PC006. Fate of the Descending Thoracic Aorta After DeBakey Type I Aortic Dissection in Patients With Genetically Triggered Thoracic Aortic Disease

Abstract

Objectives: Dissection-related aneurysmal degeneration (DRAD) is a known long-term complication of the dissected descending thoracic aorta (DTA) that may or may not occur in a given patient. Post dissection surveillance recommendations are similar for all patients with dissections since there are no reliable predictors for DRAD. The aim of this study was to investigate the incidence and treatment of DRAD in the DTA after acute DeBakey type I aortic dissection in specific groups of patients from a cohort with genetically triggered aortic disease. Methods: The GenTAC Registry is an NIH-funded, multicenter database created in 2006 to improve the diagnosis and management of patients with known or suspected genetically induced thoracic aortic aneurysms and dissections. We analyzed data from survivors of DeBakey type I aortic dissection who were diagnosed with Marfan syndrome (MFS), familial thoracic aortic aneurysm and dissection (FTAAD), or early-onset dissection occurring at age ≤50 years (TAD ≤50). Results: A total of 139 cases (75.5% male) had DeBakey I dissection (MFS = 52, FTAAD = 34, and TAD≤50 = 53), with a median follow-up of 4.5 years (range, 0.1-25 years). The dissection occurred at a younger median age in MFS (35 years; range, 14-55) compared to FTAAD (41 years; range, 23-72) and TAD≤50 (43 years; range, 17-54; P =.002). Subsequent DTA repair was performed in 59 cases (42.4%), with five during the initial hospitalization. MFS had the highest rates of subsequent DTA repair, at 61.5% compared to 38.2% in the FTAAD and 20.9% in TAD≤50 groups (P