Endovascular and Hybrid Repair in Patients with Heritable Thoracic Aortic Disease

Background: In individuals with heritable thoracic aortic disease (HTAD), endovascular repair for treatment of aortic aneurysm and dissection may be lifesaving, but is associated with increased risk of failure of endovascular repair and adverse outcomes. This study reports our experience with early and late outcomes of endovascular aortic and branch vessel repair in pa-tients with HTAD.Methods: A retrospective case series was performed by chart review of individuals with HTAD followed at Washington University School of Medicine/Barnes-Jewish Hospital who underwent endovascular aortic and/or branch vessel repair. Clinical features, imaging characteristics, and short-and long-term outcomes were collected.Results: Twenty-nine patients with HTAD (20 male; mean age 45 +/- 13 years) underwent 37 endovascular procedures between 2006 and 2020 with mean follow up of 54 +/- 41 months. Seven patients underwent two or more separate endovascular procedures. Each procedure was considered separate for data collection and analysis. Underlying conditions included Marfan syndrome (n = 16 procedures), Loeys-Dietz syndrome (n = 14 procedures), vascular Ehlers-Danlos syndrome (n = 3 procedures), and nonsyndromic HTAD (n = 4 procedures). Twenty pa-tients (69%) had prior open surgical aortic repair. Indications for endovascular aortic repair (n = 31) included urgent repairs of acute complications of aortic dissection (n = 10) or aneurysm rupture (n = 3), and elective aortic repair (n = 18; 10 chronic dissections and eight chronic an-eurysms). Six patients underwent elective endovascular repair of six branch vessel aneurysms or dissections. Six patients underwent hybrid open surgical and endovascular repair. Of the 37 procedures, 25 (68%) proximal landing zones were in the native aorta or branch vessel, 11 (30%) were in a surgical graft or elephant trunk and one was in a previously placed endograft. Thirty-six (97%) procedures were technically successful, and none required emergency surgical conversion. Two patients died: one from sepsis and one from presumed late pseudoaneurysm rupture, for a 5% per-procedure mortality rate. Two procedures were complicated by stroke and one patient developed paraparesis. Of the 31 aortic procedures, seven aortic endografts (23%) developed a stent-induced new entry (SINE) discovered with imaging at 20 +/- 15 days post -procedure. Seven endografts (23%) developed a Type I endoleak and eight (26%) developed a Type II endoleak. No Type III endoleaks were seen. Within 30 days, two endografts (of 37, 5%) required reintervention. After 30 days, fifteen additional endografts (of 37, 41%) required reintervention. Two patients (of 6, 33%) who underwent hybrid repair required reintervention.Conclusions: This study is the largest single-center case series examining outcomes of HTAD patients following endovascular repair. Urgent and elective endovascular repairs in patients with HTAD can manage acute and chronic complications of aortic aneurysm and dissection with rela-tively low risk. However, risk of early and late endoleaks and SINE is high. Close post-procedural surveillance is required, and many individuals will require additional interventions. Hybrid repair shows promise and requires further investigation.