IPC03. Outcomes of Thoracic Endovascular Aneurysm Repair in Patients With Connective Tissue Disorders

Abstract

Management of thoracic aortic disease in patients with connective tissue disorders is traditionally performed with open surgery. Thoracic endovascular aneurysm repair (TEVAR) remains controversial given the underlying connective tissue disorder, and 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. The Vascular Quality Initiative registry identified 12,207 patients treated with TEVAR from January 2010 to December 2018, including 102 with either Marfan, Ehlers-Danlos, or Loey-Dietz syndrome. Outcomes were analyzed per the Society for Vascular Surgery reporting standards. The median age was 50.6 years (interquartile range, 57.0-75.0 years), and 62 patients (60.7%) were male. Eighty-eight patients (86.3%) had Marfan, 9 (8.8%) had Ehlers-Danlos, and 5 (4.9%) had Loey-Dietz syndrome. Twenty-six patients (25.5%) were treated for degenerative aneurysmal disease and 76 patients (74.5%) were treated for type B dissections. Of the type B dissections, 33 were acute and 31 were 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 three patients who presented ruptured. There was no significant difference in terms of rates of overall perioperative complications between the subgroups of acute/chronic dissections and aneurysms (Table; P = .14). Percutaneous access was used in 61.7% of patients, with a 2.9% rate of arterial injury requiring reintervention. Follow-up data were available for 75 patients (73.3%) 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 TBAD had retrograde dissections requiring intervention. TEVAR for patients with connective tissue disorders and thoracic aortic pathology can be performed safely with low perioperative mortality, spinal cord ischemia or cerebrovascular accident. 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.TablePerioperative complicationsOverall (n = 102)DissectionAneurysm (n = 26)Acute (n = 33)Chronic (n = 31)Mortality3 (2.9%)1 (3.0%)1 (3.2%)0 (0%)Endoleak13 (12.7%)0 (0%)4 (12.9%)7 (26.9%) Type I4022 Type II4013 Type III1001 Type IV1001 Indeterminate3011SCI2 (2.0%)2 (6.1%)0 (0%)0 (0%)Limb ischemia4 (3.9%)3 (9.1%)0 (0%)0 (0%)RTOR for aortic intervention3 (2.9%)2 (6.1%)1 (3.2%)0 (0%)Arterial injury3 (2.9%)1 (3.0%)1 (3.2%)0 (0%)RTOR, Return to operating room; SCI, spinal cord ischemia. Open table in a new tab