Outcomes of Thoracic Endovascular Aneurysm Repair (TEVAR) in Patients With Connective Tissue Disorders

Objectives: Thoracic endovascular aortic repair (TEVAR) remains controversial in patients with connective tissue disorders given the concern for durability. We report on the largest series to date on outcomes of patients with thoracic aortic disease and connective tissue disorders treated with TEVAR. Methods: The Vascular Quality Initiative registry identified 12 207 patients treated with TEVAR from January 2010 to December 2018, including 102 with Marfans, Ehlers-Danlos, or Loey-Dietz syndrome. Outcomes were analyzed per the Society for Vascular Surgery reporting standards. Results: Median age was 50.6 years (interquartile range: 57.0-75.0), and 62 (60.7%) were male. Eighty-eight (86.3%) patients had Marfan, 9 (8.8%) had Ehlers-Danlos, and 5 (4.9%) had Loey-Dietz syndrome. Twenty-six (25.5%) patients were treated for degenerative aneurysmal disease and 76 (74.5%) patients for type B dissections (33 acute, 31 chronic). Most common indications for interventions in patients with type B dissection were pain (n = 41), aneurysmal degeneration (n = 16), and malperfusion (n = 8), with 3 patients who presented ruptured. There was no significant difference in perioperative complications between acute/chronic dissections and aneurysms (P = .14). Percutaneous access was utilized in 61.7% of patients, with a 2.9% rate of arterial injury requiring reintervention. Follow-up data were available for 75 (73.3%) patients at a mean follow-up of 15.6 months. Overall mortality was 5.3%. There were 30 patients with follow-up endoleak data, and 8 (26.7%) endoleaks were identified. All endoleaks were in patients treated for acute type B dissection, and all resolved after a mean of 2.1 reinterventions. Three patients treated for acute Type B Aortic Dissection (TBAD) had retrograde dissections requiring intervention. Discussion: Thoracic endovascular aortic repair for patients with connective tissue disorders can be performed with low perioperative mortality, spinal cord ischemia, or Cerebrovascular Accident (CVA). On follow-up, acute type B aortic dissections represent a higher risk subgroup with increased rates of endoleak and retrograde dissection. Closer follow-up for these patients and early reintervention may be beneficial.