Abstract 11343: Unique Phenotypes of Three-Dimensional Aortic Growth in Genetic Aortopathy

Abstract

Introduction: Patients with genetically-mediated thoracic aortic aneurysm (TAA) are at high risk of progressive aortic growth, although growth is highly variable and often difficult to accurately define using conventional diameter measurements. An emerging image analysis technique, vascular deformation mapping (VDM), provides accurate, three-dimensional (3D) mapping of aortic growth. Hypothesis: 3D growth mapping of the thoracic aorta will reveal unique patterns/phenotypes of growth in patients with genetically-mediated TAA. Material and Methods: We included adult (>18) patients at our center with GM-TAA and at least 2 CT angiograms >1 year apart. 3D aortic growth was measured by VDM. The longest CT interval with was used for analysis. Results: 61 patients (average follow-up 5.4 ± 3.6 years, cumulative 331 years) were included, with etiologies: Marfan (n=41), Loeys-Dietz (n=2), vascular Ehlers-Danlos (n=5), and familial TAA (n=13). Average age was 54.8 ± 16.6 years and 42.6% were female (n=26). The majority (n=38, 62%) had a history of open root/ascending aortic repair. Among post-surgical patients, three patterns emerged from VDM growth maps: 1) “One-and-done” - prior repair of diseased segment with no further aortic growth (n=14); 2) Adjacent segment - growth limited to native aortic segment adjacent to a surgically repaired segment (n=9); 3) Diffuse growth - significant growth involving all native aortic segments (n=15). Among non-surgical patients (n=23, 38%) there were similarly three growth patterns by VDM: 1) Stable - no growth (n=9); 2) Heterogeneous growth - multi-focal growth with intervening regions without growth (n=7); 3) Diffuse growth - contiguous growth involving all segments (n=7). Conclusion: Growth in genetic aortopathy is highly variable in extent and degree both before and after aortic repair. Unique phenotypes of 3D growth identified by VDM may have important implications for risk stratification and surgical planning.