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

INTERNATIONAL JOURNAL OF CARDIOLOGY

卷:167

Article

Kroner, Eleanore S. J.^1,2  Scholte, Arthur J. H. A.¹  de Koning, Patrick J. H.³  van den Boogaard, Pieter J.³  Kroft, Lucia J. M.³  van der Geest, Rob J.³  Hilhorst-Hofstee, Yvonne⁵  Lamb, Hildo J.³  Siebelink, Hans-Marc J.¹  Mulder, Barbara J. M.^2,4  Groenink, Maarten⁴  Radonic, Teodora^2,4  van der Wall, Ernst E.^1,2  de Roos, Albert³  Reiber, Johan H. C.³  Westenberg, Jos J. M.³ 

[1] Leiden Univ, Med Ctr, Dept Cardiol, NL-2333 ZA Leiden, Netherlands

[2] Interuniv Cardiol Inst Netherlands, Utrecht, Netherlands

[3] Leiden Univ, Med Ctr, Dept Radiol, NL-2333 ZA Leiden, Netherlands

[4] Univ Amsterdam, Acad Med Ctr, Dept Cardiol, NL-1105 AZ Amsterdam, Netherlands

[5] Leiden Univ, Med Ctr, Dept Clin Genet, NL-2333 ZA Leiden, Netherlands

关键词: Aortic dilatation;    Compliance;    MRI;    Blood Flow;    Marfan syndrome;   

DOI  :  10.1016/j.ijcard.2012.08.057

来源: Elsevier

【 摘 要 】

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 >2 mm 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. (C) 2012 Elsevier Ireland Ltd. All rights reserved.

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