Three-dimensional distensibility of the aorta derived from four-dimensional cardiovascular magnetic resonance in young and middle-aged adults with Marfan syndrome

J Cardiovasc Magn Reson. 2025;27(2):101975. doi: 10.1016/j.jocmr.2025.101975. Epub 2025 Oct 29.

Abstract

Background: Acute aortic syndromes in Marfan syndrome (MFS) often occur before reaching the surgical diameter threshold, highlighting the need for new imaging biomarkers.

Objectives: Aim was to compare cardiovascular magnetic resonance (CMR)-derived aortic three-dimensional (3D) distensibility and displacement in MFS patients with or without a history of aortic root surgery (RR or native) and healthy volunteers.

Methods: The participants underwent 3T CMR of the thoracic aorta using an accelerated non-contrast-enhanced, free breathing, 3D cine balanced steady state free precession sequence, with spatiotemporal resolution: (1.0 mm)3/∼33ms. A deep learning-based algorithm was used to obtain aorta segmentations. Non-rigid registration of these segmentations was subsequently used to calculate 3D distensibility and its separate components: 2-dimensional distensibility, longitudinal strain, and displacement in the ascending (AAo) and descending aorta (DAo).

Results: Forty-seven volunteers, 51 native, and 33 RR MFS patients were included. AAo and DAo distensibility (10-3*mmHg-1) were different for healthy volunteers vs native vs RR patients (AAo: 5.1±1.4 vs 3.6±1.4 vs. 1.4±0.7, p<0.001, DAo: 3.2±1.1 vs. 2.5±0.9 vs 2.4±1.0, p=0.001). Sinotubular junction displacement (mm) was significantly higher for healthy volunteers vs native MFS vs RR MFS patients (10.3±1.3 vs 8.7±2.1 vs 5.7±1.6, p<0.001). In native patients, age (β=-0.06 (95% CI:-0.10 to -0.01), p=0.014) and root diameter (β=-0.1 (95% CI: -0.19 to -0.02), p=0.018) were negatively associated with AAo 3D distensibility, independent of male sex, body surface area, and aortic tortuosity index.

Conclusion: Aortic 3D distensibility and displacement, derived from 4-dimensional CMR, were significantly diminished in MFS compared to volunteers and should be investigated longitudinally to assess their potential value in predicting aortic events and guiding therapy.

Keywords: 3D distensibility; 4D CMR; Aorta; Displacement; Marfan.

Publication types

  • Comparative Study

MeSH terms

  • Adult
  • Age Factors
  • Aorta, Thoracic* / diagnostic imaging
  • Aorta, Thoracic* / physiopathology
  • Aorta, Thoracic* / surgery
  • Aortic Diseases* / diagnostic imaging
  • Aortic Diseases* / etiology
  • Aortic Diseases* / physiopathology
  • Case-Control Studies
  • Female
  • Humans
  • Image Interpretation, Computer-Assisted* / methods
  • Imaging, Three-Dimensional*
  • Magnetic Resonance Imaging, Cine*
  • Male
  • Marfan Syndrome* / complications
  • Marfan Syndrome* / diagnostic imaging
  • Marfan Syndrome* / physiopathology
  • Middle Aged
  • Predictive Value of Tests
  • Reproducibility of Results
  • Vascular Stiffness*
  • Young Adult