Endovascular aortic repair in patients with marfan and Loeys-Dietz syndrome is safe and durable when employed by a multi-disciplinary aortic team

OBJECTIVES : The aim of this study was to report on mid-term outcomes after endovascular aortic repair in patients with Marfan (MFS) or Loeys-Dietz (LDS) syndrome. METHODS: We analyzed data from two European centers of patients with MFS and LDS undergoing endovascular aortic repair. Patients were analyzed based on (1) timing of the procedure (planned vs emergency procedure) and (2) the nature of the landing zone (safe vs non-safe). The primary end-point was freedom from reintervention. Secondary end-points were freedom from stroke, bleeding and death. RESULTS: A population of 419 patients with MFS (n = 352) or LDS (n = 67) was analyzed for the purpose of this study. Thirty-nine patients (9%) underwent endovascular aortic repair. Indications for TEVAR or EVAR were aortic dissection in 13 (33%) patients, aortic aneurysm in 22 (57%) patients and others (intercostal patch aneurysm, penetrating atherosclerotic ulcer, pseudoaneurysm, kinking of FET) in 4 (10%) patients. Thoracic endovascular repair was performed in 34 patients, abdominal endovascular aortic repair was performed in 5 patients. Mean age at 1st TEVAR/EVAR was 48.5 +/- 15.4y. Mean follow-up after 1st TEVAR/EVAR was 5.9 +/- 4.4 y. There was no statistically significant difference in the rate of re-interventions between patients with non-safe landing zone and the patients with safe proximal landing zone (P = 0.609). Furthermore, there was no increased probability for reintervention after planned endovascular intervention compared to emergency procedures (P = 0.916). Mean-time to reintervention, either open surgical or endovascular, after planned endovascular intervention was in median 3.9 y [95% CI 2.0-5.9y] and 2.0 y [95% CI -1.1-5.1y] (P = 0.23) after emergency procedures. CONCLUSIONS: Endovascular aortic repair in patients with MFS and LDS and a safe landing zone is feasible and safe. Endovascular treatment is a viable option when employed by a multi-disciplinary aortic team even if the landing zone is in native tissue. Graphical Abstract