Abstract 16360: Clinical Progression of Aortic Disease Among Adults Enrolled in the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions Database

Abstract

Objective: Evaluating progression of distal aortopathy among patients with GT-TAA. Methods: 1,160 patients (2007-2014) including 226 patients with &gt=2 CT/MRI scans; mean surveillance=1.5±1.4 years. Rapid progression (RP) was within the fourth quartile growth rate. Multivariable logistic regressions analyzed RP, proximal surgery and downstream growth. Kaplan Meier analysis described the event-free survival. Results: Patients were white (85%), male (58%), with Marfan syndrome (35%) and median age 44.5 years; 65% underwent surgical intervention. 3 lost to follow-up and 12 died.142 patients were RP. Median inter-scan duration 24 months. RP was 2.5-times higher with initial distal diameters &gt=3cm (p=0.032). Distal repairs more common with RP (41% vs. 20%, p=0.016); proximal interventions 55% less likely (p=0.009). Proximal interventions did not influence distal ROG but increased likelihood of distal intervention 5-fold (p=0.001). Calcium channel blockers were associated with 10.7-fold risk for RP (p=0.032). Overall, 5-year event-free survival was 37% with RP versus 34% for counterparts (p=0.706). Logistic regression found 22-fold higher likelihood of death in RP (p=0.055). Conclusions: Inter-scan intervals &lt24 months is important due to vascular-related complications. Calcium channel blockers were associated with greater ROG. Despite the need for more distal interventions, RP median event-free survival similar to counterparts. Keywords: aorta, aneurysm, genetics, surveillance