Abstract
Endovascular therapy for the management of aortic pathology in patients with degenerative connective tissue disorder (DCTD) is controversial. Current guidelines are based on a paucity of literature and registry data are lacking. This study reports on medium term outcomes of patients with diagnosed DCTD compared to those without DCTD who were included in the W.L. Gore Global Registry for Endovascular Aortic Treatment (GREAT). Patients included in the GREAT registry who underwent treatment for any thoracic or abdominal aortic pathology were included and grouped according to the presence or absence of a DCTD. Baseline demographic and procedural data were collected as well as data relating to key outcomes within 5 years follow-up, including all-cause mortality, aortic-related mortality, reinterventions and serious adverse events (SAE). Multivariable Cox proportional hazards models were built to determine if any association existed between the presence of DCTD and any key outcomes. The analysis included 92 (1.9%) with DCTD and 4741 (98.1%) without DCTD. Patients with DCTD were more likely to be female (34.8% vs. 18.5%, P < .0001) and younger (66.8 [15.1] vs. 71.7 [10.3] years, P=.013) than those without DCTD. They were also more likely to have had prior aortic intervention (22.8% vs. 13.9%, P=.015) and an associated branch vessel procedure with the index operation (30.3% vs. 18.6%, P=.005). The majority of reinterventions in both groups occurred within the first 2 years and multivariable models demonstrated that the presence of DCTD was not predictive of all-cause mortality, aortic-related mortality, reinterventions or SAE within 5 years. Within the limitations of registry data, this work demonstrates the medium term safety and durability of endovascular stent-grafts across a spectrum of aortic pathology in some patients with DCTD. More work is required to determine the applicability of these findings to specific sub-types of DCTD and aortic pathology.
Highlights
The management of aortic pathology has evolved greatly over recent years, owing largely to the ongoing advances in endovascular technology
The majority of reinterventions in both groups occurred within the first 2 years and multivariable models demonstrated that the presence of degenerative connective tissue disorder (DCTD) was not predictive of all-cause mortality, aorticrelated mortality, reinterventions or serious adverse events (SAE) within 5 years
More work is required to determine the applicability of these findings to specific sub-types of DCTD and aortic pathology
Summary
The management of aortic pathology has evolved greatly over recent years, owing largely to the ongoing advances in endovascular technology. There are still areas of controversy, for which consensus has not been reached to guide the most effective management strategies. Patients with degenerative connective tissue disorder (DCTD) and associated aortic involvement are an important example. The DCTD’s commonly associated with aortic pathology are Marfan Syndrome (MFS), Loeys-Dietz Syndrome (LDS), Ehlers-Danlos Syndrome (EDS) and Familial Thoracic Aortic Aneurysm and Dissection (FTAAD). The fragile nature of aortic tissue in these patients combined with the outward radial force and fixation requirements of aortic stent-grafts have led many to believe that endovascular intervention for these patients is likely to be associated with a significant risk of early to mid-term complications[1,2] and is not deemed to be a durable long-term option. Based on a relative paucity of data, comprising mainly of small series with limited follow-up, current European guidelines suggest that endovascular repair should be considered only for complex redo surgery or as bridging procedures in the event of emergency[3]
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