Abstract

Summary By using a comprehensive MRI-approach in patients with Marfan syndrome, increased regional pulse wave velocity in the ascending aorta can predict in 42% of the cases aortic luminal growth in the ascending aorta, while normal PWV can predict in 89% absence of luminal growth. Background The leading cause of premature death in patients with Marfan syndrome (MFS) is aortic dissection after progressive dilatation due to local increased wall stiffness, occurring most prominently in the ascending aorta. Aortic pulse wave velocity (PWV) is a marker of wall stiffness [Nollen, et al. Eur Heart J 2004]. Regional PWV can be accurately determined from in-plane multi-directional velocity-encoded (VE) MRI [Westenberg, et al. JMRI 2010]. The study objective was to test whether regional PWV can predict regional aorta dilatation at 2year follow-up (FU) in MFS.

Highlights

  • The leading cause of premature death in patients with Marfan syndrome (MFS) is aortic dissection after progressive dilatation due to local increased wall stiffness, occurring most prominently in the ascending aorta

  • Summary By using a comprehensive MRI-approach in patients with Marfan syndrome, increased regional pulse wave velocity in the ascending aorta can predict in 42% of the cases aortic luminal growth in the ascending aorta, while normal PWV can predict in 89% absence of luminal growth

  • In twenty-one MFS patients regional PWV and aortic luminal areas were assessed by 1.5T MRI (Philips, The Netherlands)

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Summary

Open Access

Normal regional pulse wave velocity predicts absence of aortic luminal growth in patients with Marfan syndrome: a comprehensive MRI-study. Summary By using a comprehensive MRI-approach in patients with Marfan syndrome, increased regional pulse wave velocity in the ascending aorta can predict in 42% of the cases aortic luminal growth in the ascending aorta, while normal PWV can predict in 89% absence of luminal growth

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