Growth Rate Assessed by Vascular Deformation Mapping predicts Type B Aortic Dissection in Marfan Syndrome

Abstract

Background: Patients with Marfan syndrome (MFS) are at a high risk of type B dissection (TBAD). Aortic growth and elongation have been suggested as risk factors for TBAD. Vascular deformation mapping (VDM) is an image analysis technique for mapping 3D aortic growth on rouine computed tomography angiography (CTA) scans. We aimed to use VDM to examine the value of aortic growth rate in the descending thoracic aorta (DescAo), among other imaging biomarkers, to identify the factors associated with risk of TBAD in MFS. Methods and Results: CTA scans spanning 2004-2023 from adult MFS patients with native DescAo were analyzed by VDM. Other measurements included multi-level thoracoabdominal aortic diameters and the length of the DescAo by centerline analysis. Among the 105 MFS patients analyzed, 63.8% were male, with median age of 40 years (range 18-73) and a median surveillance interval of 5.3 years (range 2.0-18.3). During surveillance, 12 (11.4%) patients developed TBAD. Patients with TBAD had higher radial growth rate (0.63 vs. 0.23 mm/year; p < 0.001) and elongation rate (2.4 vs. 0.5 mm/year; p < 0.001), on univariate and multivariable analysis, but pre-dissection descending aortic diameter was not significantly different. Predictors of growth rate included younger age, higher baseline maximal diameter of the DescAo, smoking history and warfarin use. Conclusions: Radial growth and elongation rates of the DescAo were independent predictors of TBAD occurrence in MFS. TBAD often occurred in at non-aneurysmal diameters. These findings emphasize the role of growth over absolute diameter in risk stratification for TBAD in MFS.