MRI-assessed regional pulse wave velocity for predicting absence of regional aorta luminal growth in marfan syndrome.

Abstract

BACKGROUND:

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.

METHODS:

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.

RESULTS:

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%.

CONCLUSIONS:

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.

• Authors: Eleanore S J Kröner, Arthur J H A Scholte, Patrick J H de Koning, Pieter J van den Boogaard, Lucia J M Kroft, Rob J van der Geest, Yvonne Hilhorst-Hofstee, Hildo J Lamb, Hans-Marc J Siebelink, Barbara J M Mulder, Maarten Groenink, Teodora Radonic, Ernst E van der Wall, Albert de Roos, Johan H C Reiber, Jos J M Westenberg
• Journal: International journal of cardiology (Int J Cardiol) Vol. 167 Issue 6 Pg. 2977-82 (Sep 10 2013) ISSN: 1874-1754 [Electronic] Netherlands
• PMID: 23000269 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

Topics

• Adult
• Aorta (pathology, physiopathology)
• Female
• Follow-Up Studies
• Humans
• Magnetic Resonance Imaging (methods)
• Male
• Marfan Syndrome (diagnosis, physiopathology)
• Middle Aged
• Predictive Value of Tests
• Pulse Wave Analysis (methods)
• Single-Blind Method
• Vascular Stiffness (physiology)
• Young Adult