-Danlos syndrome are especially challenging. This is due to the impaired aortic architecture secondary to the genetic variants. Previous studies have analyzed the outcomes after open surgery in this population. However, there is little information on their outcomes after thoracic endovascular aortic repair (TEVAR). Recent studies have demonstrated high rates of subsequent secondary interventions in these patients treated with TEVAR due to retrograde type A aortic dissection and endoleaks. These diseases are not limited to the descending thoracic aorta (DTA) treated with TEVAR. Aortic arch and thoracoabdominal aortic aneurysm repairs are frequently required in this population. This study aims to analyze the short and mid-term outcomes after TEVAR in patients with SGA compared to those without. This will help to develop specific guidelines for the proper selection and treatment of patients with SGA and thoracic aortic pathologies.

METHODS: The Society for Vascular Surgery Vascular Quality Initiative-Medicare-linked data was queried for patients who underwent TEVAR for DTA aneurysms and dissections with and without a history of SGA. Multivariable logistic regression was used to determine early post-operative outcomes. Kaplan-Meier curves were plotted to analyze mid-term survival trends, with the log-rank test used to compare outcomes between the groups within the specified time periods. Cox proportional hazards models were used to determine adjusted mid-term outcomes. Data was analyzed using Stata MP version 17.0.

RESULTS: Out of 7,876 patients involved in the final analysis, 188 patients had a history of SGA, whereas 7,688 patients did not. Patients with SGA were overall younger (70.7 vs. 74.2 years, P < 0.01), but the groups had similar distributions of other baseline demographic characteristics. After adjusting for potential confounders, patients with SGA compared with those without had similar odds of 30-day mortality, postoperative stroke, spinal cord ischemia, myocardial infarction, congestive heart failure, and respiratory complications. There were no significant differences between the groups in overall mortality and reinterventions within the first year of follow-up. Three-year survival was not significantly different between the groups (73.7% vs. 63.1%, log-rank P = 0.11). After adjustment of confounders, Cox models demonstrated no significant differences between the groups in 3-year rates of overall mortality (aHR: 0.76, 95% CI: 0.51-1.12, P = 0.16); reinterventions (aHR: 1.09, 95% CI: 0.78-1.53, P = 0.61), and aortic rupture (aHR: 1.09, 95%CI: 0.45-2.65, P = 0.85).

CONCLUSION: Despite their complex anatomical challenges, patients with SGA undergoing TEVAR have similar postoperative and mid-term outcomes compared to patients without SGA. Further studies are required to confirm these findings to support the use of TEVAR in patients with SGA.