Cited 27 times since 2012 (3.5 per year) source: Scopus International journal of cardiology, Volume 167, Issue 6, 20 September 2012, Pages 2977-2982 MRI-assessed regional pulse wave velocity for predicting absence of regional aorta luminal growth in marfan syndrome. Kröner ES, Scholte AJ, de Koning PJ, van den Boogaard PJ, Kroft LJ, van der Geest RJ, Hilhorst-Hofstee Y, Lamb HJ, Siebelink HM, Mulder BJ, Groenink M, Radonic T, van der Wall EE, de Roos A, Reiber JH, Westenberg JJ


In patients with Marfan syndrome (MFS), increased aortic wall stiffening may lead to progressive aortic dilatation. Aortic Pulse Wave Velocity (PWV), a marker of wall stiffness can be assessed regionally, using in-plane multi-directional velocity-encoded MRI. This study examined the diagnostic accuracy of regional PWV for prediction of regional aortic luminal growth during 2-year follow-up in MFS patients.


In twenty-one MFS patients (mean age 36 ± 15 years, 11 male) regional PWV and aortic luminal areas were assessed by 1.5 T MRI. At 2-year follow-up, the incidence of luminal growth, defined as mean luminal diameter increase >2mm was determined for five aortic segments (S1, ascending aorta; S2, aortic arch; S3, thoracic descending aorta, S4, supra-renal and S5, infra-renal abdominal aorta). Regional PWV at baseline was considered increased when exceeding age-related normal PWV (healthy volunteers (n=26; mean age 30 ± 10 years, 15 male)) by two standard-errors. Sensitivity and specificity of regional PWV-testing for prediction of regional luminal growth were determined.


Regional PWV at baseline was increased in 17 out of 102 segments (17%). Significant luminal growth at follow-up was reported in 14 segments (14%). The specificity of regional PWV-testing was ≥ 78% for all aortic segments, sensitivity was ≤ 33%.


Regional PWV was significantly increased in MFS patients as compared to healthy volunteers within similar age range, in all aortic segments except the ascending aorta. Furthermore, regional PWV-assessment has moderate to high specificity for predicting absence of regional aortic luminal growth for all aortic segments in MFS patients.

Keywords: MRI, Blood flow, Compliance, Marfan syndrome, Aortic Dilatation

Int J Cardiol. 2012 Sep;167(6):2977-2982