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

Area of Science:

• Cardiovascular Imaging
• Medical Diagnostics
• Genetics and Hereditary Diseases

Background:

• Marfan syndrome (MFS) significantly increases the risk of type B aortic dissection (TBAD).
• Aortic growth and elongation are suspected risk factors for TBAD development.
• Vascular deformation mapping offers a method to quantify 3D aortic growth from CT angiography (CTA).

Purpose of the Study:

• To evaluate the utility of aortic growth rate in the descending thoracic aorta for predicting TBAD risk in MFS patients.
• To identify imaging biomarkers, including growth rates, associated with TBAD risk in MFS.
• To assess the role of vascular deformation mapping in MFS aortic surveillance.

Main Methods:

• Analysis of CTA scans from 105 adult MFS patients with native descending thoracic aortas (2004-2023).
• Application of vascular deformation mapping to measure aortic radial growth and elongation rates.
• Measurement of thoracoabdominal aortic diameters and descending thoracic aorta length via centerline analysis.

Main Results:

• 11.4% of MFS patients developed TBAD during surveillance.
• Patients who developed TBAD exhibited significantly higher radial growth (0.63 mm/year) and elongation rates (2.4 mm/year) compared to those who did not.
• TBAD occurred even at nonaneurysmal descending aortic diameters (<4.0 cm); younger age, larger baseline diameter, smoking, and warfarin use predicted faster growth rates.

Conclusions:

• Radial and elongation growth rates of the descending thoracic aorta are independent predictors of TBAD in Marfan syndrome.
• Aortic growth rate is a more critical factor than absolute diameter in risk stratification for TBAD in MFS.
• Vascular deformation mapping provides valuable insights into aortic remodeling and TBAD risk in MFS patients.