Midterm outcomes of isolated thoracic aortic replacement in congenital versus degenerative aortopathy in a 15-year institutional cohort

Background: Open aortic replacement represents the only approved option to address thoracic aortopathy among patients with connective tissue disorders (CTD). The aim of our study was to investigate contemporary midterm outcomes of isolated thoracic aortic replacement in patients with CTD versus degenerative pathology in a large institutional cohort. Methods: All patients undergoing isolated open thoracic aortic replacement at a single academic center from 2005 to 2020 were included. Patients were classified as having CTD or not having CTD based on documented genetic mutations associated with congenital aortopathy. In-hospital and midterm outcomes, including mortality, paraplegia, development of new arterial pathology on surveillance imaging, and the need for future operations, were compared between groups using descriptive statistics and Kaplan-Meier survival analysis. Results: Overall, 62 patients were included with a median follow-up of 58 months (range, 19-81 months) (59 months for those with CTD vs 51.5 months for those without CTD). CTD was present in 18 patients (29%), with 16 having Marfan syndrome (77.8%). Patients with CTD were younger than patients without CTD (45.8 years vs 60.9 years) and had lower rates of smoking (5.6% vs 56.8%) and hypertension (97.7% vs 72.2%; all P < .01). Patients with CTD were more likely to have a dissection component at the time of repair compared with patients without CTD (100% vs 59.1%) and underwent repair at smaller aortic diameters (5.9 cm vs 6.6 cm; both P < .05). There were no differences in in-hospital outcomes between the two groups, including mortality (4.5% vs 5.6%) and paraplegia (2.3% vs 0%; both P > .05). At 5 years, patients with CTD were more likely to have developed aneurysmal changes distal to their thoracic repair (88.9% vs 47.7%) and extra-aortic arterial aneurysms (41.2% vs 2.3%; both P < .05). However, on survival analysis, there were no differences in freedom from additional vascular procedures (hazard ratio,1.76; P = .333) or, specifically, additional aortic procedures (hazard ratio, 1.81; P = .380) between the two groups. There was only one anastomotic complication identified on longitudinal follow-up, which occurred in a patient without CTD 8 years after the index operation. Conclusions: Although carrying significant operative risks and the potential for morbidity, open thoracic aortic replacement represents a well-tolerated, durable treatment option for patients with congenitally mediated thoracic aortic disease. Because both patients with and without CTD who required thoracic aortic replacement often need future aortic intervention, vigilant surveillance is warranted. Equivalent intervention rates between the two groups suggest remodeling of the CTD aorta is almost universally characterized by initial postrepair dilation, but the majority of these changes successfully stabilize and do not progress to higher rates of intervention.