Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance, 29 5 2025, Pages 101975 3D distensibility of the aorta derived from 4D CMR in young and middle-aged adults with Marfan syndrome. Bosshardt D, Merton R, Schreurs BA, van Kimmenade RRJ, Nederveen AJ, Cox MGPJ, Scholte AJHA, Schrauben EM, Strijkers GJ, de Waard V, Robbers-Visser D, Groenink M, van Ooij P
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 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: (1.0mm)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 - -0.01), p=0.014) and root diameter (β=-0.1 (95% CI: -0.19 - -0.02), p=0.018) were negatively associated with AAo 3D distensibility, independent of male sex, body surface area, and aortic tortuosity index.
Conclusions
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.
